The word ‘glaucoma’ comes from the Greek word b. Primary infantile glaucoma.

2
‘glau- cosis’ described by Hippocrates in 400 BC,
c. Primary juvenile glaucoma.
which means clouded or bluish-green hue. It may
d. Axenfeld-Rieger syndrome.
be due to corneal edema because of raised
intraocular tension or rapid evo- lution of cataract e. Peters’ anomaly.

Glaucom
(cataract and glaucoma were not distin- guished f. Aniridia.
until 1705). g. Other developmental anomalies.
Glaucoma is currently defined as a family of 3. Secondary glaucoma associated with other
ocular diseases characterized by progressive optic ocular and systemic disorders:

a
neuropathy with associated visual function loss. It is
often, but not al- ways, associated with increased
a. Glaucoma associated with disorders of
corneal endothelium:

1
IOP. • Iridocorneal endothelial (ICE) syndrome
Glaucoma is the second most common cause of • Fuchs’ endothelial dystrophy
irre- versible blindness in the world. • Posterior polymorphous dystrophy.
World Health Organization (WHO) survey in 1995 b. Glaucoma associated with disorders of the
has shown that 5.1 million people are bilaterally iris and ciliary body:
blind from glaucoma, 20% more have tubular • Pigmentary glaucoma
fields. Prevalence of glaucoma in India is 2.5% in
• Iridoschisis
people above 40 years (recent population-based
surveys). • Plateau iris
Glaucoma has been nicknamed as the ‘silent • Iris and ciliary body cysts.
thief of sight’ because the loss of vision normally c. Glaucoma associated with disorders of the lens:
occurs gradu- ally over a long period of time and is • Pseudoexfoliation syndrome
often only recognized when the disease is quite • Glaucoma associated with cataracts
advanced. Once lost, this dam- aged visual field • Glaucoma associated with lens dislocation.
cannot be recovered. Worldwide, it is the second d. Glaucoma associated with disorders of the
leading cause of blindness after cataracts. retina, choroid and vitreous:
With appropriate screening, usually glaucoma can • Neovascular glaucoma (NVG)
be identified early and its progress can be arrested • Glaucoma associated with retinal
with ap- propriate treatment before significant visual detachment and vitreoretinal diseases.
loss occurs.
e. Glaucoma associated with intraocular tumors:
• Malignant melanoma
CLASSIFICATION • Retinoblastoma
• Metastatic carcinoma
1. Primary glaucoma without other known ocular or • Benign tumors.
sys- temic disorders:
f. Glaucoma associated with inflammations such
a. Open-angle glaucoma:
as uveitis, keratitis, episcleritis and scleritis.
• Chronic open-angle glaucoma
g. Steroid-induced glaucoma.
• Normal tension glaucoma.
b. Angle-closure glaucoma.
c. Combined mechanism glaucoma.
2. Developmental glaucoma:
a. Primary congenital glaucoma.
 Chapter 21 Glaucoma 268

h. Glaucoma associated with ocular trauma. suspects.

i. Glaucoma associated with hemorrhage.
j. Glaucoma following intraocular surgery: Pathophysiology
• Malignant glaucoma
The exact cause of glaucomatous optic neuropathy is
• Glaucoma in pseudophakia and not known. A combination of risk factors affecting
aphakia axonal health has been identified. These include
• Epithelial, fibrous and endothelial elevated IOP, family history, race, age older than 40
proliferation years and myopia.
• Glaucoma associated with corneal
surgery
• Glaucoma associated with vitreoretinal
surgery.
k. Glaucoma associated with elevated episcleral
ve- nous pressure.

PRIMARY OPEN-ANGLE
GLAUCOMA
Definition
Primary open-angle glaucoma (POAG) can be
defined as a multifactorial progressive optic
neuropathy with a char- acteristic acquired loss of
optic nerve fibers in a person with open anterior
chamber angles. It results in:
1. Characteristic visual field abnormalities due to
cup- ping and atrophy of the optic disk
following retinal ganglion cell death.
2. Optic disk cupping.
3. Increased IOP. It is the risk factor associated with
the development of the disease and is not the
disease it- self. Patients can develop optic
neuropathy of glauco- ma in the absence of
documented elevated IOP. This condition has
been termed ‘normal- or low-tension
glaucoma’.

Glaucoma Suspects
Glaucoma suspects are people who are likely to be
having POAG or at a high risk for developing POAG
(e.g. family members of patients with POAG).
These include:
1. Some people can have elevated pressure in
the ab- sence of nerve damage or visual field
loss. This condi- tion has been termed ‘ocular
hypertension’. They are at more risk to develop
glaucoma and are considered to be glaucoma
suspects.
2. Patients with typical glaucomatous optic nerve
head (ONH) changes, but with normal IOP and
visual field, are also termed as glaucoma
 Chapter 21 Glaucoma 269
The three major theories concerning how IOP clinically treatable risk factor for glaucoma.
can ini- tiate glaucomatous damage in a According to Ocular Hyperten- sion Treatment
patient include: Study (OHTS), in patients with IOPs ranging
1. Vascular theory, which states that optic from 24 to 31 mm Hg, but with no clinical
neuropathy is due to ischemia of the optic signs of glaucoma, the average risk of
nerve. developing glaucoma over 5 years is 10%. That
2. Mechanical theory, which states that optic risk is reduced by 50%, if patients are
neuropa- thy is due to compression of the preemptively started on IOP-low- ering therapy.
axons at cribriform plate.
3. The current observation is that the
pathogenesis of glaucomatous optic
neuropathy is due to obstruction to
axoplasmic flow. It can be due to vascular or
me- chanical causes or both. It may be that
the vascular ischemia plays a major role in
the optic nerve damage in those with lower
IOP and direct mechanical com- pression in
those with higher IOP.
Intraocular pressure is the only clinical risk factor,
which is treatable. Several studies have shown
that the vi- sual field loss increases rapidly as IOP
rises above 21 mm Hg and more, and if pressures
are higher than 26–30 mm Hg.
Central corneal thickness (CCT) and diurnal
variation should be taken into account when
determining the IOP. If CCT is less, the IOP will be
recorded as lower than the actual value (more
chance of developing POAG, if CCT is
< 555 micron). A diurnal variation of more than
8 mm Hg can be seen in POAG cases. The normal
diurnal variation in IOP is 5 mm Hg or less. The
IOP recorded in routine OP checkup may be
normal, but at the peak of the diurnal variation,
the IOP may be elevated.
When POAG is associated with increased IOP,
the cause for elevated IOP is the increased
resistance to aque- ous outflow through the
trabecular meshwork.
Various theories suggested the reason for
increase in resistance to outflow, which
include:
1. Loss of trabecular endothelial cells.
2. Loss of giant vacuoles in the inner wall
endothelium of the Schlemm’s canal.
3. Loss of normal phagocytic activity.
Disk cupping and nerve fiber layer loss of up
to 40% can occur before actual visual field
loss is detected.

Risk Factors
1. Elevated IOP: It is the most important of
these risk factors because it is the main
 Chapter 21 Glaucoma 270

In some patients, the first sign of elevated

IOP can be presentation with sudden loss of Diagnosis
vision due to a central retinal vein occlusion To diagnose a patient with POAG, two of the
(CRVO). following clinical features should be present with an
2. Race: Prevalence of POAG is 3–4 times higher open-angle re- corded by gonioscopy:
in Blacks than in Caucasians. 1. Elevated IOP.
3. Family history: The prevalence in first-degree 2. Glaucomatous changes at the optic disk.
rela- tives of patients with POAG is 7–10 times 3. Typical glaucomatous field changes.
higher than in general population. The
inheritance is multifacto- rial and polygenic.
Mutation of myocilin gene in GL- CIA locus is Intraocular Pressure Measurement
seen in juvenile open-angle glaucoma and There are several methods for measuring IOP:
GLCIE locus is associated with normal tension 1. Applanation tonometry (using Goldmann applana-
glaucoma. tion tonometer).
4. Age: People older than 40 years are at risk 2. Non-contact tonometry.
factor for the development of POAG, with up to
3. Indentation tonometry (using Schiötz tonometer).
15% of people affected by the seventh
decade of life.
5. Other risk factors include: Applanation Tonometry
a. Myopia more than 4D. Applanation tonometry is the most accurate
b. Thin corneas: CCT is less than 555 microns. method. In applanation tonometry, the IOP is
c. Diabetes mellitus. inferred from the force required to flatten
d. Vasospasms, e.g. migraine. (applanate) a constant area of the cor- nea (3.06
e. Hypertension: Prevalence of glaucoma is mm). Goldmann tonometry is considered to be the
higher if diastolic perfusion pressure (sitting gold standard test and is the most widely accepted
diastolic pres- sure) is less than 55 mm Hg. method.
f. Cerebral and cardiovascular diseases. Method: The patient sits at the slit lamp with
applanation tonometer attachment. Fluorescein is
instilled into the conjunctival sac. A special
Screening disinfected prism is mounted on the tonometer head
Ideally, everyone over the age of 40 years attachment of the slit lamp and then placed
should be screened for POAG due to its chronic against the cornea. Two green semicircles are
insidious nature and also due to the fact that once visualized by looking through the slit lamp using a co-
optic nerve damage has occurred it is irreversible. balt blue filter. The force applied to the tonometer
First-degree relatives of the pa- tients, diabetics and head is then adjusted using a dial connected to a
myopes over 40 years should be regu- larly variable tension spring until the inner edges of the
examined to detect early glaucoma. green semicircles in the viewfinder meet (Figs 21.1A
and B). At this point the read- ing on the dial gives
the IOP.
Clinical Features Central corneal thickness must be determined to
People in the high-risk group should be carefully know the accuracy of IOP measured by applanation
evalu- ated periodically for any features of tonometry. The Perkins tonometer is a type of
POAG. portable applana- tion tonometer useful in children,
patients unable to co- operate with a sitting slit lamp
Sx Symptoms examination or in anesthe-
tized patients, who need to lie flat.
Patients will not usually present with any
symptoms or visual complaints until late in the
disease course in POAG because of the silent Non-contact Tonometry (Air-puff Tonometry)
nature of glaucoma. Non-contact tonometer uses a rapid air pulse to
In some patients, a positive history of chronic applanate (flatten) the cornea. Corneal applanation
head- ache, frequent change of presbyopic is detected via an electro-optical system.
glasses and de- layed dark adaptation can be Estimation of IOP is done by detecting the force of
elicited. But primarily it is the ophthalmologist’s
duty to diagnose POAG, especial- ly when a
person comes for presbyopic correction by
evaluation of the optic disk, by proper fundus
 Chapter 21 Glaucoma 271
the air jet at the instance of appla- nation. This
method is useful in mass screening of a large
number of persons and can be done by a
trained non- medical personnel also.
 Chapter 21 Glaucoma 272

FIGURES 21.1A and B: Green semicircles seen with the inner edges in contact. A. Photograph; B. Diagrammatic representation.

Indentation Tonometry rim and the optic cup.

Principle Neuroretinal rim (NRR): This is the nerve fiber layer
Indentation tonometry measures the depth of seen on the optic disk between the disk margin and the
optic cup
corneal indentation made by a small plunger
carrying a known weight. The higher the IOP, the
harder it is to push against and indent the cornea.
The weight of the plunger is 5 mg, but for very high
levels of IOP, extra weights, i.e. 7.5 or 10 mg can be
used to make the plunger push harder. The move-
ment of the plunger is measured using a calibrated
scale. The Schiötz tonometer is the most common
device to use this principle. Corneoscleral rigidity will
affect the accuracy of measurement of IOP. If the
ocular rigidity is high, falsely high values will be
obtained and if it is low, falsely low IOP reading is
obtained.
Glaucoma should be suspected if:
1. If IOP of more than 21 mm Hg and is measured
by ap- planation tonometry.
2. Diurnal variation of more than 8 mm Hg is
recorded.
3. An asymmetry in IOP of more than 5 mm Hg
between the two eyes is noted.
4. Normal tension glaucoma, if IOP is less than 21
mm Hg, but other features like optic disk cupping
and field changes are present.
5. Glaucomatous changes in the fundus
involve:
a. Optic nerve head.
b. Peripapillary area.
c. Nerve fiber layer.
Optic nerve head changes: Proper evaluation of the
ONH is important. In the examination of the optic
nerve, the things to be evaluated are the neuroretinal
 Chapter 21 Glaucoma 273

edge (Figs 21.2A and B). Normal NRR has a

uniform pink color (slightly paler on the temporal
side) and a characteris- tic configuration—broadest
inferiorly followed by the supe- rior, nasal and
temporal parts. Changes in configuration are
smooth without any sudden distortion or
notching.
Cup-disk ratio (CDR): It is the diameter of the
cup as a fraction of the disk measured in the
vertical meridian. Optic disk can be examined
with the direct ophthalmo- scope, but the ideal
method is to stereoscopically exam- ine for
evidence of glaucomatous damage with slit
lamp biomicroscopy using a 90D lens. Optic disk
changes over the years should be recorded with
stereophotographs or objective topographic
examination with scanning laser ophthalmoscope.
If these are not available, serial draw- ing over
the years should be made to assess any progress
while on treatment.
Typical glaucomatous ONH changes include
the fol- lowing:
1. The CDR, especially in vertical meridian of
more than 0.5 (Fig. 21.3).
2. An asymmetry of more than 0.2 between
the two ONHs (Fig. 21.4).
Contour of NRR changes can be:
1. Progressive enlargement of the cup (Figs 21.5A and
B).
2. Notching or thinning of disk rim particularly
at su- perior and inferior poles (because
nerve fibers at the superior and inferior poles
of the disk can often be af- fected first) (Figs
21.6A and B).
3. Optic disk hemorrhages (Fig. 21.7): It may
precede other signs of glaucoma. It is a sign
of uncontrolled glaucoma and a significant
risk factor for future pro- gressive ONH
damage. It can lead to localized dam- age of
retinal nerve fiber layer (RNFL). It is more
com- monly seen in normal tension
glaucoma.
 Chapter 21 Glaucoma 274

FIGURES 21.2A and B: Optic disk. A. Photograph; B. Diagrammatic representation.

FIGURE 21.3: Difference in the size of the cup depending on the size of the disk

Peripapillary changes: The following are the

peripapillary changes (Fig. 21.8).
Zone beta: Chorioretinal atrophy with baring of
sclera and large choroidal vessels surrounding the
disk. It is usually seen more in POAG patients. In
asymmetric glaucoma, it is more in worse affected
eye.
Zone alpha: Irregular hyper- and hypo-
pigmentation of retinal pigment epithelium
surrounding zone beta. Though it can be larger in
glaucomatous eyes, it is not as specific as zone
beta in glaucoma.
Baring of circumlinear vessel: Circumlinear
vessel is a small arteriole or vein lying
FIGURE 21.4: Asymmetric cupping superficially on NRR at its in- ner edge going toward
macula. On NRR loss, this vessel is isolated. It may
 Chapter 21 Glaucoma 275
be seen in other optic nerve diseases and in
large physiologic cups (Fig. 21.9).
 Chapter 21 Glaucoma 276

FIGURES 21.5A and B: Generalized enlargement of the cup. A. Photograph; B. Diagrammatic representation.

FIGURES 21.6A and B: Notching at inferior pole (black arrow). A. Photograph; B. Diagrammatic representation.

Retinal nerve fiber layer defects: The RNFL are radially

ori- ented striations extending from the disk
produced by the nerve fibers converging to the
optic disk (Fig. 21.10A). These are more clearly
visualized at superior and inferior poles of the disk,
where the nerve fiber layer is thickest and these
are curving in an arc-like manner. These fibers are
first affected in glaucoma. The defects can be slit
like (Fig. 21.10B), wedge shaped (Fig. 21.11) or
diffuse loss (Fig. 21.12). There will be corresponding
visual field loss also. It is best seen on fundus
examination with red-free light and dilated pupil.
The defects are seen as areas lack- ing striations.
Scanning laser ophthalmoscopy and optical
coher- ence tomography (OCT) are the newer
FIGURE 21.7: Optic nerve head hemorrhage imaging tech- niques to detect early ONH, retinal
nerve fiber and peri- papillary defects.
 Chapter 21 Glaucoma 277

Perimetry
Perimetry refers to the clinical assessment of visual
field. There are two types of perimetry:
1. Automated static perimetry: In static perimetry,
non- moving stimuli of varying luminance is
presented to each position of a number of
predetermined loca- tions; so as to get the
threshold luminance value in each position. It
is compared with age matched nor- mal. Over
the last two decades automated perimetry has
become the gold standard for assessing visual
function in a glaucoma patient, e.g. Humphrey
perim- eter, Octopus perimeter.
2. Manual kinetic perimetry: In kinetic perimetry,
a moving stimulus of fixed size and intensity is
moved from non-seeing area in the periphery
to seeing area in different meridians and a chart
FIGURE 21.8: Peripapillary changes in glaucoma is plotted along the

FIGURE 21.9: Glaucomatous cupping with baring

of circumlinear vessel

Visual Field Examination

Normal Visual Field
Visual field is described by Traquair as the island
hill of vision surrounded by the sea of darkness.
The field of vi- sion is often depicted as a three-
dimensional hill, with the peak sensitivity to stimuli
occurring at the point of fixation under photopic
conditions, decreasing rapidly in the 10° around
fixation and then decreasing very gradually for lo-
cations further in the periphery. The normal visual
field extends from the point of fixation, 60° FIGURES 21.10A and B: Retinal nerve fiber layer (RNFL). A. Normal
superiorly, 70° infe- riorly, 60° nasally and 90° arrangement of RNFL arcuate fibers (black arrow), papillomacular
temporally (Fig. 21.13). bundle (white arrow); B. Slit like defects in RNFL.
 Chapter 21 Glaucoma 278

FIGURE 21.11: Wedge-shaped defects in retinal nerve fiber layer FIGURE 21.12: Diffuse retinal nerve fiber layer loss
(loss of striations)

point of perception in each meridian. This can 6. Information to help in judging the reliability of the
be re- peated for stimuli of different size. It is not test including the rate of fixation losses, false
as sensitive as automated perimetry, e.g. positive er- rors, false negative errors and the
Bjerrum’s screen. time necessary for testing.
Certain terms used in perimetry Displays the sensitivities across the visual field: This
Luminance: This is the intensity or brightness of a informa- tion is shown in several different ways:
light stimulus. 1. A numerical plot gives the threshold for all
points checked.
Threshold: This is the degree of brightness needed
2. A gray-scale plot graphically demonstrates
for a stimulus to be detected by the person at a
regions to visual field loss by displaying the
specific location of the visual field.
regions with de- creased sensitivity in darker
Scotoma: This is a non-seeing area surrounded by tones. Areas of abnor- mally high sensitivity
seeing area. It can be a relative scotoma (when it are shown as white.
is an area of re- duced vision) or absolute scotoma 3. Total deviation plot: This plot shows the
when that area is to- tally blind. deviation of the patient’s result from that of age-
Decibel: It represents the intensity of luminance and is matched controls at each test location. Each
not a true unit of luminance and may vary between test location is graded as normal or abnormal
perimetry machines. at a defined level (p value) com- pared to a
normative population. The lower the p val- ue,
Humphrey visual field
the greater its clinical significance and the lesser
Stepwise interpretations of Humphrey visual field are
likelihood of the defect having occurred ‘by
the following.
chance’.
Patient and test details: The test details are typically 4. Pattern deviation plot: This plot is similar to total
dis- played on the top of the printout and include de- viation plot except that it is adjusted for any
the follow- ing information: general- ized depression, such as that caused by
1. Patient’s name, date of birth and age at the a cataract or miosis. Each test location is
time of the test. graded as normal or ab- normal at a defined
2. Date and time of the test. level (p value) compared to a nor- mative
3. Refractive error correction used during test. population. The lower the p value, the greater its
4. Type of test performed including the type of clinical significance and the lesser likelihood of
stimulus used, the background light level and the the defect having occurred ‘by chance’. Pattern
testing strat- egy employed. deviation plot should be carefully inspected for
5. Details on the method of fixation monitoring. early detection or progression of glaucomatous
visual field loss.
 Chapter 21 Glaucoma 279
Summary measures of visual field performance: Several
mea- sures are provided as a summary of the
visual field and they are:
 Chapter 21 Glaucoma 280

FIGURE 21.13: Normal visual field (asb, apostilbs; GHT, glaucoma hemifield test; MD, mean deviation; PSD, pattern standard deviation;
SITA, Swedish interactive threshold algorithm). Note: Refer same abbreviations for Figures 21.15, 21.17 to 21.19.
 Chapter 21 Glaucoma 281

1. Results of the glaucoma hemifield (GHT) test: spot, suggest an

GHT, devised for the Humphrey field analyzer, FIGURE 21.14: Glaucoma hemifield
compares 30-2 visual fields into 10 regions, with
five inferior re- gions representing mirror images
of five correspond- ing superior regions (Fig.
21.14). Differences between corresponding
superior and inferior zones are com- pared with
the differences present in the population of
normal controls. Possible test outcomes are:
a. Outside normal limits: The GHT is described
as ‘outside normal limits’ when differences
between a matched pair of corresponding
zones exceeds, the difference found in 99%
of the normal popula- tion or when both
members of a pair of zones are more
abnormal than 99.5% of the individuals with
the normative population.
b. Borderline: The GHT is described as
borderline when matched pairs of zones are
abnormal at the 97th percentile within the
normative database.
c. General reduction of sensitivity (Fig. 21.15):
Visual fields are described to have generalized
reduction of sensitivity when both
conditions for ‘outside normal limits’ are not
met and the best region of the VF is
depressed to a level at the 99.5 percentile
within individuals of the normative
database.
d. Abnormally high sensitivity: The GHT is
described as having abnormally high
sensitivity when the overall sensitivity in
the affected region of the VF is better than
99.5% of individuals within the nor- mative
population.
e. Within normal limits: Visual fields (VFs) are
de- scribed as being within normal limits
when none of the above conditions are
met.
Establishing reliability of results: These are as follows.
Fixation losses: It is identified as a result of
positive re- sponses to stimuli presented in the blind
 Chapter 21 Glaucoma 282
unsteadiness of gaze, which could be the depressed from previous examinations or the
interpretation of the visual field test. baseline status.
False positives: Occurs when patient responses Paracentral defects: Circumscribed paracentral defects
are noted in the absence of a presented stimulus. are early sign of localized glaucomatous damage.
Patients with high false positive rates may show It is found along the course of the nerve fiber bundle.
pale areas on the gray scale display and These are most
abnormally high sensitivity on the GHF test.
False negatives: These are identified when,
upon retesting a previously tested location with a
stimulus brighter than the measured threshold
value, no response is detected. False negatives
may be normal in eyes with advanced vi- sual
field loss, but typically reflect poor attention in
eyes with little or no visual field damage.
Visual fields were considered unreliable, if
fixation losses exceeded 25% and false positives
or false negatives exceeded 33%. High rates of
fixation losses, false positives or false negatives
indicate poor cooperation with testing
procedures.
Global indices in Humphrey perimeter: These are as follows:
1. Mean deviation indicates generalized changes
due to medial opacities like cataract.
2. Pattern standard deviation (PSD) indicates a
localized scotoma.
3. Short-term fluctuation (SF): When threshold
tests are done, intratest variation occurring
at each point is termed short-term
fluctuation.
4. Corrected pattern standard deviation (CPSD):
It is the variability in field after correction for
SF (i.e. CPSD = PSD – SF). It is recorded only
in full threshold testing.
Retinal nerve fiber paths and the field of vision: Axons
extend- ing towards the ONH from the retinal
ganglion cells form the RNFL. The RNFL
responsible for providing the supe- rior and
inferior hemifield of vision can be divided into
three divisions:
1. Nasal fiber represents the retina nasal to the ONH.
2. The maculopapillary bundle goes directly
from the macula to the temporal ONH.
3. The arcuate fibers: These fibers take an
arcuate course from the peripheral macula
and the temporal retina outside the macula
to the superior and inferior ONH.
Typical visual field defects in glaucoma
Diffuse depression: In automated perimetry, diffuse
depres- sion results in relative defects across the
entire visual field. Early diffuse depression is often
difficult to detect because thresholds may remain
within the normal range, but these may be
 Chapter 21 Glaucoma 283

FIGURE 21.15: General reduction of sensitivity

 Chapter 21 Glaucoma 284

commonly seen in upper nasal area. Usually, show an abrupt drop-off from directly adjacent
paracentral scotomas are seen in Bjerrum’s area, points.
which is an arcuate region that extends above and 5. Cloverleaf fields: This pattern of visual field
below the blind spot be- tween 10° and 20° of defects reflects poor visual attention and/or
fixation point. malingering.
Nasal step defect of Roenne: A step-like defect along
the nasal horizontal meridian results from
asymmetric loss of nerve fiber bundles in the
superior and inferior hemi- fields.
Seidel’s scotoma and enlargement of blind spot: It is a
sickle- shaped scotoma that is a superior or inferior
extension of the blind spot. Peripapillary atrophy,
which frequently ac- companies glaucomatous
damage, particularly in elderly patients, may cause
enlargement of the blind spot.
Arcuate scotoma: Loss of arcuate nerve fibers leads to
a scotoma that starts at superior or inferior poles
of blind spot, arches over the macula, widens as
they curve up or down and terminates abruptly at
the nasal horizontal me- ridian. It progresses variably
in the superior and inferior fields (Figs 21.16 to
21.19).
Temporal wedge-shaped defects: Damage to nerve
fibers on the nasal side of the optic disk may
result in temporal wedge-shaped defects. These
defects are much less com- mon than defects in the
arcuate distribution. Occasional- ly, these are seen
as the sole visual field defect. Temporal wedge
defects do not respect the horizontal meridian.
Double arcuate scotoma or ring scotoma: Upper and
lower arcuate scotomas join to form the ring
scotoma.
End stage: At the end, only central island of vision
along with a temporal island of vision is left
behind. Finally the central island will be gone and
the temporal island will be the last field to go.
Common perimetric errors
1. Incorrect patient name.
2. Incorrect patient age: As threshold values are
com- pared to age-adjusted normal values,
incorrect age entry will lead to comparisons
with the wrong set of normal values.
3. Inappropriate correction of refractive error:
Failure to properly correct for refractive error will
cause stimuli to become visually defocused.
4. Lens rim artifacts: Thick rims of the spectacles
or in- appropriate head positioning, which causes
the lens rim to block peripheral stimuli can cause
artifactual depression of the peripheral points.
Points are typi- cally severely affected (often
with threshold sensitivi- ties of 0 dB) and often
 Chapter 21 Glaucoma 285
The field results typically show high rates
of false negative responses.
6. Miotic pupils or cataracts: Ocular features,
which allow less light to reach the retina can
cause diffuse depression of the visual field,
along with statistically significant decreases in
mean deviation. A small pu- pil may simulate
a glaucomatous visual field defect by
generalized depression because of miosis or
ex- acerbate an already constricted field,
giving a false impression of progression of
glaucoma (Fig. 21.20). In cases with
concomitant cataract, attention should be
paid to the pattern deviation plot as it
adjusts for generalized depression caused
by the cataract.

Management
The modern goals of glaucoma management are:
1. To avoid glaucomatous nerve damage.
2. To preserve visual field.
3. Preserve total quality of life for patients with
minimal side effects. This requires:
a. Appropriate diagnostic techniques.
b. Regular follow-up examinations.
c. Judicious selection of treatments for the
individu- al patient, medical or surgical,
which will stabilize the vision and prevent
further visual field loss.
Although IOP is only one of the major risk
factor for glaucoma, lowering it via various
pharmaceuticals and/or surgical techniques is
currently the mainstay of glaucoma treatment.
Treatment of open-angle glaucoma is primarily
medi- cal and that of angle closure is primarily
surgical.
Ocular Hypertension Treatment Study
(OHTS) and Early Manifest Glaucoma Trial
(EMGT) are two studies
FIGURE 21.16: Arcuate scotoma
 Chapter 21 Glaucoma 286

FIGURE 21.17: Double arcuate scotoma

 Chapter 21 Glaucoma 287

FIGURE 21.18: Early arcuate scotoma

 Chapter 21 Glaucoma 288

FIGURE 21.19: Advanced superior arcuate scotoma

 Chapter 21 Glaucoma 289

FIGURE 21.20: Progression of field defects in glaucoma

 Chapter 21 Glaucoma 290

done, which help in decision making of when to Beta blockers

start treatment, especially in a patient with early Method of action: By decreasing aqueous inflow,
glaucoma. about 20%–30% reduction in IOP occurs, e.g. timolol,
betaxolol, levobunolol, metipranolol, carteolol.
Ocular Hypertension
Ocular hypertension (OHT) can be defined as
elevated IOP in the absence of identifiable optic
nerve damage or visual field loss.

Ocular Hypertension Treatment Study

The OHTS trial is a multicenter randomized
controlled clinical trial comparing observation with
medical thera- py for OHT. Result is topical ocular
hypotensive medica- tion was effective in delaying
or preventing the onset of POAG. 1 out of 22 OHT
went on to develop early field loss in 5 years
without treatment.

Early Manifest Glaucoma Trial

Multicenter randomized controlled clinical trial
compar- ing observation with betaxolol and laser
trabeculoplasty for early open-angle glaucoma. Early
glaucoma can be de- fined as raised IOP with disk
changes and early 24-2 field loss. One out of seven
patients with early glaucoma pro- gressed in 5
years without treatment.

Conclusion
If the follow-up is done properly, then there is no
need to treat all preperimetric (before
development of field de- fects) glaucoma. Before
starting treatment, consider side effects of drugs,
cost of treatment and labeling of the pa- tient as a
glaucoma patient:
• OHT with IOP < 26 mm Hg follow-up with
fields
• OHT with IOP > 26 mm Hg initiate treatment
• OHT with field changes initiate treatment.

End Point for Treatment

Target IOP: Aim for a target IOP of 20%–30%
reduction in IOP from baseline. If the pretreatment
IOP is 30 mm Hg try to bring it down to 22–20 mm
Hg, then follow-up the patient. If there is progression
in field defects, reset the tar- get IOP by 20%–30%
from the ‘new baseline’ IOP, i.e. try to bring it down
from 22 to 15 mm Hg.

Medical Management
 Chapter 21 Glaucoma 291
Side effects: Hypotension, bradycardia, heart Reduction of side effects
blocks, re- duce response to exertion, Reduction of side effects can be achieved by applying
bronchospasm, mask hypogly- cemia, one drop in cul-de-sac, by closing the eye gently,
depression, loss of libido, etc. by wiping excess of drug, by punctal occlusion and
Parasympathomimetics by asking the pa- tient not to squeeze the eyelids (to
Method of action: It is by increasing outflow via prevent lacrimal pump mechanism).
trabecular meshwork, e.g. pilocarpine, carbachol,
echothiophate, phospholine. These are rarely
used now.
Side effects: Loss of accommodation, compensated
by in- creased depth of focus, retinal detachment,
iris cyst, etc.
Alpha-agonists
Method of action: It is by decreasing aqueous
production and increasing aqueous outflow. About
20%–30% reduc- tion in IOP occurs, e.g.
apraclonidine, brimonidine.
Side effects: Burning sensation, follicular
conjunctivitis, al- lergies, headache, drowsiness,
dry mouth and nose.
Prostaglandin analogs
Method of action: It is by increasing uveoscleral
outflow. About 20%–35% reduction in IOP occurs,
e.g. latanoprost, bimatoprost, travoprost and
unoprostone.
Side effects: Redness, foreign body sensation, iris
and skin pigmentation, increase in number and
length of eyelashes.
Topical carbonic anhydrase inhibitors
Method of action: It is by decreasing aqueous
production. About 20%–30% reduction in IOP
occurs, e.g. dorzolamide and brinzolamide.
Side effects: Metallic taste, hypersensitivity and
rarely cor- neal edema.
Combining drugs
By combining two drugs, though we get 20%–30%
reduc- tion individually, together these give only
20%–30% re- duction plus an addition of 5%–
10% reduction. 1 + 1 = 2 as far as adverse
effects goes, e.g. we have combinations of
brimonidine with timolol, dorzolamide with
timolol and prostaglandins with timolol.
Add/Switch
When the first drug fails to achieve target IOP, it
is prefer- able to switch to another group than
add on as:
1. The side effects add up.
2. Long-term allergy/sensitization can occur.
Still we often need multiple drugs. In that case
try com- bination as separate first and if it is
effective, combine.
 Chapter 21 Glaucoma 292

Compliance issues pas- sage of aqueous from the anterior chamber to the
Treatment is susceptible to non-compliance as the subcon- junctival space, creating a filtering bleb and
treat- ment is lifelong and expensive, and no obvious thereby, low- ering IOP (refer Chapter 29 ‘Glaucoma
benefit like improvement in vision occurs. Compliance Surgery’).
improves with patient education, improved
accessibility to health care and rapport between
clinician and patient.
Follow-up
In general, if IOP is stable on treatment, do twice
monthly IOP checkup and disk assessment, and field
testing every 6–12 months. Complete evaluation
including systemic diseases review, gonioscopy,
and field and disk assess- ment is done yearly.

Role of Surgery
Indications for filtration surgery:
1. If target IOP is not achieved medically.
2. If patient cannot afford the medicines.
3. Regular follow-up is not possible due to lack of
access to ophthalmologist.
4. Patient shows non-compliance to medical
treatment.
5. When glaucomatous optic neuropathy worsens or
is expected to worsen at any given level of IOP
and the patient is on maximum tolerated
medical therapy (MTMT).
Argon laser trabeculoplasty
Argon laser trabeculoplasty (ALT) uses a laser
beam fo- cused through a goniolens to treat at the
border between anterior and posterior trabecular
meshwork. A full treat- ment consists of 100 spots
placed over the entire 360° of the trabecular
meshwork. This may be divided between two
sessions consisting of 50 spots over 180°. Aqueous
outflow improves after the procedure. The IOP
reduction obtained is usually in the 7–10 mm Hg
range and it may last up to 3–5 years following ALT.
Unfortunately, the de- crease in IOP is not usually
permanent. Approximately, 10% of treated patients
will return to pretreatment IOP for each year
following treatment.
Complications: A brief, but potentially significant
increase in IOP after the procedure (therefore, alpha-
agonists are often used either preoperatively or
postoperatively for prophylaxis of this occurrence);
transient iritis or corneal opacities; peripheral
anterior synechiae and hyphema.
Trabeculectomy
An alternate outflow pathway is created to increase
 Chapter 21 Glaucoma 293
Ciliary body ablation those of European origin.
Indications: This is a destructive procedure. This
procedure is indicated as a last resort for
patients who have failed medical management
and other surgeries or for those pa- tients who
have limited visual potential (often 6/60 or less) or
reached the absolute glaucoma stage.
Principle: By destroying a portion of the non-
pigmented cil- iary epithelium, aqueous humor
production is decreased. This will bring down
the IOP.
Methods: The ciliary body epithelium can be
destroyed by cyclocryotherapy, diathermy,
ultrasound, transscleral Nd:YAG or diode laser
(known as cyclophotocoagulation) or a newer
endoscopic laser.

Public Education
Glaucoma, especially POAG being a silent thief of
sight, it is necessary to diagnose POAG early by
improving public awareness. Manage and follow-
up each patient systemati- cally. Also there is no
need to treat all preperimetric glau- coma, if
these can be followed up.
All patients and their relatives should be made
aware that glaucoma is not curable, but can be
medically or sur- gically controlled, damage to
vision is not reversible and glaucoma requires
lifelong treatment and follow-up.

Primary angle-closure glaucoma (PACG) is the PRIMARY ANGLE-

type of glaucoma occurring due to closure of CLOSURE GLAUCOMA
the trabecular meshwork by the root of the iris,
occurring as an anatomi- cal variation.

Epidemiology
Incidence Data
The incidence of PACG varies among different
ethnic populations.
Primary angle-closure glaucoma is estimated to
be one tenth the number of cases as POAG among
White Cauca- sian population. Mongoloid races
have a much higher rate of prevalence for
PACG.
Descending order of prevalence (roughly)
can be: In- uit (Eskimos), East Asian (Chinese,
Filipino, Vietnamese), Indian subcontinent and
 Chapter 21 Glaucoma 294

Factors This will lead to angle closure by the root of the iris
Demographic Factors on dila- tation of the pupil even though the anterior
chamber will be of normal depth.
Age: The risk of PACG goes up with age. Most often
occurs in the sixth or seventh decade, though Mechanisms
commonly it can present by the fourth decade.
Gender: Women are more commonly affected with a Angle closure occurs often as a combination of the
rela- tive risk of 2–3 times compared to men. This follow- ing mechanisms elaborated below. In a
probably re- flects the fact that women have given case, one mechanism would prevail more
approximately 10% less ocular volume and have than others.
narrower angles.
Relative Pupillary Block
Heredity: Most cases are sporadic in nature. Some
studies do show some familial influence. The The most important mechanism is the relative
configuration of the anterior chamber is considered pupillary block. In a normal eye, the iris is slightly
to have a polygenic in- heritance and that probably in contact with the lens at the pupillary margin (Fig.
is the reason for lack of family history in most 21.22A). In a crowd- ed anterior chamber the
patients. crystalline lens is pushing the iris forward and is
partially jutting out (Fig. 21.22B). In this state, the
Refractive error: Prevalence of PACG is higher in
iris exerts a posterior pressure on the lens caus- ing
hyperme- tropic individuals.
resistance to flow of aqueous. This is maximal in
Miscellaneous: Finding that acute PACG occurs more the mid-dilated position.
in winter months has been made in the past. On the constricted state, the pupil is positioned
tan- gential to the lens and is not able to
Predisposing Factors effectively grip the lens. In the mid-dilated
position, the sphincter muscle of the iris exerts a
Primary angle-closure glaucoma essentially occurs
centripetal or inward force on the part of lens
in a small crowded eye (Figs 21.21A and B). In eyes,
jutting anterior to it (refer Fig. 21.22B). The cen-
predisposed to angle-closure glaucoma, the average
trifugal action of the dilator pupillae muscle (Fig.
corneal diameter, anterior chamber depth and axial
21.22C) also is slanted backward and a small part
length are less, but the lens size and volume do not
of the vector is hence pressing on the anterior
come down proportionally and this leads to a crowded
capsule of the lens. This gives maximal resistance to
anterior chamber, especially in the periphery at the
aqueous flow through the pu- pil into the anterior
angle of the anterior chamber.
chamber from its source, ciliary body that secretes it
Another mechanism, plateau iris, involves the into the posterior chamber. The relaxed peripheral
periph- eral iris root being inserted at an angle to portion of the iris in the mid-dilated position is
the ciliary body. pushed forward by the increased IOP in the
posterior
 Chapter 21 Glaucoma 295
FIGURES 21.21A and B: Predisposing factor. A. Normal eye with flow of aqueous shown by arrows. Aqueous is secreted by the
ciliary body into the posterior chamber and flows through the pupil into the anterior chamber to the angle of the anterior
chamber, where it traverses the trabecular meshwork to the canal of Schlemm and from these to episcleral veins; B. In an eye
predisposed to angle closure, the anterior chamber is shallow and a relatively normal sized lens juts into the same predisposing to
a pupillary block.
 Chapter 21 Glaucoma 296

of the iris to the ciliary body. As a result, the root of iris

is slanted abnormally forward. On ultrasound
biomicroscopy, these

chamber. Thus, the aqueous flow is blocked at the

pupil. This is not an absolute block and hence is
called relative pupillary block.
In this state of relative pupillary block, to
overcome the increased resistance to the flow of
aqueous from the posterior chamber to the
anterior chamber through the pupil, the pressure
gradient has to be higher than normal. The
differential pressure pushes the iris forward and the
peripheral anterior chamber is obliterated or closed
(Figs
21.23 and 21.24). Because the peripheral iris is
opposed firmly to the peripheral cornea
obliterating the angle of the anterior chamber, the
major route of outflow of fluid from the eye
(anterior chamber) will be blocked. This will stop all
aqueous outflow from the eye and the IOP can rise
dramatically.

Plateau Iris
In plateau iris condition, there is abnormal insertion
 Chapter 21 Glaucoma 297
FIGURES 21.22A to C: Relative pupillary block mechanism. A.
With pupil in its normal position the iris skims the surface of
the lens and grips it to cause resistance to flow of aqueous; B.
With pupil in mid- dilated position a little of the lens in a
shallow anterior chamber juts into the pupil and is held by the
pupillary muscles. Primarily by the sphincter pupillae and
secondarily by the posterior component of the vector of force
from the dilator. This causes a relative pupillary block to flow of
aqueous through the pupil; C. With pupil in dilated positions
the iris falls away from the lens and is unable to cause
resistance to flow of aqueous.

patients are often noted to have anteriorly

placed ciliary processes pushing the peripheral
iris root forward. Cen- tral anterior chamber is
relatively deep. When the pupil dilates, the
peripheral iris bunches up and these rolls of iris
in the periphery crowds the angle closed.

Lens Size (Phacomorphic Like)

Here the lens itself pushes the iris forward
contributing to the closure of the angle of the
anterior chamber. The lens is enlarging
throughout the life as new lens fibers are laid
down. Angle closure occurs when the lens reaches
a criti- cal size in comparison to the anterior
chamber depth.

Ciliary Body
The ciliary body is attached anteriorly to the
scleral spur. Beyond this there is no attachment to
the outer coat of the eye (sclera). When the ciliary
muscles contract, the circum- ference of the
ciliary body decreases and the ciliary body
tends to rotate anteriorly on its attachment at
the scleral
 Chapter 21 Glaucoma 298

Pathophysiology
By combination of above mechanisms, the
anterior chamber angle can close. Over time the
lens thickness is marginally increasing. This means
that the angle closure would worsen in a given
patient as age advances. Initially, the angle closes
for short spans of time. A tiny movement of the iris
would relieve the angle closure long enough,
opening the aqueous outflow channels and allows the
IOP to reduce. Later on, the duration of angle being
closed in- creases and the pressure can rise to
higher levels and it can remain high for longer
periods of time. At some point a sudden acute
angle closure occurs with severe pain,
constitutional symptoms like headache, nausea and
FIGURE 21.23: When aqueous flows into the posterior chamber, vom- iting, intense congestion, high IOP, corneal
the pressure in the posterior chamber push the peripheral iris edema and mid-dilated pupil.
forward and obliterate the angle of the anterior chamber. This
stops almost all outflow from the eye and the intraocular pressure
(IOP) can rise dramatically. Effects of Angle Closure
in Different Parts of the Eye
spur. This would rotate the peripheral iris root,
attached to it closer to the trabecular meshwork. In the Cornea (Blurring and Halos)
This would also move the entire zonular ligament When the IOP rises to approximately 30 mm Hg, a
with it and hence the lens moves forward. An cor- nea with average endothelial function
anterior rotation of the ciliary body would thus move develops epithe- lial edema. Epithelial edema causes
the lens-iris diaphragm forward and nar- row the the epithelial cells to swell up and form multiple
angle of the anterior chamber. In cases where this tiny elevations on the corneal surface, each of
mechanism contributes significantly, pilocarpine which acts optically like a prism capable of
would worsen the angle closure as it causes dispersion of light. Dispersion of light causes white
light to split up into its spectrum of colors as in a
contraction of ciliary muscles and consequent anterior
rainbow. This causes formation of ring-shaped
movement of the same.
colored halos around light sources like an
incandescent bulb with the red color
 Chapter 21 Glaucoma 299
FIGURE 21.24: Ultrasound biomicroscopic (UBM) image of pupillary block causing angle closure
 Chapter 21 Glaucoma 300

seen on the outside and blue on the inside of the Ischemia of Iris (Sphincter Paralysis Dilated Pupil,
ring (red light has a longer wavelength). At this Pigment Dispersion and Iris Atrophy)
stage there can be some degradation of quality of
vision in the form of slight blurring, foreign body At pressures in the range of 40 mm Hg or so the iris
sensation and watering due to ir- regularity of the also becomes ischemic. This causes a paralysis of the
ocular surface. iris mus- culature, especially the sphincter pupillae. This
Halos can also be seen in early stages of freezes
cataract and when mucus strands are present in
the tear film. Halos arising from the lens are due
to prismatic effect in the pe- ripheral lens fibers
near the lens equator. Thus lenticular halos would
break up if a stenopaic slit is passed across the
eye unlike a corneal edema halo as in a corneal
edema the prismatic effect is present over a
diffuse area. Halos due to mucus strands would vary
with blinking and disap- pear if the eye surface is
washed clear of mucus.

Scleral Stretch (Headache

and Constitutional
Symptoms)
Pressure rise would stretch the scleral fibers and
ocular discomfort occurs as a dull ache (40 mm
Hg approxi- mately). Further pressure rise would
convert this perior- bital ache into a more severe
headache, which is often felt all over the head as the
stretch is increased. Additionally, the scleral stretch
causes stimulation of the vagus nerve and
symptoms expected in a vagal stimulation may su-
pervene in the form of nausea, vomiting and
prostration. In fact, often the patient reaches the
internal medicine casualty with headache and
vomiting as the predominant symptoms prior to
recognition of the ocular cause by the internist.

Ischemia of Optic Nerve (Decreased Vision, CRA

Occlusion and Optic Atrophy)
When the IOP raises further, the retinal and optic
nerve functions get compromised as circulation of
blood slows down. Further reduction in visual function
results in loss of visual acuity. When the IOP goes
above 60 mm Hg there is a significant risk of total
cessation of blood flow in the central retinal artery
(CRA). This can lead to a CRA occlu- sion itself. In
such an acute event, one may see primary optic
atrophy like picture in the sequel (cupping or cav-
ernous optic atrophy occurs when the pressure
rise is gradual and not acute, and so dramatic and
the gradual reduction in blood circulation
occurs).
 Chapter 21 Glaucoma 301
the pupil in its mid-dilated state, where it is most
efficient at causing pupillary block and the
pressure rises further. Once this stage is reached,
miotic drugs like pilocarpine, which have been
classically the mainstay of therapy of angle
closure, are relatively ineffective as the muscle
cells are unable to respond. Over a few days,
the iris tissue at- rophies and the pupillary block
may be relieved. By this time, the vision can
be irrecoverably compromised.
Ischemic iris would cause the posterior lamella
of iris (pigment epithelial layer) to release iris
pigments as few of these cells lyses. This pigment
deposits all over the ante- rior segment is seen as
pigment deposits on the endothe- lium of the
cornea as a sequel on slit lamp examination FIGURE 21.25: Patch of iris atrophy
(Fig. 21.25).

Lens (Anterior Subcapsular Cataracts)

As the outflow is compromised, ultrafiltration at
the ciliary body slows down due to back pressure.
In short, aqueous secretion comes to a standstill.
The lens being avascular is totally dependent on
the nutrients and oxygen supplied by the aqueous
to it. Lack of the same causes formation of anterior
subcapsular opacities in the lens called glaucom-
flecken (Figs 21.26A and B).

Vogt’s Triad
Vogt’s triad is a set of three findings such as iris
atrophy, pigment deposits on the endothelium
of the cornea and glaucomflecken that suggests
previous occurrence of an acute congestive angle-
closure attack. This once present, persists for life
(refer Figs 21.26A and B).

Peripheral Anterior Synechiae

When the iris stays in contact with the angle
structures in the periphery of the anterior
chamber, it gets stuck to it
 Chapter 21 Glaucoma 302

FIGURES 21.26A and B: Vogt’s triad. Pigment deposits on back of the cornea (endothelium), iris atrophy and glaucomflecken (anterior
subcapsular lens opacities) constitute the Vogt’s triad. A. Diffuse illumination to show iris atrophy (solid arrows); B. Slit lamp picture to
show anterior subcapsular opacities (dotted arrows) and pigment (dashed arrows).

permanently. This leads to blockage of outflow in that with sleep that induces miosis (pupillary constriction).
area permanently. When half the circumference of If ex- amined during this episode one would find
the angle is compromised, usually the IOP stays pressures in the range of 30–40 mm Hg with corneal
raised permanently. epithelial edema and gonioscopically appositionally
closed angle.
Stages of Angle Closure
Classical Stages
Classically, angle closure has been staged with
pupillary block as the mechanism of closure with a
sequence as following. Not all patients follow all
the stages and so the staging does not reflect
the course of all patients.
1. Latent stage
In latent stage, the eye has a shallow anterior
chamber on slit lamp examination and gonioscopy.
The patient has no symptoms and the pressures
are normal.
2. Prodromal stage
In prodromal stage, there are intermittent
episodes of closure of the angle leading to
transient rise in IOP to moderate levels enough to
cause corneal epithelial ede- ma. This happens
when the pupil is mid-dilated, when the ambient light
is dim (scotopic conditions), when the pa- tient is
in a movie theater or when they are tired at the
end of the day, the pupil dilates physiologically.
This causes a relative pupillary block in
predisposed eyes, leading to pressure rise in the
range of 30s. The patient now sees halos around
lights with decrease in vision and some dis- comfort
around eyes. At this stage, these episodes occur
widely spaced and are spontaneously relieved, e.g.
 Chapter 21 Glaucoma 303
3. Stage of constant instability/intermittent closure
The process is the same as in prodromal stage
with in- creasing frequency and regularity. At this
point of time the patient realizes that there is
something amiss about their eye and seeks
medical help.
4. Acute angle closure/acute congestive glaucoma
Now the pupillary block is more severe and the
IOP rais- es high enough to make the sphincter
pupillae ischemic and paralyzed temporarily in its
mid-dilated position. The pressure rises markedly
and there is marked loss of visual acuity. But the
predominant symptom is headache caused by
scleral stretch, associated with nausea and
vomiting. The patient feels very sick and prostrate,
and may land in medical casualty.
On examination, the vision is markedly
reduced. There is circumcorneal congestion with
hazy cornea (steamy) due to stromal edema
along with epithelial edema. The pupil is mid-
dilated, vertically oval and non-reactive. If one
checks for a relative afferent pupillary defect, it
is of- ten present.
5. Chronic angle closure/chronic congestive glaucoma
Chronic glaucoma stage is characterized by the
presence of peripheral anterior synechiae. These
would compro- mise aqueous humor outflow in
that area and the IOP rises above baseline.
Usually, this happens by the time synechiae
close half the angle. Now the baseline pressure is
constantly elevated. The optic disk and field
changes, as in POAG, will start to develop over
time after this.
Though the stage of chronic angle closure
can occur after an acute angle-closure attack,
some patients develop peripheral anterior
synechiae without going through a dra- matic acute
angle closure. The mechanisms are the same,
 Chapter 21 Glaucoma 304

but the degree of closure is less. The synechiae is lit up. But in an eye with closure (or pre- disposed
would de- velop slowly from posterior part of the to closure) the anterior chamber is crowded by the
angle and work its way anteriorly closing the angle crystalline lens, pushing the central iris forward. This will
progressively. A larger cir- cumference is often cause a crescent-shaped shadow to fall on the nasal
involved. This slow process of closure is called side of the iris. If the shadow obscures half the
‘creeping angle closure’. These patients have POAG- distance
like picture as far as symptoms go. Creeping angle
closure is rarely seen in Caucasian and African
popula- tions, whereas this is the commoner form
in the Mongol- oid races and Indian
subcontinent.
6. Absolute glaucoma
Absolute glaucoma is the end stage of any
glaucoma, where all vision is lost and eye is stony
hard, blind and of- ten painful. This can occur in
all types of glaucoma.

ISGEO Classification
As there are different mechanisms contributing in
vary- ing degrees in a given person, not all patients
follow the classical stages in order. The stage of
acute closure is often skipped. This means that in
epidemiological studies it is difficult to classify
closure in the classical scheme across various
regions. So, a classification was developed only for
epidemiological studies by the ‘International Society
for Geographic and Epidemiologic Ophthalmology’
(ISGEO):
1. Primary angle-closure suspect (PACS):
Iridotrabecu- lar contact in the angle of anterior
chamber in three or more quadrants.
2. Primary angle closure (PAC): PACS plus raised IOP
or peripheral anterior synechiae on
gonioscopy.
3. Primary angle-closure glaucoma (PACG): PAC
plus disk and field changes typical of
glaucoma (as in POAG).

Examination
As in any patient with glaucoma one should record
visual acuity, pupillary responses (especially look for
a relative afferent pupillary defect), IOP,
appearance of the optic disk and visual fields.
Specific to angle-closure glaucoma, one should
look for the Vogt’s triad.
Shallow anterior chamber can be suspected on
torch- light examination with the oblique flashlight
test. Here the flashlight is shown with the light
beam parallel to the iris plane from the temporal
side. If the iris configuration is flat as in a normal
anterior chamber, almost the entire circumference
 Chapter 21 Glaucoma 305
from pupil to the nasal limbus (corneoscleral
junction), we consider the anterior chamber
shallow. This is roughly the same as saying that
with the oblique flashlight test; if the nasal
collarette of the iris is in the shadow, the eye has a
shallow anterior chamber.

van Herick’s Test

van Herick’s test is a method of estimation of
depth of an- terior chamber with the help of
the slit lamp.
Method: To perform the van Herick’s test, the slit
beam is made very bright and thin. It is set at 60°
temporally to the eye. The thickness of the cornea
is compared to the depth of the peripheral anterior FIGURE 21.27: Gonioscopy
chamber. If the anterior cham- ber depth is as
deep as the cornea is thick, then the angle is
presumed to be wide open. If there is only a slit
of aqueous then the angle is estimated to be
dangerously narrow.

Gonioscopy
The definitive way of knowing what is happening
in the angle of the anterior chamber is to visualize
it using special lenses called gonioscopes (gonio
means angle) (Fig. 21.27) along with the slit lamp
(Figs 21.28A and B, 21.29A and B). With this
technique, one can see if the anterior chamber
angle is open or closed as well as note other
features like peripheral anterior synechiae
(adhesions between iris and anterior structures,
viz. trabecular meshwork and cor- nea) (Figs
20.30A and B).
In the latent stage, the peripheral iris root is
often not- ed to be close to the trabecular
meshwork, though not ap- posed to it.
In angle-closure glaucoma, the angle is
closed by ap- position of iris root to the trabecular
meshwork. Here, in- dentation on the cornea
would open up a gap between the two tissues, as
will pupillary constriction.
 Chapter 21 Glaucoma 306

FIGURES 21.28A and B: Structure in angle of anterior chamber. A. Photograph; B. Diagrammatic representation.

FIGURES 21.29A and B: Closed angle. A. Photograph; B. Diagrammatic representation.

At a later stage, when apposition has gone for

some time, the tissues stick to each other importance and one can learn a lot about
structurally and can- not be separated and now it is pathogenesis of angle closure with these tests. In all
called synechia (plural syn- echiae). Now the closure these tests, pretest IOP and gonioscopy are done.
is in chronic angle-closure glau- coma stage. Then the pupil is stressed to pre- cipitate a relative
pupillary block and look for the rise in IOP and
Provocative Tests pupillary block by repeating gonioscopy.
Darkroom test
Provocative tests were been used clinically in the past,
but now they are rarely used as the test itself can Pretest IOP and gonioscopy are done. Then the
precipitate an acute angle-closure glaucoma attack. patient is allowed to remain in a darkened room
These are of historical for 20 minutes and IOP is checked and pupillary
block is looked for, by gonioscopy.
 Chapter 21 Glaucoma 307
FIGURES 21.30A and B: Peripheral anterior synechiae. A. Photograph; B. Diagrammatic representation.
 Chapter 21 Glaucoma 308

This is perhaps the most physiologic of these Laser iridotomy: An opening is made in the peripheral
tests as pupil dilatation in a darkened environment is iris without loss of tissue with the help of a laser
used to pre- cipitate a relative pupillary block. beam.
Prone provocative test
After pretest pressures and gonioscopy, the patient
has to lie down prone for 20 minutes. By the effect
of gravity the weight of the lens is expected to
appose the pupil more firmly to it. This should
worsen a pupillary block and raise the IOP by
closure of the anterior chamber angle.
Darkroom prone provocative test
In the darkroom prone provocative test, the prone
pro- vocative test is done in a darkroom, i.e. both the
darkroom test and the prone provocative tests are
combined to stress the pupil more.
Mydriatic test
After taking the IOP and performing gonioscopy, a
short acting mydriatic is instilled in the eye (e.g.
tropicamide). When the pupil is in the mid-dilated
position the pres- sure and gonioscopy are
repeated. The flaw is that, with the sphincter
paralyzed, the pupillary block rarely occurs as pupil
cannot grip the lens. Often pupillary block is pre-
cipitated when the pupil constricts after dilatation
and can even go in for a full blown acute angle-
closure attack. The time duration can vary by few
hours, because different persons would take different
time durations to constrict after dilatation.
Mapstone test
Here the pupil is stressed purely pharmacologically.
The 1% pilocarpine, which causes contraction of
sphincter pa- pillae, as well as 10% phenylephrine,
which causes con- traction of the dilator papillae is
concomitantly instilled in the eye. Contraction of
both the muscles can result in a pupillary block
and the same is looked for.
If the result is positive in all these test, than the
patient has to be treated. But a negative result does
not mean that the patient is safe. They were thus
relevant as one more ev- idence to proceed to open
surgery in an era when that was the only option. Now
with the less invasive laser iridotomy (Figs 21.31A to
D) becoming popular, the relevance of pro- vocative
testing has diminished.

Management
Management of primary angle-closure glaucoma is
pri- marily surgical (in contrast to POAG where it is
primarily medical).

Management Options in Angle-closure Glaucoma

 Chapter 21 Glaucoma 309
Indications populations show that only a small per- centage of
1. Acute angle-closure glaucoma. these patients eventually develop angle-closure
2. Chronic angle-closure glaucoma. glaucoma. So, this group warrants no treatment. But
3. Prophylactically in the fellow eye of acute this group is at risk compared to the general
angle-clo- sure glaucoma. population and
4. Narrow/Occludable angle.
5. Miscellaneous conditions, including
phacomorphic glaucoma, aqueous
misdirection, nanophthalmos, pigmentary
dispersion syndrome and plateau iris
syndrome.
Principle
The resistance to flow of aqueous is at the pupil.
So, if we bypass the pupil, the pathogenesis of
glaucoma by relative pupillary block could be
halted (refer Figs 21.31A to D).
Laser energy is used to create 100–150 micron
open- ing in the peripheral iris. This is usually
done in an area covered by the upper eyelid to
prevent glare by stray light entering the eye.
Lasers described for the procedure are argon
laser and pulsed Nd:YAG laser. Nowadays
pulsed Nd:YAG laser is more often used.
Procedure
Pupil is constricted with pilocarpine drops to
stretch the peripheral iris. The patient is seated at
the laser under top- ical anesthesia
(proparacaine/lignocaine drops instilled). A laser
iridotomy lens (Abram’s lens) is placed on the
eye to see the iris magnified. A suitable crypt (pit-
like depres- sion in the thin area of iris near iris
root) in the peripheral iris is chosen aimed at and
laser fired a few times to create an adequately
sized peripheral iridotomy (PI) (Fig. 21.32).
Open surgical procedures: This is the another
procedure of management.
Indications
1. When a laser PI alone is not sufficient to control IOP.
2. When other procedures fail to make an
opening on the iris with laser.
Methods
1. A surgical peripheral iridectomy.
2. Drainage procedures like trabeculectomy.
3. Cyclodestructive procedures [in the end-stage
(abso- lute) glaucoma] (refer Chapter 29
‘Glaucoma Surgery’).

Management of Angle-closure Glaucoma

Latent stage: Here the anterior chamber is shallow
predis- posing the patient to develop angle
closure. Epidemiologic studies in various
 Chapter 21 Glaucoma 310

FIGURES 21.31A to D: Laser peripheral iridotomy. A. Normal aqueous flow from ciliary body to trabecular meshwork; B. Pupillary block
causing closure of anterior chamber angle (trabecula); C. Nd:AG or argon laser is used to create an opening in peripheral iris; D. Aqueous
now flows through the peripheral iridotomy, bypassing the pupil to reach the trabecular meshwork in the anterior chamber angle.

hence needs follow-up and education regarding block.

angle- closure glaucoma symptoms.
At least once a year, they need follow-up including
IOP measurement and gonioscopy.
Prodromal stage and stage of intermittent closure: Here
there are symptoms suggesting a rise in pressure in
addition to an occludable angle on gonioscopy. One
can find a closed or occludable angle on
gonioscopy. Here a laser PI would be enough to
relieve a pupillary block. The patient should be
maintained on follow-up.
Acute angle-closure glaucoma: This is a medical
emergency and immediate measures are required to
bring down the IOP and to relieve the pupillary
 Chapter 21 Glaucoma 311
Once diagnosed, one should bring down the
IOP with systemic medications medically as
quickly as possible.
Antiglaucoma measures
Intravenous 20% mannitol 200 mL given rapidly
over 20 minutes. Intravenous acetazolamide, if
available is given.
Oral acetazolamide 500 mg immediately and
250 mg 6 hourly is started. Oral 50% glycerol 60
mL 8 hourly can be added.
But oral medications are rarely tolerated,
since the pa- tient will be having nausea and
vomiting. Topical aqueous suppressant drug timolol
0.5% twice daily can be added. Pilo- carpine 1%–2%
four times daily is also useful (once pressure starts
to come down and pupil can react) by causing
miosis.
 Chapter 21 Glaucoma 312

medical therapy for glaucoma can be instituted or

repeat trabeculectomy may be contemplated if topical
therapy is not successful in controlling the IOP.

FIGURE 21.32: Laser peripheral iridotomy opening

Analgesics and antiemetics

Meanwhile, patient’s pain and general condition may
be managed with injection Pethidine and
Phenergan or in- jection tramadol and
domperidone.
Note: Acetazolamide can cause hypokalemia and
meta- bolic acidosis. It is contraindicated in patients
with allergy to sulfa drugs. Glycerol is high calorie
liquid and diabetic control can be lost. Patients
prone to congestive cardiac failure may not
tolerate the large rapid volume of man- nitol going
into circulation. Timolol can precipitate heart blocks
and obstructive pulmonary airway disease. Prior to
administration of drugs, the presence or absence of
con- traindications should be searched for in
history.
Specific management after the acute phase
Once the IOP is brought down and the corneal edema
has decreased, the gonioscopy is done. If there is
no synechial closure or if the synechiae are less
than 180° or 6’O clock, then a PI should suffice. If the
iris is too edematous and la- ser iridotomy is not
possible on two or more attempts, one can opt for
a surgical iridectomy.
If synechiae are more than half the
circumference of the angle, one should proceed
doing a trabeculectomy. This is undertaken only
after the inflammation has sub- sided (to improve
the chances of success of surgery) till then the IOP
can be kept under control with topical and
systemic antiglaucoma medications.
After this the patient needs regular follow-up for
life. If the IOP goes up during follow-up, topical
 Chapter 21 Glaucoma 313
Chronic angle-closure glaucoma: Here there is procedures to bring down the pressure and thus to
presence of synechiae in the angle on make the patient comfortable.
gonioscopy. Plateau iris configuration: Here again a laser PI is
As mentioned above, a laser PI should suffice, if done. If the angle does not open up after the
the syn- echiae are covering less than half the pupillary block component is removed, it is called
circumference of the angle. After this the patient plateau iris syndrome. These patients respond well
needs regular follow-up for life. If the IOP goes up to pilocarpine therapy, which pulls the peripheral
during follow-up, topical medical therapy for rolls of iris out of the angle.
glaucoma can be instituted.
If the synechiae are more than half the
circumfer- ence, the chances of iridotomy alone
sufficing are less by considering the risks
involved. Classically, we were told to proceed to
trabeculectomy at this stage. We would try
medical therapy after a laser iridotomy. Only if
this is not controlling the IOP or the patient’s
compliance is poor, surgery is done.
When medical therapy and laser iridotomy are
inade- quate to control IOP, one has to proceed to
trabeculectomy.
One would proceed to trabeculectomy in
advanced disk damage also prior to trial of
medical therapy.
Drugs used
1. Beta blockers: Aqueous suppressant gives
about 20%– 30% reduction in ocular pressures.
For example, timo- lol 0.5%.
2. Parasympathomimetics: Pilocarpine drops are
avail- able as 1%, 2% and 4%. Most often
used in glaucoma therapy at 2%
concentration. Cause miosis and pull iris from
the angle opening it. Also contraction of lon-
gitudinal ciliary muscles pull on sclera spur
and open spaces in the trabecular meshwork,
reducing trabecu- lar resistance in areas of
open angle.
3. Topical carbonic anhydrase inhibitors:
Dorzolamide and brinzolamide, tid dose, reduce
aqueous secretion.
4. Alpha-agonists: This increases outflow and
decreases inflow. Prone to cause ocular
surface side effects and drowsiness
(contraindicated in infants, as they can cause
sleep apnea), e.g. brimonidine.
5. Prostaglandin analogs: In presence of a fully
closed angle their effect is not much and so
unlike in open- angle glaucoma, these are the
last choice in angle-clo- sure glaucoma.
Absolute glaucoma: Here there is a blind eye with
high pressure. If there is pain due to high
pressure, one should proceed to cyclodestructive
 Chapter 21 Glaucoma 314

CONGENITAL AND Any visual impairment in childhood impairs

DEVELOPMENTAL GLAUCOMA develop- ment of vision (Box 21.1) and leads to
amblyopia.
Glaucoma occurring in the very young are
different and there are certain features that make BOX 21.1: Causes of poor visual prognosis in
them stand apart from glaucoma in an adult: congenital glaucoma
1. The outer coats of the eye (sclera and cornea) Corneal opacities
are elas- tic in the first 3 years of life and hence Subluxation of lens
can stretch with rise in pressures. As a Anisometropia and amblyopia
consequence, glaucomatous eyes enlarge in this High risk for failure of surgeries to control intraocular pressure
age group and are often described as
‘buphthalmos’ or bull’s eye (Figs 21.33A to
D). CLASSIFICATION
2. The IOP also tend to be lower, when measured, Glaucoma in childhood can be thought of as three
for the same reason. A lowering of pressure differ- ent groups:
tends to cause some degree of shrinkage
1. Primary congenital glaucoma: Where a develop-
also.
mental anomaly of the angle of the anterior
3. The optic nerve cup is often very large, as the chamber causes resistance to outflow and it has
sclera rim of the optic disk stretches. The no association with other ocular or systemic
cupping also comes down with control of anomalies.
pressures in the eye, in contrast to older-age 2. Developmental glaucoma: It is associated with
glaucoma. oc- ular congenital anomalies, where the rise
4. The change in size of the globes causes in IOP is caused by the congenital anomalies.
change in re- fractive errors rapidly (to myopia
with high pressures and reversal of myopia
when pressures come down).
 Chapter 21 Glaucoma 315

FIGURES 21.33A to D: Buphthalmos. A. Photograph; B. Congenital glaucoma eye with Haab’s striae as shown in diagrammatic overlay
in inset. Note the grossly enlarged eyeball with corneal haze due to edema; C. Corneal diameter measured from white to white limbus;
D. Corneal diameter of 15.5 mm in a child.
 Chapter 21 Glaucoma 316

3. Secondary glaucoma in childhood: The causes are Corneal edema: This is a direct result of IOP rise,
same as in adults, but enlargement of produc- ing diffuse corneal haze and sometimes
globes and amblyopia complicates the localized stromal opacities. The stretched cornea may
situation. develop Descemet’s membrane tears called Haab’s striae
Buphthalmos was a term used synonymous with (refer Figs 21.33A to D).
pri- mary congenital glaucoma by most and
sometimes to include all childhood glaucoma, where
the eye enlarged. To avoid confusion most experts
now refer to childhood glaucoma under the above
three headings, viz. congeni- tal glaucoma (or
primary congenital glaucoma), develop- mental
glaucoma and secondary glaucoma.

PRIMARY CONGENITAL
GLAUCOMA
Epidemiology
It is estimated that primary congenital glaucoma
occurs in 1 out of 10,000 live births and represents
22.2% of all childhood glaucoma. About 80% of
these patients have onset within the 1st year of
life with 25% diagnosed at birth itself.
Open-angle glaucoma presenting after 3 years of
age do not have buphthalmic features and are often
classified as juvenile glaucoma (POAG presenting
prior to age of 35 years. It is not yet clear as to
whether the pathogenesis is that of congenital
glaucoma or of POAG).
Most congenital glaucoma occurs as sporadic
cases, though approximately 10% have a positive
family history, suggesting an autosomal recessive
inheritance with vari- able penetrance. It is assumed
that parents of a child with congenital glaucoma have
a 3% chance of having a second child with the
same disease.

Clinical Features
Classic triads of symptoms suggesting congenital
glau- coma are:
1. Epiphora (watering from eyes).
2. Photophobia (intolerance to light).
3. Blepharospasm (inability to open eyes in bright
light).
All these symptoms are caused by corneal
edema and the consequent epithelial
irregularities.
4. Additionally the large size of the cornea and
corneal haze/opacification may be noted.

Examination Findings
Corneal Changes
 Chapter 21 Glaucoma 317
These are typically a pair of parallel glassy lines
at level of Descemet’s membrane oriented
horizontally or concen- tric to the limbus and
can be single/multiple.
Differential diagnosis
Haab’s striae: Tears in the Descemet’s
membrane can oc- cur with trauma from a
forceps delivery. The tears caused by forceps
tend to be vertically oriented and more often in
the left eye, due to the more common left
occipitoanterior obstetric presentation.
FIGURE 21.34: Megalocornea
Corneal opacification and haze: May be due
to a variety of causes, viz. sclerocornea, corneal
dystrophies, inflamma- tion (e.g. interstitial
keratitis), inborn errors of metabolism
(mucopolysaccharidoses and cystinosis).
Congenital en- dothelial dystrophy mimics
glaucoma most, as these in- fants present with
diffuse corneal edema.
Corneal diameter: Often the most striking feature
is the large corneal diameters. A newborn’s
cornea has a hori- zontal diameter of 9.5–10.5
mm. This enlarges by another 0.5–1.0 mm in the
1st year of life. A corneal diameter more than 12
mm in the 1st year is suspicious (refer Figs 21.33A
to D). At any age corneal diameter of 13 mm or
more or an asymmetry between the two eyes are
suspicious findings.
Differential diagnosis
Megalocornea: Here the corneal diameter is
large (often 14 mm or more) and no other
pathology is present in the eye (often X-linked
recessive inheritance). High myopes also have
large eyes with relatively large corneas. Both these
sit- uations have normal IOP and optic nerve cup
(Fig. 21.34).

Anterior Chamber, Iris and Lens

Anterior chamber is uniformly deep. Iris often
looks nor- mal, but may have stromal hypoplasia
with loss of crypts and iridodonesis. The lens is
typically clear, but looks obvi- ously flattened out
and in late stages zonular dehiscence and
subluxation are seen. These features are explained
easily by the enlargement of the globe with
consequent circumferential stretch.
 Chapter 21 Glaucoma 318

Refractive Error therapeutic interventions.

Optic disk in a child is usually pink with very small
Progressive enlargement of the globe causes axial
cup. In congenital glaucoma the disk changes are
myopia due to sclera stretching. The stretching of the
similar to POAG, but do not indicate loss of tissue to the
zonules due to the enlargement of the eye leads to a
same extent
flattening and pos- terior displacement of the lens.
This will compensate the myopia to some extent.
Presence of Haab’s striae can lead to astigmatism.
Progressive myopic shift in the very young should
alert one to the possibility of glaucoma.

Tonometry
Tonometry in an alert child is difficult. A struggling
child would cause IOP estimation to be falsely high
due to the child’s Valsalva maneuver. One may
achieve pressure measurements in a child under
topical anesthesia when adequately distracted as
when feeding from a bottle or nursing. Handheld
tonometers like Perkins applanation tonometer or
tonopen are the best implements to esti- mate IOP
in children. Normal IOP in infants is less than in
adults and are about 10 mm Hg. By about 7 years
this becomes about 13 mm Hg as opposed to the
adult mean IOP of 16 mm Hg. In congenital and
infantile glaucoma, the pressures are usually in the
range of 30–40 mm Hg.
Quite often IOP measurements need to be done
under general anesthesia. Here one has to consider
changes in pressure due to drugs used in anesthesia,
e.g. halothane causes a significant reduction in
pressures whereas ket- amine and succinylcholine
cause significant elevation of pressures.

Gonioscopy
Any goniolens may be used though gonioprisms
like the Koeppe lens are easier to use in a supine
child. Unlike adults the infant’s trabecula is less
granular in appearance and is non-pigmented. In a
glaucomatous child the ante- rior insertion of iris in
the angle ahead of the sclera spur is seen. The
trabecula is glassy and looks like a membrane (this is
called Barkan’s membrane, which is not demonsa-
trated on histopathological examination). The
peripheral iris may be concave and is seen to have
a ‘wrap around’ configuration as it approaches
the angle.

Fundus Examination
Estimation of the ONH cupping and its response to
changes in pressures ar e the most important findings
in diagnosis and assessment of response to
 Chapter 21 Glaucoma 319
as expected from the cupping. This is because the
scleral rim in a child stretches and the disk size
itself enlarges, adding to the empty space in the
middle as cupping. The lamina cribrosa also
stretches backwards and the cup looks very
deeply excavated. With reduction in pressures the
cupping reverses to a great extent and now the
actual loss of nerve fibers in the optic disk will
reveal as the re- maining cupping.

Visual Fields
Being a psychovisual test requiring correct
responses from the patient, visual field charting is
often not possible in chil- dren. Once they are old
enough to cooperate, this can be done and the
field loss is similar to that in POAG. Typically
automated perimetry can be reliably done by
the age of 8 years. But sometimes children as
young as 4 can cooperate for testing (one should
let them try and practice on the field analyzer
from a very young age).

Visual Acuity
Visual acuity needs to be recorded frequently.
Good pres- sure control alone, even in the
absence of optic atrophy does not guarantee
good visual acuity. Corneal opacities, Haab’s
striae, refractive errors and subluxated lenses all
can lead to loss of clear central vision. In addition,
ambly- opia would set in due to anisometropia
(difference in re- fractive errors between the
eyes) and strabismus (squint), which are often
present.
Amblyopia is loss of visual acuity due to
blurred im- ages at the fovea or dissimilar
images being projected on the two foveae during
the age of visual development—less than 7–10
years. The loss is more profound at younger age
than when the child is 10 year old.
As much information as possible, is obtained
in the first examination itself and an examination
under general anesthesia is planned when
congenital glaucoma is sus- pected. Whenever, an
examination under general anes- thesia is
planned it is to be done by the surgeon trained
to operate on pediatric glaucoma and if required,
the surgery is proceeded at the same sitting to
reduce risk involved in general anesthesia.

The symptoms and signs of congenital glaucoma CLINICAL FEATURES

are given below.
Sx Symptoms
Triad of epiphora, blepharospasm and
photophobia.
 Chapter 21 Glaucoma 320

Signs sitting itself.

If the control of pressure is inadequate, a repeat
• Large eye with thin bluish sclera sur- gery is done at a new site and another quadrant
• Corneal changes—increased corneal diameter, of tra- becular meshwork is incised open. If pressure
corneal edema, corneal opacities, Haab’s
control by surgery is inadequate medical therapy is
striae
added on.
• Deep anterior chamber
• Subluxation of lens
• Moderate increase in intraocular pressure
• Deep cupping
• Myopic astigmatism
• Gonioscopy—abnormal tissue in angle of
anterior chamber.

PATHOGENESIS

Barkan’s Theory
The incomplete resorption of mesodermal tissue in
the trabecular meshwork area led to a membrane
covering the trabeculae being left behind (Barkan’s
membrane), obstructing flow of aqueous. Smooth
appearance of vis- ible trabecular meshwork
seemed to confirm this on go- nioscopy.
Goniotomy and trabeculotomy are surgeries
devised to incise into this membrane and they
work well to reduce IOP. Unfortunately, histological
studies failed to demonstrate such a membrane.
Now, most experts agree that there is an arrest
of de- velopment of the anterior chamber cells
derived from the neural crest in its angle (destined
to form the trabecular meshwork and Schlemm’s
canal) leading to aqueous out- flow obstruction. A
high insertion of iris and ciliary body anterior to the
sclera spur in the angle, may compress the
trabecular meshwork and this compacted fibers
cause re- sistance to outflow of aqueous (Figs
21.35A to C). Hence the resistance to aqueous
flow is primarily between the anterior chamber and
the Schlemm’s canal in these chil- dren (occasionally
the Schlemm’s canal is segmented and not a
complete ring, but the contribution to resistance to
flow is less from this factor).

MANAGEMENT

Management of congenital glaucoma is primarily

surgical. Once the diagnosis is established, either a
goniotomy or a trabeculotomy is proceeded with, at
the time of examina- tion under anesthesia. Some
surgeons add on a trabecu- lectomy at the primary
 Chapter 21 Glaucoma 321
depression and can lead to sleep apnea.

FIGURES 21.35A to C: A. Normal angle; B. Normal iris insertion;

A. Shows a high insertion of iris and ciliary body anterior to the
sclera spur in the angle may compress the trabecular
meshwork and this compacted fibers cause resistance out
flow of aqueous.

Medical Therapy Options

Surgery is the first option in management. Medical
thera- py is useful in the preoperative waiting
period and to man- age higher IOP after
postsurgery if and when it occurs.
Medical management utilizes the same
medications as in adults. The contraindications
and side effects are the same as in adults. The -
adrenergic agonist brimonidine is usually avoided
in infants as it causes central nervous system
 Chapter 21 Glaucoma 322

Beta blockers are used in half strength to start

with (timolol 0.25%) and are considered the first- Peters’ Anomaly
line drugs in children. To reduce systemic side
effects, all topical drugs are employed with punctal Here there is a defect in the Descemet’s membrane of
occlusion (after instill- ing drops the index finger of cen- tral cornea and endothelium. There is a
the parent is pressed on the medial canthus to corresponding
occlude the canaliculi for 30 seconds to prevent
systemic absorption from the nose via nasolacri- mal
drainage).
Prostaglandin analogs (latanoprost, bimatoprost,
tra- voprost and tafluprost) can be used especially
in older children. Experience in congenital glaucoma
with these drugs is limited.
Topical carbonic anhydrase inhibitors
(dorzolamide and brinzolamide) are well-tolerated
and can be used in children as first or second line
drug.
Miotics are rarely used now in pediatric glaucoma
(except in immediate postoperative goniotomy and
trabeculotomy). Systemic acetazolamide can be
given in a dose of 10–
20 mg/kg body weight/day in the short term.

Surgical Management
Goniotomy and trabeculotomy are the primary
surgical procedures for congenital glaucoma. If they
fail or the cor- nea is too hazy for proper
visualization, trabeculectmy is done (refer Chapter
29 ‘Glaucoma Surgery’).

DEVELOPMENTAL GLAUCOMA
Developmental glaucoma, which are present in
infancy or childhood, are associated with a definite
developmental anomaly.

Axenfeld-Rieger Syndrome
Axenfelf-Rieger syndrome is characterized by a
thickened and more anteriorly placed Schwalbe’s
line (edge of Des- cemet’s membrane), which
appears as a ridge called pos- terior embryotoxon.
Iris strands come from peripheral iris to attach to this
structure obscuring view of angle beyond it.

Rieger’s Syndrome
Rieger’s syndrome may have associated dental
anomalies as well. A developmental arrest leading to
incomplete ante- rior chamber cleavage is the
postulated cause. Half the pa- tients with this anomaly
are expected to develop glaucoma.
 Chapter 21 Glaucoma 323
thinning and opacity. Iris may be adherent to this glaucoma can be differentiated according to the site
opacity and also to the lens. The lens can be of the main outflow resistance and the
cataractous. Half the patients develop glaucoma. configuration of the chamber angle.
Management involves kerato- plasty and if
required cataract surgery to clear the optics of the
eye to allow visual development at an early
age.

Aniridia
Aniridia is bilateral absence of ‘normal’ iris. It is
a mis- nomer as there is always a rudimentary
iris stump in the periphery all around, in variable
width. This stump invari- ably rotates anteriorly
and causes a 360° secondary angle closure and
consequent glaucoma. This anomaly may be
associated with Wilms’ tumor of the kidney.

Secondary Glaucoma in Childhood SECONDARY GLAUCOMA

Most of the secondary glaucoma can occur in
childhood. A few need special mention as these
are frequently en- countered in children.

Glaucoma Following Trauma

Post-traumatic glaucoma are more common in
children as they are more often engaged in sport
activities prone to ocular trauma. Hyphema can
cause acute glaucoma and angle recession can
lead to chronic glaucoma.

Steroid-induced Glaucoma
Children are more prone to allergies (ocular and
systemic,
e.g. vernal conjunctivitis). This would entail use of
steroids and consequent steroid-induced
glaucoma in suscep- tible patients. This often
happens when a patient goes on chronic self-
medication (after the initial prescription). So, all
patients who are on steroids need to be warned
of this potentially blinding side effect.

Secondary Glaucoma (In General)

Secondary glaucoma is a group of conditions of
raised IOP with its ocular effects occurring as a
complication of some other ocular pathology.
It is important to understand secondary
glaucoma as there are critical differences in their
diagnosis and man- agement. The secondary
 Chapter 21 Glaucoma 324

Classification • Ciliary body cysts

Secondary glaucoma can be classified based on the

level of blockage to the drainage of aqueous.

Secondary Open-angle Glaucoma

1. Pretrabecular outflow resistance:
a. Epithelialization of the chamber angle.
b. Early ICE syndrome.
c. Neovascular glaucoma stage II.
2. Trabecular outflow resistance:
a. Pigmentary glaucoma.
b. Pseudoexfoliation glaucoma (PEXG).
c. Steroid-induced glaucoma.
d. Inflammatory glaucoma.
e. Phacolytic glaucoma.
f. Posner-Schlossman syndrome.
g. Fuchs’ heterochromic cyclitis.
h. Ghost cell glaucoma.
i. Hemolytic glaucoma.
j. Neurofibromatosis.
k. Siderosis.
l. Post-traumatic glaucoma with angle
recession.
3. Post-trabecular outflow resistance:
a. Sturge-Weber syndrome.
b. Arteriovenous fistulas.
c. Idiopathic.

Secondary Glaucoma with Narrow Angle

1. Anterior type with outflow resistance in
chamber angle:
a. Neovascular glaucoma stage III.
b. Advanced ICE syndrome.
2. Posterior type with pupillary block and
vitreociliary block mechanism:
a. Glaucoma with pupillary block due to
synechiae.
b. Traumatic dislocation of the lens.
c. Microspherophakia in Weill-Marchesani syn-
drome (ectopia lentis).
d. Intumescent cataract.
e. Iridoschisis.
3. Posterior type with anterior displacement of iris
lens diaphragm:
a. Glaucoma in association with:
• Choroidal bleeding
• Edema of ciliary body
 Chapter 21 Glaucoma 325
• Ciliary body tumors (malignant
melanoma, leiomyoma)
• Malignant glaucoma.
4. Glaucoma associated with contraction of
retrolental tissue:
a. Persistent hyperplastic primary vitreous (PHPV).
b. Retinopathy of prematurity.

Iridocorneal Endothelial Syndrome

Iridocorneal endothelial or ICE syndrome is a
spectrum of diseases in the eye, where the
inner layer of the cornea appears abnormal. It is
associated with corneal edema at moderate IOP
level and a variety of pathological iris al-
terations. The disease is typically unilateral, FIGURE 21.36: Cogan-Reese syndrome
especially in young women. It includes Cogan-
Reese syndrome, Chan- dler’s syndrome and
essential iris atrophy.
The iris nevus syndrome (Cogan-Reese) is
character- ized by the presence of iris nodules.
These are aggregates of melanocytic cells in the
anterior iris stroma, surround- ed by ectopic
endothelial cells and abnormal basal mem-
branes (Fig. 21.36).
The Chandler’s syndrome is a variation of
essential iris atrophy. Early in the disease,
corneal edema is present even if the IOP is
normal or only moderately elevated. En-
dothelial microscopy shows cells that resemble
battered silver. Remarkable iris changes are not
seen until the late stages of the disease.
Essential iris atrophy is a slowly progressive iris
atro- phy with hole formation, ectropion uveae
and distortion of the pupil. The pretrabecular
secondary open-angle glaucoma turns into
secondary narrow-angle glaucoma with peripheral
anterior synechiae in the late stages of the
disease.
 Chapter 21 Glaucoma 326

Treatment outflow pathways in stage I.

FIGURE 21.37: Iris new vessels
Treatment for early stages of glaucoma in ICE
syndrome is with aqueous suppressants. In late
stages, glaucoma filter- ing surgery is effective;
although late failure may develop due to
endothelialization of the fistula, which in some cas- es
may be reopened with the Nd:YAG laser. The
corneal problem will need keratoplasty.

Neovascular Glaucoma
The clinical sign of NVG is the rubeosis iridis with
elevated IOP.

Causes
Retinal ischemia is the most important mechanism
that results in the anterior segment changes causing
NVG. This leads to liberation of VEGF, which leads to
new vessel pro- liferation in the fundus as well as in
the angle and surface of iris:
• Proliferative diabetic retinopathy
• Retinal vein occlusion together cause two third of
cases
• Carotid artery occlusion
• Sickle cell retinopathy
• Eales’ disease
• Retinopathy of prematurity
• Carotid-cavernous fistula
• Radiation retinopathy
• Severe intraocular inflammation
• Central retinal artery occlusion (rarely)
• Intraocular tumors.
In stage I, new vessels are found on the
anterior sur- face of the iris (Fig. 21.37). These arise
from the iris arteries and start at the pupillary border.
The process of neovascu- larization progresses
toward the chamber angle, but does not reach the
 Chapter 21 Glaucoma 327
In stage II, the open chamber angle becomes the pupillary margin of the iris and throughout the
covered with a fibrovascular membrane. Once inner surface of the anterior chamber (Fig. 21.38). It
this occurs, IOP may rise, resulting in secondary is also associated with second- ary open-angle
open-angle glaucoma. glaucoma, known as pseudoexfoliation glaucoma.
The next stage (II–III) is a progressive closure Pseudoexfoliative material can be seen on the
of the angle. The formation of anterior
synechiae pulls the iris forward, up to
Schwalbe’s line.
In stage III, the chamber angle is completely closed.

Management
Regression of the neovascularization, prior to the
cham- ber angle coverage, may be induced by a
panretinal laser photocoagulation.
Injection of anti-VEGF factors like bevacizumab,
ra- nibizumab can lead to regression of new
vessels.
In the end stages of the disease, where
angle is closed with neovascular membrane
trabeculectomy will not be able to control raised
IOP. Filtering surgery with drain- age implants
can be done if there is visual potential or cy-
clodestructive procedures can be done, if there is
no visual potential.

Pigmentary Glaucoma
Pigmentary glaucoma is characterized by
deposition of pigment granules in the anterior
segment and loss of iris pigment epithelium. This
condition is typically seen in young male
myopes.

Clinical Features
Iris depigmentation results in radial slits parallel
to the zonules of the lens, which can be observed
at the slit lamp using retroillumination. A vertically
oriented pigment line at the center of the
posterior cornea is known as a Kruke- nberg
spindle. Pigment deposits are found in the
trabec- ulae, the periphery of the lens and along
the zonules. A classic sign is the concave iris
configuration, with a deep anterior chamber.
Medical and surgical treatment regi- mens are
the same as for POAG. Nd:YAG laser iridotomy
may be considered to overcome the postulated
reverse pupillary block, in eyes with a posterior
bowing of the iris root towards the zonules.

Pseudoexfoliation Glaucoma
Pseudoexfoliation glaucoma (PEXG) entity is
character- ized by flakes of granular material at
 Chapter 21 Glaucoma 328

Causes
In most cases of glaucoma associated with uveitis,
the anterior chamber angle is open and the increase
in IOP

FIGURE 21.38: Pseudoexfoliation glaucoma

pupillary border of the iris without dilation.

Gonioscopy shows a discontinuous pigmentation of
the trabecular meshwork, usually less dense than
seen in pigmentary glaucoma. Also, the pigment is
characteristically depos- ited on the Schwalbe’s line
or anterior to the Schwalbe’s line (Sampaolesi’s
line). Other signs of pseudoexfoliation syndrome are
insufficient mydriasis, posterior synechiae, pigment
deposition on the iris surface, deposition of pig-
ment and pseudoexfoliation material on the
corneal en- dothelium, pigment liberation after
pupillary dilation and pseudoexfoliation material
covering the ciliary processes and the zonules.
Phacodonesis, lens subluxation and cor- neal
endothelial decompensation can be present. An as-
sociated nuclear cataract is a common finding.
The treatment of PEXG is with topical
medications in early stages. Because patients with
PEXG have higher IOP, they tend to undergo
glaucoma filtering surgery more fre- quently than
patients with POAG. Cataracts occur more commonly
in patients with pseudoexfoliation syndrome.
Weakness of the zonular fibers, spontaneous lens
sublux- ation and phacodonesis also can be present.
Therefore, in these patients, cataract surgery alone or
combined cata- ract surgery and glaucoma filtering
surgery in the pres- ence of pseudoexfoliation is
associated with a higher in- cidence of
intraoperative complications, most notably zonular
dialysis, vitreous loss and lens dislocation.

Glaucoma Associated with

Inflammation
Glaucoma can occur as a complication of
uveitis.
 Chapter 21 Glaucoma 329
results from direct involvement of the increased production of trabecu- lar meshwork-
trabecular mesh- work. It can be due to local inducible glucocorticoid response (TIGR) protein,
inflammation (e.g. second- ary trabeculitis) or which could mechanically obstruct outflow. Pa- tients
as a consequence of accumulation of on chronic corticosteroid therapy can remain undi-
inflammatory debris. agnosed with an elevated IOP, which can result in
In the late stages, an increase in IOP can occur glauco- matous optic nerve damage.
as a re- sult of secondary angle closure following
pupillary block.
Glaucoma can also occur as an effect of steroid
thera- py (steroid-induced glaucoma).
Very rarely, in patients prone for narrow-
angle glau- coma use of mydriatics as part of
treatment for uveitis can trigger an angle-
closure attack.

Treatment
Includes the treatment of underlying cause of
the uveitis, along with both anti-inflammatory
(topical corticosteroids) and antiglaucoma
medications (aqueous suppressants).
Cycloplegics are used to prevent or break
already formed posterior synechiae and to
decrease choroidal effusion.
Miotics are avoided because their use may
exacerbate ciliary spasm, inflammation and
increase the likelihood of synechia.
Prostaglandins are also avoided, as they may
exacer- bate the inflammatory component.
If the patient is found to be steroid
responders, dis- continue or change the steroid
medication. If this is not possible, then more
aggressive management of the IOP is needed
until the steroid can be discontinued.

Steroid-induced Glaucoma
Causes
Steroid-induced IOP elevation typically occurs
within a few weeks of beginning steroid therapy.
More people re- spond from topically applied
drops (including topically applied creams to the
periorbital area) or intravitreal in- jection. Not all
patients taking corticosteroids will develop
elevated IOP. Risk factors include pre-existing
POAG, a family history of glaucoma, high
myopia, diabetes mel- litus and history of
connective tissue disease (especially rheumatoid
arthritis).

Mechanism
Exact pathophysiology of steroid-induced
glaucoma is unknown. The defect could be
increased accumulation of glycosaminoglycans or
 Chapter 21 Glaucoma 330

Treatment Iridocyclitis
Includes discontinuation of corticosteroids or Fuchs’ heterochromic iridocyclitis (FHI) is a chronic, uni-
switching over to a lower potency steroid lateral iridocyclitis characterized by iris heterochromia.
medication. The classic triad of FHI is heterochromia, cataract and
Antiglaucoma medications are also needed to KPs.
control the high IOP till the effect of steroids
wears off.
In the occasional cases in which the patient’s IOP
does not normalize by stopping steroid drops or in
those pa- tients, who must continue on corticosteroid
medications, prolonged use of standard antiglaucoma
medication will be required. Only rarely
trabeculectomy is required.

Posner-Schlossman Syndrome
(Glaucomatocyclitic Crisis)
Posner-Schlossman syndrome is a condition with
self- limited recurrent episodes of markedly elevated
IOP with mild idiopathic anterior chamber
inflammation.

Clinical Features
1. Uniocular involvement.
2. Recurrent episodes of mild cyclitis with keratic
pre- cipitates (KPs) and aqueous flare.
3. Duration of attack varying from a few hours to
several weeks.
4. A slight decrease in vision.
5. Elevated IOP with open angles.
6. Corneal edema with a few KPs.
7. Heterochromia with anisocoria and a large
pupil in the affected eye.
8. Normal visual fields, normal optic disk, normal
IOP and outflow facility.
9. All provocative tests are normal between
episodes.
The presence of KPs on slit lamp examination
gives the clue to the diagnosis.

Treatment
Treatment recommended include topical steroids,
topi- cal antiglaucoma drops, systemic carbonic
anhydrase inhibitors, topical non-steriodal anti-
inflammatory durgs (NSAIDs) and oral NSAIDs.

Fuchs’ Heterochromic
 Chapter 21 Glaucoma 331
Clinical Features optic nerve prior to injury). If treatment is
indicated, aqueous suppressants (e.g. beta blockers,
1. Keratic precipitates are numerous, small, non- - agonists) are the mainstay of treatment.
pig- mented, translucent and star shaped
(stellate) and are nearly pathognomonic.
These are distributed over the entire
posterior corneal surface.
2. There is minimal anterior chamber cells and flare.
3. Posterior synechiae are never present.
4. Many cases develop a posterior subcapsular
cataract, which mature rapidly.
5. Whitish vitreous cellular infiltrates, varying
from dust like to stringy veils are observed.
Any young male patient presenting with a
unilateral cataract has to be examined in
natural light for hetero- chromia and KPs.

Management
In general, treatment is not necessary for patients
with the typical low-grade inflammation.
Symptomatic flare-ups may require short-term
topical corticosteroids.
Most secondary glaucoma associated with
FHI can be controlled with antiglaucoma
medications. Glaucoma filtering procedures in
patients with FHI are less success- ful compared
with that for patients with POAG. Glaucoma
drainage implants may improve the outcome of
glaucoma surgery for patients with uveitic
glaucoma. Cataract sur- gery has good visual
prognosis.

Traumatic Glaucoma
Traumatic glaucoma include:
1. Angle-recession glaucoma.
2. Acute glaucoma associated with hyphema.
3. Late onset ghost cell glaucoma.
4. Glaucoma associated with traumatic uveitis.
5. Phacolytic glaucoma.
6. Glaucoma associated with lens dislocation.
In the acute phase, the presence of blood in
the ante- rior chamber (hyphema) or inflammation
as a result of in- jury (e.g. traumatic iridocyclitis)
may cause an increase in IOP that mandates
treatment.
Since, acute increases in IOP due to blunt
trauma may be only for a short duration,
observation and careful follow-up may be all that
is required (assuming the pres- ence of a healthy
 Chapter 21 Glaucoma 332

Angle-recession Glaucoma Surgery might be required to clear the cell load from the
trabecular meshwork. This can be accomplished by
Angle-recession glaucoma is a type of traumatic anterior cham- ber paracentesis and irrigation, pars
second- ary open-angle glaucoma. In angle recession, plana vitrectomy (PPV) and/or a trabeculectomy.
there is vari- able degree of cleavage between the
circular and the lon- gitudinal fibers of the ciliary
muscle.
On gonioscopy the ciliary band will be widened
and in long-standing cases there will be
hyperpigmentation of the angle and the widening
will not be obvious due to scarring. It can present
with and without glaucoma and it may take several
years for the glaucoma to manifest.
Management: Long-term follow-up care of patients
with recognized angle recession is warranted
because of the risk of delayed asymptomatic
onset. Of those eyes with known angle recession,
0%–20% subsequently glaucoma develops. Glaucoma
after angle recession of less than 180° is unusual;
recessions greater than 180° are associated with a
4%–9% incidence of glaucoma. Eyes with angle re-
cession of greater than 240° appear to be at the
highest risk of chronic glaucoma.
Treatment: In patients with an abnormal elevation of
IOP aqueous suppressants or prostaglandin analogs
are given.
Surgical intervention in angle-recession
glaucoma is usually indicated when maximally
tolerated medical treatment has failed. Filtration
surgery has a success rate lower than that of
POAG.

Ghost Cell Glaucoma

Ghost cell glaucoma is a secondary open-angle
glaucoma caused by degenerated red blood cells
(ghost cells) block- ing the trabecular meshwork.

Mechanism
Following a vitreous hemorrhage episode, blood
break- down products may accumulate in the
trabecular mesh- work. Hemolyzed erythrocytes
may obstruct aqueous outflow and lead to a
secondary open-angle glaucoma known as ghost
cell glaucoma. Ghost cells are general- ly 4–7
micrometers in size and less pliable than normal
RBCs. As a result of their loss of pliability, ghost
cells remain longer in the anterior chamber
because their ri- gidity makes it difficult for them
to escape through the trabecular meshwork.

Treatment
Aqueous suppressants are the first-line approach.
 Chapter 21 Glaucoma 333
Malignant Glaucoma Phacolytic glaucoma is the sudden onset of open-
angle glaucoma caused by a leaking mature or
Malignant glaucoma describes an entity hypermature cataract. It is cured by cataract
characterized by elevated IOP with a shallow or extraction.
flat anterior chamber in the presence of a patent Phacomorphic glaucoma is the term used for
peripheral iridectomy.
second- ary angle-closure glaucoma due to lens
Malignant glaucoma is rare, but one of the intumescence.
most seri- ous complications of glaucoma
filtration surgery in pa- tients with narrow-
angle glaucoma.

Mechanism
A blockage of the normal aqueous flow at the
level of the ciliary body, lens and anterior
vitreous face is believed to cause malignant
glaucoma. Posterior misdirection of aqueous
humor into the vitreous cavity occurs producing a
continuous expansion of the vitreous cavity and
increased posterior segment pressure. This
accumulation of aqueous fluid in the vitreous
cavity causes anterior displacement of the lens-iris
diaphragm in phakic and pseudophakic eyes or
forward displacement of the anterior hyaloid in
aphakic patients. The resulting shallow or flat
chamber is believed to exacerbate the condition
because of the decreased ac- cess of aqueous to
the trabecular meshwork.

Management
The first line of treatment should be medical:
1. Cycloplegic agents like atropine paralyze the
sphinc- ter muscle of the ciliary body,
increasing zonular ten- sion with flattening,
posterior movement of the lens and
deepening the anterior chamber.
2. Topical beta blockers, -adrenergic agonists
and topi- cal and oral carbonic anhydrase
inhibitors are effective in decreasing aqueous
humor production and lower- ing IOP,
presumably decreasing aqueous misdirection.
3. Osmotic agents help to decrease vitreous
volume and include oral glycerol or
isosorbide or intravenous mannitol.
4. The Nd:YAG laser can break the anterior
hyaloid to al- low free movement of fluid
from the vitreous cavity to the anterior
chamber.
5. If medical or laser treatment fails or if lens-
corneal touch occurs, surgery should be
considered. PPV with or without lensectomy,
disrupts the impermeable an- terior vitreous
face and reduces the vitreous volume.

Lens-induced Glaucoma
 Chapter 21 Glaucoma 305

The increase in lens thickness from an advanced

cataract, a rapidly intumescent lens or a traumatic
Mechanisms
cataract can lead to pupillary block. Laser iridotomy A transient rise can be due to retained
can temporarily stop an attack of acute pupillary viscoelastics or blood in the eye or due to
block. But in most pa- tients with phacomorphic postoperative uveitis. It can be managed with
glaucoma, cataract extraction is needed. Laser topical and systemic antiglaucoma medi- cations and
iridotomy should be performed first, as mydriasis
corticosteroids. An anterior chamber intra- ocular
before surgery can exacerbate the condition or
lens (IOL) is more prone to produce secondary
osmotic agents such as mannitol or glycerine
should be given before surgery to decrease glaucoma.
vitreous volume and deepen the anterior A chronic rise in IOP can occur due to angle
chamber. closure resulting from papillary block by posterior
Phacotopic glaucoma is often due to a synechiae in uveitis. Mydriatics are used to break the
traumatic pa- thology causing anterior subluxation synechiae and if that fails, Nd:YAG laser iridotomy
or dislocation of lens. It is very important to is done to form alter- ative pathway for the
recognize the pathogenic mecha- nism for a proper aqueous.
treatment. It can be pupillary block and/ or angular An open-angle type of secondary glaucoma can
block or phacolytic glaucoma. oc- cur due to vitreous in anterior chamber,
retained lens materials or pigment dispersion.
Management Transient cases can be managed with antiglaucoma
Laser iridotomy may control the glaucoma in mild medications. Prostaglandin derivatives are avoided to
cases and lens removal with/without anterior prevent cystoid macular edema. Removal of retained
vitrectomy will be required in severe displacement lens materials or anterior vitrectomy may be needed
with severe glaucoma and significant drop in vision. in some cases. In some chronic cases tra-
Improvements in lens and vitre- ous surgery have beculectomy with antifibroblastic agents like
changed the prognosis of these diseases. mitomycin may be needed.
In any case of raised IOP, it is essential to take a
Aphakic or Pseudophakic careful history of the problem and careful slit lamp
Glaucoma examination to differentiate between primary and
secondary glauco- ma. Proper diagnosis will help to
Aphakic or pseudophakic glaucoma are secondary manage the problem in the correct way with a
glau- coma occurring after cataract surgery. It is
more successful outcome.
more common in children.
Intraocular
Tumors
2
2
Girija Devi PS

Intraocular tumors comprise a broad spectrum of

benign and malignant lesions that can sometimes Iris Cysts
lead to loss of vision and even loss of life. A Primary Iris Cysts
thorough clinical examina- tion and effective use
of ancillary diagnostic tests aid the clinician in the Primary iris cysts are rare lesions arising from iris
accurate diagnosis and proper manage- ment of epithe- lium (epithelial cysts) or rarely iris stroma
these conditions. (stromal cysts).

TUMORS OF UVEAL TRACT Secondary Iris Cysts

Secondary cysts develop as a result of the following.
TUMORS OF IRIS
Implantation cysts: Due to deposition of surface
epithelial cells following surgical or penetrating
Iris Nevus trauma. They can be pearl cysts or serous cysts
Iris nevus, which is a common benign lesion appears (Figs 22.2A and B).
as a darkly pigmented lesion of the iris stroma with Drug-induced cysts: Prolonged topical use of long
minimal distortion of iris architecture. Iris nevi may acting- miotics.
present in two forms:
1. Circumscribed iris nevus—usually nodular and in- Parasitic cysts: They are rare. Cysticercosis or hydatid
volving a discrete portion of iris. cysts can rarely involve the iris.
2. Diffuse iris nevus—may involve an entire
sector or rarely the entire iris (Fig. 22.1). Malignant Melanoma of the Iris
Iris nevi usually require no treatment. If Iris melanomas account for 3%–10% of all uveal
diagnosed, suspicious lesions need close follow-up melanomas.
and photography to evaluate the growth.
Clinical Features
Malignant melanoma of the iris, usually appear as an
iso- lated pigmented or amelanotic nodule that
grows very rapidly, commonly involving the inferior
iris. They may rarely assume a diffuse growth pattern
resulting in a uni- lateral acquired hyperchromic
heterochromia and sec- ondary glaucoma. If
untreated, it may perforate the globe. Signs
suggestive of malignancy include extensive ec-
tropion iridis, prominent vascularity, secondary
glauco- ma, seedling of angle structures, extrascleral
FIGURE 22.1: Diffuse iris nevus with iris atrophy extension, in- creasing lesion size and documented
progressive growth.

Investigations
Photographic documentation of tumor growth.
 Chapter 22 Intraocular Tumors 307

FIGURES 22.2A and B: Implantation cysts of iris. A. Pearl cyst; B. Serous cyst.

Ultrasound biomicroscopy: Tumor size and anatomical CILIARY BODY TUMORS

re- lationship to normal ocular structures can be
accurately
documented. • Retinoblastoma—iris invasion with or without pseudo-
hypopyon.
Treatment
Diagnostic and therapeutic excision of the
mass:
• Iridectomy, if feasible
• Iridocyclectomy, if the lesion is involving the root
of iris and ciliary body
• Brachytherapy with custom designed plaques in
select cases
• If completely removed, the prognosis is
excellent in most cases.

Differential Diagnosis of Iris Nodule

• Iris nevus
• Iris melanoma
• Iris nodules in chronic anterior uveitis—Koeppe
and Busacca nodules
• Retained foreign bodies can become secondarily
pig- mented and it can also be associated with
chronic in- flammation
• Brushfield spots usually in Down syndrome
• Iris pigment epithelial cysts
• Epithelial invasion, serous cyst, pearl cyst or
implanta- tion cysts
• Juvenile xanthogranuloma—may be associated
with spontaneous hyphema and secondary
glaucoma
• Lisch nodules—hamartomatous lesions seen in
neuro- fibromatosis (Fig. 22.3)
• Metastatic carcinoma—rare
 Chapter 22 Intraocular Tumors 308
Ciliary Body Melanoma
Ciliary body melanomas constitute 12% of the
uveal melanomas (Figs 22.4 and 22.5).

FIGURE 22.3: Lisch nodules in neurofibromatosis 1

FIGURE 22.4: Hemangioma of iris and ciliary body

 Chapter 22 Intraocular Tumors 309

FIGURE 22.5: Ciliary body tumor seen through dilated pupil FIGURE 22.6: Sentinel vessel (black arrow) in ciliary body tumor

Clinical Presentation
Ciliary body melanoma usually present in the sixth
decade with visual symptoms or sometimes detected
incidentally.

Signs
1. Dilated episcleral vessels in the same quadrant
as the tumor (sentinel vessels) (Fig. 22.6).
2. Erosion through iris root may mimic iris
melanoma (Fig. 22.7).
3. Direct visualization of the tumor on dilated
fundus examination. FIGURE 22.7: Ciliary body tumor eroding root of iris
4. Pressure on lens causing astigmatism,
subluxation or cataract formation.
5. Extrascleral extension, posterior spread with
exuda- tive retinal detachment, circumferential
spread, etc. can also occur (Fig. 22.8).

Management
Direct examination with a three mirror contact lens,
ultra- sound biomicroscopy (UBM), biopsy, etc. are
employed to establish the diagnosis (Fig. 22.9).

Treatment
Iridocyclectomy for small tumors, brachytherapy FIGURE 22.8: Ciliary body tumor protruding into anterior
chamber (AC) through pupil
and enucleation for large tumors.

Medulloepithelioma TUMORS OF CHOROID

Medulloepithelioma is a rare embryonal neoplasm
(pre- viously called diktyoma) occurring in the first Choroidal Nevus
decade of life. Malignant forms are fatal due to
Choroidal nevi are composed of benign
intracranial spread or metastatic disease.
proliferation of choroidal melanocytes, usually
 Chapter 22 Intraocular Tumors 310
more common in the
 Chapter 22 Intraocular Tumors 311

Classification
1. Spindle cell melanomas (spindle A and spindle
B)— formed exclusively of spindle cells.
2. Epithelioid cell melanomas—most malignant.
3. Mixed cell melanomas—formed of both spindle
cells and epithelioid cells.

Patterns of Tumor Growth

The tumor can grow inwards, break through the
Bruch’s membrane and retinal pigment epithelium
(RPE) into the subretinal space with the
FIGURE 22.9: Ultrasound biomicroscopy (UBM)
development of a collar stud appearance.
picture of CB mass
Invasion of scleral channels for blood vessels
and nerves, and invasion of vortex veins resulting
white races. They are usually asymptomatic and
in orbital spread. Hematogenous spread to the
detected on routine fundus examination. liver, lungs, bone, skin and brain. Optic nerve
Typical choroidal nevus appears as an oval to invasion is rare.
circular, brown to slate gray lesion in the
postequatorial fundus of- ten with surface drusen. Clinical Presentation
Suspicious nevi are those with documented
The tumor often presents in the fifth to sixth decade
growth, those producing symptoms like blurring of
of life. It is usually primary, single and unilateral.
vision, those showing presence of surface lipofuscin,
etc. They should be kept on close follow-up, Sx Symptoms
reclassified as small melano- mas if needed and
Choroidal melanoma may be either detected
managed accordingly.
inciden- tally on a routine fundus examination or
may become symptomatic. The usual symptoms
Choroidal Melanoma are decreased vision, metamorphopsia, floaters or
Choroidal melanoma is the most common primary photopsia (ball of light).
intra- ocular malignancy in adults and accounts for
80% of all uveal melanomas (Fig. 22.10).
Signs
The tumor appears as a solitary, elevated, dome-
Pathology shaped brown or grayish mass or less commonly as
an amelanotic mass usually in the posterior pole.
The tumor is composed of a malignant
Clumps of orange pigment, lipofuscin, is often
proliferation of choroidal melanocytes either as
spindle cells or as epithe- lioid cells. seen in the RPE overlying the tumor.
Dilated blood vessels may be seen on the
surface in amelanotic masses. Exudative retinal
detachment occurs overlying the tumor.
Other signs—choroidal folds, intraocular
inflammation, hemorrhage, rubeosis, secondary
glaucoma and cataract.

Investigations
Ultrasound scan (USS): B-scan ultrasonography shows a
dome or mushroom-shaped choroidal mass with
internal homogeneity (acoustic hollowing), choroidal
FIGURE 22.10: Choroidal melanoma with extraocular spread excavation and orbital shadowing; a collar stud
configuration is al- most pathognomonic (Fig.
 Chapter 22 Intraocular Tumors 312
22.11).
 Chapter 22 Intraocular Tumors 313

The main treatment options are detailed below.

Brachytherapy (plaque radiotherapy): This therapy with ru-
thenium 106 or iodine 125 applicator is employed in treat-
ment of tumors less than 20 mm basal diameter in which
there is a reasonable chance of salvaging vision.
External beam radiotherapy: Irradiation with charged
par- ticles like protons is usually done in those
tumors, which are unsuitable for brachytherapy
because of their large size or posterior location.
Transpupillary thermotherapy (TTT): This technique uses
an infrared laser beam to induce tumor cell death
by hyper- thermia. It is a useful adjunct to
FIGURE 22.11: Ultrasound scan shows a mushroom-shaped radiotherapy.
mass in choroidal tumor Transscleral choroidectomy: It is a difficult procedure
and not widely performed.
Fluorescein angiography: Most common findings are
Enucleation: This technique is indicated in large
in- trinsic tumor (dual) circulation, mottled
choroidal melanomas, optic disk invasion, extensive
fluorescence during the avenue vision phase and
involvement of the ciliary body or angle,
late diffuse leakage and staining.
irreversible loss of useful vision and poor
Indocyanine-green (ICG) angiography: It shows motivation of patient to keep the eye.
hypofluores- cence throughout the study.
Radioactive tracers: Neoplastic tissue has an Differential Diagnosis
increased rate of phosphorus (P) uptake as • Pigmented lesions:
demonstrated by P32 uptake studies. – Large choroidal nevus
Magnetic resonance imaging (MRI): It shows – Melanocytoma
hyperintensity in T1-weighted images and – Congenital hypertrophy of the RPE
hypointensity in T2-weighted images. – Subretinal/Suprachoroidal hemorrhage.
Biopsy: It is useful when the diagnosis cannot be • Non-pigmented lesions:
estab- lished by less invasive methods. – Circumscribed choroidal hemangioma
– Choroidal metastasis
Systemic investigation: It is aimed at excluding a
– Choroidal granuloma in tuberculosis
metasta- sis to the choroids most frequently from the
lungs in both sexes and from the breast in women – Posterior scleritis.
and detecting pos- sible metastatic spread from
the choroid. In cases with clinical suspicion of Choroidal Hemangioma
metastasis, ultrasound abdomen and chest
Circumscribed Choroidal Hemangioma
radiography may be done.
A circumscribed choroidal hemangioma is not
Treatment associated with systemic disease. It is usually
asymptomatic, but can give rise to symptoms due to
The primary aim of treatment is to avoid the overlying retinal detachment.
development of a painful and unsightly eye,
preferably conserving as much useful vision as Clinical features: Circumscribed choroidal
possible. The following factors are considered in the hemangioma usually presents in two to three
treatment: decades of life as unilat- eral blurring of vision,
1. Size, location and extent of the tumor and its field defect or metamorphopsia.
effect on vision. The lesion appears as an oval orange mass at the
2. Condition of the fellow eye. posteri- or pole with overlying subretinal fluid in
3. Age and general condition of the patient. symptomatic cases. Ultrasound reveals an acoustically
4. Patient consent. solid lesion with sharp anterior surface and high
 Chapter 22 Intraocular Tumors 314
internal reflectivity. Photody- namic therapy, TTT
and radiotherapy are described in the treatment.
 Chapter 22 Intraocular Tumors 315

Diffuse Choroidal Hemangioma Retinoblastoma is caused by a mutation in the RB1

gene on the long arm of chromosome 13q14. This is a
Diffuse choroidal hemangioma occurs almost tumor
exclusively in patients with Sturge-Weber syndrome,
ipsilateral to the nevus flammeus.
The tumor usually presents in the second
decade. The fundus has a diffuse deep red ‘tomato
ketchup’ color mostly in the posterior pole.

Optic Disk Melanocytoma

Melanocytoma is a rare, unilateral, heavily
pigmented, congenital hamartoma usually seen at
the optic nerve head. It is seen more commonly in
the dark skinned and more in females. Most
cases are asymptomatic.
It appears as a brown or black, flat or slightly
elevated lesion with feathery edges that may
extend over the edge of optic disk. Complications
are optic nerve compression, central vein occlusion,
tumor necrosis and rarely malig- nant
transformation.

Choroidal Metastasis
The choroid is the most common site for uveal
metasta- sis. The most frequent primary site is the
breast in females and lungs in males. Other sites are
gastrointestinal tract, kidneys and skin.
They appear clinically as creamy white placoid
lesions with indistinct margins usually at the posterior
pole. The deposits can be multifocal in 30% cases
and bilateral in 10%–30% cases. They are usually
associated with exuda- tive retinal detachment
(Figs 22.12A and B).

TUMORS OF RETINA

RETINOBLASTOMA
Retinoblastoma is the most common primary
intraocular malignancy of childhood.

Epidemiology
The frequency of retinoblastoma ranges from 1 in
14,000 to 1 in 20,000 live births. About 90% of
cases present be- fore 3 years of age. It has no racial
predilection and occurs equally in males and females.
About 60%–70% cases are unilateral and 30%–40%
cases are bilateral.

Genetics of Retinoblastoma
 Chapter 22 Intraocular Tumors 316
melanoma, etc. About 15% of heritable cases can
have unilateral presentation.

FIGURES 22.12A and B: Choroidal metastasis. A. Metastatic

infiltration of ON head; B. Choroid with overlying exudative
RD.

suppressor gene that controls retinal cell division.

In chil- dren with retinoblastoma, retinal cell
division continues unchecked causing retinal
tumors. Both copies of the RB1 gene must be
mutated for the tumor to form. Retinoblas- toma
can be heritable or non-heritable.

Types
Heritable (Germline) Retinoblastoma
Heritable retinoblastoma accounts for 40% of
cases. Here, one allele of RB1 (tumor suppressor
gene) is mutated in all body cells. When a further
mutagenic event (‘second hit’) affects the second
allele, the cell undergoes malignant
transformation. Since all the retinal precursor cells
con- tain the initial mutation, these children
develop bilateral and multifocal tumors. They also
have a predisposition to non-ocular malignancies
like pineoblastoma (trilateral retinoblastoma) and
secondary malignancies like osteo- sarcoma,
 Chapter 22 Intraocular Tumors 317

Non-heritable (Somatic) Retinoblastoma

Clinical Presentation
Non-heritable retinoblastoma accounts for 60% of
Retinoblastoma usually occurs in infants and 90%
cases. It is unilateral, not transmissible and does not cases occur within the first 3 years of life.
predispose to secondary non-ocular cancers. The most common clinical presentation is as a
white pupillary reflex or leukocoria (amaurotic cat’s
Patterns of Tumor eye), i.e. in almost 54%–62% cases (Figs 22.14
Spread (Growth and 22.15).
Pattern) Other modes of presentation include strabismus
(18%–22%), ocular inflammation (2%–10%),
Retinoblastoma may grow mainly outwards pseudohy- popyon, hyphema, heterochromia,
separating the retina from the choroids (exophytic) or secondary glaucoma with buphthalmos, cataract,
inwards toward the vitreous (endophytic) with anisocoria, orbital inflamma- tion, orbital invasion
seeding of tumor cells throughout the eye (Figs in neglected cases, etc.
22.13A and B):
1. Optic nerve invasion with spread along the Diagnosis
subarach- noid space to the brain.
The diagnosis of retinoblastoma is essentially clinical.
2. Diffuse infiltration of the retina. In- direct ophthalmoscopy with scleral indentation
3. Metastatic spread to regional lymph nodes, must be performed on both eyes after full mydriasis
lungs, brain and bone. in all suspect- ed cases.

FIGURES 22.13A and B: Retinoblastoma. A. Exophytic growth; B. Endophytic growth.

 Chapter 22 Intraocular Tumors 318
FIGURE 22.14: Leukocoria in retinoblastoma FIGURE 22.15: Leukocoria (magnified view in the same child)
 Chapter 22 Intraocular Tumors 319

An intraretinal tumor is a homogeneous, chalky Examination should be done in all suspected cases
white dome-shaped lesion often with whitish flecks of and should include tonometry, measurement of
calcifica- tion. An endophytic tumor projects into corneal di- ameter, axial length, anterior chamber
the vitreous as a whitish mass with seeding into the examination with hand-held slit lamp, indirect
vitreous. An exophytic tumor forms as subretinal, ophthalmoscopy with 360°
multilobular mass with overly- ing retinal
detachment. Lesions may be often multiple.

Pathology
Retinoblastoma is composed of small cells with large
hy- perchromatic nuclei and scanty cytoplasm
(retinoblasts). Undifferentiated tumors show no
characteristic arrange- ment of these cells. Well
differentiated tumors show char- acteristic
arrangement patterns as following.
Flexner-Wintersteiner rosettes: Tall columnar cells arranged
around a lumen, with their nuclei lying away from the
lumen.
Homer-Wright rosettes (pseudorosettes): There is no
lumen, but the cells are arranged around a mass of
eosinophilic processes.
Fleurettes: Here the cells show some photoreceptor
dif- ferentiation with long cytoplasmic processes of
a group of cells projecting through a fenestrated
membrane to ap- pear like a wreath of flowers.
Tumor will show areas of necrosis and
calcification.

Clinical Stages
1. Quiescent stage: The tumor is confined within
the eye and presents as leukocoria or squint. The
child is apparently healthy and symptomless.
2. Stage of glaucoma: The involvement of the
angle of AC or the drainage channels will lead
to rise in intra- ocular pressure (IOP). The child
will develop buph- thalmos and the child may
be in distress due to the pain and discomfort
of raised IOP.
3. Stage of extraocular extension: The child will pres-
ent with proptosis (Fig. 22.16).
4. Stage of metastasis: This can be direct
extension along the optic nerve to involve
the brain. Blood- stream spread to cranial bones
(most common), liver and lungs, and lymphatic
spread to preauricular and submandibular
lymph nodes.

Investigations
Examination under Anesthesia
 Chapter 22 Intraocular Tumors 320
retinoblastoma or retinoma, which would provide
evidence for a hereditary predispo- sition for the
disease.

FIGURE 22.16: Extraocular and orbital invasion in a

neglected case of retinoblastoma

scleral indentation and documentation of all

findings with color drawings or photography.

Ultrasound Scan
Ultrasound A- and B-scans are used to assess tumor
size and calcification within the tumor. Calcification
occurs in 75% of cases and is almost
pathognomonic of retinoblastoma.
The B-scan ultrasound displays a cauliflower
like mass arising from the retina, with or without
a retinal detach- ment or vitreous seeds. A scan
through the mass shows a characteristic V-Y
pattern.

Computed Tomography of Brain and Orbit

Computed tomography (CT) can detect
calcification, but confers significant dose of
radiation to the child and hence routinely not
preferred (Fig. 22.17).

Magnetic Resonance Imaging

Magnetic resonance imaging (MRI) is superior to CT
in eval- uation of optic nerve and for detection of
extraocular exten- sion or pinealoblastoma, but
cannot detect calcification.
Systemic evaluation: It includes physical
examination and MR scans of the orbit and skull,
as a minimum in high risk cases. Bone scans, bone
marrow study and lumbar punc- ture is done in
all metastatic disease.

Genetic Studies
Tumor tissue from enucleated eyes and a blood
sample can be taken for DNA analysis. Parents
and siblings should be examined for untreated
 Chapter 22 Intraocular Tumors 321

Chronic toxocara endophthalmitis may cause cyclitic

membrane and a white pupil. A granuloma at the
poste- rior pole can mimic an endophytic
retinoblastoma.

FIGURE 22.17: Computed tomography showing an

intraocular mass with calcification suggestive of
retinoblastoma

Differential Diagnosis
A number of lesions can simulate retinoblastoma. The
dif- ferential diagnoses for a child presenting with
leukocoria include the following.

Persistent Fetal Vasculature or

Persistent Hyperplastic Primary Vitreous
Persistent fetal vasculature or persistent hyperplastic
pri- mary vitreous (PHPV) is typically recognized
within days or weeks of birth. The condition is
unilateral in two third of cases and may be
associated with microphthalmia, shallow AC, iris
hypoplasia and a retrolental fibrovascular mass that
draws the ciliary process inwards. B-scan helps in
diagnosis by showing the persistent hyaloid remnants
arising from the optic nerve head associated with a
closed funnel retinal detachment.

Coats’ Disease
Coats’ disease is almost always unilateral, more
common in boys and tends to present later than
retinoblastoma, i.e. in the first decade (Fig.
22.18).

Retinopathy of Prematurity
Advanced retinopathy of prematurity (ROP) can
cause retinal detachment and leukocoria. There will
be a history of prematurity and low birth weight
in most cases.

Toxocariasis
 Chapter 22 Intraocular Tumors 322
Vitreoretinal Dysplasia
Conditions like Norrie’s disease, incontinentia
pigment, etc. can be associated with a detached,
dysplastic retina forming a retrolental mass
with leukocoria.

Retinoma
Retinoma is a benign variant of retinoblastoma,
which can undergo spontaneous involution and
present as a calci- fied mass.

Retinal Astrocytoma
Retinal astrocytoma tumor appears as a small,
smooth white tumor, which may be solitary or
multiple, unilateral or bilateral. They are usually
seen in patients with tuber- ous sclerosis.

Chronic Endophthalmitis
Chronic endophthalmitis can occur during
intrauterine life or infancy due to septicemia and
vitreous exudation will give a yellow reflex
mimicking a retinoblastoma.

Classification of Retinoblastoma
The Reese-Ellsworth clinical classification is
commonly used in categorizing intraocular
retinoblastoma.

Reese-Ellsworth Classification
Group I: Very favorable:
1. Solitary tumor less than 4DD in size at or
behind the equator.
2. Multiple tumors, none larger than 4DD in
size, all at or behind the equator.

FIGURE 22.18: Advanced Coats’ disease

 Chapter 22 Intraocular Tumors 323

Group II: Favorable: histopathologically and the second eye is treated with
1. Solitary tumor, 4–10DD in size at or behind radiation. Nowadays the fo- cus is to conserve the eye
the equator. as far as possible if there is any
2. Multiple tumors, 4–10DD in size behind the
equator.
Group III: Possible to maintain sight:
1. Any lesion anterior to the equator.
2. Solitary tumor, larger than 10DD in size behind
the equator.
Group IV: Unfavorable:
1. Multiple tumors, some larger than 10DD in
size.
2. Any lesion extending anteriorly to the ora
serrata.
Group V: Highly unfavorable:
1. Massive tumors involving more than one half
of the retina.
2. Vitreous seeding.

International Classification
of Intraocular Retinoblastoma
Group A: Small tumors (< 3 mm) outside macula.
Group B: Bigger tumors (> 3 mm) or any tumor in
macula or any tumor with subretinal fluid.
Group C: Localized seeds (subretinal or vitreous).
Group D:Diffuse seeds (subretinal or vitreous).
Group E: No visual potential or presence of any
one or more of the following:
• Tumor in the anterior segment
• Tumor in or on the ciliary body
• Neovascular glaucoma
• Opaque media from hemorrhage
• Tumor necrosis with aseptic orbital cellulitis
• Phthisis bulbi.

Treatment of Retinoblastoma
Treatment of retinoblastoma is considered mainly
under three headings, i.e. treatment of:
1. Small tumors.
2. Large tumors.
3. Extraocular extension.

Treatment of Small Tumors

Earlier the only method of treatment was enucleation
and histopathological examination to confirm the
diagnosis in unilateral cases. In bilateral cases, the
worse eye is enu- cleated to confirm the diagnosis
 Chapter 22 Intraocular Tumors 324
chance for vision by treatment with Targeted Therapy
photocoagulation, chemotherapy and
radiotherapy: Gene therapy for treatment of retinoblastoma is
currently undergoing clinical trials.
1. Tumors not more than 3 mm diameter and 2
mm thickness may be treated with:
a. Photocoagulation using argon or diode laser.
b. Usually TTT as an adjunct, following
chemore- duction.
c. Cryotherapy for pre-equatorial tumors.
d. Chemotherapy for small macular tumors to
con- serve as much vision as possible.
e. Treatment of medium sized tumors.
2. Tumors up to 12 mm wide and 6 mm thick
may be treated with:
a. Brachytherapy using iodine 125 or
ruthenium 106 for anterior tumors.
b. Primary chemotherapy with intravenous
carbo- platin, etoposide and vincristine
(CEV) for 3–6 cy- cles followed by local
treatment with cryo or TTT.
c. External beam radiotherapy generally
avoided due to risk of secondary
malignancies and also due to radiation-
induced orbital growth retarda- tion in
young children.

Treatment of Large Tumors

Chemotherapy to shrink the tumor
(chemoreduction) and make it amenable to local
measures. This is especially use- ful in cases with
bilateral tumor or pinealoblastoma.
Enucleation is indicated if tumor involves more
than 50% of the globe, orbital or optic nerve
invasion or ante- rior segment involvement with
or without neovascular glaucoma. It should be
performed with minimal manipu- lation and a long
piece of optic nerve (12–15 mm) should be
obtained. The cut end of the optic nerve is
histopatho- logically examined to make sure
that it is free of any inva- sion by the tumor.

Treatment of Extraocular Extension

1. Adjuvant chemotherapy is given after enucleation.
2. External beam radiotherapy is indicated in
optic nerve invasion or extension through
the sclera.
3. Metastatic disease is treated with high dose
chemo- therapy with hematopoietic stem
cell rescue, but prognosis is poor.
 Chapter 22 Intraocular Tumors 325

Follow-up malfor- mation of the retina is a sporadic congenital

malformation involving direct communication between
After radiotherapy or chemotherapy, tumor regresses arteries and veins. Brain involvement with similar
to a cottage cheese calcified mass or a fish flesh ipsilateral lesions can occur in Wyburn-Mason
like mass or an atrophic scar. Children who are syndrome.
treated conservatively are kept on regular follow-up
till 10 years of age. Children with unilateral disease
and who had enucleation of the involved eye
should undergo regular periodic detailed
evaluation of the second eye till 10 years of
age.
Retinoblastoma can also undergo complete and
spon- taneous necrosis and regression, and phthisis
bulbi can ensue.

Prognosis
The prognosis of retinoblastoma, if untreated is
always bad. The prognosis is fair if extraocular
extension is avoid- ed. The prognosis for 5 year
disease free survival in intra- ocular retinoblastoma is
more than 90%. However, in ex- traocular extension,
the 5 year disease free survival is less than 10%.

RETINAL ASTROCYTOMA
Astrocytoma of the retina and optic nerve head is
a rare hamartoma, which does not usually threaten
vision. They are most frequently seen in tuberous
sclerosis and occa- sionally in neurofibromatosis
1.
Multiple and bilateral fundus astrocytomas occur
in about 50% of patients of tuberous sclerosis. The
tumor appears as large elevated mulberry like
lesions, which may become calcified. They usually
show fundus auto- fluorescence.

RETINAL HEMANGIOMA
Retinal capillary hemangioma is a rare vascular
tumor of the retina usually seen in association with
von Hippel- Lindau disease. It appears as a round
orange-red mass in the retinal periphery or as an ill-
defined juxtapapillary le- sion. The tumor can be sight
threatening due to macular edema, exudates or
retinal detachment.
Retinal cavernous hemangioma is a rare,
unilateral congenital hamartoma that can occur in
combination with lesions of the skin and central
nervous system (CNS). They appear as sessile
clusters of saccular aneurysms re- sembling a
bunch of grapes in the peripheral retina.
Racemose hemangioma or arteriovenous (AV)
 Chapter 22 Intraocular Tumors 326
CNS disease. Biologic agents like rituximab is
recently tried alternative.

FIGURE 22.19: Anterior chamber invasion in advanced

primary intraocular lymphoma (PIOL)

The PIOL is a subset of primary central PRIMARY INTRAOCULAR

nervous sys- tem lymphoma (PCNSL), which is LYMPHOMA
a variant of extrano- dal non-Hodgkin
lymphoma. The tumor arises from the brain,
spinal cord and leptomeninges and has a very
poor prognosis. About 20% of patients with PCNSL
have ocular manifestations, which can precede or
follow neurological involvement. The anterior
chamber invasion in advanced PIOL is shown in
Figure 22.19.

Ocular Features
Primary intraocular lymphome usually presents
in the sixth to seventh decade with unilateral or
bilateral floaters and blurring of vision. Vitritis is
usually present with some mild anterior uveitis
and presenting as a masquerade syndrome.
Multifocal subretinal infiltrates, which can oc-
casionally coalesce to form ring infiltrates, retinal
vascu- litis, exudative retinal detachment and
optic atrophy are the other ocular findings. The
absence of cystoid macular edema differentiates
this from true uveitic conditions.
Neurological features include headache,
personality changes, focal deficit, seizures, cranial
nerve palsies, etc.

Treatment
Radiotherapy is the first line treatment for PIOL,
but re- currence is common. Intravitreal
methotrexate is useful in recurrent disease.
Systemic chemotherapy can prolong survival in
 Chapter 22 Intraocular Tumors 327

Ocular
Trauma
2
3
Girija Devi PS, Jasmin LB

Ocular trauma can be physical or chemical and construction workers.

they are medical emergencies. Injury is often a
under-recognized health problem and unless
promptly and correctly man- aged, it can cause
permanent impairment of vision.
Even though the eye is protected by the bony
orbit and the lids in front, no part of the eye can
escape the effects of trauma. The majority of eye
injuries occur in people below 30 years of age and
statistics show that eye injury is a lead- ing cause of
monocular blindness in United States and one third
of these are job-related accidents. The 90% of eye
injuries are preventable. Proper safety precautions at
the workplace, inside the house, as well as careful
supervision of children’s activities can prevent
injuries to a consider- able extent. Considerable
advances have been achieved in the investigations
and successful management of ocular trauma
decreasing the visual impairment.

CHEMICAL INJURIES
Chemical injuries of the eye are real ocular
emergency, which produce extensive damage to
the ocular surface and lead to visual impairment.
Most chemical injuries are due to alkali or acid
compounds.

Alkalis
Alkali injuries occur more commonly than acid injury
as they are frequently used in fertilizers, household
cleaning agents, fire crackers and construction work.
The most se- vere alkali injuries are usually due to
ammonia. It has the potential to cause the most
severe eye damage because of its characteristic of
both lipid and water solubility. It penetrates the eye
very quickly and can reach the anterior chamber in 1
minute. The most common alkali injury is due to
cement falling in the eye, which usually happens in
Pathogenesis
Alkali injury cause ocular damage by
saponification and disruption of fatty acids in cell
membranes leading to cell death. The lipid
saponification associated with alkali inju- ries Chapter 22 Intraocular Tumors 328
allows rapid penetration of alkali substance into
tis- sue, in contrast to most acidic compounds. A
pH of 11.5 or higher is associated with severe
ocular damage.

Acids
Acidic compounds are found in household
chemicals such as cleaners, rust removers and in
car batteries. Sulfuric acid is the most common
cause of acidic chemical injury to the eye. Lead
batteries contain up to 25% sulfuric acid. During
recharging of a battery, hydrogen and oxygen are
produced by electrolysis and form an explosive
mixture. Acid used in preparation of rubber sheets
is a common cause for injury in places where
rubber plantations are situated.

Pathogenesis
Acid injuries produce precipitation and coagulation
ne- crosis of corneal epithelium, which forms a
protective barrier to further penetration. This
barrier may protect against weaker acids, but
strong acids may continue to penetrate deeply.
The severity of ocular injury depends on:
1. Area of surface contact.
2. Depth of penetration.
3. Degree of limbal stem cell injury.

Classification
Classification of chemical injuries was first
proposed by Hughes and then modified by
Roper-Hall. This helps to guide prognosis and
treatment.
 Chapter Ocular Trauma 318
23

Grade I: Involves little or no loss of limbal stem

cells and presents with little or no
evidence of ischemia (Fig. 23.1).
Grade II: Involves subtotal loss of limbal stem cells
and presents with ischemia of less than
one-half of the limbus.
Grade III: Involves total loss of limbal stem cells
with preservation of proximal conjunctival FIGURE 23.2: Grade IV chemical injury in left eye with 360°
epithe- lium and presents with ischemia of limbal ischemia hazy cornea and total cataract
one-half of the entire limbus (Fig. 23.2).
Grade IV: Involves total limbus stem cell loss as
well as loss of proximal conjunctival
epithelium and extensive damage to
entire anterior segment (Fig. 23.3).

Clinical Course
Immediate Phase
Clinically present as—conjunctival congestion, areas
of necrosis that appear white, subconjunctival
hemorrhages, large epithelial defects involving
cornea, cloudy edema- tous and opaque cornea,
elevated intraocular pressure (IOP), fibrin reaction
in anterior chamber, cataract, hy- potony due to FIGURE 23.3: Extensive epithelial loss, corneal haze and
ciliary body injury, etc. Descemet’s membrane (DM) folds

Acute Phase (0–7 Days) Late Repair Phase (> 21 Days)

Mild injuries tend to heal during this period. Severe Conjunctival scarring, trichiasis, cicatricial entropion
inju- ries show no re-epithelization. and corneal scarring can progress. Tear deficiency
develop due to loss of goblet cells or decreased
Early Phase (7–21 Days) aqueous produc- tion. Corneal sensation may be
Epithelial migration and regeneration occur in grade I decreased.
and II injuries. Fibrovascular pannus, symblepharon
and stro- mal ulceration can develop between 7 Management
Immediate Treatment
1. Irrigation with normal saline/water/balance salt
so- lution for at least 30 minutes or till pH turns
neutral.
2. Eyelid immobilization with eyelid speculum or retractor.
3. Instillation of topical anesthesia.
4. Removal of particulate matter/debris after
double eversion of lids.
5. Evaluation of the extent and depth of burn is
done under slit lamp. Fluorescein staining is
done. Limbal stem cell ischemia is looked for
and graded by the newer classification.
and 10 days.
Aim of treatment is to restore the cornea with normal
FIGURE 23.1: Grade I chemical injury with epithelial defect
and no limbal ischemia epi- thelium and a clear stroma by decreasing the
 Chapter Ocular Trauma 319
23
inflamma- tion, and enhancing the healing.
 Chapter Ocular Trauma 320
23

Treatment after Irrigation Grade I/II: Taper medical treatment.

Grade III/IV: Impending or actual perforation:
Treatment after irrigation is as follows:
1. Tissue adhesives for less than 1 mm perforations.
1. Topical steroid 2 hourly inhibits
polymorphonuclear proliferation and function. 2. Tectonic keratoplasty.
2. Topical sodium citrate 10% 2 hourly inhibits
polymor- phonuclear degranulation by calcium
chelation.
3. Tetracycline 1% ointment qid inhibits collagenase
en- zyme by chelating with zinc (Zn).
4. Oral sodium ascorbate 500 mg qid promotes
collagen synthesis.
5. Topical sodium ascorbate 20% 2 hourly promotes
col- lagen synthesis.
6. Tear substitutes 2 hourly promote epithelial
healing.
7. Cycloplegics tds or bd relieves pain.
8. Topical/Oral antiglaucoma therapy, if
needed.
9. Conjunctival/Tenons advancement for grade IV
im- proves vascularization.

Healing Patterns of Chemical Burns

After 1st week
After 1st week, reassess the patient. The severity of
injury will show the following healing patterns:
Grade I: Healed cornea with normal
epithelium. Grade II: Epithelial defect,
smaller in size.
Grade III: No epithelization, inflammation.
Grade IV: Sterile corneal ulcer + conjunctival
defect in- flammation.
Management at this stage
1. Taper steroids in the next week.
2. Remaining same treatment is continued for 3
weeks.
3. Amniotic membrane transplantation in 2nd to
3rd weeks in grade III–IV to control
inflammation and lessen symblepharon and
vascularization of cornea.
After 3 weeks to several months
Grade I/II: Healed cornea/healed with pannus.
Grade III: No healing; finally heal as a scarred and
vascu- larized cornea.
Grade IV: Sterile corneal ulcer or vascularized cornea
af- ter conjunctival advancement.
Management option
 Chapter Ocular Trauma 321
23
Vascularized Cornea The ocular trauma classification group has
developed a classification system based on BETT
1. Limbal stem cell transplantation followed by:
and features of globe injury at initial examination.
a. Penetrating keratoplasty (PK).
Mechanical trauma to the eye is subdivided into
b. Lamellar keratoplasty (LK) after 6 months.
open and closed globe in- juries because these
2. Large PK or large LK. have different pathophysiological
3. Keratoprosthesis in bilateral cases.
For symblepharon/cicatrization of conjunctiva,
amni- otic membrane transplant/mucous membrane
transplant can be done.

Ocular trauma is the cause of blindness or MECHANICAL INJURIES

partial loss of vision in more than half a million
people worldwide. The National Eye Institute
Trauma System Registry (NETSR) report on
penetrating eye injuries noted that 83% of pa-
tients were men and the average age was 27
years. Poste- rior segment trauma is the most
common cause of severe visual loss after eye
trauma.
During the last several decades, the
prognosis for pa- tients with ocular injuries,
especially those with open globe injuries has
significantly improved. This has been attribut- ed to
the advent of enhanced microsurgical technique
and instrumentation, along with an improved
understanding of the pathophysiologic
mechanism of ocular trauma.

Ocular Trauma Classification System

The ocular trauma classification group was
organized to establish a system to classify
mechanical injuries of the eye. This system
provides unambiguous definition for each term
and has become the common international
language of ocular trauma terminology improving
the ac- curacy in both clinical practice and
research.

Birmingham Eye Trauma Terminology

Birmingham Eye Trauma Terminology (BETT)
satisfies all criteria for unambiguous standard
terminology by:
1. Providing a clear definition for injury types (Table
23.1).
2. Placing each injury type within the
framework of a comprehensive system
(Fig. 23.4).
 Chapter Ocular Trauma 322
23

and therapeutic ramifications. The system

TABLE 23.1: Birmingham eye trauma terminology
categorizes trauma by four parameters (Table
Term Definition and explanation
23.2).
Eye wall Sclera and cornea
Types of Injury Although technically the eye wall
has three coats, posterior to the
The following are the types of injury as given by limbus, for clinical and practical
the above criteria. purposes, violation of only the
most external structure is taken
Visual acuity into consideration
As defined by the visual acuity measurement at the Closed globe injury No full-thickness wound of eye wall
initial examination. Testing may be done with a
Open globe injury Full-thickness wound of the eye
Snellen acuity chart for distance and near. Visual
acuity at initial exami- nation has been demonstrated wall Contusion There is no (full-thickness) wound
to be the most reliable pre- diction of functional The injury is due to either direct
energy delivery by the object (e.g.
visual outcome in open globe injury. choroidal rupture) or the changes
Relative afferent pupillary defect in the shape of the globe (e.g.
The presence of relative afferent pupillary defect angle recession)
(RAPD) is measured by swinging flash light test, Lamellar laceration Partial-thickness wound of the eye
and it grossly mea- sures optic nerve and retinal wall Rupture Full-thickness wound of the eye wall,
functions. If the affected eye is non-reactive for caused by a blunt object
mechanical or pharmacologic reasons, ob- serving the Because the eye is filled with
consensual response in the follow eye is advised. incompressible liquid, the impact
results in momentary increase in
Extent (zone) of the injury intraocular pressure (IOP)
Denotes wound location in open globe injuries or the The eye wall yields at its weakest
pos- terior extent of damage in closed globe point (at the impact site) or
injuries. elsewhere (e.g. an old cataract
wound dehisces even though the
impact occurred elsewhere); the
Investigations actual wound is produced by an
inside-out mechanism
The imaging methods used are plain X-ray orbit,
ultraso- nography, computed tomography (CT), Laceration Full-thickness wound of the eye
wall, caused by a sharp object
magnetic reso- nance imaging (MRI) and
The wound occurs at the impact site
electrophysiological tests. Plain X-ray and by an outside-in mechanism
ultrasonography give sufficient information in most
Penetrating injury Entrance wound
of the cases. If more than one wound is present,
each must have been caused by a
different agent
Retained foreign Technically a penetrating injury, but
object(s) grouped separately because of different
clinical implications
Perforating injury Entrance and exit wounds
Both wounds caused by the same
agent

Electrophysiological Test
Both, electroretinogram (ERG) and visual evoked
FIGURE 23.4: Mechanical injuries (IOFB, intraocular poten- tial (VEP), are used as valuable prognostic
foreign bodies) indicators, es- pecially in opaque media. A non-
recordable ERG means
 Chapter Ocular Trauma 323
23

TABLE 23.2: Classification of

vitreous hemorrhage, retinal detachment and
injuries
choroi- dal detachment.
Open globe injury Closed globe
2. In open globe injuries to detect occult scleral
injury
perforation, lens dislocation ruptured lens capsule,
1. Type vitreous hemor- rhage, retinal detachment,
Rupture choroidal detachment, vitre- ous leak and
Penetrating Contusion intraocular foreign bodies (IOFB).
IOFB* Lamellar laceration
Perforating Superficial foreign body Computed Tomography
Mixed Mixed Generally 1.5–2 mm thick axial cuts and 2–4 mm
2. Grade (visual acuity) thick cor- onal sections are used. To detect IOFB,
smaller cuts can be specifically asked for.
> 20/40 > 20/40
20/50–20/100 20/50–20/100 Advantages: They are as follows:
19/100–5/200 19/100–5/200 • Superior to ultrasound imaging to detect size and site

4/200 to light of IOFB

4/200 to light perception
perception • No contact with eye
NLP† • Ideal for orbital fractures.
NLP
3. Pupil Disadvantages: They are as follows:
Positive, relative APD ‡ • Contraindicated in pregnant patients
Positive, relative APD in injured
in injured eye • Intraocular structures are not well-imaged like
eye
Negative, relative APD ultra- sound scan
in injured eye • Cannot be performed in operating room or at the bed side.
Negative, relative APD in injured
4. Zone eye Computed tomography findings suggestive of open globe in-
Cornea and limbus
jury (Fig. 23.5): They are as follows:
• Eye wall deformity
External (limited to bulbar, • Intraocular gas
Limbus to 5 mm conjunctiva, sclera, cornea)
posterior into sclera • IOFB.
Anterior segment (includes
structures of the anterior segment
Posterior to 5 mm from and the pars plicata)
Magnetic Resonance Imaging
the limbus Magnetic resonance imaging investigation technique
Posterior segment (all internal
structures posterior to the pro- vides superior tissue definition and resolution.
posterior lens capsule) However, it cannot be used in the suspicion of a
ferromagnetic IOFB,
*IOFB, intraocular foreign bodies; †NLP, no light perception; ‡APD, afferent ultrasound ex- amination of traumatized eye
pupillary defect.
include:
a poor visual outcome. VEP is found to be the 1. To assess the posterior segment of eye in closed
single best predictor of visual outcome. ERG also has globe injuries, e.g. dislocated lens, rupture of lens
a role in the as- sessment of metallosis bulbi. capsule,

X-ray
X- ray has limited use in the present scenario. It can
show fractures and radiopaque foreign bodies
(RFBs).

Ultrasonography
The most valuable tool for imaging a traumatized
eye is ultrasonography. The main indications for
 Chapter Ocular Trauma 324
23
which could be dislodged and produce further
damage to the eye. With its superior resolution,
it helps in evalua- tion of occult scleral rupture,
large hemorrhagic choroidal detachments and
dense vitreous hemorrhage. It can be used to
detect radiolucent substances like wooden, plastic

FIGURE 23.5: Computed tomography showing fracture

of floor of orbit
 Chapter Ocular Trauma 325
23

and glass intraocular foreign (IOF) body. It can be EFFECTS OF BLUNT

used in pregnant patients and contraindicated in
patients with pacemaker and cochlear implant.
TRAUMA ON THE EYE

Mechanism of Blunt Injury Lesions of Conjunctiva

Anteroposterior compression of the globe at the • Subconjunctival hemorrhage
cornea result in equatorial expansion and shortening • Foreign bodies
of the globe along the visual axis. The
anteroposterior diameter of the globe decreases by,
as much as, 41% leading to corneal contact with
the lens and iris. As the anteroposterior di- ameter
decreases, the equatorial diameter increases up to
128% of normal. The extreme stretching of the ocular
tis- sue results in specific type of injury to the
eye.
Application of force to the globe is said to cause
coup injury at the site of force application and
contrecoup in- jury to areas of the globe opposite
to the site of force of ap- plication. Force waves
transmitted through the globe may be the
mechanism of contrecoup injury.
There are seven rings of tissues anterior to
equator, which are to expand, because fluid in the
eye cannot be compressed. The lens-iris diaphragm
is forced posteriorly, due to the attachment of the
structures to the scleral wall, which is moving in the
perpendicular direction; a shearing strain is created,
which may split the tissues at their roots.
The seven ring structures are:
1. Sphincter pupille: Tears can occur.
2. Anterior ciliary body, producing angle
recession or ciliary body tear, typically the
separation occurs be- tween circular and
longitudinal fibers. The longitu- dinal fibers
remain attached to the sclera and form the
new anterior boundary of the recessed chamber,
which corresponds to the broad ciliary body
band seen gonioscopically.
3. Attachment of the ciliary body muscle to the
scleral spur resulting in cyclodialysis cleft.
Aqueous is al- lowed to pass directly from
anterior chamber to su- prachoroidal.
Hypotony is common.
4. Trabecular meshwork: A tear in the anterior
portion of the meshwork resulting in a
trabecular flap.
5. Attachment of zonules, resulting in subluxation
and dislocation of the lens.
6. Attachment of retina to the ora serrata
resulting in retinal dialysis and retinal
detachment.
 Chapter Ocular Trauma 326
23
• Laceration (Fig. 23.6)
• Emphysema.

Subconjunctival Hemorrhage
Subconjunctival hemorrhage appears as a
bright red patch of conjunctival tissue with distinct
or feathered bor- ders, which resolves
spontaneously in 7–10 days.
Subconjunctival hemorrhage may be due to the
track- ing forward of the blood from the orbit
following fracture of the orbital walls or the base
of the skull. It should be differentiated from local
bleeding. When a fracture is the cause, the
densest and most extensive part of the hemor-
rhage is posterior, where no edge is seen. FIGURE 23.6: Rupture globe with hyphema and uveal
Anteriorly it may not reach the limbus. In case of tissue prolapse (white arrow)
local bleeding, the densest area is usually on the
anterior part and it starts disappear- ing towards
the equator. In bleeding from a fracture, the
color of the hemorrhage is purplish and
subconjunctival in site, whereas local bleeding is
bright red and intracon- junctival moving with
this membrane (Fig. 23.7).
The presence of subconjunctival pigmentation
in as- sociation with a hemorrhage is very
suspicious of occult scleral rupture (Fig. 23.8).

Foreign Bodies
Conjunctival foreign bodies are common. Most
conjuncti- val foreign bodies are easily removed
with a cotton-tipped applicator or 26 gauge
needle.

Laceration
Laceration may be isolated injuries or indicates a
deeper trauma. In case of conjunctival laceration a
thorough exam- ination is done to rule out an
open globe injury. The scleral
 Chapter Ocular Trauma 327
23

FIGURE 23.7: Subconjunctival hemorrhage corneal abrasion stains with fluorescein dye and its
borders are generally sharp.
The scleral defect may be from a significant distance
from the site of the conjunctival injury. So examine
the eye in a variety of gazes and if necessary
conjunctival exploration should be performed
under local anesthesia.
Larger lacerations may require closure using
absorb- able suture materials.

Emphysema
Emphysema occurs when air gets trapped under or
in the conjunctiva. In orbital fracture, air from
paranasal sinuses enter into the orbit and dissects
anteriorly under and into the conjunctiva. Patients
with orbital fractures should be advised against
nose blowing or sneezing with a closed mouth so
as to avoid orbital and subconjuncti- val
emphysema.

Lesions of the Cornea

• Corneal abrasions
• Recurrent erosion
• Corneal foreign bodies
• Corneal lacerations.

Corneal Abrasions
Superficial corneal abrasions are frequently seen with
mi- nor trauma.
Clinical features: Due to the dense innervation of
sensory nerves of the corneal epithelial surface,
corneal abrasion causes intense pain, photophobia
and lacrimation. Ir- regular corneal light reflex may
indicate presence of an abrasion. On examination, a
 Chapter Ocular Trauma 328
FIGURE 23.8: Subconjunctival pigmentation suggesting
23
scleral rupture
Routine treatment of corneal abrasion
includes the use of a broad-spectrum antibiotic
drop for infection, pro- phylaxis and cycloplegic
agent. A pad and bandage is gen- erally applied
to shield the epithelium.

Recurrent Erosion
Corneal abrasions caused by any shearing
injury (e.g. finger nail, paper cut or vegetable
matter) may dam- age epithelium—basement
membrane adhesion com- plexes and
consequently lead to recurrent or persistent
epithelial defects. Predisposing conditions such
as epi- thelial or stromal dystrophies may
contribute to recur- rent erosions.
Clinical features: Patients typically presents with
acute onset of pain, redness and tearing on
awakening. On ex- amination, an irregular area of
epithelium causing a focal breakup of the tear film
to a full thickness epithelial defect with elevated
gray margins is seen.
Treatment: Aim of the treatment is to maintain
epithelial stability and integrity until the adhesion
complex can form and hemidesmosomal
anchoring fibers extend into the basement
membrane to secure the epithelium firmly in
place. The treatment for recurrent erosion are as
follows:
1. A topical hyperosmotic agent, e.g. 5% sodium
chloride ointment applied before sleep.
During sleep, tear film becomes hypotonic
due to lack of evaporation, which make the
corneal epithelium edematous and easily
damaged with the first blink and hyperosmotic
agents help to reduce the corneal epithelial
edema and this should be used for at least 8
weeks, which is the minimum period
required for the formation of adhesion
complex.
 Chapter Ocular Trauma 329
23

2. If there is no response to topical hyperosmotics, • Iridodialysis

next step is extended wear bandage contact lens • Irideremia
for a min- imum of 6–8 weeks.
3. Debridement using a cotton-tipped applicator or
us- ing a blade will help regrowing of normal
epithelium.
4. Stromal micropuncture with a straight 20 gauge
nee- dle is used to create a partial thickness
penetration of the anterior corneal stroma.
5. Excimer laser ablating the basement membrane
and the superficial bowman’s layer.

Corneal Foreign Bodies

The symptoms caused by corneal foreign bodies
are fre- quently out of proportion to the severity of
the injury. Indi- viduals with foreign bodies remaining
on the corneal sur- face can be highly symptomatic;
conversely, high speed objects that are embedded
in or pass completely through the cornea may give
rise to no or only minimal symptoms.
Ferrous foreign bodies: They oxidize and form rust
deposits in cornea as easily as 3 hours after injury.
Because rust can retard wound healing, its removal
by scraping is recom- mended.
Caterpillar hairs: It may cause intense irritation and can
mi- grate into the deep stroma.

Treatment
Removal with a cotton tipped applicator or a 26
gauge needle in the case of deeper foreign bodies,
application of antibiotic drops or ointment till the
wound heals and patching of the eye to promote
healing as well as to relieve pain and irritation.

Corneal Lacerations
Bandage lenses are useful for non-displaced
laceration less than 3 mm in length, particularly, if
they are self seal- ing. Large lacerating wounds,
displaced wounds, wounds with loss of corneal tissue
and lacerations with accompa- nying iris or lens
incarceration must be sutured.

Lesion of Iris and Ciliary Body

• Changes in pupil and accommodation
• Vascular changes
• Interstitial tears of the sphincter
• Tears at the pupillary border
• Partial or complete dehiscence at pigmentary
layer
 Chapter Ocular Trauma 330
23
• Iridoschisis epithelium is torn.
• Traumatic cyclodialysis If the tear involves the posterior pigmentary
• Pigmentary changes. epithe- lium and sphincter muscle, hyphema is
common from the capillaries of the muscle. A
Changes in Pupil and Accommodation complete tear, involving all the layers of the iris is
the most common.
Traumatic miosis and spasm of accommodation: A
spastic miosis is a immediate sequel to trauma.
The constriction of the pupil is intense and is
usually transient, to be fol- lowed frequently by
an iridoplegia.
Traumatic mydriasis and paralysis of accommodation:
Dila- tion of the pupil is a very common sequel
of a concussion of the globe and it is usually
associated with a paralysis of accommodation
coming after the intense miosis has passed. The
pupil is moderately dilated, often eccentric, usually
with diminished reaction to light and accommo-
dation and this deformity is permanent.

Vascular Changes
Reactive hyperemia and exudation—concussion
effect on uveal vessels comprise initially an
ischemic spasm fol- lowed by a prolonged
reactive vasodilatation. Clinically evident by
circumcorneal injection and the vascular dila-
tation is associated with edema of the tissue. Slit
lamp ex- amination reveals the presence of
increased protein in the anterior chamber.
If the reaction is severe, it leads to formation
of micro- scopically visible masses of fibrin in the
anterior chamber.

Interstitial Tears of the Sphincter

Tears of the sphincter muscle without external
involvement of the tissues of the iris are usually
small and may be multi- ple. They are difficult to
see clinically, but are made obvious by
transillumination in which case, the torn area
appears as a reddish glow. There may also be
changes in the contour of the pupil involving a
minimal amount of notching.

Tears at the Pupillary Border

The tears at the pupillary border is common, the
lacera- tion may involve the anterior layers of
the stroma of the iris and leave the sphincter
intact, alternatively, the poste- rior pigmented
layer and the sphincter may be implicated, while
the anterior stroma remain intact. But much more
commonly, the whole depth of the tissue anterior
stroma, sphincter muscle and pigmentary
 Chapter Ocular Trauma 331
23

Dehiscence of the Pigmentary Layer of the Iris FIGURE 23.9: Iridodialysis (black arrow) with D-shaped pupil
(white arrow)
Common sequel of concussion injuries, which are
not visible ophthalmoscopically or by direct
observation, but only by retroillumination they appear
as bright red areas. The dehiscence is either single or
multiple, round, oval or irregular and occurs
preferentially near the root of the iris where this
tissue is thinnest.

Iridodialysis
When the trauma is of considerable severity as
from the direct blow by a stone, a ball or other
flying object or from an explosion, the iris may be
torn away from its insertion into the ciliary body to
a greater or lesser extent (Fig. 23.9).
Clinical features: As follows.
A black linear crescentic slit at the periphery of
the iris, through which the zonule and even the
periphery of the lens and ciliary process may be
visible and through which the vitreous may
occasionally herniate.
D-shaped pupil: The papillary margin will be
straight- ened at the site of the iridodialysis resulting
in a D-shaped pupil [refer Fig. 23.9 (white
arrow)].
This type of injury is accompanied by a
hyphema of considerable size due to rupture of the
large vessels sup- plying the iris. The deformity is
permanent and the ciliary region atrophies. If the
iridodialysis is large, uniocular dip- lopia will be a
symptom. If visual confusion and diplopia distress
the patient, operative measures may be indicated. If
the iridodialysis is very extensive, the detached por-
tion sometimes becomes completely rotated, so that
the pigmented back of the iris faces forward called
anteflexion
of the iris.
 Chapter Ocular Trauma 332
23
Irideremia endophthalmitis.
The root of the iris is completely torn from its attachment,
i.e. if the dialysis is complete, the condition of
irideremia or traumatic aniridia results. It is
completely detached and lies curled up into a
little ball at the bottom of the anterior chamber
where it eventually shrinks into a gray body.

Iridoschisis
A detachment of the anterior leaf of the
mesodermal stro- ma of the iris from the deeper
layers is a rare result of se- vere trauma.
The detached portion may float forward in the
anterior chamber and may even simulate an
anterior synechia.

Pigmentary Changes
Shortly after the injury, a powdering of uveal
pigment on the surface of the iris, on the
posterior surface of the cor- nea and on the
anterior capsule of the lens is the rule. Fol- lowing
an atrophy of iris, the pigmentary layer may show
an extensive ectropion, spreading widely over the
anterior surface of the structure. Pigmentary
change may occur as a long-term result of
concussion injuries, partly due to mi- gration of
pigment and possibly new formation of pigment as
a result of trauma or following iritis, i.e. the injured
iris becomes darker than its follow—known as
inverse hetero- chromia (hyperchromic), which is
particularly evident in light-colored eyes.

Dysfunction of the ciliary body may result in CILIARY BODY

loss of the normal IOP. With the loss of IOP,
phthisis invariably en- sures, leading to a poor
functional and cosmetic result.

Causes of Hypotony
Wound Leak
Wound leak is a common cause of hypotony
after open globe injury. An anterior wound
fistula may be detected using the Seidel test.

Posterior Scleral Rupture

B-scan ultrasonography is sometimes useful in
identifying discontinuities in the posterior sclera.
Leaking wounds must be surgically repaired
because they have very low rate of spontaneous
closure and have a significant risk for
 Chapter Ocular Trauma 333
23

Cyclodialysis Secondary hemorrhages tend to be more profuse

Cyclodialysis is separation of ciliary body from the than the primary hemorrhages.
sclera spur. Cyclodialysis creates free communication Traumatic hyphema rapidly and permanently
between the AC and the suprachoroidal space. absorb in 1–7 days, leaving no trace, absorption is
Presence of a cyclodialysis cleft should be mainly through the anterior surface of the iris.
suspected in any hypotonous eye that had recent
surgery or trauma. Complications
Diagnosis: The cleft between the ciliary body and the However, an iridocyclitis may develop with the
sclera spur can be identified by gonioscopy. formation of anterior and posterior synechiae.
Secondary glaucoma is a complication, if the
Ciliochoroidal Detachment hemorrhage is massive.
Ciliochoroidal detachment is commonly seen in When the hyphema is associated with rise in
presence of hypotony. tension, development of blood staining of cornea is
a permanent disability.
A long-term complication such as development of
TRAUMATIC HYPHEMA he- matogenous pigmentation of the iris causes a
heterochro- mia iridum wherein the iris of the
An accumulation of free blood in the anterior injured eye becomes darker.
chamber is a common result of contusion (Figs
23.10A and B).
Treatment

Primary Hyphema A small hyphema gets absorbed by itself. Patient

should be hospitalized and advised bed rest. Using
Primary hyphema appears at the time of accident mydriatics have is controversy. Since, absorption of
and it settles gravitationally, and varies in height blood is partly through the anterior surface of the
from 1 to 2 mm to filling completely the anterior iris, the greater the available surface, the more
chamber. Such hemor- rhages usually absorb rapidly
rapid is the process and mydriatics may retard this
and are particularly seen in case of children.
action. But, hyphema is associated with iritis and
this warrants the use of mydriatics:
Secondary Hemorrhage 1. Patient should be hospitalized, advised bed rest
The picture may be complicated by the occurrence and head end of the bed should bed
of a fresh bleeding usually on the 2nd or 3rd, or elevated.
sometimes on the 4th or 5th post-traumatic day. The 2. Place a shield over the involved eye, do not
secondary type is seen most frequently in older patch be- cause this prevents recognition of
patients. sudden visual loss in the event of rebleed.
 Chapter Ocular Trauma 334
23

FIGURES 23.10A and B: Traumatic hyphema

 Chapter Ocular Trauma 335
23

3. Atropine 1% solution bid or tid to prevent Two types of rosette-shaped opacity can follow
contraction of ciliary body and pupil, and trauma; those occurring very shortly after the injury
subsequent disruption of damaged blood (early rosette) and those appearing after sometime
vessels. (late rosette).
4. Use topical steroids to prevent iritis.
5. For increased IOP, start with a beta blocker, e.g.
timo- lol, avoid prostaglandin analog and miotics
since they may increase inflammation.
If topical therapy fails, add acetazolamide 20
mg/kg/ day in divided dose/day or mannitol 1–2
g/kg intrave- nously over 45 minutes.

Surgical Measures
A controlled AC paracentesis is safe and this
will re- move blood from the AC as well as control
the second- ary glaucoma.
Indications for surgical evacuation of
hyphema:
1. Corneal stromal blood staining.
2. Hyphema that does not decrease to less than
50% by 8 days [to prevent peripheral anterior
synechiae (PAS)].
3. For IOP > 60 mm Hg for > 48 hours despite
maximal medical therapy.
4. For IOP > 25 mm Hg with total hyphema for >
5 days (to prevent stromal blood staining).
5. For IOP of 24 mm Hg for > 24 hours (or any
transient increase in IOP > 30 mm of Hg) in
sickle cell trait/ disease patients.

CONCUSSION EFFECTS
ON THE LENS AND ZONULE

Vossius Ring
Vossius ring is an imprint of the pupillary border of
iris upon the lens capsule corresponding to the
extreme mio- sis, which develops on receipt of the
injury. This annular deposition appears only in the
young because the iris in the young possesses
considerable elasticity to deposit the imprint and it
serves as an indicator to prior blunt trauma.

Traumatic Cataract (Rosette-

shaped Opacity or Stellate
Cataract)
 Chapter Ocular Trauma 336
23
Early Rosette
The early rosette opacity may appear sometimes
in the an- terior, sometimes in the posterior
subcapsular region and sometimes in both region
simultaneously, which appear within few hours,
after the injury. In early rosette, the lines of
opacity feather out from the suture, which
themselves lie in the center of each petal (Fig.
23.11).

Late Rosette
Late rosette opacities are seen few years after the
trauma and are usually found lying deep in the
cortex or in the adult nucleus, separated from the
capsule by a clear zone of varying thickness FIGURE 23.11: Traumatic cataract with capsular injury
(Figs 23.12A to C).
In late concussion rosette; the lines of
opacity feath- er out from the sutures in such a
way that the petals are formed from adjacent
suture, which themselves lie be- tween the
petals.

Subluxation and
Dislocation of the
Lens
In subluxation of lens, phacodonesis may be the
initial clini- cal sign. Once the zonular attachment is
damaged, lens be- come more spherical and
myopic, sometimes astigmatism and impairment of
accommodation is the visual result.
If the subluxation is greater, the lens may
slip from its axial position and its equatorial
edge may appear as a crescent in the pupillary
aperture dividing it into phakic and aphakic parts
and the AC will have unequal depth.
In complete traumatic luxation, the lens may
follow dif- ferent routes into the anterior
chamber, the vitreous, the inter-retinal space,
outside the eye if the globe is ruptured and comes
to rest in the subconjunctival or Tenon’s space.
 Chapter Ocular Trauma 337
23

FIGURES 23.12A to C: Rosette cataract. A. Photograph; B and C. Diagrammatic representation of early and late rosette, respectively
(Courtesy: Eye rounds online Atlas of Ophthalmology).

Dislocation into the Anterior Treatment

Chamber A traumatic cataract is soft and can be aspirated through
the large aspiration port of the phaco tip especially in
The lens lies in front of the iris as a transparent
a
body, sometimes almost filling the anterior
chamber, some- times lying in its lower part with
its rim showing a golden luster so that it resembles
a large globule of oil in the aque- ous with the pupil
spasmodically contracted.

Complications
A sudden onset fulminating glaucoma due to
pupillary block of an intractable iridocystitis and
endothelial dam- age can follow in AC dislocation.
Dislocation into vitreous is twice as common as
anterior dislocation. In this position of lens, eye may
remain quiet, but severe iridocystitis and secondary
glaucoma can occur. At first, the lens is mobile in the
vitreous (lens natans) and may even travel from the
vitreous into the anterior chamber through the
dilated pupil by changing the posi- tion of the
patient from the prone to the supine position
(wondering lens), but eventually organized
membranes
tend to anchor it (lens fixata).

Extraocular Dislocation of the

Lens
Following rupture of globe, lens may lie under the
con- junctiva (lenticule phacocele) and gets absorbed
eventu- ally leaving some by calcareous deposits. But
if the capsule is intact, it may remain practically
unattended almost in- definitely.
 Chapter Ocular Trauma 338
23
young patient. Ophthalmic viscosurgical device
(OVD) tamponade of vitreous can be used for
areas of zonular incompetence.
If vitreous has migrated into the anterior
chamber, the surgeon should perform an anterior
vitrectomy before starting phacoemulsification or
cortical aspiration in order to avoid vitreous
aspiration with resulting retinal traction.
If there is no enough capsular support to allow
phaco- emulsification, a capsular tension ring
(CTR) can be in- serted into the capsular bag.
This device provides ade- quate support for
nuclear and cortical removal, as well as for in-the-
bag intraocular lens (IOL) insertion.
When the nucleus is markedly subluxed or
dislo- cated and vitreous fills a substantial part
of the anterior chamber, the surgeon should
consider removing the lens through a pars plana
approach.
An anterior chamber IOL or transclerally
fixated pos- terior chamber lens may be
necessary in case of inade- quate capsular
support for a posterior chamber lens.
Anterior dislocation of lens is an ophthalmic
emer- gency and lens removal after medical
control of secondary glaucoma is needed to
prevent decompensation of cornea due to
endothelial touch of the lens and permanent
sec- ondary glaucoma due to PAS.

Concussion Changes in the Vitreous

Changes in vitreous body after a concussion injury
are dis- ruption of the framework and liquefaction of
gel (syneresis).
Vitreous hemorrhage following tearing of the
retina with or without detachment.
Detachment of the vitreous and herniation of
vitreous into anterior chamber.
 Chapter Ocular Trauma 339
23
migra- tion of pigments. Sometimes pigments are fine
CONCUSSION and dust, and at other times it is corpuscular or
EFFECTS ON THE arranged in clumps, being arranged without
RETINA reference to blood vessels.
Damage to the retina after concussive injuries to
the globe, affecting particularly the macula and
periphery is common.

Commotio Retinae (Berlin’s

Edema)
Clinical Features
A milky white area of transient cloudiness with an
ill- defined margin extending over the posterior
region of the fundus due to the development of
retinal edema is a common sequel of concussions of
the globe. It occupies a considerable area usually
surrounding the optic disk and particularly always
involving the macula, which shows a bright red spot
in center in brilliant contrast to the gray back
ground as cherry red spot. The cloudiness increases
progressively for 24 hours after the injury,
thereafter it slowly fades away. The central vision
is lowered consid- erably and with the
disappearance of edema, the vision may again
become normal, but unfortunately the lesion may be
followed by the appearance of pigmentary depos- its,
cystoids formation at the macula and even a hole.
The macula is most frequently affected because of
the rich- ness of the underlying capillary network
and because of the anatomical peculiarities of the
structure of the retina in this region.

Traumatic Macular Cyst and Hole

Postcontusion necrosis may result in cystoid
macular edema and the development of cystic space
that coalesce to produce a large cyst. Rupture of
inner layer of a larg- er macular cyst may produce
a macular hole. If both the inner and outer walls of
a macular cyst disappear a full thickness hole
may develop.
Peripheral atrophic retinal changes seen after
ocular concussion as localized retinal atrophic
change as well as widespread change. These
atrophic and pigmentary changes resemble those
seen in myopia or congenital and acquired syphilis.

Diffuse Pigmentary Changes

The posterior region of the fundus is affected with
 Chapter Ocular Trauma 340
23
Vascular Change in Retina rupture may occur di- rectly at the site of
application of the force or indirectly on
Vascular lesions include hemorrhages, vascular
occlu- sions and the formation of traumatic
aneurysms.
Small hemorrhages from the retinal vessels,
usually single, but sometimes multiple are
common after ocular contusions. The most
common are intraretinal hemor- rhage usually
small round extravasation in the outer reti- nal
layers or flame-shaped extravasations in the nerve
fi- ber layer, but preretinal hemorrhages either
subhyaloid or extravasation into the vitreous
are not unusual.

Traumatic Aneurysms
Traumatic aneurysms in the retina are rare sequel
of con- tusion of the globe.

Peripheral Retinal Tears

Peripheral retinal tears are common source of
post- trau- matic retinal detachment. These
tears tend to be large, more irregular and located
at the site of direct ocular con- tusion. They can
subsequently lead to retinal detachment for a
variable period after the trauma.
In most cases, contusion related retinal tears
are lo- cated inferotemporally, probably because
the bony orbit affords less protection at this
location.

Retinal Dialysis
Retinal dialysis is defined as a break or separation
occur- ring at the anterior edge of the ora serrata.
Retinal dialysis is the most frequent traumatic
retinal break. It almost al- ways occurs at the
time of the injury. The most common location is
the inferior temporal quadrant.

Choroidal Hemorrhage CONTUSION EFFECTS

ON THE CHOROID
Hemorrhages in the choroid are frequent sequel
of in- juries. It appears as a rounded dark blotch
with blurred edges over which the retinal vessels
course. It slowly tends to absorb leaving
considerable pigmentation and usually some
degree of atrophy.

Rupture of Choroid
The choroid is prone to rupture from the effects
of blunt trauma applied to the globe and the
 Chapter Ocular Trauma 341
23

the opposite side of the globe. The choroid is shapes and are always
susceptible to rupture because of the inelastic FIGURE 23.13: Choroidal rupture
characteristic of Bruch’s membrane (Fig. 23.13).

Direct Rupture of the Choroid

A large, broad and usually irregular lesion lies near
the periphery exposing the white sclera, bordered
initially by hemorrhage and eventually by heavy
pigmentary changes.

Indirect Rupture of the Choroid

Indirect rupture of the choroid is more common
and initial appearance is usually obscured by
hemorrhages. When multiple tears occur, the central
one is usually the largest and the most peripheral
smallest.
The rupture is usually crescentic in shape and
runs concentrically with the margin of the disk. As
a rule, it is broadest in its middle and narrows toward
each end. Over the rupture, the retina usually
remains structurally intact and the vessels course
over the rent in the choroid without any break in their
continuity. When choroidal rupture is accompanied
by retinal rupture it is called retinitis sclop- etaria or
chorioretinitis sclopetaria, or chorioretinitis plas- tic
sclopetaria.
Eventually the overlying retina becomes atrophic
and degenerated showing neuroglial proliferation,
wandering of pigment and the new formation of
vessels. After some- time it becomes adherent to
and is incorporated in the fi- brous tissue, which
fills the gap in the choroid.

Traumatic Choroiditis
Lesions may be small and discrete, round or oval
and sharply defined or they may be diffused and
widespread, assuming the irregular and map-like
 Chapter Ocular Trauma 342
23
associated with a considerable degree of
pigmentary pro- liferation and subsequent
atrophy.
Ciliochoroidal detachment (Fig. 23.14) is
commonly seen in presence of hypotony.

Papillitis: Causing considerable swelling of the disk CONCUSSION

may be associated with ocular contusion. EFFECTS AT THE
Optic atrophy: It may be a sequel to widespread OPTIC DISK
retinal or choroidal damage.

Rupture and Avulsion of the Optic

Nerve
The most common cause of a rupture and
avulsion of the optic nerve is a penetrating
injury of the orbit.

Traumatic Hypermetropia CONCUSSION

EFFECTS ON
Traumatic hypermetropia causes are paralysis of
accom- modation due to an injury of the ciliary
REFRACTION
nerves or in more severe cases to organic lesions
in the ciliary muscles, rais- ing of retina with
organized material after a rupture of the choroid
or posterior dislocation of the lens with an in-
creased depth of the anterior chamber.

Traumatic Myopia
Traumatic myopia is the commonest refractive
change following a concussion injury. An
increase in myopia of

FIGURE 23.14: B-scan choroidal detachment

 Chapter Ocular Trauma 343
23

1D–6D can occur and starts disappearing within a

week or two and becomes normal within a Delayed-onset Glaucoma
month.
Delayed-onset glaucoma include angle recession, ghost
cell, hemolytic, hemosiderotic, lens subluxation, phaco-
Ciliary Spasm morphic, lens particle and lens induced.
Ciliary spasm can occur in majority of cases due to Angle recession: In the presence of hyphema, angle
irrita- tion of muscle fibers or the III nerve, sometimes recession is found in 71%–100% of eyes. Only 7%–9%
to paresis of cervical sympathetic. patients with angle recession develop glaucoma. Risk of
development is
Damage to the Suspensory
Apparatus
Damage to the suspensory apparatus of the lens
cause an increase in lenticular curvature. If the
zonules are rup- tured and the lens remain in the
place, the myopia may amount from 5D to 6D and
be permanent, while a higher degree may be
caused by an anterior dislocation of lens.

TRAUMATIC GLAUCOMA

Glaucoma Associated with Closed

Globe
Glaucoma associated with closed globe injuries
are:
• Early onset
• Delayed onset.

Early-onset Traumatic Glaucoma

Early-onset traumatic glaucoma is due to
inflammation, trabecular meshwork disruption
and hyphema.
Inflammation: It can induce high IOP due to
outflow ob- struction by the inflammatory debris,
cells or protein in the anterior chamber and
possibly due to inflammation at the trabecular
meshwork. Inflammation is usually self- limiting and
close monitoring along with aqueous sup-
pressants, topical steroids and cycloplegic should be
used.
Trabecular meshwork rupture: The meshwork can be
su- perficial or full thickness. Gonioscopy is the
most useful technique for diagnosis.
Hyphema: It is caused by the tear in the iris root and
bleed- ing from the small branches of the arterial
circle of the iris. High IOP results from RBC
obstruction, inflammation and disruption of the
outflow pathway.
 Chapter Ocular Trauma 344
23
greater, if more than 180°–240° are affected. meshwork and peripheral anterior synechia.
Increased out- flow resistance is caused by
either growth of membrane covering the angle or Flat Anterior Chamber
scarring. Gonioscopic examination shows broad
ciliary body band, torn iris processes and an Aqueous loss may cause flat anterior chamber.
abnormally white scleral spur. Prolonged flat anterior chamber leads to peripheral
anterior synechia
Ghost cell glaucoma: In the presence of vitreous
hemor- rhage, ghost cell glaucoma develops
within 1–3 weeks post injury. It is caused by old
and degenerated RBCs ob- structing the aqueous
outflow pathway because they are more rigid to
pass through.
Hemolytic glaucoma: It occurs after several days to
weeks of intraocular hemorrhage produced by
hemoglobin laden macrophages, free hemoglobin
remnants of lysed RBCs and clogging the
trabecular meshwork.
Hemosiderotic glaucoma: Tissues of eye-like
trabecular meshwork, endothelial cells absorb
iron liberated from the lysed RBCs. If the normal
ferritin-apoferritin system is saturated, toxic
granules of inorganic iron accumulates within the
cells and cause hemosiderosis and obliteration of
intertrabecular spaces.
It is a rare condition associated with prolonged
history of intraocular bleed.
Lens related glaucoma: Injury to zonules result in
sublux- ation or dislocation. In anterior
displacement, lens may cause papillary block and
result in reduced visual acuity, myopia, closed
angle on gonioscopy. Treatment is reliev- ing the
pupillary block by removal of subluxated lens.
In posterior displacement, pupillary block by
vitreous occur leading to secondary glaucoma.
Phacomorphic glaucoma: There will be swelling of
lens due to disruption of lens capsule leading to
rapid hydration of lens and pupillary block or
angle block result. The clini- cal features are
irregular AC, dense cataract and increased lens
thickness measured by a scan compared to the
follow eye. Treatment is cataract extraction.
Lens particle glaucoma: Lens particle
released into AC obstruct trabecular meshwork
and cause glaucoma. It is commonly seen in
open globe injury.

Glaucoma
Associated with
Open Globe Injury
Inflammation Glaucoma
Inflammatory cells may block the trabecular
 Chapter Ocular Trauma 345
23

and angle closure. Prevention is the best option by as shown by circumcorneal injection and miosis.
me- ticulous wound reconstruction, intraoperative FIGURE 23.15: Corneal foreign body penetrating into the
reforma- tion of anterior chamber and postoperative anterior chamber
mydriasis and topical steroids.

Epithelial Down Growth

Epithelial down growth is caused by epithelial or
fibrous down growth due to delayed or improper
wound repair resulting in obliteration of
trabecular meshwork.

Associated with IOFB

Iron foreign body leads to siderotic glaucoma, copper
foreign body leads to tissue damage due to aseptic
inflammation.

EXTRAOCULAR FOREIGN
BODIES
The most common accident in ophthalmology is
retention of foreign body on the surface of eye.
Common foreign bod- ies, which enter eye are pieces
of metal and stone, in indus- trial workers, while
husks, pieces of straw, grain, thorns, leaves, insects or
their wings are common in agricultural surroundings.
Foreign bodies particularly if sharp and metallic
and travelling at high speed, get embedded
themselves more deeply in the tissues of the cornea
or the episclera or sclera, while others with sufficient
momentum penetrate the coats of the eye and
enter the globe (Fig. 23.15).
If the cornea is affected by a superficial foreign
body, symptoms present as sharp burning pain,
reflex gush of tears with momentary blindness and
the lids close in bleph- arospasm. On the other hand,
if particles penetrate deeply and remain impacted or
perforate the globe, less symptoms are produced. In
all these cases there is evidence of uveal irritation
 Chapter Ocular Trauma 346
23
In any case with corneal abrasion in the metallosis and even loss of the eye despite the best
upper half of cornea, the upper lid has to be efforts at treatment. Visual prognosis is best when
everted and examined for the presence of any the IOFB is removed during the initial wound
foreign body. repair surgery or as soon as possible.
In the conjunctiva, sharp gritty particles The majority of IOFBs are small, sharp projectile
adherent to the inner surface of the upper lid, pro- duced in hammering metal or stone, up to
particularly those ly- ing in the subtarsal fold, 90% of IOFBs
continuously abrade the cornea in the movements
of blinking and cause symptoms more severe
than results from those impacted on the corneal
surface.
In the sclera, the impaction of foreign bodies is rare.
They are usually found in the palpebral aperture.

Complication of
Extraocular Foreign
Bodies
Important complications of extraocular foreign
bodies are the introduction of infection at the
time of the injury, the formation of corneal ulcers
leading to corneal opacity and traumatic
implantation cyst.

Treatment of Extraocular
Foreign Bodies
Superficial Foreign Bodies
A superficial foreign body is removed by a moist
cotton- tipped applicator after putting topical
anesthesia.
Embedded foreign bodies: Removed with ordinary
stainless steel hypodermic needle supported
on the syringe.
Deeply embedded foreign bodies: Removal of deeply
em- bedded foreign bodies should be undertaken
in the oper- ating theater as a major surgical
procedure. Severe com- plications such as corneal
perforation and pushing of the particle into the
anterior chamber should be anticipated and
managed immediately.

Intraocular Foreign Bodies

Intraocular foreign bodies (IOFBs) are common
prob- lem in ocular injuries. IOFB most commonly
affect young men, while hammering, shooting a
shot gun, witnessing an explosion or using a
machine tool. The majority occur in the
workplace.
Retained IOFB represents a true emergency
and can lead to severe vision loss caused by
endophthalmitis, reti- nal detachment and
 Chapter Ocular Trauma 347
23

are metallic and 55%–80% of these are magnetic. FIGURE 23.16: Intraocular foreign body in the vitreous
Most frequently they enter the eye through the
cornea (65%). Other common locations include the
sclera (25%) and the limbus (10%). The IOFBs most
frequently lodges in the vitreous cavity (Fig. 23.16)
(61%), but can also be located within the anterior
chamber (Fig. 23.17) (15%), retina (14%), lens
(8%) or subretinal space (5%).

Clinical Features
The presence of an IOFB is to be suspected in any
pen- etrating injury until it is excluded by
investigations like CT, MRI, etc. The probabilities are
high in injuries caused due to breaking stones with a
metallic hammer. An IOFB has to be suspected if
there is a through wound track in the cornea or
iris.

Management
Investigations such as CT, MRI and X-ray of skull has
to be done to confirm the presence as well as to
locate the exact site of the foreign body.
Management of an IOFB injury requires immediate
clo- sure of the globe and removal of IOFB. Delay in
removal of IOFB for more than 24 hours in primary
repair and delay in IOFB removal, produces a 4-fold
increase in the risk of endophthalmitis and severe
vision loss.
Timing of IOFB removal depends on the factor
such as; size of foreign body, amount of intraocular
reaction, material of the IOFB on duration between
injury and reporting time.

Treatment Methods
If a small magnetic IOFB, clear media, easily
accessible anterior location and no associated ocular
damage, then primary magnetic extraction is
done.
 Chapter Ocular Trauma 348
23
disorganization of the normal anatomy due to
trauma may cause difficulty in assessing

FIGURE 23.17: Intraocular foreign body in the anterior

chamber (black arrow)

A pars plana vitrectomy is indicated in case of

opaque media, associated retinal detachment or
vitreous hemor- rhage, endophthalmitis, non-
magnetic foreign body, in- carcerated foreign body
and failed magnetic extraction.

Complication
Complications of IOFB injuries include
endophthalmitis, retinal detachment with
proliferative vitreoretinopathy, siderosis bulbi
and chalcosis.

Siderosis
Siderosis is a tissue reaction caused by a
retained iron foreign body. Electrolytic
dissociation of iron and com- bination with
tissue proteins result in cellular death (Fig.
23.18).
There will be gradual loss of vision due to
development of secondary glaucoma and retinal
atrophy if the iron for- eign body is not
removed.
The iris will take a rusty brown color and this
discolor- ation is often the first indication that the
eye is harboring an iron foreign body (Fig. 23.19).
The lens will also show rusty brown discoloration
and slowly turn cataractous. The retina will show
retinitis pigmentosa like changes. There will be
gradually rise in IOP and the eye will slowly
become blind, if left untreated.

Endophthalmitis following intraocular trauma is TRAUMATIC ENDOPHTHALMITIS

dif- ferent from other forms of endophthalmitis
because of several reasons. Firstly
 Chapter Ocular Trauma 349
23

FIGURE 23.18: Siderosis bulbi FIGURE 23.19: Foreign body in the lens

the clinical features and in making an accurate Topical antibiotics (e.g. fortified gentamicin and forti-
diagnosis in early stage. Secondly, the organism fied cefazolin or fortified vancomycin 1 hourly).
producing the in- fection is more virulent. Thirdly, Intravitreal antibiotics (e.g. amikacin 0.4 mg in 0.1
the protocol for manage- ment remains ill defined mL and vancomycin 1 mg in 0.1 mL or clindamycin 1 mg
(Fig. 23.20). in 0.1 mL) these may be repeated every 48–72 hours as
Ocular trauma has been reported to contribute needed.
17%– 40% of all cases of culture positive
endophthalmitis.
Onset of endophthalmitis is about 1–2 days in
fulmi- nant cases caused by Bacillus cereus and
streptococci, 3–4 days in acute cases caused by
Staphylococcus epidermi- dis and gram-negative
organisms and about 5–7 days for chronic
endophthalmitis caused by fungi.
Infection caused by Bacillus cereus is
characteristic, usually there is a history of trauma
with a metallic foreign body lodged within the eye.
The patient develops severe orbital pain within 24
hours of the injury and this is associ- ated with a
significant proptosis, chemosis and periorbital
inflammation. A corneal ring infiltrates and ring
abscess occur frequently. Most patients become
febrile with a moderate polymorphonuclear
leukocytes. The only other endophthalmitis producing
organism capable of causing similar constitutional
symptoms is Clostridium.
Collection of intraocular samples for laboratory
investigations is necessary as in cases of postsurgical
endophthalmitis. The procedures adopted are
para- centesis, vitreous aspiration and vitreous
biopsy.
Treatment should not be delayed for want of
diagnos- tic specimens, start systemic antibiotics
(e.g. ciprofloxa- cin, 400 mg IV12 hourly and
cefazolin, 1 g IV qid).
 Chapter Ocular Trauma 350
23
The benefit of pars plana vitrectomy is
unknown for traumatic endophthalmitis. However
pars plana vitrecto- my reduces infectious load
and provide sufficient material for diagnostic
culture and pathologic investigation.
Early vitrectomy has been advocated in all
cases with retained IOFB. Steroids should not be
used until fungal or- ganisms are ruled out.

Most of the serious injuries of the ocular adnexa FRACTURES OF THE ORBIT
involve fractures in the region of the orbit.
Common causes for fracture of the orbit are fall
from a height, road traffic acci- dents and blow
with a fist. High incidence of multiple and serious
injuries is associated with head and facial trauma
has been noted. So as a general rule immediate
examina- tion of all systems are necessary.

FIGURE 23.20: Traumatic endophthalmitis following

penetrating injury
 Chapter Ocular Trauma 351
23

From the ophthalmological point of view injuries presence of bony fragments in the orbit,
may be divided into two types: direct and indirect. downward due to loss of supporting action of the
Direct injury is due to blunt violence falling directly
ligament of Lockwood, which stretches like a
upon the orbit and the indirect injury results from
hammock from the medial to the lateral bony
an involvement of the or- bital bones, in a radiating
margins of the orbit. When the exophthalmos is gross
fracture of the vault of the skull or the bones of the
following downward displacement of orbital con-
face.
tents, a pseudoptosis of the upper lid and deepening
of the supratarsal fold will occur.
Blow Out Fractures (Fig. 23.21)
Blow out fractures result from a sudden increase in Diplopia
intra- orbital pressure, secondary to a blow to the
Usually due to restriction of vertical movements and
eye and soft tissues of the orbit from a non-
main factors responsible are incarceration within the
penetrating convex object such as Tennis ball, a fist
fracture- line of the extraocular soft tissues,
or other bodies of greater diam- eter than the
particularly inferior rec- tus and oblique muscles,
orbital margins.
displacement of the suspensory ligament of
The tissue of the orbit is suddenly compressed Lockwood, the periorbital, the muscle sheaths and
and the increased pressure is transmitted to the their connections. While in fracture of medial wall,
walls of which the more delicate portions are
the medial rectus muscle and its sheath get
fractured and blown outwards. Floor is the usual
involved re- sulting in limitation of horizontal
site of fracture, bony fragments and orbital soft
movement of the globe.
tissue may be displaced down- wards into the
maxillary antrum, usually giving rise to a
hemorrhage. Sometimes blow out fractures Forced Duction Test
involve the medial orbital wall in the area of the The muscle is gently grasped with a forcep after
lamina papyracea and the orbital plate of the anesthe- tizing the eye and the eye is moved in
ethmoid, resulting in the me- dial displacement of the direction of ac- tion of the muscle. There will
the bony fragments and orbital soft tissue with be limitation of movement if there is entrapment of
local hemorrhage in the plane of the ethmoid air the muscles in the fracture. Infe- rior rectus is
cells (Fig. 23.22). usually involved due to its midline position over the
infraorbital canal where such fractures most fre-
Clinical Picture quently occur, but as there is some fibrous
connection between the inferior rectus and inferior
Displacement of Globe oblique muscle both muscles are usually
Either proptosis due to intraorbital hemorrhage or affected.
ex- ophthalmos due to fracture of floor with
herniation of orbital contents into the maxillary Sensory Loss
antrum or nasal cav- ity, or vertical displacement Infraorbital anesthesia over to the region of skin
that may be upward by the and oral mucosa supplied by the infraorbital nerve
support
 Chapter Ocular Trauma 352
23

FIGURE 23.21: Movement of left eyeball is restricted on elevation FIGURE 23.22: Coronal cut CT showing orbital floor fracture with
soft tissue entrapment
 Chapter Ocular Trauma 353
23

a fracture in the central part of the floor of the Traumatic optic neuropathy can be classified into
orbit. The absence of such sensory loss in the three types:
presence of other signs of fracture of the orbital floor 1. Optic nerve avulsion where the optic nerve is
indicates that the injury may be either medial or partially or completely separated from the
lateral to the infraorbital canal. In case of lateral globe.
orbital floor fracture sensory loss occur in the
distribution of the zygomatic nerve.

Epistaxis
Epistaxis occurs in nasomaxillary injury or a fracture
of medial orbital wall.

Cerebrospinal Rhinorrhea
Clear watery discharge from the nose or ear
following an injury to the head indicates that a
communication exists between the subarachnoid
space and the exterior. In all such cases the advice
of a neurosurgeon should be sought at an early
stage.

Management
Antibiotics and anti-inflammatory drugs are given to
con- trol the inflammation and to prevent
infection.
If there is significant diplopia and exophthalmos,
sur- gery is indicated. The periosteum is elevated
from the floor of the orbit and all entrapped orbital
contents are separated and the defect in the bone is
repaired using sili- cone or teflon mesh.

TRAUMATIC OPTIC
NEUROPATHY
Trauma may affect any segment of visual pathway.
Optic nerve is about 47–50 mm in length and can be
divided into four parts as intraocular (1 mm),
intraorbital (30 mm), in- tracanalicular (6–9 mm)
and intracranial (10 mm).
The intracanalicular part is the most vulnerable to
ex- ternal blunt trauma. It is immobilized in the canal,
which is fixed to the surrounding periosteum and
bone.
Intraorbital optic nerve injury rarely occurs
because of the laxity of the optic nerve in this
area and is cush- ioned by surrounding orbital fat.
Intracranial optic nerve is rarely damaged indirectly
due to its mobility within the cranium and it is
cushioned by the surrounding cerebro- spinal fluid.

Classification
 Chapter Ocular Trauma 354
23
2. Direct injury caused by the impact on the optic If an obvious compressive lesion is present on
nerve or nerve sheath from a penetrating imaging studies (e.g. bone fragment, hematoma)
foreign body, a dis- placed bone fragment or decompression should be considered.
a retro-orbital hematoma. If there is no visual impairment within 48 hours or
3. Indirect injury, here forces are transmitted to the patient’s vision worsens despite steroids, optic
the op- tic nerve within the optic canal. nerve de- compression should be considered.

Optic Nerve Avulsion

Visual acuity is reduced to a variable degree in
partial avul- sion. In total avulsion, the vision is
reduced to no percep- tion of light. The fundus
changes with time. Immediately after the injury,
the disk is often obscured by overlying vitreous
hemorrhage. Overtime in cases of total avulsion
the sclera canal is seen devoid of optic disk and
this defect is gradually filled with glial tissue,
which may extend into the vitreous. Associated
ocular finding include dilated and amaurotic
pupil.
Visual acuity ranges from a minimal decline to
no per- ception of light.
Apart from defective vision, signs include
relative af- ferent papillary defect, defective color
vision and field de- fect. Disk pallor develops
within 3–4 weeks after injury.

Electrophysiology
The VEP may be used to document conduction
delays in comatose patients and the initial VEP
may correlate with the final visual acuity.
High resolution CT: Both the coronal and axial cuts
should be performed to detect facial and optic
canal fractures.
MRI: Although inferior to CT in the detection of
bone de- fects, MRI is an adjunct to CT in
imaging the intracranial segment of the optic
nerve for disruption or hematoma.
Complete neurologic assessment is required, as
many of these patients have associated brain
injuries.

Management
Medical therapies with intravenous high dose of
cortico- steroids have been reported to be
effective in some cases. Methylprednisolone 20–30
mg/kg/day or dexamethasone 3–5 mg/kg/day.
Treatment should be instituted within 24– 48 hours
and if improvement occurs, oral steroid therapy
can be started and tapered off over the next
2 weeks.
 Chapter Ocular Trauma 355
23

Optic Canal Decompression Typical features are granulomatous bilateral uveitis,

mutton fat keratic precipitates (KPs), vitreous cells,
Decompression of optic nerve within the optic
yel- low-white choroidal infiltrates and Dalen-Fuchs
canal may be performed through various approaches
nodules.
(e.g. transcra- nial, transorbital, transethmoidal). The
goal of surgery is to reverse any component of
compression (edema, hema- toma, bone fragment)
of the intracanalicular portion of the optic nerve.

Optic Nerve Sheath Decompression

The goal of surgery is to decompress the orbital
portion of the optic nerve.

SYMPATHETIC OPHTHALMIA

Sympathetic ophthalmia is a rare, bilateral diffuse

granu- lomatous uveitis that presents insidiously after
open globe injury or surgery. The injured eye is
known as the exciting eye and the follow eye
developing inflammation weeks to years later
known as the sympathizing eye.

Incidence
The exact figures are difficult to ascertain due to
delayed presentation, lack of histopathological
confirmation in clinical sympathetic ophthalmitis
and absence of clini- cal evidence in
histopathologically positive cases of sym- pathetic
ophthalmitis. Due to meticulous suturing tech-
niques, prompt repair of corneoscleral wounds and
use of steroids, the incidence of sympathetic
ophthalmitis is now reduced drastically.

Clinical Features
The average onset is 3 months after injury. About
90% oc- curs in 1 year, but can occur as early as 2
weeks and as late as 50 years. Onset is never earlier
than 2 weeks since this is a hypersensitivity
reaction.
The clinical features comprises of a spectrum
ranging from mild to severe. The patient first seeks
advice for pho- tophobia and lacrimation or transient
defective near vision due to weakness of
accommodation in the uninjured eye.
The first sign may be the presence of keratic
precipi- tates on the back of the cornea or the
presence of retrolen- ticular flare and cells, which
are noticed at early stage.
 Chapter Ocular Trauma 356
23
The exiting eye may show traces of old decision to enucleate an eye is diffi- cult and
iridocyclitis and still possess useful vision or controversial. The need to remove a traumatized
may have shrunken completely.
The B scan often reveals choroidal
thickening, fluo- rescein angiography shows early
hyperfluorescent sites of choroidal leakage.
Indocyanine green is an important ad- ditional test
and shows multifocal hypofluorescent dots, which
become prominent with time.

Differential Diagnosis
• Phacoanaphylactic endophthalmitis
• Vogt-Koyanagi-Harada syndrome: Absence of
history of trauma/surgery.

Histopathology
• Diffuse granulomatous infiltrates throughout
uveal tissue
• Dalen-Fuchs nodules: These are epithelial
granulomas seen between the Bruch’s
membrane and the retinal pigment
epithelium
• Choroidal thickening
• Absence of uveal necrosis
• Choriocapillaris and retina being spared
• Immunohistochemical studies show histolytic
cells with degenerating retinal pigmented
epithelium (RPE) cells and lymphocytes
• Predominant CD4 in early stage and CD8
lymphocytes evident in late stage.

Etiopathogenesis
Sympathetic ophthalmia is an autoimmune
disorder. Re- lease of uveoretinal antigen following
penetrating injury exposes it to conjunctiva. It is
then processed through the lymphatic channels,
which act as immune stimulants. The normal
suppressor mechanism is bypassed and an auto-
immune uveitis results in genetically susceptible
individ- uals. Genetic predisposition to
sympathetic ophthalmia is evidenced by increased
frequency of human lymphocytic antigen-A11
(HLA-A11) in histologically proven sympa- thetic
ophthalmitis.

Prevention
The best way to prevent sympathetic ophthalmia
is early enucleation of a badly damaged eye
before the sensitiza- tion occurs. But the
 Chapter Ocular Trauma 338
23

eye is very remote in present times. Other

measures in- clude meticulous and prompt closure
Other Immunomodulators
of corneoscleral wounds, trauma to uveal tissue Cyclosporine has been used as a second line drug
should be minimized and prolapsed tissue should be in the treatment of sympathetic ophthalmia in
excised or reposited. The role of prophylactic case with poor response to corticosteroids.
steroids is controversial. Cyclosporine 5 mg/kg/day helps in inhibition of T-cell
production in this T-cell medi- ated disease. Other
Management agents used in addition to steroids in- clude
Azathioprine, Methotrexate and Chlorambucil with
Early detection and prompt initiation of high-dose varying success.
ste- roids holds the key to a good outcome.
Topical steroids and mydriatics can be used to Outcome
control anterior uveitis. Systemic corticosteroids in
high doses, at least 1–1.5 mg/kg of prednisolone Early diagnosis and aggressive therapy are correlated
should be promptly commenced, followed by a slow di- rectly with better visual outcome. Other causes
taper to a maintenance dose for several months, of poor vision include glaucoma, cataract, macular
up to at least a year. scarring, cystoids macular edema. Due to relapsing
nature of this disease, long-term follow-up is
Treatment may sometimes be commenced with
required, even in patients who are symptom free
pulsed steroids, three infusions of intravenous
for years.
methylpredniso- lone 1 g over 3 days, followed by
After resolution the fundus may appear normal,
oral steroids. Relapse has been noted commonly
but pigment migration and optic atrophy can
on taper of corticosteroids.
occur.
 Chapter Ocular Trauma 339
23

SECTION 5
Diseases of the Adnexa
Lid
s
2
4
Girija Devi PS, Lekshmi P Moorthi

The lids are the movable folds provided in front of plate. The tarsal plates contain verti- cally arranged
the eye. Their main function is protection of the large sebaceous glands called meibomian
eyeball, a mere touch or even a loud noise will
cause reflex closure of the lids. The lid movements
are essential to spread the tears over the eyeball
and also to wash away any dust, foreign bodies,
debris, etc. into the lacrimal sac by the action of
the lid closure. Unknowingly, the eyes are
continuously washed with tears by the lid
movements.

APPLIED ANATOMY
The lids are covered by skin in front and mucous
mem- brane behind (Fig. 24.1). The junction
between the skin and the mucous membrane is the
‘intermarginal strip’ the transitional zone lining the
lid margins. In between the skin and the mucous
membrane there are muscles, the fibrous plate
called tarsus, the blood vessels and nerves. The
detailed anatomy is described in Section 1
‘Anatomy and Physiology of the Eye’.
The skin of the lids has many anatomical
peculiarities. It is the thinnest in the body and it
has no subcutaneous fat. Being very elastic and
loosely attached to the under- lying structures, it
can get easily distended with fluid or blood, which
can gravitate to it from the scalp or neigh- boring
structures.
It has fine hairs and it is well lubricated with fine
seba- ceous glands, but has only small sweat
glands. At the lid margin these structures undergo
modification. The hair is modified into eyelashes—
curved short strong hairs usu- ally in two or more
rows. The sebaceous glands supply- ing these
eyelashes are also suitably modified to form the Zeis’
glands. The sweat glands are also modified into large
glands at the lid margin to form the Moll’s glands.
The ducts of the Moll’s glands open into the hair
follicles of the lashes or into the ducts of the Zeis’
glands. The fibrous tis- sue near the lid margin is
strong and almost like cartilage. This is called tarsal
glands or tarsal glands. Their ducts open along a
straight line at the lid margin.
There is a fine gray line immediately anterior
to the openings of the meibomian glands. This is
an important an- atomical landmark of the lids.
The lids can be split into two layers at the gray line
—the anterior and posterior lamellae. The anterior
lamella consists of the skin and orbicular-
is muscle. The posterior lamella consists of the
tarsal plate, conjunctiva and the eyelid retractors.
The eyelid retractor in the upper lid is the levator
palpebrae superioris. The lower lid retractor is a
fascia extending from the inferior rectus muscle,
which splits to enclose the inferior oblique muscle
and then reunite to form a fibrous sheet, which at-
taches to the inferior border of the tarsal
plate.

FIGURE 24.1: Anatomy of upper lid

 Chapter Lids 342
24

The lids are richly supplied by blood vessels. FIGURE 24.2: Angioneurotic edema
Hence wounds of the lids heal well even if heavily
contaminated. Even small tags of tissue, however
devitalized it may ap- pear, will survive and no
tissue of the lid need be sacrificed in repair of
lacerated wounds of lids.

DISEASES OF THE LIDS

Lid Edema
Since the skin of the lids is very elastic and
loosely at- tached to the underlying tissue, fluid can
easily collect in the lid. Many conditions can lead
to lid edema.

Inflammatory Lid Edema

Edema of the lids is common in any inflammation
of the lids, adnexa or eyeball. The extent of edema
depends on the severity of the inflammation.

Reactionary Lid Edema

In any inflammation of the surrounding structures like
an infected wound on the scalp or a boil on the
forehead a re- actionary lid edema can occur. The
lids may get so swollen that it is difficult to examine
the eyeball. But on retracting the lids, the eye will
be normal. There will be no tender- ness or
induration of the lids. Searching the neighboring
areas will reveal the culprit. Once the infection at
this fo- cus subsides the lid edema will also
disappear.

Angioneurotic Edema
Angioneurotic edema (Fig. 24.2) is a severe allergic
reaction to some allergens like medicines, cosmetics
or a wasp bite. There will be history of sudden onset
and itching. The lids will be swollen and closed, but
there will be no tenderness or induration of the lids.
On retracting the lids there may be congestion and
chemosis, if it is a local allergic reaction.
Treatment: If it is wasp bite, anaphylactic shock can
occur. So systemic steroids have to be given.
Otherwise topical
 Chapter Lids 343
24
steroids and/or antihistamines and removal of the Treatment: Clean the lashes with baby shampoo or
offend- ing allergen are sufficient to control warm 3% sodium bicarbonate solution regularly. In
the problem. acute exac- erbations steroid ointments can be
applied after cleaning.
Passive Lid Edema
A puffiness of the lid can appear, especially in the
morning on waking up in conditions of generalized
fluid collection due to renal disease or cardiac
failure. Passive lid edema can occur in local
circulatory obstruction as in cavernous sinus
thrombosis.

Ecchymosis or Black Eye

Since lids are highly vascular and tissues are lax,
any con- tusion injury can lead to bleeding into
the subcutaneous tissue. The subcutaneous
blood will give a bluish color to the lids and the
lid swelling will depend on the extent of the
bleed. This condition is called ‘black eye.’ This
bluish discol- oration can disappear within 1–3
weeks depending on the amount of blood.
Bleeding need not be from the lid itself. Any
blood from the neighboring structures like bleeding
un- der the scalp or even the blood from the other
eye can gravi- tate to the lids due to the looseness
of the tissue. In this situa- tion the black eye will
appear only 1–2 days after the trauma.

Treatment
No treatment is required. This blood will get
absorbed in 1–3 weeks. If the bleeding is massive
serratiopeptidase can be given to facilitate
reabsorption of the blood.

Inflammations of the Lid

Blepharitis
Anterior blepharitis
Seborrheic blepharitis: It is similar to seborrhea or
dandruff of the scalp. Patients, usually complain of
itching of the lid margins. The lid margins are
usually hyperemic and there are tiny scales in
between the lashes. The lashes fall off
frequently, but they are replaced without any
distortion (Table 24.1).
TABLE 24.1: Blephiritis
Anterior blepharitis Posterior
blepharitis Squamous/seborrheic blepharitis
Meibomian seborrhea Ulcerative blepharitis
Meibomianitis
 Chapter Lids 344
24

But their use must be kept to the minimum due to the FIGURE 24.3: Ulcerative blepharitis
com- plications associated with prolonged steroid
ointment.
Ulcerative blepharitis: This condition is due to chronic
in- flammation of the follicles of the lashes usually
due to Staphylococcus aureus.
The presenting complaints will be mild swelling of
the lid margins, irritation and crusting of the lid
margins. The symptoms will be aggravated with
redness, lacrimation and photophobia when the
infection spreads to the ocular surface to produce
blepharoconjunctivitis (Fig. 24.3).
On removal of the crusts, small ulcers can be
seen in between the roots of the lashes. There will be
redness and swelling of the lid margins. The lashes
will fall off and do not grow again leading to areas
with no lashes on the lid margin (madarosis). The
scarring produced by prolonged inflammation can
lead to distortion of the lashes and tri- chiasis
(lashes rubbing on the ocular surface). Conjuncti-
vitis and toxic marginal keratitis can occur.
Treatment: Clean the lid margin with baby shampoo
or warm sodium bicarbonate solution and apply
antibiotic ointment. The antibiotic treatment has to
be continued for 2–3 weeks or longer, since
organisms can survive deep in the hair follicles and
lead to recurrence. In resistant cases a C and S
study must be done to decide on the antibiotic
needed. The toxic keratitis may require weak steroids
like fluorometholone drops 2–3 times daily for
control.
Sequele: If not properly managed, the infection can
be- come chronic and lead to serious sequelae. The
lid margin abnormalities like madarosis and
trichiasis can occur.
The lid margin can become thickened leading to
droop- ing of the lids—tylosis. If the lower lid is
involved it can lead to eversion of the lower punctum
and epiphora, and ectropion.

Posterior Blepharitis
Posterior blepharitis is characterized by abnormal and
ex- cessive meibomian secretion.
 Chapter Lids 345
24
Meibomian seborrhoea: The excessive and abnormal inflamma- tion of a Zeis’ gland. Commonly it is
mei- bomian secretion leads to froth in the staphylococcal infec- tion. Since, Zeis’ gland opens
precorneal tear film and there will be oil droplets into a lash follicle an abscess forms at the root of
at the openings of the mei- bomian glands. These the lash (Fig. 24.5).
abnormalities can cause tear film instability and
irritation, burning and soreness of the eyes.
Meibomianitis: The conjunctiva on the tarsal surface
will be congested and prominent meibomian
glands will be seen through the tarsal conjunctiva
as pale yellow streaks. Lid massage will lead to
thickened cheesy secretions coming out from the
ducts. Obstruction of the ducts of meibomian
glands with this thickened secretions lead to
multiple cha- lazia formation.
Treatment
Warm compresses to melt the secretion and
mechanical expression by lid massage. Antibiotics
applied topically will help to control secondary
infection. Doxycycline or tetracycline for 6–12
weeks will help to correct the abnor- mal
secretions of the meibomian glands. This will
control the infection also.
Sequelae: Multiple chalazia, tear film instability and
mar- ginal corneal infiltrates.
Phthiriasis palpebrarum (Fig. 24.4): It is an infestation
of the eyelashes with the pubic louse (Pthirus
pubis). The infec- tion usually spreads in people
living in unhygienic and overcrowded
surroundings as in overcrowded hostels.
The infestation produces irritation and
itching. The crab-like lice and its nits can be seen
attached to the roots of the lashes.
Treatment:
1. Trimming of the lashes at their roots to
remove all the lice and its nits.
2. Touching the lashes with petrol (after
application of plenty of antibiotic ointment
to protect the eye) 1% mercuric oxide
ointment or cryotherapy.
3. Delousing of the patients by removing all
body hair and the cleaning of the clothes in
power laundry or by boiling is essential to
prevent recurrence.
4. Treatment of all infested family members or
the peo- ple in the hostel is also required.

Infections of the Glands of the Lids

Hordeolum Externum
Hordeolum externum (HE) is a suppurative
 Chapter Lids 346
24

with hot fomentation and topical antibiotics for 2–3

weeks may eradicate the persistent Staphylococcus
infection.
Complications: As follows:
1. Spread of infection to the surrounding tissues
leading to preseptal cellulitis or orbital
cellulitis.
FIGURE 24.4: Phthiriasis palpebrarum
2. Rarely, even cavernous sinus thrombosis can
occur since the infection is in the danger zone
of the face.

Hordeolum Internum
Hordeolum internum (HI) is a suppurative
inflammation of the meibomian gland. Since, it is a
much larger gland the inflammatory signs and
FIGURE 24.5: Hordeolum externum (HE) with pus symptoms are much more than in HE (Figs 24.6A
pointing at lid margin and B).
Symptoms: Patient usually complaints of pain, worse on
Symptoms: Children are more commonly affected. lid movements and swelling of the lids.
Pain and swelling of the affected lid will be the Signs: On examination, there will be some lid edema
main complaint of the patient.
and tenderness at the site of the affected gland. But
Sign: The affected lid will show edema. No definite there will not be any palpable swelling, since the
visible or palpable swelling is present. Tender inflamed gland is confined within the tarsal plate.
swelling is seen at the lid margin with pus On examination of the palpebral conjunctiva,
pointing at the root of a lash. initially there will be localized con- gestion at the
Treatment: In the initial stages before an abscess is site of the involved gland and later, as pus forms
formed, hot compresses and antibiotic ointment yellow spot will be visible through the palpebral
application will control the inflammation. conjunctiva. The pus may burst spontaneously
If pus points, it can be evacuated by pulling out through the pointed spot and the inflammation
the af- fected lash or incision and curettage can be will subside. Sometimes the infection will spread
done, if the in- fection has spread to the surrounding to the surrounding tissues, and a palpable and
tissues and a larger abscess has formed. tender swelling will appear and the pus may burst
In recurrent attacks, an uncorrected refractive through the skin surface also.
error or an undetected diabetes (especially in an Treatment: Hot compresses and topical antibiotic
adult with HE) has to be ruled out or a generalized oint- ment or drops may control the infection.
poor health with di- minished resistance to
If pus points on the conjunctival surface a small
Staphylococcus infection may be the reason. A course inci- sion will be sufficient to drain the pus.
of systemic antibiotics combined
Incision and curettage is needed, if a localized
abscess has formed.
Complications: Lid abscess, preseptal cellulitis and
cavern- ous sinus thrombosis.
 Chapter Lids 347
24

FIGURES 24.6A and B: Hordeolum internum with pus pointing on palpebral conjunctiva
 Chapter Lids 348
24

Chalazion (Tarsal Cyst or Meibomian Cyst) Rarely the chalazion will get resolved
Chalazion (tarsal cyst or meibomian cyst) is a chronic spontaneously. This usually happens in small chalazia
in- flammatory granuloma of the meibomian gland in children. Usu- ally an incision and curettage is
required for its resolution. Sometimes small amount
(Fig. 24.7). It is common in children than adults.
of granulation will be seen protruding through the
People with chronic meibomianitis, seborrheic
duct of the gland this is called
dermatitis and acne rosacea are prone to developing marginal chalazion.
chalazion, which is often multiple. The granuloma will burst spontaneously through
Obstruction of the duct of the gland with the conjunctival surface to form a papillomatous
thickened secretion may lead to accumulation of fleshy mass at the site, this is called chalazion
giant cells, plasma cells and histiocytes. The swelling granuloma.
is usually encapsulated and gradually increases in Treatment
size (Table 24.2). 1. Triamcinolone acetonide injection: 0.1
mL diluted with equal quantity of lignocaine
Clinical Features given into the chala- zion may result in
spontaneous resolution. The injec- tion can be
Symptoms: The usual complaint will be a disfiguring repeated after 2 weeks.
swell- ing on the lids, which has been present for a 2. Incision and curettage (I and C)
few weeks to months. There will not be any pain method: The lid is anesthetized with injection
or discomfort. of xylocaine (Fig. 24.8A). A chalazion clamp is
applied to the lesion so as to fix and evert the
Sign: A visible and palpable non-tender firm
lid (Fig. 24.8B). Chalazion clamp also helps in
swelling will be seen on the lids in the area of the
hemostasis. With a sharp knife like a BP blade a
tarsal plate. Smaller chalazia are more palpable small vertical incision is made over the
than visible. The tarsal con- junctiva over the chalazion at the conjunctival side without
swelling will be showing some grayish or muddy extending to the lid margin (Fig. 24.8C). Any
discoloration, but pus will not be seen. The skin fluid filling the cavity will flow out. A chalazion
over the swelling will be freely mobile. The scoop is used to scoop out all semisolid
swelling does not usually extend to involve the lid granulation inside (Fig. 24.8D). The verti- cal
margin and does not extend beyond the posterior incision is important to prevent damage to
edge of the tarsal plate. other glands because of the vertical
arrangement of the meibomian glands.
After all materials have been curetted out, a
firm pressure dressing is given for 1–2 hours to
control bleeding. If the chalazion is large and
pointing more on the skin surface, an incision
may be put on the skin surface also for
complete removal of all material. On skin
surface the incision is made horizontally along a
skin crease to minimize scarring.
After removing the dressing topical antibiotics
TABLE 24.2: Differential diagnosis of hordeolum applied
are internum,forhordeolum
a few days.externum
and chalazion If there is a chalazion granuloma, the
protruding granulation should be excised
FIGURE 24.7: Chalazion before doing the inci- sion. If there is a
marginal chalazion, the duct of the gland
should be curetted out.

Hordeolum Hordeolum externum Chalazio

internum (HI) (HE) n
Pain ++ + –
Tenderness + + –
 Chapter Lids 349
24
Swelling Lid edema only Swelling at root of lashes ++
Palpable swelling – – ++
Conjunctival surface Pus points Mild congestion Muddy discoloration over the swelling
Duration Short Short Long
 Chapter Lids 350
24

FIGURES 24.8A to D: Steps of incision and curettage. A. Local anesthesia infiltrated; B. Chalazion clamp applied and lid everted;
C. Curettage done with a chalazion scoop; D. Vertical incision put with a Bard-Parker (BP) blade.

3. Systemic tetracycline: In patients with lids oc- cupying the position of the meibomian glands.
chronic mei- bomianitis and multiple chalazia, They may
tetracycline or doxycycline has to be given for 4–
12 weeks to prevent recurrence.
Complications:
1. Bleeding after I and C.
2. Persistence of the swelling, if the curetting is
incomplete.
3. Recurrence of the swelling. Usually, it is a
chalazion arising from a neighboring gland. If it
is a true recur- rence at the same site, an
adenocarcinoma of the mei- bomian gland has
to be suspected, especially if the patient is
elderly. An excision biopsy has to be done to
exclude or confirm adenocarcinoma by
histopatho- logical examination.

ANOMALIES IN THE
POSITION OF THE LIDS
AND LASHES
Congenital Anomalies
Distichiasis
Distichiasis is an extra row of lashes in all the four
 Chapter Lids 351
24
rub on the cornea and has to be removed by
cryotherapy or radiofrequency epilation
needle.

Epicanthus
There is a semilunar fold of skin extending from the
upper lid to the lower one covering the inner
canthus. It is normal in Mongolian races. This fold
makes the distance between the two eyes appear
wider than normal and gives an ap- pearance of
a pseudoconvergent squint. This fold may dis-
appear as the child grows or it can be surgically
corrected.

Blepharophimosis
Blepharophimosis (Fig. 24.9) is a congenital
condition where the patient has bilateral ptosis
with reduced lid size both in the vertical and
horizontal dimensions. In addi- tion to narrow
palpebral fissure, there is epicanthus inver- sus,
flat nose, ptosis and telecanthus. Epicanthus inversus
means the fold of skin arises from the lower lid
and goes upwards. This condition usually runs
in families.
Treatment: Plastic reconstruction of the lids done
in mul- tiple stages at early childhood will correct
the deformity to some extent.
 Chapter Lids 352
24
increased due to abnormally widely separated bony
orbits. It will give an

FIGURE 24.9: Blepharophimosis syndrome

Telecanthus
Telecanthus is characterized by widely separated
medial canthus due to an abnormally long medial
canthal ten- don. It can occur alone or as part of
blepharophimosis syndrome. It has to be
differentiated from hypertelorism where the eyes
are widely separated due to widening of the
distance between the bony orbits.

Coloboma of the Lids

Coloboma of the lids (Figs 24.10A to F) is a
congenital de- fect in the lid. It can be partial or
full thickness defect and can be unilateral or
bilateral. It may be associated with other
congenital anomalies of the eye and there may be
a dermoid cyst at the limbus in the area of the
coloboma. Coloboma can lead to exposure of the
cornea on lid clo- sure. So the surgical correction has
to be done as early as possible after birth.

Epiblepharon
There is an additional horizontal fold of skin close
to the lid margin, which covers the lid margin and the
lashes es- pecially on the medical aspect of the lid.
On pulling the skin of the lids away from the lid
margin, the normal lid margin becomes visible and
the lashes assume normal position temporally. Thus
epiblepharon can be differenti- ated from congenital
entropion. Spontaneous correction occurs in most of
the cases as the infant grows up. If not, it has to be
surgically corrected. Mean while antibiotic oint-
ments can be prescribed to be applied frequently
to pro- tect the cornea.

Hypertelorism
Hypertelorism is a rare congenital condition where
the distance between the two medial canthi are
 Chapter Lids 353
24
appearance of pseudoconvergent squint. In 2. Contralateral lid retraction may give an apparent
telecanthus, a similar appearance can be ap- pearance of ptosis in the normal eye.
produced by abnormally long medial canthal
tendons.

Cryptophthalmos
Here, there is no palpebral fissure. The skin of
the fore- head is continuous with the skin of the
cheek and the eyes are hidden. It is often
associated with severe congenital anomalies of
the eye and orbit.

Congenital or Acquired Anomalies

Ptosis
Ptosis is a drooping of the upper eyelid. It can
be congeni- tal or acquired and also unilateral
or bilateral.
Classification:
1. Congenital ptosis:
a. Simple.
b. Complication associated with other
anomalies like:
• Blepharophimosis syndrome
• Marcus Gunn jaw-winking
• Ocular motor anomalies.
2. Acquired
ptosis:
a. Neurogenic:
• III nerve palsy
• Horner’s syndrome
• III nerve misdirection syndrome.
b. Myogenic:
• Myasthenia gravis
• Myotonic dystrophy
• Ocular myopathy.
c. Aponeurotic:
• Involutional
• Postoperative.
d. Mechanical:
• Tumors of the lid
• Dermatochalasis
• Symblepharon.
Pseudoptosis
Pseudoptosis (Figs 24.11A and B) is an apparent
appear- ance of ptosis, but the levator function
will be normal.
Causes:
1. Lack of support to the lid due to a small
eye as in phthisis bulbi and enophthalmos or
an empty socket after enucleation.
 Chapter Lids 354
24

FIGURES 24.10A to F: Coloboma of the lids. A. Congenital dermoid and coloboma; B. Dermoid in the eye with coloboma; C.
Accessory auricles in the child (Goldenhar syndrome); D. After coloboma repair; E. Traumatic coloboma; F. Traumatic coloboma
after repair.

3. Brow ptosis: Brow is at a lower position leading Congenital ptosis

to a narrow palpebral fissure, usually caused by Congenital ptosis is the commonest type of ptosis
VII nerve palsy. and it can be unilateral or bilateral. It has a
4. Dermatochalasis: This condition is seen in hereditary tendency and many people in a family
middle or old age, there is an abundance of will be affected (Figs 24.12A to C).
skin in upper lid with herniation of orbital fat,
Clinical features: In severe weakness of the levator
which hangs as loose folds over the palpebral
the up- per lid fold will be absent. This may be the
fissure.
only sign that can be elicited in an uncooperative
Pseudoptosis can be differentiated from a true small child. There will be compensatory wrinkling of
ptosis by demonstrating normal levator action in the forehead and brow el- evation as well as
cases of pseu- doptosis.
 Chapter Lids 355
24
elevation of the chin.
 Chapter Lids 356
24

FIGURES 24.11A and B: Pseudoptosis. A. In an empty socket; B. Corrected when using artificial eye.

Usually congenital ptosis may be associated In eyes with fair and good levator action, levator
with weakness of the superior rectus muscle, muscle resection will gives good cosmetic
since both muscles develop as a single block. If
appearance. In ptosis with poor levator action,
the lids are covering the pupil there is risk of
frontalis sling operation has to be done. In this surgery
development of amblyopia and surgery has to be
the lid is mechanically lifted up by sus- pension at the
done early. If the pupils are not covered, the child
can be followed up till the preschool age since brow from the frontalis muscle using various materials
some spontaneous improvement will occur as the such as fascia lata, non-absorbable sutures or sili- cone
child grows. Surgery may be done before band. This will give good appearance in primary gaze,
starting school. but on looking down there will be limited lid
movement and lid closure during sleep may be
Treatment: The treatment is surgical. The time of
defective. In unilateral cases, the cosmetic appearance
surgery depends on the severity of the problem.
is generally unsatisfactory. In such unilateral cases
Time of surgery: If the ptosis is severe enough to with poor levator function, it is better to give crutch
occlude the pupil this can result in amblyopia. If spectacles, which will mechanically
the child is con- stantly keeping the chin elevated to lift up the upper lid.
clear the visual axis, this can lead to permanent
In patients with Marcus Gunn jaw-winking
structural changes in the neck. In both these
phenom- enon, if the cosmetic problem is
situations surgery has to be done before the age of 2
significant the surgical plan has to be suitably
years. Otherwise surgery is ideally done before
modified. The levator muscle has to be disinserted
starting school.
from the tarsal plate and frontalis sling surgery
Type of surgery: The type of surgery depends on has to be done to manage both ptosis and jaw-
whether it is unilateral or bilateral and whether the winking together. In severe unilateral cases, bilateral
levator action is poor, fair or good. The aim is to leva- tor disinsertion and frontalis sling surgery will
obtain symmetrical look between the two eyes. be needed to produce a symmetrical look
between the two eyes.

FIGURES 24.12A to C: A. Minimal ptosis right eye (RE)

with good levator palpebrae superioris (LPS) function; B.
 Chapter Lids 357
Moderate ptosis in LE; C. Bilateral congenital ptosis with poor
24
LPS action and compensatory frontalis overaction.
 Chapter Lids 358
24

Acquired ptosis FIGURE 24.13: Aponeurotic ptosis with upper eyelid crease
Neurogenic ptosis at a higher level
Neurogenic ptosis is due to III nerve palsy or due
to sym- pathetic palsy as in Horner’s syndrome. In III
nerve palsy the motility of other muscles supplied
by III nerve will be affected and the pupil may be
affected or spared. On elevating the ptotic lid, the
patient will experience diplo- pia. In ptosis due to
Horner’s syndrome other features of Horner’s
syndrome will be present.
Myogenic ptosis
Myogenic ptosis is bilateral. In myasthenia gravis,
there will be variation in the ptosis in different
times of the day. Ptosis will be more when the
patient is tired or toward the evening. The
Tensilon test will be positive.
Aponeurotic ptosis
Cause
Aponeurotic ptosis is due to weakness of the
aponeurosis of levator muscle. There will be
stretching, disinsertion or dehiscence of the
aponeurosis, which makes lifting of the lid difficult
when the levator muscle contracts. This can occur
after some surgeries, trauma or in old age. If it is due
to old age, which is the most common type, it will
be bilat- eral (Fig. 24.13).
Clinical features: The upper eyelid crease will be at a
higher level or absent in severe cases of
aponeurotic ptosis. The upper sulcus will be deep
and the lid above the tarsus will be thin. This is due
to the disinsertion of the attachments of the levator
aponeurosis to the tarsus and at the same time
the skin attachments remain intact.
Mechanical ptosis
There will be a mechanical restriction to the
movements of the lids as in upper lid tumors,
edema of the lids or adhe- sion of the lids to the
globe (symblepharon) (Figs 24.14A and B).
 Chapter Lids 359
24

FIGURES 24.14A and B: Mechanical ptosis. A. Shows

ptosis; B. Lid tumor, which is the reason for the mechanical
ptosis is visible an elevating the lid.

Investigations:
1. Margin reflex distance: This is the
distance between the center of the pupil
and the upper lid margin. Normally it is 4–
4.5 mm. In ptosis it will be less and it may
be negative in severe ptosis (the lid line is
below the pupil).
2. Measurement of the palpebral fissure
height: This is the distance between the
upper lid and lower lid at the level of the
pupil when the patient is looking straight.
Normally it is 7–10 mm in men and 8–12 mm
in women. If the difference between the two
eyes is 2 and 4 mm, the ptosis is mild, 4–7
mm, it is moderate and when more than 7
mm it is severe.
3. Measurement of levator action: The
lid movement is measured with a
transparent ruler held vertically before the
eye in the pupillary plane. The compensa-
tory action of the frontalis is prevented by
firm pres- sure on the eyebrow with the
thumb of the examiner. The patient is
asked to look maximally down and then
upwards as far as possible without moving
the head. The difference in the reading on
the rule at ex- tremes of gauze gives the
levator function. Normally it is around 15
mm. If the measurement is 4 mm or less, it
is poor levator action, 5–7 mm it is fair and if
it is more than 8 mm it is good levator
action. In infants these measurements may
not be possible. If the lid
 Chapter Lids 360
24

fold is present, it can be taken that fair levator Fasanella-Servat operation

action is present and if the lid crease is absent, Fasanella-Servat operation is done in:
it denotes poor levator function. 1. Mild degree of congenital ptosis (2 mm).
4. Measurement of action of associated 2. Horner’s syndrome.
muscles: Like frontalis, orbicularis, superior 3. Mild or moderate degrees of myogenic ptosis.
rectus and other ex- ternal ocular muscles
must be done. In people with myasthenia
frontalis over action and wrinkling of the forehead
will be absent. The orbicularis action may be
weak in myopathies.
5. Bell’s phenomenon: It is tested by asking
the patient to close the lid, while the lids are
kept open by the fingers of the examiner. If
the Bell’s phenomenon is good, the eye will
role upon attempted closure of the lids. If Bell’s
phenomenon is weak, there is greater risk for
developing exposure keratitis after frontalis sling
surgery. Absent Bell’s phenomenon will lead to
expo- sure of the cornea during sleep, if the lid
closure is de- fective after surgery.
6. Marcus Gunn jaw-winking phenomenon:
It has to be looked for in all congenital ptosis
patients. It is seen in 5% of congenital ptosis.
There is elevation of the eyelid on stimulation
of the ipsilateral pterygoid muscle by chewing
or opening the mouth. In severe form it can be
very embarrassing to the patient.
Treatment of acquired ptosis
Neurogenic ptosis: The underlying neurological
problem, if possible has to be treated. The patient
has to be regularly followed up for spontaneous
recovery. When the defect be- comes stabilized and
no more recovery is taking place, sur- gery has to be
contemplated. In III nerve palsy, the squint has to
be surgically corrected before ptosis is taken up.
Otherwise diplopia will occur on elevation of the lid by
sur- gery. If there is residual levator action levator
surgery can be done. Otherwise frontalis sling surgery
has to be done.
Myogenic ptosis: The ptosis is usually bilateral. The
under- lying condition has to be treated. In
myasthenia gravis, steroids, immunosuppressants or
thymectomy will give good results. In conditions like
myopathies crutch spec- tacles can be used to lift
the lid.
Aponeurotic ptosis: The aponeurosis may be
reattached to the anterior surface of the tarsus
and this may be com- bined with levator
resection to get optimum results.
Mechanical ptosis: Treat the underlying pathology.
Surgeries for ptosis
 Chapter Lids 361
24
Technique: The upper lid is everted and a curved are pulled up to the upper incision through a tunnel
artery for- ceps is applied to include conjunctiva, underneath skin and orbicularis muscle. A curved
2–3 mm of the upper edge of the tarsus and artery forceps can be used, if silicon band or
corresponding amount of Müller’s muscle. The prolene suture is used. The sling material is pulled
clamped tissue is removed by cutting with scis- up to give the desired lift to the eyelid and the
sors and the wound is sutured with absorbable material is pri- marily anchored to the frontalis
sutures. muscle above the eye- brow with sutures. The skin
Levator resection incisions are then closed.
Levator resection is done when there is fair to
good leva- tor function. This can be done from
the conjunctival or skin surface. The conjunctival
approach is used when the amount of levator
resection needed is 10 mm or less. The skin
approach gives better exposure and this approach
is used when larger resections have to be
done.
On an average, levator resection of about 3–4
mm cor- rects 1 mm ptosis. For congenital ptosis
the minimum amount of resection should be 10
mm. For a ptosis of 4–7 mm, the average
resection is 15–22 mm.
A horizontal skin incision is put near the upper
border of the tarsus. The orbicularis muscle is split
to expose the aponeurosis of the levator. The
muscle is separated from the upper tarsal border
and the muscle is dissected out from the
surrounding structures. The orbital septum is
carefully separated from the muscle to expose the
desired amount of muscle. The desired amount of
muscle to be re- moved is marked. The lateral
attachments of the muscle are also cut for free
mobilization of the muscle. Then 3 mattress
catgut sutures are passed above the level of exci-
sion of the muscle, then the amount of muscle
is excised and the three preplaced mattress
sutures are anchored to the anterior surface of the
tarsal plate. The orbicularis and skin are sutured
in layers (Figs 24.15A to C).

Frontalis Sling Surgery

Frontalis sling surgery (Figs 24.16A and B) is done
when the levator action is poor. Materials used
for suspension are fascia lata from the patient
himself, 5–0 prolene suture or silicon band.
Three incisions are made on the upper lid skin
parallel to the lid margin till the tarsal plate is
exposed. Two inci- sions are put just above the
eyebrow deep up to the fron- talis muscle. Three
strips of fascia lata or silicon bands are fixed to
the tarsal plate. If prolene suture are used, bites
are taken from the tarsal plate and the suture is
fixed to the tarsal plate. A fascia lata introducer is
passed from the incision above the eyebrow to
reach the lower incisions and the strips of fascia
 Chapter Lids 362
24

Lagophthalmos
Lagophthalmos is an incomplete closure of the
palpebral aperture when an attempt is made to
close the eye.
Causes:
1. Abnormalities of the lids:
• Paralysis of the orbicularis
• Symblepharon and scarring
• Coloboma—congenital or traumatic
• Severe ectropion.
2. Abnormal protrusion of the eyeball:
• Thyrotoxicosis
• Orbital tumor
• Acute orbital cellulitis
• Carotid—cavernous fistula
• Buphthalmos.
3. Absence of reflex blinking:
• In patients in coma or under anesthesia
• Terminally ill patients
• It can result in exposure keratitis.
In these conditions improper lid closure will
lead to drying of the corneal epithelium,
keratinization of corneal epithelium and exposure
keratitis.
Treatment:
1. Treat the underlying pathology, if possible.
2. Keep the cornea well lubricated with frequent
instilla- tion of lubricant drops or ointments.
3. If proper protection of the cornea is impossible
or ex- posure keratitis sets in, a lateral
tarsorrhaphy is done

FIGURES 24.15A to C: A. Levator muscle disinserted from

the tarsal plate; B. Levator to be resected is measured with
calipers;
C. Mattress sutures are passed through the cut end of levator
palpebrae superioris (LPS) and the upper part of the anterior
surface of tarsal plate (Courtesy: Stallard’s eye surgery).
 Chapter Lids 363
FIGURES 24.16A and B: Frontalis sling
24
surgery. A. In the immediate postoperative;
B. Late picture.
 Chapter Lids 364
24

in lagophthalmos. In neuroparalytic keratitis a

para- median tarsorrhaphy is done.
Acquired Ectropion
Method: Two raw surfaces 6 mm in length are Involutional ectropion
produced in corresponding position of the upper Involutional ectropion (Fig. 24.18) is the commonest
and lower lid mar- gins by removing a strip of type of ectropion. It usually affects the lower lid.
intermarginal strip of conjunc- tiva. The two raw Mechanism: Horizontal lid laxity combined with laxity
surfaces are brought together and kept in of the medial and lateral canthal tendons lead to
approximation with sutures so that adhesions this con- dition. Horizontal lid laxity can be
develop between the raw surfaces of the lid margins. demonstrated by the snap test. Pull the central
The sutures will be removed after 2 weeks (Figs part of the lower lid away from the eye. On
24.17A to C). releasing, the lid should snap back into posi- tion
without blinking. If the lid takes time to go back to
normal position or needs blinking to bring it back to
ECTROPION posi- tion, horizontal lid laxity is present.
Involutional entropion and ectropion
Ectropion is an eversion or rolling out of the lid
margin away from the eyeball. Both conditions have almost similar etiological
factors. There is horizontal lid laxity in both
conditions.
Types People who develop ectropion have an
1. Congenital. atrophied or smaller than normal tarsal plate and
2. Acquired: a normal or in- creased tone of the
preseptal/pretarsal orbicularis and the mechanical
a. Involutional or senile.
effect is a rolling out of the lids.
b.Cicatricial.
People who develop entropion have a normal or
c. Paralytic. larger than normal tarsal plate and normal or
d. Mechanical. decreased tone of the pretarsal orbicularis and the
vector forces mechani- cally pull the lid margin
Congenital Ectropion inwards.
Congenital ectropion is a very rare condition. It is Management
usually associated with other congenital anomalies Management depends on the cause for the ectropion.
like coloboma of the lids and microphthalmos. The surgical procedure depends on the severity of
the problem and the portion of the lid where the
malposition is most pronounced.
 Chapter Lids 365
24
FIGURES 24.17A to C: Tarsorrhaphy. A. Immediately after surgery; B. Late picture; C. Diagrammatic
representation (Courtesy: Stallard’s textbook of surgery).
 Chapter Lids 366
24
FIGURE 24.19: Excision of tarsoconjunctival spindle

FIGURE 24.18: Involutional ectropion

Electrocautery puncture: In mild degree of senile

ectropion especially involving the medial canthus, a
row of electric cautery burns are applied to the
conjunctival surface be- low the lower punctum. The
contraction produced by the scarring will pull the lid
inwards at the medial end and the punctum close to
the globe.
Excision of a tarsoconjunctival spindle (Fig. 24.19): In
moder- ate degrees of senile ectropion involving
the medial end of the lower lid, excision of a
horizontally oval area of tar- sus and conjunctiva
parallel with and inferior to the lower punctum and
suturing the wound edges can be done. This will
produce an inward pull on the inferior punctum.
Full thickness lid shortening: In generalized ectropion
a wedge- or V-shaped full thickness area from the
lower lid with the broader side of the wedge at the
lid margin is excised. The site is selected at the
area where the lid is maximally everted. The lid is
closed in layers. Sometimes the medial canthus
tendon laxity also has to be corrected.
Kuhnt-Szymanowski procedure (Fig. 24.20): This is done
when there is severe ectropion with redundant
skin.
Method: The lid is split along the gray line from the
junc- tion of the medial and central one thirds to
the lateral can thus. At the lateral canthus a
triangular area is mapped by extending the incision
from the lateral canthus upwards
 Chapter Lids 367
24
and outwards for an appropriate length depending
on the excess skin. From the lateral canthus a
vertical incision downwards is made, which is twice
the length as the hori- zontal extension. The ends
of the two incisions are joined by a third incision
to complete the triangle. The skin and muscle in
this triangle is removed. A wedge flap with its
base at the lid margin is excised from the inner lid
flap. The outer split lid flap is pulled up and
outwards to cover the defect formed by the
excision of the triangle of skin and muscle, and
sutured (Figs 24.21A to C).
Cicatricial ectropion
Cicatricial contracture of the skin of the lids and
surround- ing area of the face will pull the lid
outwards and cause ectropion (Fig. 24.22A).
FIGURE 24.20: Diagrammatic representation of wedge resection

FIGURES 24.21A to C: Modified Kuhnt-Szymanowski procedure.

A.Lid is split along the gray line and a wedge-shaped place
is excised from the posterior lamella; B. The wedge is
sutured and a triangular flap of skin is removed at lateral
canthus; C. Skin edges are sutured (Courtesy: Stallard’s eye
surgery).
 Chapter Lids 368
24

Causes: Burns of the face, scarring from injuries, by excision of a full

congeni- tal ichthyosis, etc.
FIGURES 24.22A and B: Cicatricial ectropion.
Management: To correct the cicatricial ectropion all A. Cicatricial ectropion; B. After repair.
scar tissue has to be carefully excised to make the
lid freely mobile. A full thickness or partial thickness
skin graft may be used to cover the raw area
created by the excision of the scar tissue. A
temporary tarsorrhaphy may be done to prevent
contracture during the healing stage (Fig. 24.22B).
Paralytic ectropion
Paralytic ectropion is due to paralysis of the VII
nerve as in Bell’s palsy, tumors like acoustic
neuroma, parotid tu- mors, trauma, etc. Lack of
muscle tone will pull the lower lid away from the
globe due to the effect of gravity. There will be
associated lagophthalmos and brow ptosis (the
brow at a lower position compared to the normal
side).
Clinical features: The ectropion of the lower lid leads to
loss of contact of the lower punctum with the globe.
So the tears will not be able to drain into the lacrimal
sac when the orbi- cularis contracts and this will lead
to epiphora. Frequent wiping of the eye will lead to
aggravation of ectropion and epiphora. The
exposed conjunctiva will undergo kerati- nization
and will develop a raw beefy appearance. There
will be chronic conjunctivitis, which will aggravate
the epiphora. The skin of the lid becomes
eczematous due to constant wetting and this will
aggravate the ectropion, and the whole events
become a vicious cycle.
Management
In minimal degrees a lateral tarsorrhaphy will be
suffi- cient. In long standing cases with severe
ectropion and lid laxity a lid shortening procedure
 Chapter Lids 369
24
thickness wedge from the lid has also be done. lower lid to the eyeball.
The medial and lateral canthal tendons will have to
be strengthened by fascia lata suspension
anchoring to the periosteum of the orbital rim.
Mechanical ectropion
Mechanical ectropion is caused by tumors of the
lids, which mechanically evert the lid.
Management of the un- derlying cause will solve
the problem.

Entropion is a rolling of the lid margins. This will ENTROPION

lead to a misdirection of the lashes and they will
rub against the cornea—‘trichiasis’. This in turn
can lead to epithelial ero- sion and corneal ulcer.

Classification
Entropion can be:
1. Congenital.
2. Involutional or senile.
3. Cicatricial.

Congenital Entropion
Congenital entropion is a rare condition. The
cause is a vertical shortening of the posterior
lamella of lid. A curved fold of skin extends from
the medial canthus to lateral canthus. This fold
rides up and turns the lashes inwards. The lashes
in babies are fine and they rarely cause any
abrasion of the cornea. As the baby grows, this
entropion gets corrected spontaneously. If not,
surgery may be done. Management: The rare
condition often corrects itself. Sur- gery is done in
cases, which fail to get corrected spontane- ously
or corneal problems are produced.
Technique: A horizontally oval strip of skin and
orbicularis muscle is excised 3 mm below the lid
margins and extend- ing from the level of lower
punctum to the lateral canthus. The skin and
muscle are sutured with absorbable sutures.

Involutional (Senile) Entropion

Involutional entropion (Fig. 24.23) involves the
lower lid. The upper lid, which has a broader
tarsus, often remains in normal position. This
turning in of the lower lid leads to trichiasis (Fig.
24.24), corneal erosion, ulceration and
vascularization.
Causes
1. The horizontal lid laxity caused by
stretching of the tarsal plate and the canthal
tendons lead to weak ap- position of the
 Chapter Lids 370
24
‘the snap back test’, ‘modified Wheeler’s procedure’
or ‘modi- fied Fox procedure’ is done.

FIGURE 24.23: Involutional entropion

FIGURE 24.24: Trichiasis in entropion

2. Overriding of the preseptal portion of the

orbicularis over the pretarsal portion of the
orbicularis leads to inward turning of the lid
margin during lid closure.
3. Laxity of the lower lid retractors and short
postlamella of the lid allow the lower border of
tarsal plate to move away from eyeball.
Treatment
Jone’s procedure: This procedure is done for cases in
which horizontal lid laxity is minimal and weakness
or dehis- cence of the lower lid retractors is the
primary pathology. In this procedure the lower lid
retractors are tightened and reattached to the
lower border of the tarsal plate.
Technique: A horizontal skin incision is done 4 mm
below the lash line in the lower lid. The incision is
deepened and the orbicularis muscle is split to
expose the lower border of the tarsal plate and the
orbital septum below the tar- sal plate. The orbital
septum is cut transversely below the lower border of
the tarsal plate and the orbital septum and the
orbital fat beneath it are displaced downwards to
ex- pose the capsulopalpebral fascia. Three sutures
are passed through the lower border of the tarsal
plate and through the exposed capsulopalpebral
fascia and tightened. Then the skin and muscles are
sutured in layers. If there is re- dundant skin and
muscle, an elliptical area of skin and or- bicularis is
resected before suturing.
In eyes with horizontal lid laxity as revealed by
 Chapter Lids 371
24
Modified Fox procedure
The lower lid is split along the gray line to
anterior and posterior lamellae. A triangle of
tarsal plate and conjunc- tiva with its apex at the
lid margin and base at the lower border of the
tarsus is resected (Fig. 24.25).
A triangle of skin and orbicularis muscle is
excised temporal to the lateral canthus with its
base near the later- al canthus and slanting
upwards. The edge of the resected tarsus and
conjunctiva are sutured with absorbable su- tures.
Similarly the cut ends of the orbicularis and the
skin are also sutured.
Modified Wheeler’s procedure FIGURE 24.25: Modified Fox procedure
An incision is done along the whole length of
the lower lid just below the lid margin. A 3 mm
band of underlying orbicularis muscle is dissected
free from the skin and the tarsal plate and cut
vertically in the middle. From the ex- posed
tarsal plate a triangular piece with its apex toward
the lid margin is excised as in Fox procedure.
The tarsal plate is sutured. The cut ends of the
orbicularis are pulled over one another till the
lid is tightened and the over- lapped edges of
the muscle are sutured and a bite is taken from
the lower border of the tarsal plate before the
sutures are tightened. The skin is then closed with
interrupted silk sutures (Figs 24.26A and B).

Spastic Entropion
In this condition there is spasm of the orbicularis
muscle leading to the overriding of the preseptal
portion of the or- bicularis over the pretarsal
portion and thus results in roll- ing of the lower lid
margin. Any condition leading to severe
photophobia and corneal irritation can lead to
spastic en- tropion, especially in the elderly.
Causes can be corneal ulcers, postoperatively
following ocular surgery, etc. The condition may
revert back to normal when the cause for
photophobia subsides. It may result in
permanent
 Chapter Lids 372
24
entropion will per- sist. In this situation surgical
correction will be required.

FIGURES 24.26A and B: Modified Wheeler’s operation. A.

Tarsus and the strip of orbicularis cut; B. Cut ends of tarsus sutured
and the orbicularis overlapped and sutured (Courtesy: Stallard’s eye
surgery).

involutional entropion, especially if there is

associated weakness or dehiscence of
capsulopalpebral fascia (lower lid retractors).
Treatment
1. Lubricants to lessen irritation of the cornea by
the rubbing lashes.
2. Temporary measures:
a. Keep the lid in normal position by traction
on the skin by a strip of adhesive tape
stretching from the lower lid to the cheek.
b. Injection of botulinum toxin into orbicularis
muscle in severe cases—3 doses of 5 units
medially, central- ly and laterally. The effect
lasts for 6 months.
Once the cause for photophobia is corrected,
the lid may revert back to normal position. If there is
involution- ary changes in lower lid retractors, the
 Chapter Lids 373
24
Cicatricial Entropion
Causes: As follows:
1. Severe scarring and contracture of the
palpebral con- junctiva due to thermal or
chemical injury.
2. Cicatricial inflammations like trachoma, Stevens-
John- son (SJ) syndrome. FIGURE 24.27: Cicatricial entropion of upper lids in trachoma
The lid margin is pulled inwards and this
involves both upper and lower lids (Fig. 24.27).
Treatment: This can involve both lids and the
management depends on the severity of the
problem, and the extent of scarring and
adhesions on the conjunctival surface.
Mild cicatricial entropion
Skin and muscle operation is done for mild
entropion of the medial end of the upper eyelid.
An elliptical area of skin and orbicularis muscle is
excised from the upper lid 3–4 mm above the area
of localized entropion. Interrupt- ed sutures, which
pass through the underlying tarsal plate are used
to close the defect. This will evert the lid margin
and keep the lashes away from the cornea.
In moderate cases where the tarsus is not
thickened or deformed complete dissection and
excision of all scarred conjunctiva and
subconjunctival scar tissue is done and the raw
surface is covered with a conjunctival graft from
the other unaffected eye or a thin mucous
membrane graft or amniotic membrane graft in
bilateral cases.
In severe cases where the tarsus is deformed.
The sur- gical correction can be by:
1. Tarsal paring and eversion.
2. Tarsal rotation operation.
Tarsal rotation operation
In this operation, a strip of tarsus with the
conjunctiva is rotated outwards to form a new
intermarginal strip so that the lashes are directed
outwards away from the cornea (Fig. 24.28).
After putting three traction sutures at the
upper lid margin, the lid is everted and an incision
is made through the full thickness of the tarsus, 3
mm from the lid margin. This strip of tarsus is
freed from the orbicularis by careful dissection
and is rotated through 90°.
 Chapter Lids 374
24

Essential Blepharospasm
Essential blepharospasm is characterized by involuntary
spasm of the orbicularis and the facial muscles. Elderly
fe- males are more affected. In severe cases the
patient will be practically blind due to the continuous
spasms of orbicularis.
Treatment: Injection of botulinum toxin into both lids
and above eyebrow. Injections have to be
repeated.

BENIGN AND MALIGNANT

TUMORS OF THE LIDS

FIGURE 24.28: Tarsal rotation operation Benign Lesions

1. Cysts:
Three mattress sutures are passed through this
a. Chalazion.
strip of tarsal plate in this rotated position and they
b. Dermoid cysts.
are tied at the lid margin and these sutures ends
c. Cysts of Zeis’ glands.
are then brought out through the skin of the lid 5
d. Cysts of Moll.
mm from the lid margin and tied and cut.
e. Milia.
Tarsal paring and eversion f. Comedone.
An incision is made 3 mm above the lid margin 2. Benign epidermal tumors:
though the skin and orbicularis to expose the tarsal a. Squamous cell papilloma.
plate. With a sharp knife, the tarsus is pared down b. Seborrheic keratosis (basal cell papilloma).
along its entire length from its upper edge toward the c. Actinic keratosis.
incision. Three mattress sutures are then passed from 3. Benign pigmented tumors:
the upper lid margin and the orbicu- laris and then a. Nevi.
the pared tarsal plate to come out through the 4. Benign vascular tumors:
lower edge of the incision and the skin of the lids a. Capillary hemangioma.
just above lashes. On tying these sutures, the lid b. Cavernous hemangioma or port-wine stain.
margin is bent outwards (Fig. 24.29). 5. Miscellaneous benign lesions:
a. Pyogenic granuloma.
b. Xanthelasma.
c. Neurofibroma.

Malignant Tumors
1. Basal cell carcinoma.
2. Squamous cell carcinoma.
3. Keratoacanthoma.
4. Sebaceous gland carcinoma.
5. Melanoma.
6. Kaposi sarcoma.

Benign Lesions
Cysts
FIGURE 24.29: Sutures passed after pairing of the tarsal plate
 Chapter Lids 375
24
Chalazion: Commonest cystic lesion of the lids
arising from the meibomian glands (already
described).
 Chapter Lids 376
24

Dermoid cysts: Typically appear as congenital FIGURES 24.30A and B: A. Squamous cell papilloma; B.
subcutane- ous cystic lesions in the outer aspect of Benign wart at lid margin.
the upper lid. It may be attached to the periosteum
of the orbit. If cosmeti- cally disfiguring, the
lesions can be excised.
Cyst of Zeis’ glands: Small non-translucent cysts seen in
the anterior lid margin.
Cysts of Moll: They are small translucent cysts at the
ante- rior lid margin.
Milia: They are tiny white papules, often multiple,
seen on the skin of the lids. They are retention
epidermal cysts containing keratin.
Comedones: They are black heads seen in crops on the
skin of lids, in people with acne vulgaris or elderly
persons. They are dilated orifices of hair follicles
plugged with se- bum and keratin.

Benign Epidermal Tumors

Squamous cell papilloma: They appear as
pedunculated reddish growths or as a sessile lesion
(Figs 24. 30A and B). Treatment: Excision of the
growth.
Basal cell papilloma or seborrheic keratosis: They are
com- posed of proliferation of the basal cells of the

epidermis.
 Chapter Lids 377
24
They appear as brown plaques with a verrucous
surface and a ‘stuck-on’ appearance (Fig. 24.31),
usually seen in elderly persons. Similar lesions will
be seen on other parts of the face, neck and
upper limb.
Treatment: Simple excision and biopsy to rule out
basal cell carcinoma or melanoma.
Actinic keratosis: Commonly seen in elderly white
skinned persons living in tropical countries and
exposed to excessive sunlight. Appear as nodular or FIGURE 24.31: Seborrheic keratosis
wart like lesions with a scaly surface and may show
cracks and fissures. Similar lesions will be seen in
other parts of the body exposed to sunlight. There is
a risk of transformation into squamous cell
carcinoma.
Treatment: Simple excision and histopathological
exami- nation to rule out malignancy.

Benign Pigmented Tumors

Nevus
Nevus (Figs 24.32A to D) can be congenital or acquired.
Congenital nevus: It can be small or involve
extensive ar- eas of the body and contain hairs
(Fig. 24.32A). They can involve corresponding
areas of both upper and lower lids called kissing
or split nevus (Fig. 24.32B). The large le- sions
have a risk of developing malignant
transformation.
Treatment: Surgical excision of larger lesions with
skin grafting, if required.
Acquired nevus: They can have different clinical
features and histopathological features depending
on the level of involvement.
Junctional nevus: Appear as light brown macules or
papules. The nevus cells are located at the
junction of the epidermis and dermis. The risk of
malignant transformation is low.
Compound nevus: These are raised lesions with
uniform light brown to dark brown color (Fig.
24.32D). The nevus cells extent to the dermis
from the epidermis and the po- tential for
malignant transformation is low.
 Chapter Lids 378
24

FIGURES 24.32A to D: Nevus. A. Congenital nevus; B. Kissing or split nevus; C. Nevus of Ota with heterochromia iridum;
D. Compound nevus.

Intradermal nevus: This is the commonest type and congenital subcutaneous lesion, which consists of a well
usually seen in elderly persons. The nevus cells are demarcated
situated in the dermis and the lesions are
papillomatous with little or no pig- mentation. There is
no risk of transformation to malignancy.
Treatment: Excision, if it is disfiguring or suspicion of
ma- lignancy is aroused by increase in growth or
vascularity. Excision must be complete or there is risk
of recurrence.

Benign Vascular Tumors

Capillary hemangioma or strawberry nevus: They appear
soon after birth as raised bright red lesions on the
lids. It may extend to the skin of the face or into
the orbit. It may also be associated with
hemangiomas of internal organs. Large lesions on the
upper lid can cause mechanical pto- sis and block the
pupil and result in amblyopia. Capillary hemangiomas
blanch on pressure and this sign differenti- ates it
from cavernous hemangioma.
Cavernous hemangioma or port-wine stain: It is a
 Chapter Lids 379
24
patch, which consists of blood spaces of varying
caliber. Some of them are form part of the Sturge-
Weber syndrome.
Since they are subcutaneous lesions they
appear bluish in color. The color tends to
become darker with age due to spontaneous
atrophy and thickening of the overlying skin. In
course of time the overlying skin be- comes
thickened and nodular.
Cavernous hemangioma does not blanch with
pressure and this feature differentiated it from
capillary hemangioma.
Treatment
Capillary hemangioma: Small capillary
hemangiomas usu- ally disappear in 3–4 years. If
they are large and cause me- chanical ptosis, this
can lead to amblyopia and require treatment.
Treatment may be undertaken, if they are large
and disfiguring. Local injections of triamcinolone
plus be- tamethasone into the tumor may show
resolution. Large diffuse lesions can be treated
with systemic steroids. High doses for several
weeks to months may be required. Su- perficial
radiotherapy may also be useful.
 Chapter Lids 380
24

Cavernous hemangioma: Laser therapy, early in life Neurofibroma: Plexiform neurofibroma can involve
may help in decreasing the skin discoloration.
the lids along with the surrounding areas of the face
Photodynamic therapy can be tried in large
in people with neurofibromatosis type 1 (Figs 24.34A
lesions.
and B). The lid will be considerably swollen and has
a feeling of bag of worms due to the hypertrophied
Miscellaneous Benign Lesions nerves in the swelling. It can lead to mechanical
Pyogenic granuloma: It is a rapidly growing mass of ptosis and amblyopia, in addition to the gross
vascu- larized granulation tissue (Fig. 24.33). They disfigurement. The orbit may also be involved along
usually appear following surgery, trauma or infection, with the lids.
or sometimes with no specific cause. Treatment: Simple excision is difficult in diffuse lesions.
Clinically they appear as reddish polyps that
bleed readily following trivial trauma. Histologically,
they con- sist of granulation tissue containing many
Malignant Tumors
vascular chan- nels and inflammatory cells. Basal Cell Carcinoma
Treatment: Simple excision. Basal cell carcinoma (BCC) is the commonest
Xanthelasma: It is a common condition seen in malignancy in the lids.
middle aged or elderly persons, often associated
Risk factors: Caucasians are more affected. Exposure
with hypercho- lesterolemia. Yellowish raised plaque-
to sunlight for long periods increases the risk.
like lesions is seen on the medial aspects of the
lids, often bilateral and both lids are involved. Clinical features: The lower lid especially near the
Histologically, it consists of accumula- tion of lipid medial canthus is preferably affected. Usually
laden histiocytes in the dermis. starting as a small nodule it soon ulcerates and the
Treatment: Excision can be done, if the patient finds ulcer has a raised, rolled out indurated edges. The
these patches are disfiguring. CO2 or argon laser ulcer spreads slowly and invade the surrounding
therapy is also useful. Patients with xanthelasma structures including the orbit and bones, hence the
have to be investigated for high cholesterol levels. If name rodent ulcer (Fig. 24.35). The spread is lo- cal
there is hypercholesterol- emia, it has to be and the lymph nodes are not usually affected. Incom-
controlled. Otherwise xanthelasma will plete removal can lead to recurrence and the growth
recur after excision. tends to be more aggressive.
Histology: The malignancy arises from the basal
layer of the epidermis and proliferates as large
palisades into the dermis.
Clinical types:
1. Nodular type: Slow growing nodule with dilated
ves- sels over it.
2. Noduloulcerative type: This is the typical rodent ulcer.
3. Sclerosing type: It is rare and appears as an
indurated plaque and sometimes look like chronic
FIGURE 24.33: Pyogenic granuloma blepharitis.
 Chapter Lids 381
24

FIGURES 24.34A and B: Plexiform neurofibroma. A. Front view; B. Lateral view.

 Chapter Lids 382
24

2. Photodynamic therapy is emerging as a

treatment with reasonable efficacy and gives
good cosmesis for large or multiple SCC.
3. Radiotherapy or cryotherapy can also be tried.

Keratoacanthoma
Keratoacanthoma is a rare tumor found in fair-
skinned people exposed to sunlight for long periods.
Histologically it is considered to be a variant of SCC.
Clinically it appears as a rapidly growing pink
FIGURE 24.35: Basal cell hyperkeratotic lesion. It may show a period of
carcinoma regression after rapid growth for a few months.
Treatment During this period it may appear as a keratin filled
: crater.
1. Excision with normal surrounding area and show the characteristic ‘keratin pearls.’
recon- struction of the lids is the best Treatment: As follows:
treatment.
1. Wide excision complete excision is often difficult
2. It is sensitive to radiation also. Radiation is due to ill-defined margins of the lesion.
preferred for large tumors where surgical
reconstruction is dif- ficult or for recurrence. The
results of radiation treat- ment can be
misleading. It may show remission su-
perficially, but continue to spread deeply.
3. Exenteration is indicated for tumors involving
the sclera or extraocular muscles.

Squamous Cell Carcinoma

Squamous cell carcinoma (SCC) in this lids is less
common than BCC, but tends to be more aggressive
with spread to the preauricular or submandibular
lymph nodes. It may arise from preexisting ca-in
situ or actinic keratosis or arise without any pre-
existing conditions. SCC preferen- tially arises from
sites where the character of the epithelium shows a
transition. So, in the lids, the lid margins are prefer-
entially affected (Figs 24.36A to C).
Clinical features: Since it can arise from pre-existing
con- ditions or denovo it has no specific clinical
features. It can arise as a hyperkeratotic nodule or
as ulcerative type, which is difficult to differentiate
from BCC and the diag- nosis is often confirmed by
histopathological examina- tion. The rapid growth,
the surrounding feeder vessels and the involvement
of the regional lymph nodes favor a clini- cal
diagnosis of SCC.
Histology: It arises from the squamous cell layer of
the epi- dermis. It is composed of groups of
malignant epithelial cells with prominent nuclei
within the dermis. Well differ- entiated tumors will
 Chapter Lids 383
24

FIGURES 24.36A to C: Squamous cell carcinoma (SCC). A.

Ulcerative type on lid margin; B. Nodular type; C. After excision.
 Chapter Lids 384
24

Treatment: Complete excision, radiotherapy or

cryothera- py. Intralesional 5-fluorouracil can also
be tried.

Sebaceous Gland Carcinoma

Sebaceous gland carcinoma (SGC) the lids it usually
arises from the meibomian glands. It is more common
in the up- per lid. Rarely it can arise from the Zeis’
glands or seba- ceous glands in the caruncle:
1. In the ‘nodular type’ in the early stages it
appears as a chalazion (Figs 24.37A and B).
Unlike a simple chala- zion, which usually occurs
in children or young adults, this chalazion like FIGURES 24.37A and B: Meibomian carcinoma simulating a
tumor arises in elderly people—fe- males are chalazion; A. Appearance from skin side; B. Appearance on everting
more affected. Yellowish lobules of cells on I and the lid.
C or recurrence after I and C should arise
suspicion.
2. Rarely it can appear as a spreading SGC, which
may simulate chronic blepharitis.
3. Rarely a ‘pagetoid spread’ can occur where it
spreads into the fornices or bulbar conjunctiva
and may look like an inflammatory condition
(Figs 24.38A to C).
Treatment: Wide excision with 3–4 m normal
surrounding tissue and reconstruction of the lids. In
deeply spreading type, excenteration is indicated or

intralesional mitomycin
 Chapter Lids 385
24

FIGURES 24.38A to C: A. Meibomial carcinoma (nodular type);

B. After wide excision; C. After suturing.

can be tried. There is a high risk of recurrence

and 10% cases show lymph node
involvement.

Melanoma
Melanoma is a rare tumor and is less than 1% of
eyelid tu- mors. It can be variably pigmented. Half
the cases are non- pigmented and diagnosis is
arrived by histopathological examination (Fig.
24.39).
 Chapter Lids 386
24

Treatment: Cryotherapy on epilation.

Madarosis
Madarosis is an absence or decrease in lashes. It
can be congenital or acquired. Common causes for
acquired madarosis is burns, infiltrating lid tumors,
radiation treat- ment, lepromatous leprosy,
FIGURE 24.39: Melanoma of upper lid hypothyroidism, etc.

Any increase in size of a pigmented lesion, Poliosis

irregular margins, recent color change or presence
of multiple tu- mors should arouse the suspicion Poliosis is a premature localized whitening of hair,
of melanoma. which can involve the eyebrows and lashes also.
Treatment: Patient should be evaluated for any Vogt-Koyanagi- Harada syndrome, Waardenburg
systemic spread. Wide excision with local lymph syndrome, vitiligo and chronic anterior blepharitis
node removal is the best method of treatment. are the common causes.

Kaposi Sarcoma Trichomegaly

Kaposi sarcoma is a vascular tumor seen in patients Trichomegaly is an excessive eyelash growth. The
with acquired immunodeficiency syndrome (AIDS). com- mon cause is the use of topical prostaglandin
Sometimes it is the only clinical manifestations of HIV analogs like latanoprost, porphyria or AIDS and
infection. Clini- cally it appears as a reddish or rarely it can be familial.
purple lesion on the lids.
Treatment: Excision or radiotherapy. Trichiasis
Trichiasis is a posterior misdirection of the lashes
ABNORMALITIES OF THE where it can traumatize the cornea. This can lead to
EYELASHES epithelial ero- sions, ulceration, pannus formation and
Congenital Distichiasis opacification of the cornea.
Congenital distichiasis is a rare congenital The common causes are scarring of the lid margin
abnormality where the meibomian glands will form due to chronic blepharitis, trachoma, herpes zoster
a complete hair follicle containing unit and the ophthal- micus (HZO), SJ syndrome, etc.
person will have an extra row of lashes. These Treatment: Management options for trichiasis:
lashes are usually thinner and shorter and rarely
cause damage to the cornea. 1. Simple epilation with forceps—the lash will
grow again and irritate the cornea.
Treatment 2. Electroepilation: This is useful if the in turned
lashes are few. The electrolysis needle is
In cases where it is causing problem the extra row of introduced 2–3 mm along the root of the cilia
lashes can be destroyed by cryotherapy after under operating microscope and low current is
splitting the lid into anterior and posterior lamellae
passed to destroy the hair follicle
along the gray line or by application of
radiofrequency waves. permanently.
3. Cryosurgery of lashes and follicles.
Acquired Distichiasis 4. Radiofrequency ablation of lashes.
5. Argon laser ablation.
Differentiation of the meibomian glands to hair
6. Wedge resection of the segment of the lid.
follicles can occur in chemical injuries, SJ syndrome
and ocular cicatricial pemphigoid.
 Chapter Lids 387

2
24

Lacrimal
Apparatus

5
Girija Devi PS, Sahasranamam

of the lacrimal gland. It is made up of lobules,

ANATOMY resembling the structure of the parotid gland. It is
composed of secre- tory epithelial cells. The gland
The lacrimal apparatus (Fig. 25.1) of the eye consists
is divided into two parts— the superior orbital part
of es- sentially two parts:
and the inferior palpebral part. The ducts of the
1. Secretory system (tear production). lacrimal gland, which are about 10–12 in number,
2. Excretory system (drainage of tears). open in the lateral part of the superior fornix. The
glands secrete tears composed of water, electrolytes
Secretory System and lysozyme.
Secretory system consists of the lacrimal gland and its
ducts and the accessory lacrimal glands. The lacrimal Accessory Lacrimal Glands
glands start functioning fully only by about 6 weeks
Accessory lacrimal glands are small glands, which
after birth. So, the newborn infants do not
se- cretes tears and maintain the basal secretion of
produce tears, when crying.
tears. They are glands of Krause and Wolfring.

Excretory System Glands of Krause

Excretory system is composed of the lacrimal puncta, The glands are about 20 in number in the upper lid
the lacrimal canaliculi, the lacrimal sac and the and about 8–10 in the lower lid, situated within the
nasolacrimal duct (NLD) draining into the inferior stroma of the conjunctiva, mainly near the
meatus of the nose. fornix.

Lacrimal Glands Glands of Wolfring

Lacrimal glands are tubuloracemose glands The glands are few in number situated near the
situated at the upper and outer angle of the bony upper bor- der of tarsal plate.
orbit in the fossa
Lacrimal Puncta
Lacrimal puncta are two small round or oval openings
sit- uated on a small elevation called lacrimal
papilla, about 8 mm from the inner canthus on
each lid margin. Punc- tual region appears paler
than the surrounding area. The puncta are in line
with the openings of the meibomian glands. The
puncta are slightly inverted and opposed to the
lacrimal lake and are visible only if the lid is
everted.

Lacrimal Canaliculi
The upper and lower canaliculi in either lid are narrow
FIGURE 25.1: Lacrimal apparatus tubu- lar passages, which pass medially to enter the
lacrimal sac.
 Chapter 25 Lacrimal Apparatus 366

The two canaliculi may open separately in the secretomotor fibers, i.e. the greater superficial petrosal
lacrimal sac or may join to form a common nerve is derived from the facial nerve via the
canaliculus. The puncta and canaliculi are surrounded sphenopalatine ganglion.
by fibers of orbicularis oculi, the action of which on The arterial supply of the sac and duct is derived from
blinking helps in tear drainage. the superior and inferior palpebral branches of the
ophthalmic
Lacrimal Sac
The lacrimal sac is situated in the anterior part of
the me- dial wall of orbit, in the lacrimal fossa. The
lacrimal fossa is formed by the lacrimal bone and
the frontal process of the maxilla. The lacrimal sac
is about 15 mm in length and 5 mm width. The
portion of the sac above the open- ing of the
canaliculi is known as the fundus of the sac, the
portion below is called body of the sac. The sac
is con- tinuous with the NLD inferiorly.

Nasolacrimal Duct
Nasolacrimal duct is a membranous tubular structure
ap- proximately 15–20 mm long, extending from lower
part of the sac to the inferior meatus of the nose.
The direction of the NLD is downwards, posteriorly
and laterally. The direc- tion can be drawn as a line
joining the medial canthus of the eye to the first
upper molar. The valve of Hasner is a mucous
membrane fold, present at the lower end of the
nasolacri- mal duct. It prevents air from the nasal
cavity, entering the lacrimal sac during sneezing or
blowing of the nose.

Blood Supply of the Lacrimal

Gland
The arterial supply is by the lacrimal branch of the
oph- thalmic artery and infraorbital branch of the
maxillary ar- tery. The venous drainage is by the
lacrimal vein, which drains into the superior
ophthalmic vein.

Lymphatic Drainage
The lymphatics from the lacrimal gland join the
conjunc- tival and palpebral lymphatics and drain
to the preau- ricular nodes.

Nerve Supply
Sensory supply is through the lacrimal branch of the
oph- thalmic division of the trigeminal nerve,
sympathetic sup- ply is derived from the carotid
plexus from the cervical sympathetic and the
 Chapter 25 Lacrimal Apparatus 367
artery, the angular artery and infraorbital artery. infections like mumps or
Venous drainage is into the angular vein,
infraorbital veins and nasal veins.
Nerve supply to the lacrimal sac and NLD
comes from infratrochlear and anterior, superior
alveolar nerves.

Secretion of Tears
Normally, the rate of secretion of tears is such
that there is normal wetting of the ocular
surface. The basal secre- tion is mainly from the
accessory lacrimal glands. When a foreign body or
other irritant enters the eye, the secretion of tears
is greatly increased and the mucus in the tear film
coats the foreign body to prevent damage to the
ocular surface. Secretion of tears is also increased
in emotional states. The reflex secretion is from
the main lacrimal gland. Almost 25% of the tears
are lost by evaporation. The remaining 75% is
carried into the nasal cavity via the lac- rimal
drainage system. The lacrimal pump mechanism
(contraction of the orbicularis oculi pulls on the
lacrimal sac and draws tears into it) should
function normally for
drainage of tears into the nose.

Disease of the Lacrimal Gland

Common diseases of the lacrimal gland include:
1. Acute dacryoadenitis.
2. Dacryops.
3. Mikulicz’s syndrome.
4. Tumors.

Disease of the Lacrimal Passages

Common diseases of the lacrimal passages include:
1. Epiphora.
2. Congenital anomalies of the puncta and canaliculi.
3. Dacryocystitis—acute and chronic.

Acute dacryoadenitis is an acute inflammation of ACUTE DACRYOADENITIS

the lac- rimal gland affecting either the
palpebral or the orbital parts separately or more
commonly, the whole lacrimal gland is inflamed.
Incidence of dacryoadenitis is rare and
occurrence of abscess is still rarer.

Etiology
1. Acute dacryoadenitis is a rare condition
occurring in association with viral
 Chapter 25 Lacrimal Apparatus 368

influenza and also infectious mononucleosis, DACRYOPS

etc. sometimes leading to suppuration and
fistula for- mation. Dacryops is a cystic swelling of the lacrimal gland
2. Orbital cellulitis, erysipelas of the face, etc. may (pal- pebral lobe), due to retention of lacrimal
also lead to this disease. secretion due to
3. Primary acute dacryoadenitis may occur without
any obvious cause.

Symptoms
There is marked pain, redness and swelling in the
upper and outer part of the orbit along with
mechanical ptosis.

Signs
1. Palpation will reveal a tender swelling at the outer
part of the upper lid, spreading toward the temple
and cheeks.
2. There is congestion and chemosis of the
conjunctiva in upper and outer part with mucoid
or mucopuru- lent discharge.
3. Abduction of the affected eye will be painful
and sometimes restricted.
4. The preauricular glands may be enlarged and
tender.
5. On lifting the upper lid and looking down and
in, the inflamed gland can be visualized.

Complications
1. Suppuration can lead to abscess and fistula
forma- tion, if not promptly treated.
2. Rarely, the gland may undergo degeneration and
at- rophy, resulting in dry eye.

Differential Diagnosis
Acute dacryoadenitis should be differentiated from lid
ab- scess, hordeolum internum, hordeolum externum,
acute purulent conjunctivitis with chemosis and
orbital celluli- tis by the focus of inflammation on
the outer and upper quadrant of the orbit and
painful limitation of abduction of the eye.

Treatment
Treatment consists of systemic broad-spectrum
antibiotics and anti-inflammatory agents and hot
fomentation. Incision and drainage is done in cases of
lacrimal abscess formation.
 Chapter 25 Lacrimal Apparatus 369
blockage of one of the lacrimal ducts. It presents
as a non- tender, mobile and fluctuant swelling
in the upper lid on the lateral aspect.

Treatment
Treatment is excision through conjunctival approach.

Mikulicz’s syndrome presents with a classical MIKULICZ’S SYNDROME

clinical pic- ture with symmetrical enlargement of
the lacrimal and/ or salivary glands (parotid
glands), usually with lymphoid tissue hyperplasia.
The etiology is unknown, but it is seen in
uveoparotid inflammations (Fig. 25.2).

Benign Tumor TUMORS

The most common tumor is pleomorphic adenoma
[mixed tumor (Figs 25.3A to C)]. The tumors are
basically arising from the ducts of the gland. The
benign mixed tumor usu- ally occurs at around
30–50 years of age. It presents as a slowly
progressive painless palpable mass in the upper
lid. It may result in mechanical ptosis.

Treatment
Treatment is excision of the tumor.

Malignant Tumors
The malignant tumors can be:
1. Pleomorphic adenocarcinoma.
2. Adenoid cystic carcinoma.
3. Lymphoreticular tumors (bilateral as in
leukemias, Hodgkin’s disease and
lymphosarcomas).

FIGURE 25.2: Mikulicz’s syndrome

 Chapter 25 Lacrimal Apparatus 370

FIGURES 25.3A to C: Pleomorphic adenoma (mixed tumor). A. Photograph; B and C. Scanned pictures.

Treatmen Cause
t s
Treatment for adenocarcinomas is radical excision
Trachomatous canaliculitis occurs in areas where
fol- lowed by radiotherapy, if needed.
tra- choma is rampant. Infection by Actinomyces
Lymphoreticular tu- mors need systemic treatment
israelii, an- aerobic gram-positive bacteria is a
for the respective disease, local radiotherapy for the common cause in non- endemic areas.
lacrimal gland and if the size of the tumor is
large, it needs excision.
Clinical Features
EPIPHORA There will be chronic conjunctivitis with pouting
and in- flammation of the lacrimal puncta. The
Epiphora is excessive tearing of the eyes due to chronic conjunc- tivitis will not be responding to
imperfect drainage through the lacrimal drainage conventional treatment
system.
It may be due to lacrimal punctal abnormalities like
con- genital absence of puncta, punctal stenosis or
ectropion. It may be due to obstruction at the level of
canaliculus, lacrimal sac or NLD. It may occur in the
form of atresia (non-canaliza- tion) canaliculitis,
dacryocystitis, trauma, etc. Nasal patholo- gies may
also block the NLD.

Treatment
Treatment is directed toward the specific cause.
Excessive tearing due to hypersecretion of tears is
called lacrima- tion. The difference between
hypersecretion and defective drainage is given in
Table 25.1.
 like conjunctivitis, ectropion, eversion of the
puncta keratitis and uveitis,
acute congestive
glaucoma, etc. Obstruction of lacrimal drainage
Emotional states system
Reflex hypersecretion Punctal stenosis
Lacrimal Apparatus 371
Chapter 25 Canaliculitis
due to irritants,
foreign bodies, etc. Nasolacrimal duct obstruction
Sympathetic Lacrimal pump failure
CANALICULITIS stimulation elsewhere Lower lid laxity
Lower motor neuron (LMN)
Canaliculitis is an inflammation of the canaliculi and facial palsy
will be associated with epiphora (Fig. 25.4).
 Chapter 25 Lacrimal Apparatus 372

Congenital Dacryocystitis
(Dacryocystitis Neonatorum)
Inflammation of the lacrimal sac in the newborn
may present with an acute or chronic clinical
picture.

Etiology
There may be failure in canalization of the NLD or
its lu- men may be blocked by epithelial debris or a
membrane may be blocking the lower part of NLD.
It may be a bilat- eral or unilateral. Many
FIGURE 25.4: Canaliculitis with inflamed pouting punctum obstructions open spontaneously within 4–6 weeks
after birth.
of conjunctivitis. The pouting and inflamed
canaliculus will lead to the diagnosis. Pressure over Clinical Features
the canaliculus will express mucopurulent There is epiphora, usually evident from 2nd week
discharge and sometimes concretions. Aggregates of life. Normally, the tears are secreted 3–4 weeks
of the organism will produce granular after birth. There may be purulent discharge in
concretions. infected cases and may be mistaken for
conjunctivitis. Mucopurulent discharge and persistent
Treatment epiphora are two important signs of the disease.
There may be regurgitation of mucopurulent discharge
The treatment of Actinomyces canaliculitis on pressure over lacrimal (the sac area), which
consists of slitting the canaliculus, expressing out confirms that there is obstruction below the level of
the contents and topical antibiotics. The organism is the lacrimal sac.
sensitive to penicillin drops or levofloxacin drops.
Canaliculitis may be difficult to eradicate and the Treatment
pa- tient should be warned regarding the chronic
Conservative treatment is indicated in early cases.
nature of the disease and treatment over multiple This includes observation, lacrimal sac massage and
sittings. topical antibiotics.
Massage over the lacrimal sac area and clean
DACRYOCYSTITIS the discharge as and when it occurs. Educate the
mother/ parents regarding the illness and teach
Dacryocystitis is the inflammation of the lacrimal the mother to apply pressure over the lacrimal
sac. It may be congenital or acquired. sac area by the thumb. Then bring the thumb
downward, pressing toward the ala of the nose
(Figs 22.5A and B). This is repeated many
 Chapter 25 Lacrimal Apparatus 373

FIGURES 25.5A and B: Direction of massaging over the lacrimal sac

 Chapter 25 Lacrimal Apparatus 374

times daily. Massage helps to increase the pressure of canaliculi and the duct, as it may cause fibrosis
inside the sac and helps to open up the or peri- canalicular inflammation (Fig. 25.6).
membranous occlu- sions in many cases. But
make sure that it is done prop- erly. Broad-
spectrum antibiotic eyedrops are instilled Procedure
frequently after expressing out the contents of the The procedure is done under general anesthesia.
sac, by pressure over the sac area. 80%–90% cases The upper canaliculus is usually probed. This is to
may be cured by this treatment. Conservative avoid damage to the lower punctum and
treatment may be contin- ued up to about 1 year canaliculus, which is more important in drainage of
of age in many cases. tears. The punctum and canaliculus are dilated
Diligent medical management for 3–4 months is with a Nettleship dilator. A small probe No. 1 or 2 is
given deciding on surgical treatment. Diligent medical inserted vertically downwards into the canaliculus
manage- ment cures the problem in majority of for 2 mm. It is gently, but firmly passed inwards
cases. until bony stop of lacrimal fossa is felt. The probe
Aim: To open up or recanalize the NLD. If is then rotated toward the midline and pushed
conservative methods fail, we have to resort to down the NLD, till it reaches the floor of the nose.
surgical modalities. This includes: The whole procedure will take only a few minutes
and if per- formed in the right way, will cure many
1. Probing of the NLD.
cases of con- genital dacryocystitis.
2. If repeated probings fail, intubation with
silicone stent (e.g. Crawford stent) may be
performed. The sili- cone tubing may have to be Complications
kept in the NLD for about 6 months. Failure to open the NLD, creation of a false
3. Balloon dilatation of the duct (balloon passage and spread of infection to surrounding
dacryoplasty) is another modality of surgical tissues leads to orbital cellulitis.
intervention, which can be adopted. Since the
equipment and procedure is expensive, it is
limited to complicated cases. ACUTE DACRYOCYSTITIS
4. Dacryocystorhinostomy (DCR) may have to
be done when the other measures fail and the Acute dacryocystitis is an acute suppurative
child is 3–4 years old. inflammation of the lacrimal sac.

Probing of Nasolacrimal Duct Etiology

If there is persistent epiphora and discharge after 3 Acute dacryocystitis can occur as an acute
months of conservative treatment, probing of the NLD exacerbation of a chronic dacryocystitis or it may
may have to be performed. Ideally it should be done
occur without previous history of watering from
within the 1st year of life, before permanent structural
the eye (Fig. 25.7).
damage of the canalicu- lus occurs. Care should be
taken to avoid injury to the walls
 Chapter 25 Lacrimal Apparatus 375

FIGURE 25.6: Probing of nasolacrimal duct FIGURE 25.7: Acute dacryocystitis

 Chapter 25 Lacrimal Apparatus 376

Clinical Features rhinospo- ridiosis, which may be seen in people who

take bath in stagnant water, like ponds.
The patient presents with an acute onset of swelling, The disease is more common in females, probably
red- ness, pain and tenderness of the skin over the due to narrow bony canal in females.
lacrimal sac area. No regurgitation may be present,
as the canaliculi are blocked due to edema.
Conjunctival congestion, muco- purulent discharge
from the eye and submaxillary lymph node
enlargement may be present. Fluctuant swelling
may be present on palpation over the sac area, when
there is abscess formation. But this may be difficult
to elicit, due to severe tenderness.

Complications
1. Lacrimal fistula may result, if the abscess bursts
open or if it is incised repeatedly.
2. Osteomyelitis of the lacrimal bone.
3. Orbital cellulitis.
4. Cavernous sinus thrombosis (not very
common).

Treatment
1. Hot compresses, systemic broad-spectrum
antibiotics, analgesics and anti-inflammatory drugs
are effective.
2. In case of lacrimal abscess, a vertical incision
can be made over the sac area in the lower
part.
3. In case of lacrimal fistula, fistulectomy and
removal of lacrimal sac is done.
4. Once the acute inflammation is over, DCT or
DCR is done to prevent recurrence.

CHRONIC DACRYOCYSTITIS

Chronic dacryocystitis is a chronic suppurative

inflamma- tion of the lacrimal sac that usually results
from obstruc- tion of the NLD.

Etiology
Chronic dacryocystitis is usually due to stricture of
the NLD as a result of chronic inflammation of the
nasal mu- cosa or obstruction by nasal polyps,
grossly deviated nasal septum or a hypertrophied
inferior turbinate bone. The stagnant tears in the
lacrimal sac may get infected by pyo- genic
bacteria.
Another cause for chronic dacryocystitis is
 Chapter 25 Lacrimal Apparatus 377
Clinical Features
The disease may present with chronic epiphora or
muco- purulent discharge from the eye. It may
also present as a swelling (mucocele) of the
lacrimal sac. Regurgitation of mucopurulent fluid
by pressure over the sac area may be present.
If the common canaliculus is also blocked, a
firm cys- tic swelling will be seen in the
lacrimal area (encysted mucocele) and there
will be no regurgitation on pressure over the
sac.

Complications
1. Non-healing corneal ulcer or hypopyon corneal
ulcer may occur due to spread of infection
from the sac to a corneal abrasion.
2. Postoperative endophthalmitis after
intraocular surgery.
3. Lacrimal abscess may occur as a result of
infection by pyogenic organisms.
4. A lacrimal fistula (Fig. 25.8) may form
following spon- taneous bursting of the
lacrimal abscess or following incision and
drainage of a lacrimal abscess.

Investigations
1. A thorough nasal examination by an ENT
surgeon is important to exclude deviation of
septum, polyps, any abnormal mucosal
growth and atrophic rhinitis.
2. Radiological examination to visualize the
lacrimal pas- sage though not commonly done
include the following:
a. Dacryocystography is done using Lipiodol,
Uro- grafin, etc. which outlines the lacrimal
drainage system. X-ray films are taken to
find out the size of the sac and site of
obstruction.

FIGURE 25.8: Lacrimal fistula

 Chapter 25 Lacrimal Apparatus 378

b. Subtraction macrodacryocystography with is split along the line of incision. The lacrimal fascia is
cana- licular catheterization gives better ex- posed and incised. Lacrimal sac is freed by blunt
results. dissec- tion from the surrounding tissues and drawn
c. Lacrimal scintigraphy with radioactive forwards. The sac may be excised close to the NLD.
techne- tium-99 (99Tc) instilled into the eye The upper end of the NLD may be curetted. Orbicularis
as a drop, can be traced with a gamma muscle is sutured
camera.
These tests are now done infrequently because of
bet- ter methods of evaluation including endonasal
evalua- tion, computed tomography (CT) scan,
magnetic reso- nance imaging (MRI), etc.

Treatment
Surgery is the treatment of choice and includes:
• Dacryocystectomy (DCT)
• Dacryocystorhinostomy (DCR)
• Canalization with special tubes like the Lester
Jones tube.

Dacryocystectomy
A complete excision of the lacrimal sac is done in
this pro- cedure.
Indications: This is not the surgery of choice, but may
be done in cases of an atrophic, fibrous sac,
atrophic rhini- tis, etc. It is preferred in elderly
patients who may be hav- ing associated dry eye
also.
Advantage
1. Dacryocystectomy is the ideal surgery in
rhinosporid- iosis, since DCR may result in the
spread of infection to the nasal cavity.
2. It is the surgery indicated in tumors of the sac
and specific infections of the sac-like TB.
Disadvantage
1. The disadvantage of this surgery is that the
natural lacrimal drainage system is
abolished.
2. Epiphora may persist though it may become
less- er. But recurrent sac infections and the
danger of a source of infection close to the
eye is eliminated.
Method
The lacrimal sac and the area surrounding it are
anesthe- tized by an injection of 2% Xylocaine with
adrenaline. A curvilinear incision is made starting 2
mm above the me- dial palpebral ligament, 3 mm
medial to the medial can- thus and 4 mm
downwards and outwards. It coincides with the
anterior lacrimal crest (Fig. 25.9). Orbicularis muscle
 Chapter 25 Lacrimal Apparatus 379
with catgut and the skin is sutured with continuous
subcu- ticular sutures after attaining hemostasis
(Fig. 25.10).

Dacryocystorhinostomy
The usual DCR done is a traditional external DCR
(trans- cutaneous). The surgery is usually done
under local anes- thesia, especially in adults.
General anesthesia or moni- tored sedation may
be used in some cases.
In this surgery, an anastomosis is made
between the medial wall of the lacrimal sac and
the nasal mucosa in the middle meatus. This is
done after making an opening in the bone, which
forms the floor of the lacrimal sac.

FIGURE 25.9: Lacrimal sac in the lacrimal fossa under the

medial palpebral ligament

FIGURE 25.10: External dacryocystectomy in progress

 Chapter 25 Lacrimal Apparatus 380

Procedure Disadvantages of external DCR

1. The nasal cavity of the same side is packed with
1. More hemorrhages intraoperatively.
gauze soaked in Xylocaine and adrenaline. Local
anesthe- sia with 2% Xylocaine and adrenaline is 2. Occasional incision scar.
used for local skin infiltration. 3. Interference with lacrimal pump function because
2. The canaliculi are dilated and lacrimal sac is of medial canthal changes.
irrigated with saline. Modification to conventional DCR includes:
3. The initial steps are same as for DCT. transcana- licular endolaser DCR, endonasal DCR,
4. The lacrimal sac is carefully separated from the etc.
lacri- mal fossa and displaced laterally to expose
the bone. Transcanalicular endolaser DCR
5. The periosteum over the lacrimal crest is incised The procedure is performed under general
and lacrimal bone is exposed. anesthesia. The nasal mucosa is anesthetized and
6. The bony crest is removed with a bone gouge and an endoscope is used for examination of the
ham- mer and an opening, roughly 1 cm diameter, lacrimal pathways. The site of osteotomy is
is created in the lacrimal fossa extending from the determined with transillumination of the lateral
anterior to the posterior crest and downwards up nasal wall. By applying laser energy via an optic
to the opening of the NLD. The underlying fiber passed through the canaliculi, an opening is
nasal mucosa is exposed. created from the sac into the nasal mucosa. A
7. A vertical incision is put in the exposed nasal bicanalic- ular silicone stent can be inserted as
mucosa to create an anterior and posterior flap. the last step. Suc- cess of procedure is absence of
Similarly, the sac is also slit vertically to epiphora (subjective) or patency of the lacrimal
produce two flaps. drainage system on irrigation (objective).
8. The nasal mucosa of the middle meatus is
anasto- mosed with the sac by suturing the Endonasal DCR
anterior flap of the sac to the anterior flap of This is performed under local or general
the nasal mucosa. The pos- terior flap of the anesthesia by direct visualization of the nasal
mucosa of the lacrimal sac and nasal mucosa mucosa using endo- scope. Lignocaine with
are also sutured together. adrenaline is injected under the nasal mucosa at
9. Syringing is done to test the patency of the the proposed site of osteotomy. The en- doscope
passage. gives magnification as well as illumination. The
Complications opening into the lacrimal sac from the nasal
1. Hemorrhage from the nasal mucosa, which is mucosa is made manually or with laser. Mainly the
usually very vascular is a common thin lacrimal bone is removed in endonasal laser
complication. To prevent this, nasal packing DCR and no sutures are put.
may be maintained for a day.
Advantages of an endonasal DCR (internal DCR):
2. Failed DCR may occur following an improper
bony ostium, improper suturing of the flaps or • Lack of visible scar
persistent nasal pathology like a polyp. • Shorter recovery time
• Less discomfort.
Advantages of external DCR
For this surgery either the ophthalmologist
1. The lacrimal sac is fully exposed and intrasac
should have special training in endonasal surgery or
pathol- ogy identified.
he may take help of an ENT surgeon.
2. The rhinostomy is large (at least 10 mm) and
the mu- cosal flaps are sutured.
3. Success rate is higher compared to newer
techniques.
Orb
it
2
6
Girija Devi PS, Sija S

obvious source of inoculation is noted.

ORBIT
Orbital cavity is a small area containing many
important blood vessels and nerves in addition to
eyeball and any pathological process here has
serious consequences.

Infectious Inflammatory
Disorders
Infections can be of various types depending on the
tissue involved. It can be classified as:
1. Orbital cellulitis:
a. Preseptal cellulitis.
b. True Orbital cellulitis.
2. Dacryoadenitis.
3. Subperiosteal abscess.
4. Tenonitis.

BACTERIAL
INFECTIONS OF
THE ORBIT
Orbital Cellulitis
The most common cause of cellulitis is bacterial
infections of the orbit or periorbital soft tissues
(Fig. 26.1). It occurs from three primary sources:
1. Direct spread from an adjacent sinusitis or
surround- ing structures.
2. Direct inoculation following trauma or skin
infection.
3. Bacteremic spread from a distant focus (otitis
media, pneumonia).
Although periorbital infections are typically
classified as being either preseptal or orbital
cellulitis, they often represent a continuum with
common underlying cause, requiring similar
treatment regimens. It must be empha- sized that
infectious cellulitis, whether preseptal or orbit- al, is
most commonly caused by underlying sinusitis, if no
Preseptal Cellulitis
Infectious preseptal cellulitis is defined as
inflammation and infection confined to the eyelids
and periorbital struc- tures, anterior to the orbital
septum. The orbital structures posterior to the
septum are not infected, but may be sec-
ondarily inflamed.
Clinical features
Eyelid edema, erythema and inflammation may
be severe. Swollen closed lids will be tender and
indurated and proper examination of the eye will
be difficult. Usually the globe is uninvolved, but
the swollen closed lids give an apparent ap-
pearance of proptosis. On examining the eyes,
usually with the help of lid retractors, eyeball will
appear normal. Pupil- lary reaction, visual acuity
and ocular motility are not dis- turbed. Pain on eye
movements and chemosis are absent. If an abscess
forms, pus will be pointing at some part of the lids.
Causes
Although preseptal cellulitis in adults is usually
due to penetrating trauma or a cutaneous source
of infection like hordeolum internum or externum,
in children, the most common cause is
underlying sinusitis.
Management
Computed tomography (CT) evaluation of orbit
and si- nuses is essential, if eyelid swelling is
profound enough to

FIGURE 26.1: Orbital cellulitis

 Chapter 26 Orbit 375

preclude examination of the globe and thereby 4. Restriction of ocular movements and pain with
excluding orbital cellulitis is difficult. The patient ocular motility.
should be treated in consultation with primary
care physician.
Oral antibiotics and nasal decongestants, in cases
of associated sinusitis are typically effective therapy.
Hospi- talization and intravenous antibiotics are
indicated if the cellulitis progresses despite
outpatient therapy, as cases of preseptal cellulitis
can progress to orbital cellulitis.
In teenagers and adults, preseptal cellulitis
usually arises from a superficial source (e.g.
traumatic inocula- tion, infected chalazion or
epidermal inclusion cyst), and responds quickly to
appropriate oral antibiotics and warm compresses.
Surgical drainage may be necessary, if preseptal
cel- lulitis progresses to a localized abscess.
Incision and drainage can usually be performed
directly over the ab- scess, but care should be
taken to avoid damage to leva- tor aponeurosis. To
avoid contaminating the orbital soft tissues, the
surgeon should not open the orbital septum.
Complications
• Lid abscess
• Orbital cellulitis
• Cavernous sinus thrombosis.

True Orbital Cellulitis

In infectious orbital cellulitis, disease is present
posterior to the orbital septum.
Etiology
In more than 90% of cases, orbital cellulitis occurs
as a secondary extension of infection from
surrounding structures:
1. Acute or chronic bacterial sinusitis is the
common source of infection.
2. In children, it can be from the teeth.
3. Other sources can be deep injuries of orbit
including surgeries like retinal detachment (RD)
or squint sur- gery.
4. Spread of infection can be from the eye
itself as in panophthalmitis or perforated
corneal ulcers.
5. Infection can come from distant foci by
septicemia or bacteremia.
Clinical features
1. General symptoms like fever and malaise.
2. Lid edema with tense indurated lids and marked
che- mosis.
3. Proptosis, which is usually axial.
 Chapter 26 Orbit 376
Vision will be normal in the early stages, but
decrease in visual acuity, color vision and visual
field abnormalities as well as pupillary
abnormalities suggest compressive optic
neuropathy, demanding immediate investigation
and aggressive management.
A significant percentage of adult cases of
orbital cel- lulitis proceed to abscess formation,
which may present as progressive proptosis or
globe displacement. Abscesses usually localize in
the subperiosteal space, adjacent to the infected
sinus, but may extend through the periosteum
into the orbital soft tissues. Such abscesses should
be sus- pected, if patients on IV antibiotics do
not show daily im- provement (Fig. 26.2).
Management
Early diagnosis and aggressive management is required.
Investigations
A CT scan of the head and orbit is mandatory to
localize the source of infection as well as to look
for any abscess formation and complications like
cavernous sinus throm- bosis.
Treatment
IV broad-spectrum antibiotics
Patient should be hospitalized and intravenous
broad- spectrum antibiotics should be started.
Antibiotic therapy should provide broad-spectrum
coverage because, infec- tion in adults usually
includes multiple organisms that may include
gram-positive cocci, Haemophilus influen-
zae, Moraxella catarrhalis and anaerobes.
Surgical management
Early surgical intervention to drain the involved
sinus is usually indicated, especially if orbital
findings progress during IV antibiotic therapy. In
contrast, orbital cellulitis in children is more often
caused by a single gram-positive organism and is
less likely to require surgical drainage of the
infected sinus.
Computed tomography scan will help to
identify and localize the abscess. Pus obtained
can be sent for

FIGURE 26.2: Orbital cellulitis with abscess formation

below the eyeball
 Chapter 26 Orbit 377

microbiological studies and the antibiotics can be tropical countries and in areas where these infestations
suitably modified. are com- mon, any case of proptosis or orbital
Not all subperiosteal abscesses require surgical inflammation has to be investigated by
drain- age. Isolated medial or inferior subperiosteal ultrasonography (US) and CT scan to exclude their
orbital ab- scesses in children younger than age 9 presence.
with underlying isolated ethmoid sinusitis, intact
vision and moderate proptosis typically respond
to medical therapy.
Surgical drainage coupled with appropriate
antibiotic therapy is recommended in older patients
or more severe presentation and usually leads to
dramatic clinical im- provement within 24–48 hours.
Concomitant sinus sur- gery is indicated, if
sinusitis is present.
Delay in treatment may result in blindness,
cavernous sinus thrombosis, cranial neuropathy,
brain abscess and death.
Complications
1. Cavernous sinus thrombosis: Orbital infections
rarely spread posteriorly to the cavernous sinus.
Cavernous sinus thrombosis is heralded by the
rapid progression of proptosis and by
anesthesia in both the first and second divisions
of the trigeminal nerve. There will be worsening
of the general condition and lateral rectus palsy
may develop in the other eye also.
2. Meningitis and brain abscess may develop.
3. Optic nerve compression, spread of infection
to the optic nerve, central retinal artery CRA
or vein oc- clusion can lead to rapid drop in
vision and even blindness.
4. Spread inside the eye can lead to
panophthalmitis or endophthalmitis.
5. Bacteremia and septicemia can develop
leading to distant infections.

FUNGAL INFECTIONS OF ORBIT

Mucormycosis and aspergillosis can cause rapidly
pro- gressing and fatal infection of the orbit and
should be managed on an emergency basis (refer
Section ‘Systemic Diseases and Eye’).

PARASITIC INFECTIONS OF
ORBIT
The common parasitic infestations of the orbit
are:
• Trichinosis
• Cysticercosis
• Echinococcosis or hydatid cyst.
Parasitic infestations are more common in
 Chapter 26 Orbit 378
Trichinosis times daily for 8–15 day) combined with systemic
steroids is also effective. Before starting medi- cal
Trichinosis is caused by the round worms of the therapy a detailed fundus evaluation to rule
species Trichinella spiralis. Eating out
undercooked pork meat or sausages containing
the trichinosis larvae lead to human infestation.

Clinical Features
Constitutional symptoms like fever and headache,
gastro- intestinal (GI) symptoms like abdominal
cramps, diarrhea and vomiting, joint and muscle
pains will be present. Lar- val encystment can
occur in the lids, conjunctiva or ocular muscles
leading to lid edema, proptosis, pain and diplopia
on ocular movements. Rupture of the cysts can
produce severe inflammatory symptoms.

Treatment
Systemic steroids combined with albendazole 25
mg/day or mebendazole 200–400 mg/day for 10
days may be effec- tive. Steroids will control the
inflammation, but elimina- tion of the adult worm
is difficult and larvae will continue to be
produced resulting in recurrence of symptoms.

Cysticercosis
Cysticercosis is an infestation by the pork
tapeworm, Tae- nia solium. Human beings are
the carriers and the infesta- tion is spread
through water and food contaminated with the
eggs passed in the feces of an infected person.
The eggs hatch once they reach the stomach
and the larvae can reach any part of the body
via bloodstream and lead to cyst formation. One
or more cysts may form in the con- junctiva,
ocular muscles, vitreous or retina. It can lead to
proptosis, limitation of ocular movements and
diplopia. Death and rupture of the cysts can lead
to severe inflam- matory reaction.

Management
Orbital US and CT scans are essential to confirm
the diag- nosis. The cystic lesion with the central
hyperechoic spot formed by the scolex is
diagnostic.

Treatment
Removal of the cyst by orbitotomy is the
treatment of choice. Cysticidal therapy with
praziquantel (50 mg/ day, three times daily for
14–30 day) or albendazole (15 mg/day, three
 Chapter 26 Orbit 379

intraocular cysts and a central nervous system 1. The inflammation can primarily affect the
(CNS) evaluation to rule out intracranial cysts is lacrimal gland (dacryoadenitis).
essential, since death of the cysts can lead to
2. One or more extraocular muscles (myositis).
severe intraocular inflammation and loss of
3. The sclera and posterior tenons (scleritis).
vision.
4. The optic nerve sheath (inflammatory optic neuritis).
5. It can be restricted to the superior orbital fissure
Echinococcosis or Hydatid Cysts and cavernous sinus (Tolosa-Hunt
Hydatid cysts is the cyst formed by the larvae syndrome).
of Echi- nococcus granulosus, which lives in the Sx Symptoms
intestine of cats and dogs.
Hydatid cysts can form in the orbit leading to • Deep-rooted boring pain
progres- sive proptosis. • Visual acuity may be impaired
• Conjunctival inflammation and eyelid erythema
Management • Orbital imaging may confirm the diagnosis.
Diagnosis is confirmed by CT and US scan and by
En- zyme-linked immunosorbent assay (ELISA) test Signs and Symptoms
for Echi- nococcus antibodies.
Depend on the involved tissue, however deep-rooted
Treatment bor- ing pain is a typical feature of the process.
Extraocular movement restriction, proptosis,
Treatment is excision of the cyst without rupturing conjunctival inflamma- tion and chemosis are common
its wall. Rupture of the cyst can lead to severe and eyelid erythema and soft tissue swelling may be
inflammation. present. Visual acuity may be impaired, if the optic
nerve or posterior sclera is involved. On the whole,
NON-SPECIFIC there will be some signs of inflamma- tion, but not
ORBITAL INFLAMMATION so acute or severe as in orbital cellulitis, but also
some features of a tumor, clinically or radiologically
(ORBITAL PSEUDOTUMOR)
evident mass-like lesion, but biopsy or fine-needle
aspi- ration cytology (FNAC) shows some non-specific
Non-specific orbital inflammation (NSOI) also known
cellular
as orbital pseudotumor, is currently defined as an
reaction.
idiopathic tumor like inflammation consisting of a
pleomorphic cel- lular response and a fibrovascular A typical clinical presentation is often diagnostic
tissue reaction. NSOI is usually confined to the orbit, and orbital imaging may confirm the diagnosis. A
but may extend to the sinus- es and the intracranial thorough systemic evaluation should be undertaken,
space. It has a variable, but gener- ally self-limited if there is any uncertainty regarding the diagnosis.
course. Both children and adults may be affected Not all patients with NSOI present with the classic
(Figs 26.3A and B). signs and symptoms. There may be a typical pain,
The subclassification of NSOI is made on the limited inflammatory signs or a fi- brotic
basis of the anatomical target: presentation termed sclerosing NSOI. Such lesions
more commonly require biopsy for diagnosis.
 Chapter 26 Orbit 380

FIGURES 26.3A and B: Orbital pseudotumor. A. Clinical picture; B. CT scan showing thickening of muscles including tendon.
 Chapter 26 Orbit 381

Investigations optic nerve compression is progressive.

Tumor necrosis factor alpha (TNF- blockers have
Biopsy is not necessary in all cases because the been used in recurrent cases or for those patients in
findings on clinical, ultrasonographic and radiologic whom corticosteroids are contraindicated.
examination may be sufficiently diagnostic to institute
therapy. Biopsy is reserved for cases that fail to
respond rapidly to corti- costeroids or for those with
recurrence, after treatment is discontinued.
Simultaneous bilateral idiopathic inflam- mation in
adults suggests the possibility of systemic vas-
culitis. In children, however approximately one
third of cases of NSOI are bilateral and are rarely
associated with systemic disorders.
Histopathologically: NSOI is characterized by a
pleomor- phic cellular infiltrate consisting of
lymphocytes, plasma cells and eosinophils with
variable degrees of reactive fi- brosis. The
sclerosing type demonstrates a predominance of
fibrosis with sparse inflammation.
Computed tomography scan: In dacryoadenitis, CT
scan reveals diffuse enlargement of the lacrimal
gland. In myo- sitis, the extraocular muscle tendons
of insertion may be thickened in up to 50% of
patients with NSOI in contrast, thyroid-associated eye
disease (TED) typically spares the muscle
insertions.
An inflammatory infiltrate of the retrobulbar fat
pad is commonly seen and contrast enhancement of
the sclera may be caused by tenonitis (producing
the ring sign).
B-scan ultrasonography often shows an acoustically
hol- low area corresponding to an edematous
Tenon’s capsule.

Treatment
Initial therapy consists of systemic corticosteroids.
Acute cases generally respond rapidly with an abrupt
resolution of the associated pain. The use of steroids
can be tapered as soon as the clinical response is
complete, but this ta- pering should be done slowly.
Rapid reduction of systemic steroids may cause a
recurrence of inflammatory symp- toms and signs.
An incomplete therapeutic response or recurrent
dis- ease suggests the need for orbital biopsy, which
can pro- vide histopathologic confirmation and
exclude specific inflammatory diseases.
After the diagnosis is confirmed, orbital
irradiation, antimetabolites or alkylating agents such
as methotrexate or cyclophosphamide and continued
steroid therapy may be useful for controlling the
disease.
Rarely orbital decompression is necessary, if
 Chapter 26 Orbit 382
Sclerosing NSOI is a distinct subset of disease 3. The visual acuity and fields were usually
with pre- dominant fibrosis and minimal cellular normal al- though the optic nerve might be
inflammation. It re- sponds poorly to steroids and to affected.
low-dose radiotherapy and typically requires more 4. The pupils are relatively spared.
aggressive immunosuppression with cyclosporine,
methotrexate or cyclophosphamide.

Cavernous Sinus Thrombosis

The cavernous sinus has several tributaries
connecting it to the eyes, ear, face, sinuses and
most part of the cere- brum. Infection in any of
these areas can reach cavernous sinus and
produce inflammation and thrombosis. Staphy-
lococcus aureus is the causative organism in
70% of cases.

Clinical Features
Cavernous sinus thrombosis is closely simulates an
orbital cellulitis, but the constitutional symptoms
are much more severe. There will be high fever,
headache and cerebral symptoms. The second
eye will also be involved in 50% of cases and a
lateral rectus paresis will be the earliest sign. A
swelling behind the ear due to involvement of the
mastoid emissary vein is another early sign.
Unlike in case of orbital cellulitis there will be
severe neuralgic supraorbital pain due to
involvement of the ophthalmic division of the
trigeminal. Disk edema with engorgement of
the retinal veins may be present.

Investigation
Computed tomography scan will confirm the diagnosis.

Treatment
Aggressive management is required to prevent
loss of vi- sion as well as life. Broad-spectrum
antibiotics and anti- coagulants should be
administered. Though the signs and symptoms are
mainly in the eye, it is primarily an intracra- nial
problem, and it has to be managed by the
neurologist and the ophthalmologist together.

Tolosa-Hunt Syndrome
The syndrome is a non-specific inflammatory
process with granulation tissue in the region of
superior orbital fissure. It is characterized by:
1. Recurrent unilateral retro-orbital pain.
2. Palsies of the extrinsic ocular muscles
involving the III, IV and VI cranial nerves, the
sympathetic and the V nerve.
 Chapter 26 Orbit 383

Males and females are equally affected. It is • Pulsatile or non-pulsatile

more common in the 5th decade. The symptoms • Static, intermittent or progressive.
last for some days or weeks with spontaneous
remissions lasting some months or years.
Pseudoproptosis
Treatment Pseudoproptosis is an apparent appearance of proptosis.

Dramatic response to systemic steroid therapy within

Causes
a very short time is seen, which is another important
char- acteristic. 1. Severe enlargement of one eye alone as in
unilateral high myopia or buphthalmos.
PROPTOSIS 2. Unilateral lid retraction.
3. Facial asymmetry.
Proptosis is an abnormal protrusion of the eyeball. 4. Enophthalmos or ptosis of one eye, which makes
Exoph- thalmos is a term conventionally used for the uninvolved eye more prominent.
proptosis seen in TED (Box 26.1).
Thyroid-associated Eye Disease
BOX 26.1: Causes of Proptosis
Thyroid eye disease is the most common cause for
Congenital conditions uni- lateral as well as bilateral proptosis. So, thyroid
Crouzon syndrome
function tests have to be done in the investigations
Apert syndrome
of all cases of proptosis.
Orbital dermoid
Inflammatory conditions
Orbital cellulitis Unilateral Proptosis
Mucormycosis Unilateral proptosis can be due to:
Dacryoadenitis • Thyroid eye disease
Tolosa-Hunt syndrome • Orbital cellulitis
Wegener’s granulomatosis
• Parasitic infestations
Orbital pseudotumor
• Benign or malignant tumors
Vascular conditions
• Cavernous sinus thrombosis in the early stages.
Cavernous hemangioma
Caroticocavernous fistula
Orbital varices Bilateral Proptosis
Tumors Bilateral proptosis can be due to:
Lacrimal gland tumors • Thyroid eye disease
Optic nerve glioma • Caroticocavernous fistula and cavernous sinus
Meningioma throm- bosis in late stages
Lymphoma • Parasitic infestations
Neurofibroma
• Bilateral orbital tumors as in lymphomas,
Metastatic tumors
secondary deposits or orbital extension of bilateral
Frontal and ethmoidal mucocele
retinoblastoma
Endocrine diseases
• Pseudotumor in children
Thyroid exophthalmos
• Craniofacial synostosis like Crouzon syndrome.
Traumatic conditions
Orbital fractures
Orbital hemorrhage Acute Proptosis
Orbital emphysema Acute proptosis usually appears within a few hours to
few days. It can be due to:
Proptosis can be:
• Orbital cellulitis
• Unilateral or bilateral
• Orbital hemorrhage
• Acute or chronic
• Orbital emphysema
• Axial or eccentric
• Caroticocavernous fistula.
 Chapter 26 Orbit 384

Axial Proptosis optic foramen. With the advent of CT scan and

MRI, the importance of X-rays has come down.
A condition (Figs 26.4A and B) where the eye is
4. Ultrasonography scan easily delineates the size and
pushed straight forward is seen in:
location of a space occupying lesion.
• Thyroid eye disease
• Orbital cellulitis
• Intraconal mass lesions.

Eccentric Proptosis
A condition where the eye is displaced sideways is
seen in extraconal mass lesions.

Pulsatile Exophthalmos
The condition is seen in:
• Caroticocavernous fistula
• Meningoencephalocele with bony defects of
orbit
• Highly vascular tumors.

Static Proptosis
Static proptosis condition is rare and seen in
congenital conditions like dermoid cysts and
craniofacial synostosis with shallow orbits.

Intermittent Proptosis
The condition is seen in orbital varices.

Investigations in a Case of
Proptosis
1. Exophthalmometry: A simple instrument called
Hertel exophthalmometer is used to measure the
displacement of the globe. Normal measurement
is 18–21 mm and a measurement of more than
21 mm or a difference of more than 2 mm
between the two eyes is significant.
If exophthalmometer is not available, a
transpar- ent ruler held at the lateral canthus,
parallel to the nose can be used to make an
approximate measurement.
Naffziger’s sign—look down at the patient
from above and behind, so that you are looking
down at the patient’s eyebrow and nose from
above. Any proptosis can be made out (Fig.
26.4C).
2. Thyroid function tests to rule out any thyroid
eye disease.
3. Plain X-ray of skull and orbit to highlight any
fractures or erosions of the bones. Special view
like water’s view is required to visualize the
 Chapter 26 Orbit 385

FIGURES 26.4A to C: Proptosis. A. Left axial proptosis; B.

Bilateral axial proptosis with lid retraction in thyrotoxicosis; C.
Naffziger’s sign with prominent right eye.

5. Computed tomography scan gives useful clue

regard- ing the anatomical nature of the mass
lesions, espe- cially those arising from the
bones and also helps to visualize calcification
and any extension to the sinus- es or
intracranial areas.
6. Magnetic resonance imaging helps better
anatomical identification of soft tissue masses
than CT scan, but it is contraindicated in the
presence of a metallic for- eign body.
Magnetic resonance imaging angiogram is
indicat- ed in special situations like orbital varices,
any vascular anomalies and to identify the feeder
vessels in highly vas- cular tumors.

Treatment
1. This depends on the underlying cause. So, all
investi- gations have to be done to make a
correct etiological diagnosis.
 Chapter 26 Orbit 386

2. If it is thyroid exophthalmos, thyrotoxicosis has to grows. The more superficial cysts usually become
be treated. Antithyroid drugs have to be symp- tomatic in childhood, but deeper orbital
monitored care- fully so as to avoid going in for dermoids may not become clinically evident until
hypothyroidism, since this can aggravate the adulthood. Orbital dermoid cysts are lined by
protrusion of the eyeball. epidermis only and are usually filled with keratin,
3. If it is due to a tumor, surgery is required. and they do not contain dermal ap- pendages.
Preseptal orbital dermoid cysts occur most
General Measures commonly in the area of the lateral brow adjacent
to the frontozygo- matic suture. Dermoid cysts
1. Artificial tears have to be instilled frequently to commonly present as pal- pable smooth, painless,
avoid drying of the cornea. oval masses that enlarge slowly. They may be freely
2. If there is corneal exposure, lateral tarsorrhaphy mobile or they may be fixed to perios- teum at the
is in- dicated to protect the cornea. underlying suture (Figs 26.5A and B). Medial lesions
3. Systemic steroids can be given in addition to in the infant should be distinguished from congen-
antithy- roid drugs to decrease the inflammatory ital encephaloceles and dacryoceles.
infiltration and control the protrusion in Dermoid cysts that do not present until adulthood
severe cases. are often not palpable because they are situated
4. If all these measures fail and optic nerve posteriorly in the orbit, usually in the superior and
compression or exposure keratitis sets in, temporal portions, adjacent to the bony sutures.
surgical decompression of the orbit has to be The globe and adnexa may be displaced causing
done. progressive proptosis and erosion or remodeling of
bone can occur. Less commonly, the clini- cal
Enophthalmos presentation may be orbital inflammation, which is in-
cited by leakage of oil and keratin from the cyst.
Enophthalmos is an inward displacement of the
Management
eyeball. It has to be differentiated from
microphthalmos—a con- genitally small eye or Dermoid cysts are usually removed surgically.
phthisis bulbi, a disorganized eye- ball due to Superficial dermoids can be excised through an
disease. In enophthalmos, the abnormality is in the incision placed in the upper eyelid crease or directly
over the lesion. If possible,
orbital walls or its contents and the eye is normal.

Causes
1. A blowout fracture of the orbit: This is a
fracture of the orbital walls without involving
the orbital mar- gins, usually caused by a blunt
trauma of consider- able force. The inferior or
the medial wall of the or- bit is involved.
There will be herniation of orbital soft tissue
into the sinuses and entrapment of the ocular
muscles in the fracture. This will lead to
enophthal- mos and limitation of ocular
movements.
2. Atrophy of orbital fat due to extreme
malnutrition, ra- diation treatment or repeated
steroid injections into the orbit.
3. Sclerosing orbital lesions like metastatic
scirrhous carcinoma and sclerosing orbital
pseudotumors.

ORBITAL NEOPLASMS
 Chapter 26 Orbit 387
Congenital Orbital Tumors
Dermoid Cyst
Dermoid and epidermoid cysts are among the
most com- mon orbital tumors of childhood. These
FIGURES 26.5A and B: Dermoid cyst. A. Dermoid with hair;
cysts are present congenitally and they enlarge B. Orbital dermoid.
progressively as the child
 Chapter 26 Orbit 388

the cyst wall should be maintained during surgery.

Rup- ture of the cyst can lead to an acute
inflammatory process, if part of the cyst wall or any
of the contents remains with- in the eyelid or orbit. If
the cysts wall is ruptured, the sur- geon should
remove the entire wall and then thoroughly
irrigate the wound to remove all cyst contents.

amblyopia. Cosmetic deformity is often significant.

Teratoma Management
Teratomas are rare tumors that arise from all 1. Magnetic resonance imaging demonstrates the char-
three germ layers. These tumors are usually cystic acteristic fine intralesional vascular channels and
and can cause dramatic proptosis at birth. The also
globe and optic nerve may, as a consequence, be
maldeveloped. Exenteration is sometimes
performed because of the fear of malignan- cy.
Some cystic teratomas can be removed and ocular
function preserved.

Vascular Tumors
Capillary Hemangiomas
Capillary hemangiomas are common benign primary
tu- mors of the orbit in children. They should be
distinguished from cavernous hemangiomas, which
are the most com- mon benign orbital tumors in
adults (Figs 26.6A and B).
Capillary hemangiomas are seen primarily in
children in the first year of life, often appearing in
the 1st week or 2 weeks after birth, enlarging
dramatically over the first 6–12 months of life.
They are more common in girls and premature
newborns. After the first year, these vascular tumors
begin to involute, 75% of lesions resolve during the
next 4 years of life.
Clinical features
The clinical appearance of a periorbital capillary
hem- angioma depends on the depth of tumor
under the skin. Superficial capillary hemangiomas
produce an elevated red discoloration with a dimpled
texture (refer Fig. 26.6A). Deeper ones cause a
bluish discoloration or may pres- ent merely as a
progressively enlarging mass without any overlying
skin change.
Periocular hemangiomas are commonly associated
with hemangiomas on other parts of the body.
Common sites of involvement are superonasal
quadrant of the orbit and me- dial upper eyelid.
Capillary hemangiomas of the eyelids and orbit may
cause anisometropia, strabismus or deprivation
 Chapter 26 Orbit 389

FIGURES 26.6A and B: Capillary hemangioma. A. Gross anatomy;

B. Histopathology showing vascular channels.

defines the speed of blood flowing within

the lesion. Capillary hemangiomas usually
have high blood flow derived from multiple
fine feeder vessels.
2. Ophthalmic indications for treatment of
capillary hemangiomas are anisometropia,
strabismus and amblyopia.
3. Radiation therapy, pulsed dye laser therapy
and high- potency topical corticosteroids has
been used in the treatment of capillary
hemangiomas.

Cavernous Hemangioma
Cavernous hemangiomas are the most common
benign neoplasms of the orbit in adults (Figs
26.7A and B). Mid- dle-aged women are most
commonly affected. Proptosis is usually slowly
progressive, although growth may accel- erate, if
the patient is pregnant.
Complications
Retinal striae, hypermetropia, optic nerve
compression, in- creased intraocular pressure and
strabismus may develop:
1. Histopathologically, the lesions are
encapsulated and are composed of large
cavernous spaces containing
 Chapter 26 Orbit 390

FIGURES 26.7A to D: Cavernous hemangioma. A. Before surgery; B. After surgery; C. Histopathology;

D. CT scan in cavernous hemangioma.

red blood cells and the walls contain smooth embolic visual loss.
muscle (Fig. 26.7C).
2. MRI demonstrates an enhancing lesion with small
in- tralesional vascular channels containing slowly
flow- ing blood.
3. Arteriography and venography has no much role
be- cause the lesion has a very limited
communication with systemic circulation.
4. Computed tomography shows a homogeneously
en- hanced well-encapsulated mass (Fig. 26.7D).
Old le- sions may contain radiodense
phleboliths.
Management
Treatment consists of surgical excision of the lesion
com- prising ocular function. These tumors rarely
undergo spontaneous involution.
When therapy is indicated, initial treatment
consists of local steroid injection, usually an equal
mixture of 0.5 mL betamethasone and 0.5 mL
triamcinolone. Adverse effects include necrosis of
the skin overlying the hemangioma, subcutaneous
fat atrophy, systemic growth retardation and
 Chapter 26 Orbit 391
Systemic steroids have also been used,
especially in lesions that extend more deeply
into the orbit. Injecting deep orbital lesions
carries a higher risk of orbital hemor- rhage and
retinal emboli.
Lesions that are smaller, those that are
subcutane- ous and nodular, and those
refractory to steroids can be managed with
surgical excision. The use of systemic
interferon- has been reported, but poorly
tolerated in most cases.

Lymphangioma
A lymphangioma is a relatively uncommon
tumor that usually becomes apparent in the 1st
decade of life. It may occur in the conjunctiva,
eyelids, orbit, oropharynx or si- nuses.
Lymphangiomas often enlarge during upper respi-
ratory tract infections, probably because of the
response of the lymphoid tissues within the lesion.
They may pres- ent with sudden proptosis caused
by spontaneous intral- esional hemorrhage.
Histologically, these tumors are characterized
by large serum-filled spaces that are lined by
flattened, delicate
 Chapter 26 Orbit 392

endothelial cells. These tumors have an infiltrative

Arteriovenous Fistula
pattern and not encapsulated (Figs 26.8A to D).
Management Arteriovenous fistulas are abnormal
communications between previously normal arteries
In blood cysts associated with lymphangiomas,
and veins. These ac- quired lesions may be caused by
spontane- ous regression is common. Surgical
trauma or degenerations.
intervention should be deferred unless vision is
affected. If optic neuropathy or corneal ulceration
threatens vision, aspiration of blood through a hollow- Caroticocavernous Fistula
bore needle or by open surgical explora- tion can be The common occurrence of a caroticocavernous
attempted. fistula (CCF) is due to the partly fixity of the
Because of the infiltrating nature of vessels in this area to the bone so that, a fracture
lymphangioma, a subtotal resection is generally frequently causes direct injury to the artery, to the
needed to avoid sacrificing important structures. The thinning of the wall of the carotid, as it enters the
CO2 and contact neodymium- doped yttrium skull and to the unique anatomical arrange- ment
aluminum garnet (Nd:YAG) lasers are use- ful whereby the artery runs within the lumen of the
adjuncts for surgery, which improves hemostasis and vein. Moreover the circle of Willis is the common
can be used to cause shrinkage and scarring of some site for the occurrence of intracranial aneurysms,
un- which provides point of weakness (Fig. 26.9).
resectable areas of tumor. Bipolar cautery can also be Caroticocavernous commu- nications can be
ap- plied to shrink the tumor during surgical divided into:
excision. • Traumatic and spontaneous
In more extensive diffuse lesions of the orbit, a • Direct or indirect
tran- scranial approach may allow total or subtotal • Low flow or high flow.
excision. Traumatic cases: Occur most frequently in males,
consti- tuting 80% of the total cases. 75% of these
Arteriovenous Malformations are associated with a fracture of the base of the skull,
mostly involving the body of the sphenoid bone to
Arteriovenous malformations are developmental which the artery and vein are intimately apposed.
anoma- lies composed of abnormally formed
anastomosing arter- ies and veins without an
intervening capillary bed.
 Chapter 26 Orbit 393
FIGURES 26.8A to D: Lymphangioma. A and B. Clinical pictures; C. CT scan in lymphangioma; D. Histopathology.
 Chapter 26 Orbit 394

usually diminution of vision, and a marked unilateral

pulsating proptosis are the char- acteristic features
(Figs 26.10A and B).

FIGURE 26.9: Types of caroticocavernous fistula

Spontaneous fistulas: Occur due to the

communication between the carotid and sinus, an
event, which usually presumably results from the
bursting of an aneurysm or a weakness of the wall of
the vessel. Majority of spontane- ous fistulas occur in
females with systemic hypertension and
atherosclerosis.
Direct CCF: Occurs when the communication is
between the internal carotid artery and the
cavernous sinus.
Indirect CCF: Occurs when a branch of internal
carotid or the external carotid artery communicates
with the cavern- ous sinus.
Mechanism of the symptomatology of the
condition is clear. Rupture of the carotid allows
direct escape of arterial blood under high pressure
into the cavernous sinus, the venous pressure is
increased and the flow of blood reversed.
Ophthalmic veins are dilated, causing stasis and
edema in the orbit producing pulsatile pro- ptosis
synchronous with the systolic beat. Arterial pres- sure
is lowered and venous pressure is raised resulting in
acute reduction in the perfusion pressure of the
circula- tion and hypoxia. Usually the ipsilateral orbit
and eye are affected, but the proptosis may be
bilateral or become evident on the other side or
even alternate between the two. This is due to the
communication channels between the two
cavernous sinuses.
Clinical feature
Clinical feature is usually typical. Frequently, the
onset is sudden either on recovering consciousness
from a head injury or without apparent cause. A
swishing noise in the head, considerable pain and
 Chapter 26 Orbit 395
Signs in a high-flow fistulas: Tortuous epibulbar and
fore- head vessels, bruit that may be audible to
the examiner, a pulsatile proptosis and paralysis of
cranial nerves III, IV or most commonly VI with
associated muscle palsies.
Fundus examination reveals engorged veins,
disk edema, choroidal effusions and vitreous
hemorrhage may occur. Increased episcleral
venous pressure will lead to a secondary
glaucoma.
Investigations
Computed tomography scan show diffuse
enlargement of all the extraocular muscles
resulting from venous en- gorgement and a
characteristically enlarged superior oph- thalmic
vein (refer Fig. 26.10A).
Low-flow fistulas: It often close spontaneously. Recent
data suggest that patients with low-flow fistulas
are at a higher risk of intracranial hemorrhage
because of the arterializa- tion of the venous
system.
Selective arteriography is used to evaluate the
arterio- venous fistulas of the orbit and
cavernous sinus.
Treatment
Embolization using coils to obstruct the fistula is
gener- ally accomplished through an endovascular
transarterial route.

Orbital Varix
An orbital varix can occur primarily as dilatations
of pre- existing venous channels. It can be:
1. Primary varices due to a congenital venous
malfor- mation in the orbit.
2. Secondary varices due to an arteriovenous
shunt ei- ther intracranially or within the
orbit itself.
FIGURES 26.10A and B: Caroticocavernous fistula. A. MRI scan;
B. Photograph.
 Chapter 26 Orbit 396

Clinical features The chief clinical feature is gradual, painless, unilat-

Clinical feature presented by a case of orbital varix is eral, axial proptosis associated with loss of vision
dra- matic and typical; the significant sign is a and an afferent pupillary defect. Other ocular findings
transient pro- ptosis, which may last a few seconds, may
a few hours or a few days. It is always unilateral and
usually left sided due to the narrowness of the jugular
foramen on that side. Proptosis increases when the
patient’s head is dependent or after a Valsalva
maneuver. Patients may exhibit enophthalmos at rest,
when the varix is not engorged due to pressure
atrophy of the orbital tissue resulting from recurrent
attacks. Vision is usually unimpaired. Mydriasis in the
affected eye is com- mon and venous engorgement
of the retina is the rule.
Investigations
The diagnosis can be confirmed via contrast-
enhanced spiral CT scan. Rapid spiral CT scan during
the Valsalva maneuver or other means of
decreasing venous return shows characteristic
enlargement of the engorged varix. Phleboliths can
sometimes be seen on plain film radio- graphs. If
the diagnosis is suspected, biopsy should be
avoided because significant bleeding can be
anticipated.

Treatment
Treatment is usually conservative. Surgery is
reserved for relief of significant pain or for cases in
which the varix threatens vision, because of
compressive optic neuropa- thy. Complete surgical
excision is difficult, as the varix is intertwined with
normal orbital structures and directly communicates
with the abundant venous reservoir in the cavernous
sinus. Intraoperative embolization of the lesion aids
surgical removal followed by excision. Embolization
with coils inserted through a distal venous cut down
has also been reported to diminish symptoms.

Neural Tumors
Neural tumors include:
• Optic nerve glioma
• Neurofibromas
• Meningiomas
• Schwannomas.

Optic Nerve Glioma

Rare benign tumors occur predominantly in children
in the 1st decade of life. Malignant optic nerve
gliomas are very rare and tend to affect adult
males. Approximately, 25%–50% of optic nerve
gliomas are associated with neurofibromatosis.
Clinical features
 Chapter 26 Orbit 397
include optic atrophy, optic disk swelling and
strabis- mus. Intracranial involvement may be
associated with de- creased function of the
hypothalamus and pituitary gland. In most cases,
optic nerve gliomas are self-limited and show
minimal growth. Initial signs and symptoms of ma-
lignant gliomas include massive swelling and
hemorrhage
of the optic nerve head, and severe retro-orbital pain.
Gross pathology of resected tumors usually
reveals a smooth, fusiform intradural lesion. The
benign tumors in children are considered to be
juvenile pilocytic as- trocytomas.
Investigation
Optic nerve gliomas can usually be diagnosed
by means of orbital imaging. CT scan and MRI
usually show fu- siform enlargement of the
optic nerve (Figs 26.11 and 26.12A and B). MRI
may be more accurate in defining the extent of
an optic canal lesion and intracranial disease.
Treatment
1. Treatment of optic nerve glioma is
controversial. Pre- sumed optic nerve glioma
particularly with good vi- sion on the
involved side may be carefully followed up,
if it is confined to the orbit.
2. Excision is considered in situations like rapid
growth, intracranial optic nerve involvement,
increase in in- tracranial pressure and massive
proptosis with cor- neal exposure.
3. Radiation therapy is considered, if the tumor
cannot be resected and if symptoms
progress.
4. Combination chemotherapy using
actinomycin D and vincristine has also been
reported to be effective in patients with
progressive chiasmatic/hypothalam- ic
gliomas.

Meningioma
Meningiomas are invasive tumors that arise from
arach- noid villi and usually originate
intracranially along

FIGURE 26.11: Optic nerve glioma

 Chapter 26 Orbit 398

FIGURES 26.12A and B: Optic nerve glioma; A. CT scan; B. Optic atrophy.

sphenoid wing with secondary extension into the orbit Primary orbital meningiomas usually originate in the
through the bone, the superior orbital fissure or the op- arachnoid of the optic nerve sheath. They occur
tic canal. Ophthalmic manifestations are related to the common- ly in women in their 3rd and 4th decades of
location of the primary tumor. Meningiomas arising life. Symptoms include gradual, painless, unilateral
near the sella and optic nerves cause early visual de- loss of vision. Relative afferent pupillary defect and
fects and papilledema or optic atrophy. Tumors arising decreased visual acuity are the typical signs. Proptosis
near the pterion often produce a temporal fossa mass and ophthalmoplegia may be present at presentation.
and proptosis. Primary optic nerve meningiomas can The optic nerve head may appear normal, atrophic or
produce early profound vision loss without any propto- swollen and optociliary shunt vessels may be visible
sis (Fig. 26.13A). (Figs 26.13B and C).
Sphenoid wing meningiomas produce hyperostosis of Computed tomography and MRI show diffuse
the involved bone and hyperplasia of associated soft tubular enlargement of the optic nerve with contrast
tissues. enhance- ment. In some cases, CT scan shows
calcification within
 Chapter 26 Orbit 399
FIGURES 26.13A to C: Optic nerve meningioma. A. Clinical picture; B. Optociliary shunt vessel; C. CT scan.
 Chapter 26 Orbit 400

the meningioma, a sign that on plain X-ray is

termed tram-tracking. Rhabdomyosarcoma
Malignant meningioma is rare and results in rapid Rhabdomyosarcoma is the most common primary
tu- mor growth that is not responsive to surgical orbital malignancy of childhood. The average age of
resection, ra- diotherapy or chemotherapy. Both the onset is 8–10 years (Fig. 26.15A).
extent of visual loss and the presence of intracranial
extension are important factors in treatment Clinical Features
planning.
The classic clinical features are one of a child with
Treatment sudden onset and rapid evolution of unilateral
Treatment of optic nerve sheath meningiomas proptosis. There is often a marked adnexal response
must be individualized, if minimally affected and no with edema and discol- oration of eyelids. Ptosis and
intracranial extension is present. If the tumor is strabismus may also be pres- ent. A mass may be
confined to the orbit and visual loss is significant or palpable particularly in the superona- sal quadrant
progressive, radiation ther- apy should be of the eyelid.
considered. Surgery is reserved for patients with If a rhabdomyosarcoma is suspected, the
severe visual loss and profound proptosis. workup should proceed on an urgent basis. CT and
MRI can be used to define the location and extent
Schwannomas of the tumor (Figs 26.15B and C). CT is particularly
helpful, if the tumor has caused bony destruction,
Schwannomas (also known as neurilemmas) are although the orbital walls re- main intact in most
prolif- erations of Schwann cells that are cases. A biopsy should be undertaken, usually
encapsulated by peri- neurium. Hypercellular through an anterior orbitotomy.
schwannomas sometimes recur even after what is
Rhabdomyosarcomas arise from undifferentiated
thought to be complete removal, but they seldom plu- ripotent mesenchymal elements in the orbital
undergo malignant transformation. These tumors are soft tissues and not from the extraocular
usually well encapsulated and can be excised with muscles.
relative ease. They may be grouped into four categories:
• Embryonal
Neurofibromas • Alveolar
Neurofibromas (NF) are tumors composed chiefly • Pleomorphic
of pro- liferating Schwann cells within the nerve • Botryoid.
sheaths. Plexi- form neurofibromas are infiltrative
tumors that usually occur in NF1 (Figs 26.14A and Treatment
B). They are well vascular- ized and can seldom be The standard treatment of orbital rhabdomyosarcoma
completely removed by surgical excision. Discrete was orbital exenteration and the survival rate was
neurofibromas can usually be excised surgically poor. But re- cently, radiation therapy and systemic
without recurrence. chemotherapy has
 Chapter 26 Orbit 401

FIGURES 26.14A and B: Plexiform neurofibroma. A. Frontal view; B. Lateral view.

 Chapter 26 Orbit 402

FIGURES 26.15A to C: Rhabdomyosarcoma; A. Clinical picture; B. CT scan findings; C. Histopathological findings.

become the mainstay of primary treatment. Survival are often located anteriorly in the orbit or beneath the
rate is better than 90%, if the orbital tumor has not conjunctiva,
evolved or extended beyond the bony orbital walls.

Lymphoproliferative Disorders
Lymphoproliferative lesions of the ocular adnexa
consti- tute a heterogeneous group of neoplasms that
accounts for more than 20% of all orbital tumors.
Most are non- Hodgkin lymphomas.
The vast majority of orbital lymphomas (Figs
26.16A to D) are B-cell derived. T cell lymphoma is
rare and more lethal. Based on the revised European-
American lymphoma classi- fication (REAL), the
following represent the four most com- mon orbital
lymphomas:
1. Mucosa-associated lymphoid tissue (MAT).
2. Chronic lymphocytic lymphoma (CLL).
3. Follicular center lymphoma.
4. High-grade lymphomas include large cell
lymphoma, lymphoblastic lymphoma and Burkitt’s
lymphoma.
The typical lymphoproliferative lesion presents as
a gradually progressive painless mass. These tumors
 Chapter 26 Orbit 403
where they may feature the typical salmon-patch
appear- ance. Lymphoproliferative lesions,
whether benign or malignant, usually mold to
surrounding orbital structures rather than invade
them. Disturbances of extraocular mo- tility or
visual function are unusual. Orbital imaging re-
veals characteristic putty-like molding of tumor to
normal structures up to 50% arise in the
lacrimal fossa.

Management
Although systemic corticosteroids are useful in
idiopathic orbital inflammation (pseudotumor),
they are not recom- mended in the treatment of
lymphoproliferative lesions.
Radiotherapy is the treatment of choice for
patients with localized ocular adnexal
lymphoproliferative dis- ease. A surgical cure,
usually, cannot be achieved because of the
infiltrative nature of lymphoid tumors.

Secondary Orbital Tumors

Tumors from the neighboring structures can
extend to the orbit and can result in proptosis.
Tumors of the lids or intraocular tumors like
retinoblastoma can extend to the orbit and lead
to proptosis.
 Chapter 26 Orbit 404

FIGURES 26.16A to D: Lymphoma. A. Clinical picture; B. Histopathological findings; C. CT scan findings;

D. Lymphoma advanced stage.

Mucoceles of Paranasal Sinuses 1. The subperiosteal surgical space, which is the

The openings of the paranasal sinuses can get blocked poten- tial space between the bone and the
and they can get distended with secretions. The frontal periorbital.
and ethmoidal sinuses are separated only by thin 2. The extraconal surgical space (peripheral surgical
bones from the orbit and the frontal and ethmoidal space), which lies between the periorbital and the
mucoceles can bulge into the orbit to form bulging in muscle cone with its fascia.
the upper and outer quadrant of the orbit displacing the 3. The intraconal surgical space (central surgical
eye downwards and upwards (Fig. 26.17). space), which lies within the muscle cone.

Malignant Nasopharyngeal Tumors

More than one third of the cases of nasopharyngeal
tumors can produce orbital symptoms. Paresthesia or
numbness on the area of distribution of the infraorbital
nerve on the lower lid and cheek may be an early
symptom. Extension of the tumor into the orbit will
cause proptosis with dis- placement of the globe
upwards.

ORBITAL SURGERIES

Surgical Spaces
There are five surgical spaces within the orbit, which
are shown in Figures 26.18A and B: FIGURE 26.17: Frontal mucocele
 Chapter 26 Orbit 405

FIGURES 26.18 A and B: Surgical spaces of the orbit. A. Anteroposterior view; B. Crosssectional view. 1. Subperiosteal space;
2. Peripheral orbital space; 3. Intraconal space; 4. Sub-Tenon’s space; 5. Subarachnoid surgical space.

4. The episclera (sub-Tenon’s) surgical space, which lesions.

lies between Tenon’s capsule and the globe.
5. The subarachnoid surgical space, which lies
between the optic nerve and the nerve
sheath.

Orbitotomies
A myriad of clinical disorders can affect the orbit. The
tim- ing and approach of surgical intervention are
based on the nature of the orbital disease process
defined by clinical and imaging study.
Basic indications for orbital surgery are:
• Incisional biopsy
• Excision of a cyst or mass
• Repair and reconstruction
• Abscess drainage
• Decompression
• Exenteration.
The common surgical approaches to the orbit
include anterior, lateral or superior orbitotomy.
The location of the mass within the surgical space
and its extent, its relation to the extraocular muscles
and optic nerve and the character of the lesion
determine the spe- cific choice.

Anterior Orbitotomy
Anterior orbitotomy techniques are used for the
incisional biopsy of palpable orbital tumors or the
excision of anteri- orly located well-defined
 Chapter 26 Orbit 406
Lateral Orbitotomy
Lateral orbitotomy with or without removal of
lateral wall gives excellent approach to the
intraconal and ex- traconal spaces of the orbit,
lateral to the optic nerve. The two most popular
approaches are the Berke-Reese inci- sion and
the Stallard-Wright incision (Fig. 26.19).
The Berke-Reese incision involves a 3–5 mm
horizon- tal incision after a complete lateral
canthotomy.
The Stallard-Wright incision is curvilinear
extending from lateral half of the eyebrow,
toward the lateral bony orbital rim. Stallard-
Wright incision gives a better access to lacrimal
gland fossa tumors and does not require re-
construction of the lateral canthus.

Orbital Decompression
The goal of orbital decompression is to allow the
enlarged muscles and orbital soft tissue to expand
into periorbital spaces, to relieve optic
neuropathy and to decrease pro- ptosis. It is
done usually in thyroid ophthalmopathy.

Enucleation
Enucleation is the removal of the entire globe,
while pre- serving other orbital tissues.

Indications
1. Enucleation is indicated for primary intraocular
malig- nancies; not amenable to alternative
modes of therapy.
 Chapter 26 Orbit 407

scleromalacia.
6. For eye donation in a dead person.
Guidelines for enucleation
Earlier the eye was removed leaving an empty
socket. After 30–45 days, when the healing has
completed, a readymade artificial eye is fitted. The
cosmetic improve-

FIGURE 26.19: Incisions for orbitotomy (Courtesy: Stallard’s

Textbook of Surgery)

Retinoblastoma and choroidal melanoma are the

ocu- lar tumors that most commonly require
enucleation. Evisceration should not be performed
in cases of sus- pected intraocular malignancy.
Surgeon must take care to avoid penetrating the
globe during surgery and he/she must handle
the globe gently to minimize the risk of
disseminating tumor cells. In case of suspected
retinoblastoma, the surgeon should obtain a long
seg- ment of optic nerve with the enucleation
specimen to increase the chance of completely
resecting the tumor.
2. Blind eyes with opaque media, suspected of
harboring an occult neoplasm unless another cause
of ocular dis- ease can be surmized.
Ultrasonography is useful in eval- uating these
eyes and planning proper management.
3. In severely traumatized eyes, enucleation within
first 14 days of the injury may be considered, if
the risk of sympathetic ophthalmia and harm to
the remaining eye is judged to be greater than
the likelihood of recov- ering useful vision in the
traumatized eye. Although there is some
conflicting evidence, removal of an eye that
has already stimulated sympathetic ophthalmia
is unlikely to prevent progression of the
disease.
4. Painful blind eyes as in neovascular glaucoma,
when conservative measures fail and unsightly
eyes as in huge anterior staphyloma. Enucleation
with an orbital im- plant will relieve the symptoms
and give good cosmetic appearance. Evisceration
can also be done in these situ- ations, but
enucleation is better, if the chance of an intra-
ocular malignancy is not completely ruled
out.
5. For endophthalmitis, evisceration is not
possible if there is scleral abscess or
 Chapter 26 Orbit 408
ment was poor, since the prosthesis rarely
matched the other normal eye in appearance and
the movement of the prosthesis on ocular
movements was practically nil.
Now, the concept is to put in an orbital
implant at the time of enucleation (Figs 26.20A to
E). This implant gives

FIGURES 26.20A to E: Enucleation with and without an

implant. A and B. Enucleation without implant; C. Same eye
with artificial eye; D. Enucleation with implant; E.
Appearance with prosthesis in place with fair motility.
 Chapter 26 Orbit 409

volume to the orbital contents and fixing the ocular with conscious sedation. In either case, a retrobulbar
mus- cles to the implant gives some mobility to the block us- ing a 50:50 mixture of 2% lidocaine with
prosthesis worn over it. epinephrine and 0.75% bupivacaine provides
Muscles sutured into the normal anatomical vasoconstriction intraopera- tively, as well as sustained
locations, either directly to the implant or to analgesia postoperatively.
homologous sclera or autologous fascia surrounding
the implant, allow superior motility and prevent
migration.
Volume loss in the adult anophthalmic socket may
be adequately replaced by a 20–22 mm sphere
implant.
The prosthesis is now custom made to match the
other eye and with the mobility given by the
implant, the cos- metic appearance is excellent.
A functionally and aesthetically acceptable
anophthal- mic socket must have the following
components:
1. An orbital implant of sufficient volume centered
with- in the orbit.
2. A socket lined with conjunctiva or mucous
membrane with fornices deep enough to hold
a prosthesis.
3. Eyelids with normal appearance and adequate
tone to support prosthesis.
4. Good transmission of motility from the implant to
the overlying prosthesis.
5. A comfortable ocular prosthesis that looks similar
to the normal eye.

Enucleation in Childhood
Enucleation in early childhood, as well as congenital
an- ophthalmos or microphthalmos, may lead to
underdevel- opment of the involved bony orbit with
secondary facial asymmetry. When enucleation is
necessary in childhood, a large implant should be
used to replace orbital volume. Autogenous dermis-
fat grafts are used successfully as an- ophthalmic
implants in children, as they appear to grow along
with the expanding orbits.

Enucleation Technique
Prior to performing enucleation surgery, it is crucial
that the patient be advised of the advantages and
disadvantag- es of enucleation surgery, implant
migration or extrusion and the need for fitting of an
ocular prosthesis, 6–8 weeks postoperatively.
The correct eye is marked immediately before
surgery.

Anesthesia
Either general anesthesia or local anesthesia is given
 Chapter 26 Orbit 410
Procedure
1. A lid speculum is placed to retract the lids. Indications
2. A 360° conjunctival peritomy is created with 1. Panophthalmitis.
scissors. Tenon’s capsule is bluntly dissected 2. Bleeding anterior staphyloma.
off the globe in all four quadrants.
3. One by one, the four recti muscles are
secured with a muscle hook and a 5-0
polyglactin suture is passed through the
muscle near the insertions and the mus- cles
are cut from the globe.
4. The globe is rotated laterally with the traction
sutures and a clamp is inserted medially and
applied to the optic nerve to crush the
central retinal artery. The nerve is then cut
with curved scissors and the eye is removed.
Every attempt should be made to obtain as
long a section of optic nerve as possible.
5. Bleeding can be controlled by digital pressure
or with bipolar cautery.
6. The appropriate size implant is chosen and
soaked in antibiotic solution. The extraocular
muscles can be su- tured directly to a porous
polyethylene implant either by passing the
suture needles through the implant ma- terial
or through the predrilled suture tunnels.
7. Tenon’s capsule is closed with interrupted 6-
0 poly- glactin sutures and conjunctiva is
closed with a run- ning 6-0 polyglactin
suture.
8. A conformer is placed and the lids are
closed. A pres- sure patch is applied.
9. Avoid excessive dissection near the orbital
roof and apex to reduce the chance of
damaging the extraocular muscles or their
innervation and resulting in ptosis.
Complications
1. Bleeding intraoperatively or in the immediate
postop- erative period.
2. Infection.
3. Extrusion or the implant.
4. Socket granuloma.

Evisceration
Evisceration is the removal of the contents of the
eye leav- ing the sclera and the extraocular
muscles intact.
Evisceration should be considered only if the
presence of an intraocular malignancy has
been ruled out.
 Chapter 26 Orbit 411

3. Painful blind eye (for better cosmesis

compared to enucleation, since an implant
Techniques
placed within the sclera will have better Evisceration can be performed either with retention
motility). of the cornea or with excision of the cornea. The
cornea can be retained, if it is of normal thickness
Contraindication and shows no active corneal disease.
Any blind eye is suspected to have malignant growth After insertion of the speculum, the eye is fixed
inside. by for- ceps. A cataract knife enters the cornea,
just anterior to the limbus. With a few snips of the
scissors, the cornea is detached and removed. A
Advantages
scoop of appropriate size is in- serted between the
1. Less disruption of orbital anatomy. sclera and uveal tract and is swept cir- cumferentially
2. Better motility of the prosthesis. to separate the ciliary body from the scleral spur, the
3. Better treatment of endophthalmitis because choroids from the sclera and posteriorly to tear
subse- quent orbital cellulitis or intracranial through the intraocular portion of the optic nerve.
extension of in- fection is reduced. The in- traocular contents are scooped out. All uveal
4. A technically simpler procedure. tract must be thoroughly removed. Retained
5. Lower rate of migration, extrusion of the implant fragments are potentially danger for sympathetic
and reoperation. ophthalmitis.
A very satisfactory cosmetic result may be
Disadvantages achieved by including an implant within the scleral
1. Evisceration should never be performed, if a cup. Tenon’s cap- sule is sewn over this with
tumor is suspected. horizontal line of interrupted sutures and the
2. Sympathetic ophthalmia is rarely caused by a conjunctiva is closed separately.
reaction to uveal tissue in the eviscerated
socket. Exenteration
3. Evisceration affords a less complete specimen
Exenteration involves the removal of the soft tissues
for pathologic examinations.
of the orbit including the globe (Figs 26.21 and
26.22A and B).

FIGURE 26.21: Incision for exenteration

 Chapter 26 Orbit 412
FIGURES 26.22A and B: Exenteration. A. Postexenteration. B. With spectacle attached prosthesis (Courtesy: www.medicalartprosthetics.com).
 Chapter 26 Orbit 395

4. Sarcomas and other primary orbital

malignancies that do not respond to non-
surgical therapy.
5. Orbital phycomycosis.

Types
Subtotal: The eye and adjacent intraorbital tissues are
re- moved, so that the lesion is locally excised
(leaving peri- orbital and part or all of eyelids).
This technique is used for some locally invasive
tumors for debulking of dissemi- nated tumors or for
partial treatment in selected patients.
Total: All intraorbital soft tissues including periorbita
are removed with or without the skin of the
eyelids.
Extended: All intraorbital soft tissues are removed,
together with adjacent structures (usually bony walls
and sinuses). Following removal of the orbital
FIGURES 26.23A and B: Implants after enucleation contents, the bony socket may be allowed to
spontaneously granulate and epithelialize or may
be covered by a split thickness skin graft, which
Indications
may be placed onto bare bone or over a tem-
1. Destructive tumors extending into the orbit from poralis muscle or temporoparietal fascial flap.
the sinuses, face, eyelids, conjunctiva or
intracranial space. Orbital Implants
2. Intraocular malignant melanomas or
Prosthesis
retinoblastoma that has extended outside the
globe. An ocular prosthesis is fitted within 4–8 weeks after
3. Malignant epithelial tumors of the lacrimal enu- cleation. The ideal prosthesis is custom fit to the
gland. exact di- mension of the orbit after postoperative
edema has sub- sided (Figs 26.23A and B).
SECTION 6
Disorders of Ocular Motility
Strabism
us
2
7
Anitha Balachandran, Girija Devi PS

CLASSIFICATION
INTRODUCTION
No classification is perfect or all inclusive and several
The term strabismus is derived from the Greek word methods of classifying eye alignment and motility disor-
‘stra- bismos’ meaning ‘to squint, to look obliquely or ders are used.
askance’. Strabismus means ocular misalignment,
whether caused by abnormalities in binocular vision
or by anomalies of neuromuscular control of
ocular motility.
Orthophoria is the ideal condition of ocular
balance. In reality, orthophoria is seldom
encountered; a small het- erophoria can be
documented in most persons.
Heterophoria is an ocular deviation kept latent
by the fusional mechanism (latent strabismus).
Heterotropia is a deviation that is manifest and
not kept under control by the fusional mechanism
(manifest strabismus).

PREFIXES
Eso: The eye is rotated so that the cornea is
deviated na- sally because the visual axes converge.
This is also called convergent strabismus (Figs
27.1A and B).
Exo: The eye is rotated so that cornea is deviated
tempo- rally because the visual axes diverge. This is
known as di- vergent strabismus (Figs 27.2A and
B).
Hyper: The eye is rotated so that cornea is deviated
superi- orly. This is also called vertical strabismus (Figs
27.3A and B).
Hypo: The eye is rotated so that the cornea is
deviated in- feriorly. This is also called vertical
strabismus.

SUFFIXES
Phoria: A latent deviation.
Tropia: A manifest deviation.
According to Fusional Status
1. Phoria: A latent deviation in which fusional
control is always present.
2. Intermittent tropia: A deviation in which
fusional control is present part of the time
(Figs 27.4A and B).
3. Tropia: A manifest deviation in which
fusional con- trol is not present (Fig. 27.5).

FIGURES 27.1A and B: Esotropia. A. Clinical picture;

B. Diagrammatic representation.

FIGURES 27.2A and B: Exotropia. A. Clinical picture;

B. Diagrammatic representation.
 Chapter Strabismus 400
27

FIGURES 27.4A and B: Intermittent exotropia. A. No

deviation;
B. Deviation of right eye is present.

FIGURES 27.3A and B: Hypertropia. A. Clinical

picture;
B. Diagrammatic representation.

According to Variation
with Gaze Position or
Fixating Eye
1. Comitant (concomitant): The deviation does
not vary in size with direction of gaze or
fixating eye.
2. Inconcomitant (non-comitant): The deviations
vary in size with direction of gaze or fixating
eye.

According to Fixation
1. Alternating: Spontaneous alternation of fixation
from one eye to other (Figs 27.6A and B).
2. Monocular: Definite preference of fixation with
one eye (Fig. 27.7).
 Chapter Strabismus 401
27
FIGURE 27.5: Constant exotropia

FIGURES 27.6A and B: Alternating exotropia. A.

Right eye is diverting; B. Left eye is diverting.

According to Age of Onset

Congenital/infantile: For a deviation documented
prior to 6 months, the term infantile may be more
appropriate.

According to Type of Deviation

1. Horizontal: Esodeviation or exodeviation.
2. Vertical: Hyperdeviation or hypodeviation.
3. Torsional: Incyclodeviation or excyclodeviation.
4. Combined: Horizontal, vertical, torsional or
any combination.

Heterophoria is a condition where there is a HETEROPHORIA

tendency for misalignment of visual axes, but
remains latent and can be corrected by the
fusional capacity. This ability is shared between
the two eyes. But the tendency for deviation be-
comes manifest by dissociation test.
 Chapter Strabismus 402
27

Diagnosis
The diagnosis of heterophoria depends on abolishing
fu- sion by dissociation test, so that without fusional
control, the eyes assume their natural position of
rest.

Cover Test
Cover test is the simplest test, which gives valuable
FIGURE 27.7: Monocular (right) esotropia infor- mation.
Principle: In phorias the parallelism of the eye is
If the latent deviation is one of convergence, it is
main- tained by the power of fusion. When the
called esophoria, if it is divergence, exophoria and
image to one eye is blocked, the fusional
if vertical, hyperphoria. It is impossible to be sure
mechanism will not be acting and the covered eye
whether there is absolute hyperphoria of one eye
goes to its natural position. When the cov- er is
or hypophoria of other. If the deviation is torsional,
removed and the stimulus for fusion comes back, the
the condition is cyclophoria.
eye will resume parallelism.
When a distant or near object is regarded and
Predisposing Factors both eyes are uncovered, there is no deviation.
1. Ill health, fatigue of eyes due to eyestrain, certain One eye is cov- ered, while other eye continues to fix
oc- cupation like computer workers/tailors, at the object. If there is heterophoria, the eye under
painters, etc. cover deviates. The cover is then quickly removed
2. Age: and the eye under cover is ob- served. As soon as
a. In infancy, binocular reflexes are only the cover is removed, the eyes under cover move
developing, so there is a tendency for back and corrects the deviation and regain the
divergence or exophoria. position of binocular fixation. The speed of
b. In childhood, accommodation and movement of recovery shows the amount of ability of
convergence reflexes are powerful, so there the fusional control. The other eye reacts similarly
is a tendency for deviation inwards or when the test is repeated by covering the other eye
esophoria. and the deviation in both remain the same. Cover
test should be done with spectacles for both near
c. In presbyopic age, as the near point of
distant ver- sion recedes, the convergence and distant objects (Figs 27.8A to D and 27.9).
becomes weaker and there is tendency for Both eyes are straight, but on covering right eye it
deviation of eye outwards. devi- ates under cover, which is revealed immediately
3. Influence of refraction: on remov- ing the cover. Both eyes become straight
a. In hypermetropia there is a tendency for again. On cover- ing the other eye, the covered eye
esopho- ria due to excessive use of also deviates under cover and the eyes become
accommodation to cor- rect hypermetropia. straight again on removing the cover. If the eye
b. In myopia there is a tendency for exophoria, deviates outwards under cover and moves inwards
since they are not using accommodation to on removing the cover, the patient is having exo-
see near ob- jects clearly. phoria or latent divergent squint. Similarly, in
4. Anatomical defects of muscles, ligaments or esophoria, the eye moves outwards on removing the
fascia in orbit. cover. So, the de- viation is opposite to the
movement of the eye on remov-
Sx Symptoms ing the cover.

Since the parallelism of visual axes is

Maddox Rod Test
maintained by tonic contraction of appropriate
muscles, symptoms of eyestrain are encountered The test depends on altering the appearance of
in higher degrees of phoria (asthenopic retinal im- age in one eye, so that no stimulus is
symptoms). For the same reason cyclopho- ria is given for fusion. Of these, the simplest is the
the most symptomatic. Maddox rod.
Blurring of vision, while reading, the letters The patient is placed at 6 mm from a bright
seem to run together.
Intermittent diplopia due to intermittent phases
of manifest squint, when overstrained muscles
relax and assume position of rest.
 Chapter Strabismus 403
27
spot of light. Maddox rod consists of four or five
cylinders of red glass joined side-by-side within
a supporting frame. A Maddox rod is now placed
in a trial frame in front of one of the eyes. The
eye with the Maddox rod sees the spot light as
a red straight line at right angles to the axis of
rod. If the
 Chapter Strabismus 404
27

FIGURES 27.8A to D: Latent squint. A. Eyes straight ahead in normal situation; B. Covering one eye leads to dissociation; C.
Note that the eye has deviated outwards under cover, which is revealed immediately after removal of the cover; D. Once fusional
mechanism is activated by removal of the cover, eyes will become straight ahead again.

FIGURE 27.9: Cover-uncover test to detect heterophoria

Maddox rod is placed with the axes horizontal, the red is then rotated so that cylinders are vertical; the red
line is vertical. Thus, the images of spotlight in the line will be below or above the spot. If there is
two eyes be- come dissimilar and fusion becomes hyperphoria. In each case, amount of deviation is
dissociated. If there is orthophoria (Figs 27.10A to D), measured by the strength of prism required to
the bright spot will appear to be in the center of correct it or it is measured on a Maddox tangent
vertical red line, if there is eso or exo- phoria red scale set on wall. Maddox rod test can be done for
line will be to one side of the spot. Maddox rod near and distance (Figs 27.11A to C).
 Chapter Strabismus 405
27

FIGURES 27.10A to D: A. Orthophoric in normal situation; B and C. In heterophoria, both eyes deviate under cover; D. On
removing the cover, both eyes resume fixation.

Maddox Wing Test ver- gence power for fusional control of the phoria
can also be measured. For this, prism vergence tests
The deviation in latent squint is often different in are done with
near vi- sion and distant vision. An exophoria
appearing more for near is regarded as
insufficiency of convergence, which gives rise to
symptoms when much near work is under- taken.
The deviation in near is tested using a Maddox wing
test (Fig. 27.12). At 33 cm from eye, when viewed
through the two slit holes of the instrument, the
fields, which are exposed to each eye is separated
by a diaphragm. The right eye sees a white arrow
pointing vertically upwards and a red arrow
pointing horizontally to left. The left eye sees a
horizontal row of figure in white and vertical row is
red. These are calibrated to read the deviation in
degrees. In orthophoria, the white arrow pointing to
white horizontal line and the red arrow pointing to
red vertical line should be at zero. Any deviation
indicates eso or exo, the amount of which can be
read off on the scale.

Prism Vergence Test

Besides the actual measurement of heterophoria the
 Chapter Strabismus 406
27
the patient seated at 6 m from light. Prisms
deviate the di- rection of light rays toward its
base and a person looking through the prism will
see it toward its apex. Highest prism,

FIGURES 27.11A to C: Maddox rod test; A. Maddox rod;

B. Retinal image with no heterophoria; C. Retinal image with
heterophoria.
 Chapter Strabismus 407
27

CONCOMITANT STRABISMUS
mechanisms. Hyperphoria in less degree should
unhesitatingly be treated in this way since in this
condition, the fusional power is limited and
hence exercises are useless.

FIGURE 27.12: Maddox wing

which can still give single vision, gives the verging

power for the particular direction tested. Base-out
prism gives the converging ability and the one with
base in, will mea- sure the divergence power. The
converging power is usu- ally 40–45 diopters (D) and
if it falls below 20D it is taken to be insufficient. The
diverging power is limited and should be 4D–5D and
normal limits of superduction and subduc- tion
(vertical vergence) are from 2D–3D.

Treatment of Heterophoria
The lower degrees of esophoria and to a less
extent of exo- phoria are almost universal. These
cause no symptoms and need no treatment. In
larger degrees of phorias and symptoms
producing phorias:
1. The refractive error must be corrected.
2. Orthoptic treatment to increase the fusional
range and muscle power using prism exercises or
with syn- optophore.
3. Operative treatment: Weak muscle is
strengthened (resected) and its antagonist
(stronger) muscle is made weaker (recessed).
This treatment is consid- ered when deviation
is large. Even if the deviation is not completely
corrected, deviation can be reduced to such a
degree as to abolish asthenopia.
4. Relieving prism: If there is no relief with any
of the above treatments, symptoms may be
relieved by or- dering prisms in spectacles to
correct the defect, i.e. prisms with apices in
the direction of deviation. This should be
avoided as far as possible. Prisms neither
correct the deviation, like surgery nor improve
the person’s ability to handle the error better,
like exer- cises. It generally tends to increase
the defect by re- laxing the fusional
 Chapter Strabismus 408
27
Concomitant strabismus is a dissociation of the
eyes where in the deviation remains the same
in all directions of gaze (Duke-Elder).

Etiology
1. Obstacles in the sensory path of binocular
reflex: De- fective vision in one eye makes it
easy for the defective eye to lose fixation:
a. Uncorrected errors of refraction.
b. Opacities in the media: Cornea, lens or vitreous.
c. Diseases of retina or optic nerve.
2. Disturbance in muscular equilibrium due to
malin- sertion or defective development of
one or more ex- trinsic muscles.
3. Decompensation of a heterophoria producing
mani- fest squint.
4. Dissociation between accommodation and
conver- gence relationship. For example, in
hypermetropia as more accommodation is
exerted to correct the error there is more
tendency for convergence and so a con-
vergent squint may develop. Similarly, in
myopia, as accommodation is not exerted to
correct the error or for near work,
convergence is weak and there is ten-
dency for divergent squint.
5. Central causes:
a. Defective development of fusional faculty.
b. Hyperexcitability of central nervous system
(CNS): As in seizure disorders, mental
retardation, etc.
Sx Symptoms
Usually, there is no symptom and the deviation of
eyes is detected by the parents or relatives.
Although the im- age of an object does not fall on
the fovea in the squint- ing eye there is no
diplopia. The reason is that this im- age is
automatically suppressed. The main feature of
concomitant squint is failure of binocular single

Signs
There are two important signs for concomitant squint:
1. The primary deviation is equal to the
secondary devi- ation: The primary deviation is
the angle of deviation of the squinting eye,
when the normal eye fixes an ob- ject. The
secondary deviation is the angle of deviation
of the normal eye, when the squinting eye is
made to fix the object. The amount of
deviation also remains the same in all
directions of gaze.
 Chapter Strabismus 409
27

2. There is no limitation of movements of eyeball FIGURE 27.13: A left constant esotropia

in any direction.

Age Incidence
Usually, concomitant squint develops during the
periods when reflexes governing binocular vision are
established,
i.e. within 5 years of age.

Types of Concomitant Squint

Depending on Deviation
• Convergent: Esotropia
• Divergent: Exotropia
• Vertical: Hypertropia or hypotropia.

Depending on Fixation Preference

Unilateral strabismus
If one eye habitually fixes and other eye squints, the
case is one of unilateral strabismus. This leads to
decreased usage of the squinting eye and leads to
decreased vision of that eye and amblyopia (Fig.
27.13).
Alternating strabismus
In alternating strabismus when one eye fixates, the
other eye deviates either inward or outward,
depending on the type of squint, but either of the
eyes can take up fixation. One important feature of
altering squint is normal vision in each eye.

Investigations of Strabismus
History
1. Age of onset of squint: Squint due to
congenital pa- resis appears at very early age.
Accommodative type of convergent squint
manifests at about 2–5 years of age, when
accommodative mechanism is stronger. In- fantile

esotropia is a convergent squint manifesting at

 Chapter Strabismus 410
27
about 6 months of age. In general, older uncover test (Figs 27.14A and B).
the age of onset and shorter the squinting True or apparent squint: When the
period, better is the prognosis. supposedly fixating eye is covered, while when
2. Any history of illness, fatigue, trauma or the patient fixes at a target, there is
delayed de- velopmental milestones. movement of the squinting eye to
3. Is the squint intermittent or constant?
4. Which eye is deviating predominantly?
5. Family history of squinting or poor eyesight
in the family.

Examination of Patient
1. Inspection: The first step is to ensure that the
apparent deviation is indeed real.
An apparent squint or pseudostrabismus is
due to the configuration of palpebral aperture,
e.g. in chil- dren with telecanthus or epicanthal
fold, medial can- thi approach the corner
eccentrically and the appear- ance of a
convergent squint results. More commonly
such an appearance is due to the divergence
between visual axis and optic axis. In
emmetropic eye, the visu- al axis cuts the
optic axis nasally, since the macula is
temporal to the optic disk. So, angle formed
between them called angle gamma is
positive. In hyperme- tropic eyes angle
gamma is also positive, but greater than
emmetropia; in myopia since visual axis and
op- tic axis coincides or latter even cuts
temporal to it, the angle gamma is negative.
Neither of these can be seen and direction of
line is judged by position of reflex at pupil. A
light shone onto cornea will cause a reflex just
nasal to center of cornea. This is termed
positive angle kappa. Greater the size of
positive angle, eye appear to look divergent. If
angle kappa is negative, eye appear to look
convergent. So in high hypermetropia, there
is an apparent divergent squint and in high
myopia there is an apparent convergent
squint.
Unilateral ptosis gives the appearance of
pseudo- hypertropia. Asymmetry of face is a
cause for pseudo vertical squint.
2. Which eye is deviated, right or left?
3. Is the deviation inwards, outwards or vertical?
4. Any opacity in cornea lens or reaction of pupil.
5. The next step is to confirm the presence of
deviation, identify the direction of deviation,
to differentiate a unilateral from alternating
type and to differentiate a concomitant from
a paralytic squint (Table 27.1).
All these can be analyzed by a cover-
 Chapter Strabismus 411
27

TABLE 27.1: Distinguishing features of non-

paralytic and paralytic
strabismus
Non-paralytic Paralytic
(concomitant) (inconcomitant)
strabismus strabismus
Primary deviation is equal Secondary deviation is greater
to secondary deviation than primary deviation
Ocular movement shows no There is limitation in the
limitation direction of action of
paralyzed muscle
No diplopia Diplopia is present
Normal head posture Abnormal head posture
False projection is absent False projection is present
No vertigo or vomiting Symptoms like vertigo,
nausea and vomiting

take up fixation, if there is a true squint. In Measurement of the angle of deviation is important in
apparent deviation, there is no movement of all cases of concomitant squint as a guide to treatment.
the supposedly deviating eye to take up The following methods are used.
fixation. Neutralization method by prism: Handheld prisms are
Type of squint: Depending on the placed in front of deviating eye with the base of
deviation of movement of the eye to take up prism
fixation, the nature of strabismus is confirmed.
That is, eye moves inwards to take up fixation in
a divergent squint and outwards in a
convergent one (Figs 27.15A to E).
Uniocular or alternating: In uniocular squint it
is one eye, which is always maintaining
fixation. Only during the cover test the
deviating eye take up fixa- tion, but goes back
to the deviated position when the cover is
uncovered. In alternating squint, both the eyes
take up fixation alternately.
In concomitant deviation, the primary
deviation is equal to the secondary deviation
unlike in incon- comitant where primary
deviation is less than sec- ondary deviation.
6. Ocular motility is tested in all direction of gaze
to find out any limitation of movement in any
direction.
7. Recording of the visual acuity to find out any
error of refraction. If so, refractive error has to be
assessed by complete cycloplegic
retinoscopy.
8. The anterior and posterior segments of the eye
has to be examined in detail to rule out any
sensory cause for the deviation.

Measurement of the Angle of Deviation

 Chapter Strabismus 412
27

FIGURES 27.14A and B: Cover-uncover test to

detect true squint. A. On covering the apparently fixing
eye, the deviating eye becomes straight. On removing the
cover, the deviating eye moves back to its original deviated
position. B. Cover test in alternating squint. Right eye
appears to have convergent squint. After occluding the left
eye, right eye becomes straight. On removal of the cover,
the covered left eye shows convergent squint and the right
eye is straight.

placed in the direction opposite to deviation or

in other words, apex of prism in the direction of
deviation, e.g. in convergent squint, prism is held
base out. Cover test is then performed. Stronger
prisms are used till the end point is reached
when prism negates ocular movements to take up
fixation. The angle of deviation is read from the
strength of the prism. A set of prism bar is shown
in Figure 27.16.
A rough estimate of angle of squint is
obtained by the reflection test of Hirschberg. This
test is useful in uncoop- erative patients and in
infants. A light is shone into the eye and the
deviation of corneal light reflex from the center
of the pupil is noted in the squinting eye. If the
reflex is situ- ated at the temporal border of the
pupil angle is about 15% and if it is at the limbus,
the angle measures about 45%.
By synoptophore: The angle of squint measured
with this equipment is very accurate.
 Chapter Strabismus 413
27

FIGURES 27.15A to E: Cover-uncover test to detect the

types of squint. A. Note that right eye is convergent; B. On
occluding the left eye, right eye moves to take up fixation; C.
On removing the cover note that left eye is convergent; D.
Now on covering the right eye, left eye takes up fixation; E.
The right eye goes back to the convergent position.

Treatment of Concomitant Squint b. Bifocals: If there is no significant refractive

error and eyes are straight for distance, but
1. Correction of any error of refraction by suitable
esotropia is present for near, bifocals with
spec- tacle correction for constant use, after
+3D should be pre- sented. The most
complete cy- cloplegic retinoscopy:
satisfactory form is the executive type of
a. Spectacles: These help in forming a sharp
bifocals where intersection cuts the lower
retinal image, which helps in fusing the two
portion of pupil.
images. It also helps in achieving the
balance between ac- commodation and
convergence in hypermetro- pia and
myopia. A convex lens will decrease the
increased accommodation in hypermetropia
and thereby convergence. A concave lens
will stimu- late accommodation and thereby
convergence, and helps in controlling the
divergence in myopia. The patient should be
re-examined in a month’s time. FIGURE 27.16: A set of prism bar
 Chapter Strabismus 414
27

2. Occlusion of the fixing eye: When vision is tex is activated.

tested, the squinting eye is usually (Fig. 27.17) FIGURE 27.17: Prism is kept with apex toward the direction
amblyopic in a constant unilateral squint and of deviation. Base in and apex out in exotropia.
an effort is made to improve the vision in it by
continuous exercise. To make this eye being
used, the other must be prevent- ed from seeing
clearly. The only satisfactory method of
ensuring this is by occlusion effected by a
patch covering the better eye for at least 6
hours a day. It may have to be continued for 6–
12 weeks when im- provement is found to occur.
It may be discontinued, if no improvement has
occurred after 6 months of strict occlusion. After
the age of 9 years conventional occlusion is
usually unsuccessful. Younger the pa- tient and
better the visual acuity, when occlusion is
started, shorter the duration of occlusion
required. The end point of occlusion therapy is
equalization of visual acuity of two eyes or
development of alteration in a unilateral
squint.
3. Alternative methods to occlusion include:
a. Atropine penalization—where atropine eye
oint- ment 1% is used to blur the vision,
especially for near in the better eye. It is
found to be useful only if vision in
amblyopic eye is good and hence this
modality is useful mostly for mild-to-
moderate amblyopia. When properly done
this technique is found to be equally
effective as occlusion for am- blyopia
treatment.
b. Manipulation of glasses: This is most effective
for high myopia. The correction in
amblyopic eye is not changed, but
correction in better eye is re- placed by
plane lens and thus, the child is forced to
use the affected eye.
c. Stripe therapy [complementary and
alternative medicine (CAM) therapy]: This
consists of view- ing of rotating high
contrast gratings of varying widths before
the amblyopic eye. In this way, en- tire
population of visual neurons in the visual cor-
 Chapter Strabismus 415
27
Orthoptic Exercises purely cosmetic.
Surgical treatment of alternating concomitant
There are three stages of exercises: squint without error of refraction is purely
1. Antisuppression exercises in which diplopia cosmetic; as fusion does not develop in this type,
aware- ness is created and the patient unless the case is seen when the patient is very
recognizes that he/she is squinting, when young or immediately after the squint has been
diplopia is developed. first noticed. Both the eyes have to be operated to
2. Once diplopia awareness is created, fusional correct the deviation, since it is usually large.
exercises are given to improve the fusional
ability and control the deviation. This can be
through synoptophore ex- ercises or exercising
prisms. The prisms of increasing strength
placed base out in front of the eye will increase
the converging ability and help in controlling
a diver- gent squint (positive fusional
amplitude) by adducting prism). Likewise,
prism of increasing strength base will help in
developing divergent potential and help (ab-
ducting prisms) to improve the negative
fusional am- plitude and in controlling
convergent squints.
3. Improvement of relative fusional convergence:
This is not the same as improvement of
fusional ampli- tude. Wherever the amount
of accommodation (A) is changed,
accommodative convergence (AC) is ei- ther
exerted or relaxed by a predetermined
amount (the AC/A ratio), but if
accommodation is constant, fusional
convergence can be exerted (positive rela-
tive convergence) or relaxed (negative
relative con- vergence) relative to that
amount of accommodation. The former, if
improved, can control exodeviation; the latter
is improved in esodeviation, so that
sufficient accommodation can be used for
clear vision without associated use of
convergence. These are achieved by using
stereogram card exercises.

Operative Treatment
Surgical treatment of concomitant unilateral
squint is indicated when the angle of squint is
10° or more when wearing correcting lenses and
in children, when orthoptic training has failed to
correct the deviation within a rea- sonable
time.
As a general rule, if indicated, it should be
undertaken early and as soon as possible before
the child develops irrevocable vision loss due to
amblyopia. Postponement until the child is 10
years or more of age usually results in
permanence of amblyopia and failure to establish
binocu- lar vision. The surgery in those cases is
 Chapter Strabismus 416
27

Type of Surgery such that muscle becomes straightened and

The overacting muscle is weakened by recession: The taut. About 1 mm of resection of lateral rectus
mus- cle is detached from its insertion and corrects 1°–2° of angle.
reattached at a posi- tion behind the site of insertion. c. Similarly, in a uniocular divergent squint, the
This will weaken its action. The weak muscle is lat- eral rectus is recessed and medial rectus
strengthened by resection: The muscle is detached resected.
from its insertion, the required amount of muscle is d. In hypertropia, the superior rectus is recessed
cut and removed, and the shortened muscle is and inferior rectus is resected.
reattached at its original position: e. In hypotropia, the inferior rectus is recessed
1. In unilateral convergent squint: and superior rectus resected. Always
a. The medial rectus is weakened or recessed recession is done before resection.
by shifting its insertion backwards. About 1 2. In alternating convergent squint: Bilateral medial
mm of recession of medial rectus corrects rec- tus recession in patients with equal vision is
about 2°–3° of angle of squint, though this both eyes and in which the deviation for distance
may vary with each surgeon. The medial is greater than that for near.
rectus should not be re- cessed more than 3. In alternating divergent squint: Bilateral lateral
5 mm. If so, weak convergence occur rectus recession is done in patients with equal
leading to discomfort in reading and near vision is both eyes and in which the deviation for
work, and to headaches.
distance is greater than that for near.
b. The lateral rectus (LR) is strengthened or
The recession and resection types of squint
resected (Figs 27.18A to D), if a portion of
surgeries are shown in Figures 27.19A and B.
the muscle is removed and reattached to
the original insertion

FIGURES 27.18A to D: Unilateral convergent squint surgery. A. Lateral rectus (LR) isolated and pulled up with muscle
hook and cut with scissors; B. The amount of recession measured with calipers; C. The LR reattached with sutures at the
recessed position; D. Conjunctiva sutured back.
 Chapter Strabismus 417
27

Signs and Symptoms

Sx Symptoms
Diplopia: It is the chief complaint. It is most
marked in the direction of action of the paralyzed
muscle. Binocu- lar diplopia results; diplopia is
FIGURES 27.19A and B: Squint surgery. A. present when both eyes are open. Diplopia may
Recession; be crossed (heteronymous) or uncrossed
B. Resection. (homonymous). If the eyes are divergent
(uncrossed) diplopia is crossed as in III nerve
NON-COMITANT paresis. If the horizontally acting muscles are
OR PARALYTIC affected, the two images are seen side by side, if
vertically acting muscles are affected one image
SQUINT appears at a lower level than other. If oblique
Definition muscles areb.affected,
Injury to muscle.
one of the images appears
tilted. c. Disease of muscle or myopathy, as in
Non-comitant squint is a deviation caused by
thyrotoxic
Vertigo and nausea: These myopathy,
symptoms ocular myopathy.
are due to
paralysis or restriction of extraocular muscles and
it is a condi- tion in which deviation of eye varies diplo- pia and false projection of images. These
in different direc- tions of gaze. unpleasant symptoms are counteracted partially
by altering the position of head or completely by
shutting or cover- ing the affected eye. In
Etiology congenital strabismus, these symptoms are not
Any lesion of the cranial nerve supplying the muscle. obtrusive since the vision in one eye is
The lesion may be at any of the following suppressed. In acquired cases, they are at first
locations:
1. Lesion of motor nerve nucleus:
a. Congenital, absence of nucleus.
b. Vascular causes affecting the nucleus
(midbrain).
c. Toxic causes like endogenous (diphtheria),
exoge- nous (alcohol, lead, botulism, thiamine
deficiency).
d. Inflammatory lesion affecting muscles, e.g.
en- cephalitis, neurosyphilis.
2. Lesions of the nerve root just beyond the nucleus:
In- flammation, neoplasm, vascular cause.
3. Lesions of nerve trunk:
a. Trauma: Direct injury to trunk, pressure on
the nerve by hematoma, stretching of nerve
due to ce- rebral edema or intracranial
hemorrhage.
b. Neuritis: As in diabetes, vitamin deficiency or
lead poisoning.
c. Toxic causes: Alcohol.
d. Pressure on the nerve by aneurysm or
neoplasm.
4. Lesion of muscle itself:
a. Congenital absence or maldevelopment of
muscle.
 Chapter Strabismus 418
27

Signs
Limitation of extraocular movement: In paralysis of an
ocu- lar muscle, the ability to turn the eye in the
direction of normal action of muscle is diminished
or lost. Limitation is tested roughly by fixing
patient’s head and telling him/ her to follow the
movements of surgeon’s finger. The fin- ger
should be held vertically to test horizontal
movements and horizontally in testing vertical
movements.
Primary deviation is less than the secondary deviation:
Pri- mary deviation is the deviation of the
squinting eye and secondary deviation is the
deviation of the unaffected eye when the
deviating eye is forced to take up fixation (Figs
27.20A and B). The relative movements of the
two eyes when each is used for fixation are of
importance. For ex- ample, if the right lateral
rectus is paralyzed and the left eye is covered,
then on attempting to fix an object situated to
right with the paralyzed eye, left eye will
deviate very much to the right under cover. The
deviation in the left eye is the secondary
deviation; the deviation of right eye when the left
eye fixates is the primary deviation. The reason for
this difference is due to the fact that equal
motor energy goes to the synergic muscles of
both eyes in any ocular
 Chapter Strabismus 419
27
head to compensate for defective vertical
movements of one eye. It is distinguished from true
torticollis due to contraction

FIGURES 27.20A and B: Primary and secondary deviation.

A. Deviation of the squinting eye when the normal eye is used
for fixing; B. When the normal eye is occluded and the
squinting eye is forced to take up fixation, the normal eye
undercover is deviating more due to the increased nervous
energy exerted for making the paralyzed eye to work.

movement. When the paralyzed muscle tries to fix an

object in the field of action of the affected muscle (an
object on the right side in a case of right lateral rectus
palsy), extra motor energy is necessary to fix with the
squinting eye and this will be shared by the normal
eye and so it deviates more.
Visual acuity: This is normal in each eye and there
is no amblyopia.
False orientation: This is a necessary accompaniment
of binocular diplopia. Suppose a patient whose right
lateral rectus is paralyzed, shuts the left eye and
attempts to fix an object situated to the right.
When the patient is asked to point at the object
with the extended index finger, the finger will pass
considerably to the right of object. This is called
false projection. It depends on some principle as
increase of secondary deviation. The object is
projected according to amount of nervous energy
exerted. As this is greater than that exerted normally,
the object is projected too far in the direction of
action of paralyzed muscle. As soon as he realizes
that the finger has overshot the target, suitable
adjustment will be made.
Anomalous/compensatory head postures: The patient
holds the head so that the face is turned in the
direction of action of paralyzed muscle. For example,
in paralysis of right lat- eral rectus the patient keeps
his head turned to the right. This is to decrease the
need for looking toward the right side and thus, to
lessen the diplopia and its attendant un- pleasant
consequences as much as possible. The position is
unconsciously adopted. Any anomalous posture may
mask an underlying deviation.
Ocular torticollis is a term applied to tilling of
 Chapter Strabismus 420
27
of sternomastoid muscle. In that, there is simple occurs on looking to paralyzed side.
tilling of head without rotation of the chin
toward the opposite shoulder and sternomastoid
is not unduly contracted. It occurs in cases of
congenital origin, but it also follow trau- matic
fractures, if binocular single vision can be obtained
by adjusting head posture. The vertical squint is
made manifest by placing the head straight when
diplopia is also elicited.
Sequelae of external ocular muscle palsy: If
the pa- ralysis is of some duration, certain
changes occur in the muscles. These are:
1. Overaction of contralateral synergist.
2. Contracture of the direct antagonist.
3. Secondary inhibitional palsy of the
contralateral an- tagonist.
For example, in a case of right lateral rectus palsy
there is overaction of left medial rectus
(contralateral synergist), contracture of right
medial rectus (direct antagonist) and secondary
palsy of left lateral rectus (contralateral antago-
nist). Similarly, in case of left superior oblique
palsy there are overaction of right inferior
rectus, contracture of left inferior oblique and
secondary inhibitional palsy of right superior
rectus.
Varieties of ocular paralysis: If one muscle alone is
affected, it is generally the lateral rectus or
superior oblique, since each of these is supplied by
an independent nerve. Afflic- tion of several
muscles are due to paralysis of III nerve.

Total Ophthalmoplegia
Total ophthalmoplegia is the condition when the
entire motor apparatus of the eye, including the
intrinsic mus- cles (ciliary muscle, sphincter and
dilator pupillae), the extrinsic muscles and the
levator palpebrae superioris, is paralyzed.

External Ophthalmoplegia
External ophthalmoplegia is the condition when
only the extrinsic muscles including the levator are
paralyzed spar- ing the ciliary muscle, sphincter
and dilator pupillae:
1. Paralysis of lateral rectus: This is the
commonest. The clinical signs are:
a. The eyeball is rotated inwards.
b. There is restriction of movement of
eyeball out- wards.
c. There is face turn toward the paralyzed side.
d. Homonymous (uncrossed) diplopia
 Chapter Strabismus 421
27

2. Paralysis of superior oblique: d. History of trauma/head injury.

a. The eyeball is deviated upwards and e. History of any anomalous head posture.
inwards; there is limitation of movement
downward and toward the sound side.
b. The face is turned downwards (chin
depressed) and toward the sound side.
Head is tilted toward the sound side.
c. Homonymous diplopia in looking down and
in, the false image is lower with upper end
tilted to- ward true image. The patient has
great difficulty in going downstairs and
vertigo is the particular symptom.
When the eyes are crossed, the diplopia is
un- crossed and vice versa.
3. Paralysis of III nerve: In complete paralysis of III
nerve there is ptosis, which prevents diplopia.
On raising the lid with a finger, the eye is seen
to be deviated out- wards and rotated
internally owing to the tone of two unparalyzed
muscles, viz. lateral rectus and superior oblique:
a. Limitation of movements in all directions
except outwards.
b. Pupil is mid-dilated and inactive.
c. Accommodation is completely lost.
d. Paralysis of III nerve is often incomplete
and in- dividual muscles may occasionally
be affected alone.
Misdirection in regeneration of III cranial nerve
is common after total interruption of function
usually by head trauma. The ocular signs are:
1. Pseudo-Graefe lid sign: As eye attempts to
move downwards, upper lid retracts.
2. Pseudo-Argyll Robertson pupil: There is a slow
light re- flex, but better constriction of pupil to the
near reflex.
3. Horizontal-gaze lid dyskinesis: The upper lid
retracts as eye is adducted and falls as the eye
is abducted.
4. Adduction on attempted vertical gaze.

Investigations of a
Case of Paralytic
Squint
1. History:
a. Regarding the onset and associated illness
that may be a precipitating factor.
b. History of symptoms like vertigo,
vomiting.
c. History of diplopia, which is the most
common complaint.
 Chapter Strabismus 422
27
2. Examination:
a. In case of any anomalous head posture,
head should be made straight before
examination to make the deviation
evident.
b. The first procedure is to cover one eye
and deter- mine, if the diplopia is
uniocular or binocular.
c. Cover test to find out whether secondary
devia- tion is more than the primary
deviation.
d. If diplopia is binocular, the patient should
fix at the surgeon’s finger and the field
of fixation of each eye is tested. The
limitation of movements in each
deviation is carefully documented.
e. In such cases diplopia must be
investigated by more specific tests.

Diplopia Charting
A spectacle containing a red glass for the right
eye and a green glass for the left eye is worn by
the patient to distin- guish the images (Fig.
27.21). In the darkroom a bar of light through a
stenopaic slit in a handheld torch is moved in the
field of binocular fixation at a distance of 4 ft (120
cm) from the patient, patient’s head being kept
stationary. The posi- tion of the images is
accurately recorded on a chart with nine squares
showing the nine gaze positions. The follow- ing
data are derived:
• Areas of single vision and diplopia
• Distance between two images in the areas of diplopia
• Whether the images are on the same level or not
• Whether the image is inclined or both are erect
• Whether the diplopia is crossed or uncrossed (Fig.
27.22). These data, if concordant, are sufficient
to diagnose the paralysis. The false image, which
is frequently tilted, is the fainter of two. Find the
position of gaze when the separation of the
image is maximal. In that position the farthest
displaced image belongs to eye with muscle palsy.
By covering one eye, it can be shown as to
which eye this image belongs.
There are other methods to find out exactly,
which muscle is affected by testing using a Hess
chart, Les screen

FIGURE 27.21: Patient wearing red-green goggles

 Chapter Strabismus 413
27

2. To avoid diplopia, occlusion of the better eye

may be done, so that movement to the
paralyzed side may be initiated by the affected
eye without diplopia and contracture of the
antagonist of the paralyzed muscle does not
develop.
3. Relieving prisms may be given, but this
treatment is rarely of much use, since there is
variation in amount of deviation in different
gaze position and variation over time.
FIGURE 27.22: Testing diplopia
4. Surgery: Sufficient time should be allowed for
recov- ery before decisions on surgery is taken. It
or Lancaster screen. Synoptophore examination can
is indicated when the deviation has become
also be done in different position.
stabilized and no more spontaneous recovery
is taking place, and be- fore permanent
Treatment of Paralytic Squint structural changes like contracture develop in the
1. Etiological treatment should be directed to the synergic muscles. The operative mea- sures
cause of palsy. consist of weakening the antagonistic muscles by
recession and strengthening the synergic
muscles by resection.
 Chapter Strabismus 414
27

SECTION 7
Ophthalmic Surgeries
Surgery for
Cataract
2
8
Girija Devi PS

correct, but the lack of fine instruments of modern

EVOLUTION OF CATARACT era
SURGERY
Cataract surgery has a long and interesting history
dating from the medieval period. The first
documented cataract surgery was done in the 5th
century BC by the renowned Indian physician of that
period ‘Sushruta’. At that time the lens was supposed
to be occupying the center of the eye- ball and
‘cataract’ is some bad ‘humor’ that flowed in front of
the lens.

COUCHING
The surgery practiced at that period was
‘couching’. The aim was to displace the so called
abnormal humor from its position in front of the lens.
The patient was restrained by someone, while the
surgeon sits in front of the patient and a small
incision is made inferiorly just behind the corneo-
scleral junction. A couching needle is introduced
through this wound, and the cataractous lens is
pushed backwards and inferiorly leaving the pupillary
area aphakic, and thus giving a momentary
improvement in vision to a person blind due to
cataract.
Due to lack of asepsis and the inflammation
induced by the lens material liberated into the eye,
very few people retained the vision.

EARLY PHASE
(EXTRACAPSULAR CATARACT
EXTRACTION)
By 17th century, the correct position of the lens was
iden- tified and cataract was recognized as the
opacification of the lens.
Jacques Daviel is the Father of Modern Cataract
sur- gery. He developed instruments with which
incision was put through the inferior cornea, which
was then extend- ed with scissors. The cornea is
lifted up, the interior cap- sule is incised, the
nucleus expressed out and the ante- rior chamber
(AC) was also washed out. The concept was
and fine suture material to close the wound,
and lack of knowledge about aseptic
precautions lead to a high rate of postoperative
complications. But this technique was widely
accepted. The von Graefe’s cataract knife
devel- oped by von Graefe in 19th century
created a better in- cision, which reduced the
incidence of infection and iris prolapse. Even
then, since the wound is not sutured, the patient
has to lie down in a supine position without
mov- ing the head for at least 2 weeks for the
wound to heal satisfactorily without
complications.

Even with a better incision using the von CATARACT

Graefe’s knife, the retained cortical matter
produced increased inflam- mation and
interference with regaining satisfactory vi- sion.
This led the surgeons to search for methods for
re- moving the opaque lens in its entirety. Samuel
Sharp first performed a successful intracapsular
cataract extraction (ICCE) in 1753. How to rupture
or lyse the suspensory lig- ament to release the
lens was a problem. Sir Henry Smith, an English
Surgeon, who was working in Punjab in India
started using a muscle hook to apply pressure
externally at the inferior limbus to ‘tumble’ the
lens out through a lim- bal incision made at
superior limbus. His technique came to be known
as the ‘Smith Indian technique’ and gained wide
popularity. The next improvement was the use
of a toothed lens forceps to gently grasp the
anterior capsule, and rock it side to and to
rupture the zonules. The next device to be
introduced was ‘erysiphake’, which is a small
suction cup applied to the anterior lens capsule to
create a vacuum and suction force, and the
lens is removed.

Modern Intracapsular Cataract MODERN CATARACT SURGERY

Surgery
The introduction of cryoapplication started the
modern era of ICCE. A cryoprobe, which is
cooled with nitrous
 Chapter 28 Surgery for Cataract 418

oxide to temperatures below 0°C, is applied to the

anterior capsule. An ice ball forms fixing the Extracapsular Cataract
anterior capsule to the tip of the cryoprobe and the
zonules are ruptured with gentle rocking movements,
Extraction in Modern Era
and the lens attached to the tip of the cryoprobe is The ECCE has come back with modern sophisticated
removed. Lens extraction became easy with this equipments and the intraocular lenses (IOLs), it
equipment. has
With modern sterilization techniques, fine suture
ma- terial to suture the incision and operation
microscopes, which increased the precision of the
surgery, ICCE be- came a successful operation
for cataract.
This surgery is still in practice in many parts of
the developing countries where large volume of
blind popu- lation has to the treated without the
recent sophisticated equipments for today’s
cataract surgery.
It is still useful in the management of subluxated
cata- ractous lens.
Though a successful procedure, ICCE had
certain limitations:
1. Not useful in young people who have strong
zonules, which are not easily torn and firm
adhesion of the vit- reous to the posterior
capsule, which can lead to vit- reous loss.
Zonules can be lysed with enzymes like -
chymotrypsin, but due to the strong attachment
of the vitreous to the posterior capsule vitreous
loss with all its complication will follow ICCE in
children or young adults.
2. The patient is still dependent on strong convex
lenses for visual rehabilitation after ICCE. The
vision through the high convex lens is associated
with several prob- lems and patients are usually
not happy with aphakic spectacle correction.
Many of them refuse to wear the aphakic
spectacles and hence have no functional vi- sual
improvement.
3. The ICCE requires a fairly large incision, which is
as- sociated with complications like delayed
wound heal- ing, iris prolapse, vitreous loss and
most importantly, significant astigmatism even in
uncomplicated cases.
4. Loss of barrier between the posterior and
anterior segment leads to anterior movement of
the vitreous. This plays a role in the high
incidence of postoperative complications like
cystoid macular edema and rheg- matogenous
retinal detachment.
These problems associated with ICCE led to the
renais- sance of extracapsular cataract extraction
(ECCE).
 Chapter 28 Surgery for Cataract 419
become a surgery with minimum complications routine for the patient.
and ad- equate visual rehabilitation. The surgery,
which was done sutureless earlier, because fine Intraocular Lens Implantation
sutures and were not avail- able, has become
sutureless again because the decrease in the size The visual rehabilitation after ICCE using high-
of the incision and the valve-like technique of powered convex lens is very unsatisfactory due to
making the incision made suturing unnecessary. the image mag- nification and distortion of images,
Once it was done without anesthesia because no while looking through
local or systemic anesthetic was available. Now
it can be done with no an- esthesia, only local
anesthetic drops are required. ECCE of the
modern era can be:
1. The conventional ECCE with suturing of the wound.
2. Manual small incision cataract surgery (MSICS)
where the nucleus is removed manually
through a valve like sclerocorneal incision
followed by aspiration of all re- sidual lens
matter.
The introduction of many new instruments
made the new ECCE a safer technique.
These include:
a. Cystitomes that can be fashioned from 26
gauge needles to do controlled
capsulotomy (opening in the anterior
capsule).
b. Irrigation and aspiration cannula, which
infuse fluid into the eye and aspirate the
cortical matter simultaneously.
c. The introduction of ophthalmic viscoelastic
sur- gical devices (OVDs), which help to
maintain the AC, and protect the corneal
endothelium during manipulations inside
the eye, increased the suc- cess rate and
decreased the complications.
The added advantage that highly
expensive equipments like
phacoemulsifier are not required for MSICS,
made it highly suitable for developing
countries like India and this surgery is very
popu- lar here.
3. Phacoemulsification: In MSICS, the incision has
to be big enough to manipulate the nucleus
out of the eye. In phacoemulsification,
ultrasound waves are used to fragment the
nucleus into microscopic particles and they
are aspirated, while fluid is infused into the
eye—all done through a single small
handpiece of the phacoemulsifying equipment.
This allowed cataract surgery to be done
through a small incision 2.8 or even lesser in
size. The IOLs are also suitably modi- fied
foldable or injectable type to introduce
through this small incision. Smaller the
incision size, lesser the postoperative
astigmatism and earlier the return to normal
 Chapter 28 Surgery for Cataract 420

the side of the glasses. This led people to search for So, patients have to remain partially blind for a
better alternatives to spectacles, especially methods variable period of time waiting for the right time
to replace an artificial lens for the God-given lens, for surgery. With the advent of modern techniques,
which is removed. Harold Ridley was the first this scenario has completely changed. Cataract surgery
surgeon who introduced IOLs made of polymethyl and the replacement of the lens with artificial lens
methacrylate, into the posterior chamber after ECCE. can be
The initial attempts for IOL implan- tation met with
high rate of complications due to poor lens shapes
and chemical reactions produced by the methods of
sterilization of the lenses. Continued research in
this field led to improvements in lens design and its
steriliza- tion techniques, and now IOL implantation is
hardly as- sociated with any complication in the hands
of an experi-
enced surgeon.
The introduction of foldable and injectable
lenses al- lows implantation of IOL through the
small incision of phacoemulsification surgery.
The visual rehabilitation with IOLs is excellent
except for one factor, i.e. lack of accommodation.
Even little chil- dren who have to undergo cataract
surgery for congenital or traumatic cataract have to
wear bifocal spectacles to facilitate near vision. Now
multifocal and accommodative lens are available,
which help to see clearly at all distances with less
dependence on glasses. These lenses are not as
perfect as God-given lenses and with the
continuing re- search near perfect lenses can be
expected in future.
The cataract surgery now has come to full circle.
From the days of couching, which was done
through a 2 mm incision without anesthesia, it has
come to the days of phacoemulsification done
through 2 mm or less incision with only topical
anesthetic drops. The modern cataract surgery has
now become a simple and almost outpatient
procedure for the patients, but demands a high
level of skill and precision, and consequently a
long and steep learning curve for the surgeon.

EVALUATION FOR
CATARACT
SURGERY
When to do Surgery?
In the era of ICCE, surgery was done only when the
len- ticular opacity has progressed to mature
cataract stage. The reason was that the rupturing of
the zonular fibers to remove the lens intact was
easier when the cataract has reached maturity.
 Chapter 28 Surgery for Cataract 421
done even in the early stages of lenticular 2. In patients requiring posterior segment surgeries
opacification. The time of surgery depends on like retinal detachment surgery or vitrectomy,
the visual requirements of the patient and the cataract surgery is indicated if the opacification
interference in the activities of daily living by the of the lens is interfering with clear visualization
visual disturbance caused by the cataract. of the posterior segment.

Indication for Surgery

Visual Disability
If the reduced visual acuity caused by the cataract
is inter- fering with daily activities, surgery is
indicated. This will vary from person to person
depending on education, job requirements,
routine activities, etc. All posterior subcap- sular
cataracts (PSCs) can grossly interfere with the
visual acuity especially the near vision and reading
and an office worker will require early surgery to
continue his/her offi- cial activities. Similarly PSC
and anterior cortical cataract will produce glare
during night driving, and a person who is a driver
by profession will require surgery to continue
the profession even before significant drop in
vision has occurred. At the same time an old
illiterate person leading a sedentary life or a
manual laborer will be able to con- tinue routine
activities till the visual acuity is considerably
decreased. So the right time for cataract
surgery is a de- cision to be taken by the
patient and the doctor together, depending on
the interference in routine activities and the
desire to improve the vision.

Professional Requirements
Certain professions like that of a pilot will require
perfect vision and surgery has to be done if the
best corrected vi- sual acuity (BCVA) is less than
normal, and cataract is the reason for the
decrease in vision.

Cosmetic
A total cataract in an eye with long-standing
retinal de- tachment or glaucomatous optic
atrophy will have to be surgically removed for
cosmetic purpose even though there is no
chance of improving vision by surgery.

Medical Indication
1. Condition like phacolytic glaucoma,
phacomorphic glaucoma or dislocation of the
lens into the AC de- mand lens removal to
cure the secondary glaucoma.
 Chapter 28 Surgery for Cataract 422

Preoperative Evaluation diseases. This is particularly important in people with

diabetes who may have diabetic retinopathy. A past
A patient with some decrease in vision and some
history of recurrent attacks of redness and pain, which
catarac- tous changes in the lens has to be
subsided with or without treatment is important
properly evaluated to solve the following issues:
1. Is the drop in vision solely due to cataract or is
there some other pathology?
2. Even in patient with other ocular problems
affecting vision, e.g. glaucoma, is cataract
surgery going to pro- duce a significant
improvement in vision?
3. Is the lens capacity secondary to some
systemic or ocular problem?
4. Whether the general condition is fit for
undergoing cataract surgery?
5. Is there any local ocular problem that can
adversely af- fect the procedure, e.g. chronic
dacryocystitis, dry eye, etc.
The preoperative evaluation is done to get
satisfactory answers to the above questions.

Clinical History
A proper and careful elicitation of history is important
in:
1. Determining the type of cataract.
2. Whether there are other ocular problems
affecting the vision as well as the outcome
after surgery.
3. Any systemic problem that has to be taken care
of be- fore surgery.

Nature of Visual Loss

Cataract is the most common cause for gradual loss
of vi- sion. But all patients having gradual loss of
vision are not suffering from cataract. Other common
causes for gradual loss of vision like primary open
angle glaucoma, uncor- rected refractive error or
macular degeneration may be the reason for
defective vision, or these problems may be
coexisting with cataract.
History of any sudden loss of vision or sudden
drop in vision at some point in the course of
gradual loss of vision is important and may point
toward some other pathology like retinal detachment,
vascular occlusion or exudative type of macular
degeneration.
Possibility of any posterior segment problem—
then patients have to be clearly evaluated for
deciding whether there are any co-existing posterior
segment problems and whether the lenticular opacity
is sufficiently advanced so that cataract surgery will
give some visual benefits in spite of the other ocular
 Chapter 28 Surgery for Cataract 423
since this may indicate the presence of chronic out any ocular problem that may adversely affect the
anterior uveitis, and the cataract may be a visual recov- ery (e.g. macular degeneration), as well
complicated cataract. as adversely affect the natural course of cataract
A history of previous trauma to the eye is surgery (e.g. chronic dacryo- cystitis, which can
important for two reasons. This may mean lead to postoperative infection).
that:
1. The cataract is of traumatic origin.
2. The patient may have other damage to the
eye due to trauma like secondary glaucoma,
macular degenera- tion or retinal
detachment, which may adversely af- fect
the postoperative visual recovery.
A careful elicitation of clinical history is
important in all uniocular cataracts and
cataracts in young people to rule out a
traumatic, complicated or metabolic cataract.

Medical History
A complete medical history is an important part
of the preoperative evaluation:
1. A careful history is important regarding
medical prob- lems like diabetes mellitus
(DM), hypertension (HT), coronary artery
disease, chronic obstructive pulmo- nary
disease (COPD), bleeding disorders or any gen-
eral medical problems, which necessitate long-
term steroid therapy.
2. Any history of taking any immunosuppressants
or an- ticoagulants is also important since drug
modification is necessary during the time
of surgery.
3. Any history of drug allergy is also important
since this necessitates careful drug allergy
testing for all medi- cines and local anesthetics
that have to be used at the time of surgery.

Preoperative Clinical Examination

Systemic Examination
The general health of the patient should be
assessed to determine whether he/she can
safely undergo cataract surgery. If the patient is
suffering from DM, HT, coronary heart diseases,
chronic airway obstruction or psychiatric disorder,
these illnesses have to be medically controlled
before surgery. A general medical check-up, and
routine blood and urine examination are essential
to rule out any of the systemic problems.

Ocular Examination
A careful ocular examination is essential to rule
 Chapter 28 Surgery for Cataract 424

Visual acuity and refraction: The distance as well as the can accompany pseudoexfo- liative glaucoma with
near visual acuity should be measured both unaided cataract.
as well as with the most suitable spectacle correction Pupil: Examination of the pupil is very important in the
decided by re- fraction (BCVA). preoperative evaluation of cataract. The pupillary
Lids and ocular adnexa: Look for any lid abnormalities reaction will be brisk even in hypermature cataract. A
like lagophthalmos, ectropion or entropion. They swinging
have to be corrected before cataract surgery. Any
infection like hor- deolum internum or externum, or
blepharitis has to be controlled before under taking
surgery. Dacryocystitis, if present, can lead to
postoperative endophthalmitis. Sy- ringing of
nasolacrimal ducts should be done to rule out
chronic dacryocystitis. If dacryocystitis is present a
dac- ryocystectomy (DCT) or
dacryocystorhinostomy (DCR) should be done
before undertaking cataract surgery.
Ocular motility: A pre-existing squint means the
patient may be having amblyopia in the squinting
eye. A cataract surgery in that eye cannot be
expected to give good visual improvement.
Conjunctivitis: If present should be treated and
controlled before planning surgery.
Cornea: Careful examination of the cornea under slit
lamp is essential to rule out any increase in corneal
thickness or corneal edema, which denotes a
compromised endothe- lial layer. The surgical
trauma can aggravate this problem and hamper
visual recovery. If the patient had undergone a
previous refractive surgery, the corneal thickness and
clarity have to be assessed. Special techniques for
IOL power calculation has to be made in these
patients.
Any keratic precipitates, old or new is a
warning sign about the presence of uveitis. The
cataract in this situation may be a complicated
cataract due to uveitis. All inflam- mation has to be
controlled before surgery and the surgery is usually
undertaken under cover of systemic steroids.
Anterior chamber: A shallow AC indicates narrow
angle or an intumescent lens and gonioscopy
should be done to rule out narrow angle glaucoma.
Gonioscopy is essential to rule out angle
abnormalities or abnormal vessels in the angle if an
AC IOL is planned. Look for any cells or flare in the
AC, which means an active anterior uveitis, which is a
contraindication for cataract surgery.
Iris: Look for posterior synechiae, which indicates a
coex- isting or previous anterior uveitis. Inflammation
has to be controlled before planning cataract surgery.
Look for any exfoliative material on iris, which
mean weak zonules or secondary glaucoma, which
 Chapter 28 Surgery for Cataract 425
flash test should be done to rule out any
relative afferent pupillary defect.
Look for any iris shadow, while examining
the pupil. Throw the light from one side so that the
light falls obliquely on the lens near the edge of
the pupil. If the lens is partially opaque, as in an
immature cataract, the clear lens matter will act as
a mirror with the opaque lens fibers underneath
simulating the silvering at the back of a mirror and
a cres- centic shadow of the iris will be seen at the
edge of the pupil on the same side as the light (Fig.
28.1). If the lens is totally opaque (mature
cataract) no shadow will be seen.
Crystalline lens: The lens has to be examined under
the slit lamp to assess the level of opacity whether
posterior sub- capsular, nuclear or cortical
cataract. Assess whether the lenticular opacity
correlates with the decrease in vision. If the
cataract is insignificant and the decrease in
vision is considerable, the patient has to be
carefully evaluated for some other problem
affecting vision.
Look for any subluxation or phacodonesis of
the lens, which mean the zonular integrity is
compromised.
Fundus evaluation: The evaluation of fundus after
dilata- tion by both direct and indirect
ophthalmoscope is essen- tial to evaluate the
optic disk (for any cupping or pallor), the retinal
vessels (for any evidence of fresh or old vascu-
litis or vascular occlusion), macula (for any
macular de- generation) and the peripheral
retina for any peripheral retinal degeneration
or retinal detachment.
In patients in whom the cataract is too dense
for prop- er visualization of the fundus, special
investigations like B scan must be done in
addition to clinical tests like:
1. Projection of light.
2. Gross color vision like identifying the red or
green color of a fairly large object.
3. Macular function tests to access the functional
integ- rity of the retina.
Electroretinography (ERG) and visually evoked
poten- tial (VEP) may be indicated in specific
circumstances.

FIGURE 28.1: Demonstration of iris shadow

 Chapter 28 Surgery for Cataract 426

Routine Investigations In a darkroom two pen lights 2 cm in diameter are

held close together 60 cm away from the eye and
1. Checking intraocular pressure (IOP) is mandatory gradually sep- arated until the patient can perceive
in all patients to undergo cataract surgery. If two lights.
there is any suspicious optic disk cupping or
elevation of IOP, fur- ther tests are indicated to
rule out glaucoma. If glau- coma is detected or
already existing, it is important to decide
whether:
a. Cataract and glaucoma surgery may be done
as a combined procedure.
b. Glaucoma surgery is done first and cataract
sur- gery later.
c. Cataract surgery alone is done and
glaucoma managed by topical antiglaucoma
medications.
2. Projection of light: This test is important in all
patients with advanced cataract, so that visual
acuity is re- duced to PL or HM. If projection of
light is inaccurate, a posterior segment
problem like glaucoma or optic atrophy has to
be suspected.
3. Gross color vision: Even in the presence of
advanced cataract, the patient will be able to
identify primary colors like red, green or blue.
4. Visual field testing by confrontation method
should be done in all cataract patients. If there is
a visual field loss, patient has to be evaluated
for glaucoma, optic nerve disease or retinal
detachment.

Special Tests
Macular Function Tests
The visual recovery after surgery depends to a large
extent on the functional integrity of the macula. So
a series of special tests are designed to assess the
macula, especially in cases where the lenticular
capacity is dense enough to prevent proper
visualization of retina.

Maddox Rod Test

Maddox rod test is a simple, but very reliable test.
The Maddox rod consists of a series of cylinders
placed side by side in a frame. It converts a point
of light into a line. The patient is asked to look at a
light at a distance of 1/3 m through the Maddox rod
with the other eye occluded. Any brakes, gaps or
distortion of the line denotes a macular pathology.

Two Point Discrimination Test

 Chapter 28 Surgery for Cataract 427
With HM vision—two lights are perceived Electroretinography and Visually
when they are 12 cm apart: Evoked Potential
• CF to 3/60–7 cm apart
• More than 3/60–5 cm apart. When other tests are inconclusive,
electroretinography or visually evoked potential
testing can be done to evaluate retinal and optic
Color Perception Test
nerve functions.
The patient wears red-green goggles and a light
is shown through the spectacle. Blue color is
tested with the blue light of a blue field
endoscope. If the patient can perceive at least
one color that indicates a good macular function.

Blue Field Entoptic

Phenomenon (Flying
Corpuscle) Test
The retina is uniformly illuminated with the blue
light of an endoscope held close to the eye. The
number of white cor- puscles moving in the
parafoveal capillaries will be visual- ized as an
entoptic phenomenon if the photoreceptors and
capillaries at the macula are intact. A normal
response is 15 or more corpuscles moving in a
pulsatile manner. Many pa- tients will find
difficult to understand this test.

Purkinje Vascular Entoptic Test

Like blue field test this test, is also subjective. A
bright source of light is shined through the
patient’s closed eye- lids. If the patient can
perceive images of the retinal vascu- lature, the
retina is probably attached and healthy.

Estimation of Potential Visual Acuity

The possible postoperative visual acuity can be
measured by two methods.
Laser interferometry: In this method monochromatic
he- lium-neon laser light from two sources
produce a fringe pattern on the retina. The
transmission of these patterns is not affected by
the lenticular opacity. By gradually de- creasing
the spacing of the pattern, the potential visual
acuity is estimated. The limitation of this
technique is that a small foveal lesion that can
considerably diminish the visual acuity may go
undetected.
Potential acuity meter: A miniature Snellen’s chart is
pro- jected into the eye and the patient is asked
to read the let- ters on the chart. PAM tends to
be most accurate in eyes with visual acuity of
6/60 or better.
 Chapter 28 Surgery for Cataract 428

Preoperative Measurements day of surgery. Some surgeons do not use any systemic
antibiotics at all. Some surgeons like to give
Biometry is done to calculate the IOL power. Corneal acetazolamide tablets 250 mg on the previous night
pow- er is first detected using keratometry or corneal and the morning before surgery, to keep the IOP low.
topogra- phy equipment. The actual length of the
Again, many surgeons do not use this. Pa- tient is
eye is measured using A scan—ultrasonography.
asked to apply some broad spectrum topical an-
From these two measure- ments (keratometry
tibiotic drops starting from the previous day of
reading and axial length of the eye) IOL power can
surgery.
be calculated. All ultrasound machines have built in
software to calculate the IOL power if the cor- neal
keratometry value is also given to the machine.
Ac- curate IOL power calculation is essential for
proper visual rehabilitation after cataract
surgery.
Corneal pachymetry is the method to measure the
cor- neal thickness. If there is endothelial dysfunction
the cen- tral corneal thickness will be more than
640 µ and these patients are at a higher risk for
developing postoperative endothelial
decompensation and corneal edema. Specu- lar
microscopy is used to determine the endothelial
cell density per millimeter square as well as its
morphologi- cal abnormalities like polymegathism
(increase in size) or pleomorphism (irregular shape).
Eyes with such abnor- malities are at increased risk
for developing postoperative endothelial
decompensation.

Preoperative Patient Preparation

Before surgery the patient and their family
members should be informed about the procedure,
the pre- and post-operative care, the visual
improvement that can be expected and the risk for
any complication, sight threaten- ing as well as life-
threatening. A written informed consent should be
obtained from the patient before surgery.
Any medical problem like DM, HT, COPD, any
cardiac condition, etc. should be well-controlled
before surgery.
Any ocular or periocular infections like
blepharitis, conjunctivitis, chronic dacryocystitis
should be treated and cured in advance before
deciding on the surgery. Pa- tients with chronic
dacryocystitis should undergo DCR or DCT and
postoperative inflammation should subside before
cataract surgery. If the patient has glaucoma this
should be medically controlled before cataract
surgery.
The preoperative medications and preparation of
the eye will vary from center to center and from
one surgeon to another. Patient is usually started on
a broad spectrum systemic antibiotic on the previous
 Chapter 28 Surgery for Cataract 429
Since non-steroidal anti-inflammatory drops The disadvantages are the longer time interval
may pre- vent intraoperative pupillary be- tween the injection and the onset of action,
constriction, this may be started 2–3 days peribulbar swelling and slightly less effectiveness
before surgery. On the day of surgery, the than retrobulbar anesthesia.
pupils are dilated with tropicamide The advantages are lesser incidence of
phenylephrine drops and sometimes complications compared to retrobulbar anesthesia,
cyclopentolate drops are also ap- plied. The but the risk of globe perforation is not totally
eye- lashes may be clipped before surgery or eliminated.
they are left alone and kept away from the
surgical field with the help of adhesive surgical
eye drapes. Povidone- iodine eyedrops are
instilled just before surgery because they have a
broader spectrum of action against bacteria, fungi
and virus than any antibiotic drops.

Anesthesia for Cataract Surgery

Cataract surgery has evolved from surgery
without any anesthesia in the historical era
through retrobulbar anes- thesia, peribulbar and
sub-Tenon’s anesthesia to today no anesthesia
technique, i.e. no anesthetic injection is given,
only topical anesthesia drops are used.
Retrobulbar anesthesia: The local anesthetic solution
is xy- locaine 2% injection mixed with 1 in
1,000,000 adrenaline and hyaluronidase 150 units
in 20 mL of xylocaine. 2–4 mL of this mixture is
injected inside the muscle cone either through
the lower lid at the junction between the lateral
and inferior walls of the orbit or through the
lower fornix. The retrobulbar block may be
supplemented with facial block injected near the
temporomandibular joint. The fa- cial block is to
paralyze the orbicularis muscle and prevent
squeezing of the lids, which can increase the IOP,
and can cause vitreous loss during surgery. Good
anesthesia and akinesia are obtained with
retrobulbar injection.
Complications: Retrobulbar hemorrhage, accidental
globe perforation or optic nerve injury.
Accidental intravenous (IV) injection of
xylocaine can lead to cardiac arrhythmias and
inadvertent intradural in- jection is associated with
seizures and respiratory arrest.
Peribulbar anesthesia
To lessen the complications of retrobulbar
anesthesia, peribulbar anesthesia was introduced.
Here the anesthet- ic injection is given outside
the muscle cone into the pe- ripheral orbital
space from where it will slowly diffuse into the
muscle cone and produce anesthesia and
akinesia.
 Chapter 28 Surgery for Cataract 430

Sub-Tenon’s infusion or injection of xylocaine based flap tends to retract and expose the sutures,
Here the anesthetic drug is injected or given as some sur- geons prefer a limbus-based flap. A limbus-
repeated bolus through a cannula introduced into the based flap will interfere with proper visualization of the
sub-Tenon’s space. It provides good anesthesia, but AC and in putting the sutures, but it will keep the
only moderate aki- nesia during surgery. The sutures nicely covered in the postoperative period. After
complications are almost absent. the flap is made, bleeding vessels are cauterized with
Topical anesthesia the thermal cautery or wet- field cautery.
Using proparacaine drops—this requires good
coopera- tion from the patient, and can be used
only in intelligent and cooperative patients. It is not
useful in anxious and ap- prehensive patients. Topical
anesthesia is supplemented with the use of
preservative-free xylocaine injected into the AC
(intracameral xylocaine) once the AC is entered
into.
General anesthesia
General anesthesia is required in young children
and in adults whose cooperation cannot be
expected as people with mental retardation or
psychiatric disorders.

TYPES OF CATARACT SURGERY

Intracapsular cataract surgery is practically obsolete
in modern times, but it is still practiced in some
remote areas of undeveloped countries where the
facilities for surgery are minimal. It is still useful in
cases of subluxation or dis- location of lens.

Method
Intracapsular cataract extraction is usually done
under retrobulbar anesthesia. After giving the
retrobulbar injec- tion, cleaning the eyelids and
surrounding skin is done with povidone-iodine
solution, and draping is done with sterile towels. A
disposable plastic drape if available can be used,
which will minimize the exposure of the skin and also
help to keep the lashes away from the surgical
field.
Separation of the lids can be done with sutures
put on the upper and lower lids or using a wire
speculum.
A suture is passed underneath the superior
rectus muscle and this is fixed to the surgical
drapes using an artery forceps. This superior rectus
bridle suture helps to steady the globe and rotate it
downwards so that the supe- rior limbus is well-
exposed to put the incision.
A fornix-based conjunctival flap is fashioned using
a conjunctival forceps and scissors. Since a fornix-
 Chapter 28 Surgery for Cataract 431
Incision
The incision is made at the superior limbus and Enzymatic Zonulolysis
extends up to 180° (9 O’–3 O’clock). A large In patients below 50 years the zonules will be
incision is required to remove the whole lens stronger and difficult to rupture. Enzymatic
intact. An initial groove is made with a sharp zonulolysis can be done before lens removal by
Bard-Parker knife or razor blade fragment. AC is injection of -chymotrypsin into the AC.
entered into with the sharp point of the number
15 BP knife or razor blade fragment, usually at 12
O’clock po- sition. The initial entry wound is
extended to either side using corneal scissors.
This is an ‘ab externo’ incision. In the earlier days
when von Graefe’s knife was used, the in- cision
was ‘ab interno’. The knife enters the eye at 3
O’clock or 9 O’clock and comes out at the
opposite limbus, and it is slowly moved upward
till it is completely out of the AC, meanwhile
making a limbal incision from 3 O’ to 9 O’ clock
position. This ‘ab interno’ incision is difficult to
make correctly and likely to injure the cornea, iris
or lens. A suture is placed into the cornea at 12
O’clock merid- ian with 8-0 black silk or 10-0
prolene. By pulling this su- ture the cornea can
be lifted up and folded back to expose the lens.
The cryoprobe is applied to the anterior lens cap-
sule and an ice ball is formed, which attach the
capsule to the tip of the probe. With gentle
rocking movements the zonules are ruptured, and
the lens is lifted up and out through the limbal
incision. A small peripheral iridectomy is done
after lens removal. The purpose is to make an
al- ternate passage for the aqueous from posterior
to the AC if the pupil gets blocked with the
vitreous. The AC may be reformed with an air
bubble or balanced salt solution. If IOL is to be
placed it has to be an anterior chamber IOL,
which is supported at the angle. Then AC is
formed with air or viscoelastic material and the
IOL is placed in the AC with the supporting
haptics in the horizontal merid- ian. It is made
sure that the pupil is round and the iris is not
caught in the haptic of the lens. The limbal
wound is then closed with interrupted or
continuous 8-0 black silk or 10-0 nylon. If
viscoelastic was used it has to be removed before
wound closer is completed. If viscoelastics is left
behind this can lead to secondary glaucoma in the
imme- diate postoperative period (viscoelastics
and IOLs were not available in the era of ICCE.
They are part of modern
times and used if ICCE is done now).
A subconjunctival injection of 0.5 mL of
gentamicin and dexamethasone is given by many
surgeons at the end of the surgery to counter
postoperative infection as well as inflammation.
 Chapter 28 Surgery for Cataract 432

Postoperative Care eyes will pro- duce diplopia. Binocular vision is

impossible un- less the other eye also undergoes
Topical antibiotic and steroid drops are given at cataract surgery.
frequent intervals initially and slowly tapered off
within 6–8 weeks. In ICCE usually a mydriatic like
homatropine or atropine is also given 2–3 times daily.
The sutures can be removed after 6–8 weeks.

VISUAL
REHABILITATION
AFTER ICCE
Conventional ICCE is done without IOL
implantation. Simple removal of the cataractous lens
alone is not going to benefit the patient significantly
as far as vision is concerned. Without the crystalline
lens, only the corneal refraction will be taking place
and the light rays will be coming to focus
considerably behind the retina and the eye will be
strongly hypermetropic. In a previously emmetropic
person the uncorrected visual acuity will be around
CF at half meter. If the patient was previously
myopic, the myopia will com- pensate the
hypermetropia to some extent and the vision will
be better, depending on the degree of myopia.
The methods of visual rehabilitation include:
1. Spectacle correction.
2. Contact lens.
3. A secondary IOL implantation done at a later date
after all postoperative inflammation has subsided.
This can be a ACIOL (Fig. 28.2) supported at the
angle of AC iris clip lenses supported by the
haptics clipping the iris or a scleral fixated IOL,
which is placed behind the pupil after anterior
vitrectomy, and kept in place with sutures through
the ciliary body and sclera fixing the haptic of the
lens in the ciliary sulcus.

Spectacle Correction
An aphakic person will require strong convex
lenses to correct the hypermetropia.

Disadvantages of Aphakic
Spectacle Correction
1. Magnification of the image size: The strong
convex lenses increase the image size by 25%–
30%. This pro- duces two problems:
a. If the other eye has good vision the difference
in the size of the images between the two
 Chapter 28 Surgery for Cataract 433
2. Loss of accommodation: Once the lens is
removed all power of accommodation is lost.
For near and intermediate vision, the person
is totally depend on glasses whatever be the
age of the patient. This is

FIGURE 28.2: Anterior chamber intraocular lens (ACIOL)

If this is not done and the other eye has

good vi- sion, the aphakic spectacle has to
be abandoned, and the patient has to
depend on the vision of the unoperated
eye alone, until the vision in that eye
decreases due to cataract or some other
pathol- ogy, or cataract surgery is done
in that eye also.
What happens if a patient who is
aphakic in one eye undergoes cataract
surgery with IOL im- plantation in the
other eye?
The problem of diplopia sets in due to
the dif- ference in the size of the images,
if he/she wears aphakic glasses. Either the
patient has to abandon aphakic glasses
and use the pseudophakic eye alone or a
secondary IOL implantation has to be
done in the aphakic eye also.
b. If the other eye has poor vision due to
cataract, or some other problem or both
eyes are aphakic, the magnification of the
images produce another problem. We
assess the distance of objects by the size
of the images formed on the retina and
the magnification produces a false spatial
orientation. Even simple tasks like picking
up a piece of paper or pouring tea into a
cup becomes a difficult task and all
movements become clumsy. There is a
long learning curve before the patient can
adjust to the new spatial orientation. Some
people never learn to adjust to this and go
about without wear- ing glasses. They
prefer the blurred uncorrected image to
the clearer magnified images of an unfa-
miliar world.
 Chapter 28 Surgery for Cataract 434

more relevant in young children who have to from 65° to 90° are unrefracted by spectacle glasses (Courtesy:
undergo cataract surgery for congenital cataract. Cataract surgery and its complications by Norman S Jaffe).
The benefit of retaining accommodation with
blurred vision has to be weighed against clearer
vision with no accommo- dation before
embarking on surgery.
3. Spherical aberration: The spherical aberration
of the thick convex lens gives a pincushion
effect to the visual field. Straight lines appear
curved and the whole world is converted into a
parabola and every eye movement make the
objects move like a writh- ing snake. The patient
can see clearly only by looking straight through
the center of the lens, and he/she has to turn the
head and not the eye to see to the sides.
4. Restriction of visual field: With spectacle
correction the visual field is restricted due to:
a. Small size of the aphakic lenses due to the
heavi- ness of the thick lens and spherical
aberrations of the periphery, the aphakic
glasses are given as

FIGURE 28.3: Aphakic spectacle corrected vision in straight

ahead gaze. Rays from 50° to 65° fail to enter the eye
producing a ring scotoma. The car in this area is invisible. Rays
 Chapter 28 Surgery for Cataract 435
small lenticular lenses. This leaves a large
portion of the peripheral field
uncorrected.
b. Roving ring scotoma—due to the
prismatic ef- fect of the periphery of a
spherical lens there is a ring of scotoma
extending from 30° to 50°. When the eye
moves to one side the scotoma moves in
the opposite direction and hence some
objects, which were previously invisible
(Fig. 28.3) be- come suddenly visible and
other objects, which were previously
visible disappear. This is called ‘Jack-in-
the-box phenomenon’ (Fig. 28.4). This is
very disturbing to some patients and
they feel giddy and nauseated, and refuse
to wear aphakic glasses. The peripheral
field from 65° to 80° goes unrefracted
since this is outside the spectacles.
These various disadvantages of aphakic
spectacle cor- rection led to research for better
options.

FIGURE 28.4: Demonstration of roving ring scotoma. When the

gauze is directed laterally, lateral rays from 30° to 50° fail to
enter the eye. Rays from 50° to 90° are unaffected. Due to the
central shift of the scotoma, the man disappears and the car
suddenly comes into view (Jack-in-the-box phenomenon).
 Chapter 28 Surgery for Cataract 436

Contact Lenses Iridodialysis can occur when the initial entry wound is
ex- tended with corneal scissors. This can lead to
Contact lenses are better option than glasses. The hyphema.
magni- fication with contact lens is only 5%, which is
tolerated by our visual mechanism. There is no
spherical aberration, distortion of images or
restriction of fields with contact lenses.
But many of the patients undergoing cataract
surgery are elderly patients who often lack the
finger dexterity to insert and remove the contact
lenses themselves. So, the use of contact lenses is
often limited to younger patients.
In short, the logical solution for the removal of the
natu- ral crystalline lens is the replacement by an
artificial lens.

COMPLICATIONS OF
INTRACAPSULAR CATARACT
EXTRACTION
Higher incidence of complications is associated with
ICCE compared to ECCE and its various
modifications.

Preoperative Complication
Associated with retrobulbar injection, retrobulbar
hem- orrhage, accidental globe perforation, etc.
(mentioned earlier).

Intraoperative Complications
Inadequate Incision
A large incision 160º–180º in size is required for ICCE
for easy delivery of the whole lens. A smaller incision
can lead to endothelial damage, Descemet’s
membrane stripping, difficulty in delivery of the lens
leading to capsule rupture and vitreous loss.

Irregular Incision
Often occurs from inexperienced hand. It will lead
to de- layed wound healing, iris prolapse, marked
astigmatism, epithelial ingrowth, etc.

Excessive Bleeding from the

Corneal Wound and Iris
Excessive bleeding from the corneal wound and
iris can occur especially in people on anticoagulants
or drugs like Aspirin, which prevents clotting of
blood.

Iridodialysis
 Chapter 28 Surgery for Cataract 437
If the dialysis is large, it can displace the pupil pupil and getting incarcerated in the wound leads to
or cause uniocular diplopia in the postoperative this phenomena.
period. Large iri- dodialysis should be sutured. Pupillary block by the vitreous: It can occur leading to
shal- low AC and secondary glaucoma.
Inadequate Pupillary Dilatation
Inadequate pupillary dilatation can occur in
people on long-term pilocarpine therapy for
glaucoma, pseudoexfo- liation and senile miosis.
This will cause difficulty in deliv- ering the lens
leading to sphincter tear or capsule rupture. This
can be avoided by doing a sector iridectomy.

Vitreous Loss
Vitreous loss can occur if the IOP is not kept low
preopera- tively with acetazolamide or
mannitol.
Intraoperative rise in IOP can occur:
1. In short-necked people on lying down.
2. If too much xylocaine is injected during
retrobulbar block.
3. If the lid suture or speculum is applying
pressure on the eyeball.
4. If the patient is anxious and restless.
Problems caused by vitreous loss
Vitreous loss per se is not the problem. Vitrectomy
is done as a surgical procedure in many
vitreoretinal disorders. The space left behind by
the removed or lost vitreous will be filled up
with aqueous humor.
But the vitreous loss during surgery can lead to
a series of complications due to forward
movement of the vitreous through the pupil and
the incarceration in the incision.
Vitreous loss leads to problems by two mechanisms:
1. Vitreous incarceration in the wound unless a
very me- ticulous vitrectomy is done.
2. The moving forward of the vitreous exerts
traction at its attachments to the retina
mainly at the vitreous base at pars plana,
and also at its posterior attach- ments at
macula and the edge of the optic disk.
The adverse effects of vitreous loss
are due to these sequelae.
Vitreous incarceration in the incision leads to
delayed wound healing, iris prolapse, epithelial
and fibrous down- growth onto the posterior
surface of the cornea, and iris and marked
astigmatism. Vitreous touch on the corneal
endothelium can lead to corneal
decompensation and corneal edema.
Updrawn pupil: The vitreous coming through the
 Chapter 28 Surgery for Cataract 438

Secondary glaucoma: It can occur due to the Lens capsule rupture can occur, while attempting to
pupillary block as well as the blockage of the re- move the lens with the cryoprobe or the capsule
angle by the vitreous filling the AC. forceps. The surgery will then have to be converted
into ECCE.
Chronic iritis: The vitreous in the AC can cause
irritation to the iris and lead to mild chronic
anterior uveitis and per- sistently irritable eye.
Cystoid macular edema (CME): The traction exerted on
the macula as well as the release of prostaglandins
can lead to CME.
Retinal detachment (RD): The traction exerted at the
retinal periphery at the vitreous base can lead to
retinal degenera- tion, hole formation and
subsequent retinal detachment.
Even if there is no vitreous loss at the time of
surgery, the loss of barrier between the posterior
and the anterior compartment after the lens removal
will lead to a gradual moving forward of the
vitreous into the AC, and this can lead to cystoid
macular edema and RD. ECCE retain the barrier
between the two compartments, and decrease the
incidence of CME and RD.
Management of vitreous loss
All the problems related to vitreous loss can be
prevented to a large extent by meliculous removal
of all vitreous in front of the papillary plane
manually by cutting with a Vanna’s scissors or with
the help of anterior vitrectomy machine.

Suprachoroidal or Expulsive Hemorrhage

In people with high IOP or high blood pressure (BP)
in the preoperative period and people with
atherosclerosis, the sudden lowering of the IOP at the
time of the entry into the AC can lead to rupture of
the choroidal vessels and mas- sive intraocular
bleeding.
All intraocular contents, i.e. retina, vitreous, lens
and uveal tissue will be thrown out by the blood
gushing out of the eye. An expulsive hemorrhage
will lead to loss of the eye and vision unless an
early drainage of the blood through a scleral
incision is made (suprachoroidal drain- age) and the
wound is closed immediately to give a tam-
ponade effect as soon as the choroidal bleeding is
sus- pected. Once well-established, this massive
bleeding can be controlled only by evisceration of
the eye.

Lens Capsule Rupture

 Chapter 28 Surgery for Cataract 439
Nucleus Drop
Nucleus drop complication is more common in
ECCE than ICCE.
A capsule tear extending to the posterior
capsule can lead to lens matter and even the
nucleus falling into the vitreous cavity. The soft
cortical matter in the vitreous will slowly get
absorbed even though it can lead to secondary
glaucoma or uveitis. But the nucleus will not get
absorbed and it has to be removed by a second
surgery with the help of the vitrectomy machine by
the vitreoretinal surgeon. A nucleus left behind in
the vitreous cavity can lead to intractable
secondary glau- coma, uveitis and RD, and
finally loss of vision.

Postoperative Complications
Early Phase
1. Postoperative shallow AC can occur due to
wound leak, if the corneoscleral incision is
irregular and not sutured properly. It can also
occur associated with choroidal detachment
and malignant glaucoma.
2. Iris prolapse is due to inadequate wound
closure or due to rise in IOP in the
immediate postoperative pe- riod or due to
restlessness of the patient. If detected
within 24 hours after surgery, the prolapsed
iris may be repositioned and resuturing of the
incision is done. If detected later,
repositioning can lead to infection since the
prolapsed iris may be contaminated with
microorganisms. So the prolapsed iris is cut
off (iri- dectomy) and the wound is
resutured.
3. Hyphema—bleeding can occur from iris
vessels or the incision into the AC, if the
patient is restless or the suturing is not
proper. This blood will get absorbed. Patient
may be given acetazolamide tablets to control
any rise in IOP due to the blood in AC.
4. Endophthalmitis: Can be acute or chronic or
sterile (Fig. 28.5):

FIGURE 28.5: Postoperative endophthalmitis

 Chapter 28 Surgery for Cataract 440

a. Acute endophthalmitis develops in 2–5 days Treatment: Atropine eyedrops four times daily may
af- ter surgery. cure mild cases. Relaxation of the ciliary muscles by
Patient presents with pain, redness, loss of atropine stretches the suspensory ligament and
vision and photophobia. On examination this will pulls the lens backwards. Aspiration of the fluid
be corneal edema, exudates on the iris or through the pars plana or a pars plana vitrectomy
pupillary area and hy- popyon. The pupil will may be needed in intractable cases.
give a yellow reflex due to vit- reous exudation.
The pathogen is often bacterial or- ganisms and
the source is contaminated instruments or fluids
used during surgery or the commensals from the
skin of the lids or neighboring structures.
Treatment: Immediate vitreous or aqueous tap
should be done to isolate the organism by
smear and culture. At the same time intravitreal
injection of 0.4 mg in 0.1 mL amikacin and
vancomycin (1 mg in 0.1 mL) is also given.
These antibiotics are also given IV or as sub-
conjunctival injection or as fortified drops. If
vision is considerably reduced, vitrectomy is
done to remove all the exudates and toxic
material in the vitreous ear- ly before toxic
damage to the retina occurs.
b. Chronic endophthalmitis may present a few
weeks after surgery. The inflammation is
less acute and drop in vision will be the
main com- plaint of the patient. The
pathogenic organism is usually fungus.
c. Sterile endophthalmitis or toxic anterior seg-
ment syndrome (TASS) is a sterile
inflammation that appear within 12–24
hours after surgery due to introduction of
toxic material as a contaminant on the
instruments or fluids used in surgery.
Treatment: To control the inflammation with
intensive topical and systemic steroid
therapy.
5. Secondary glaucoma can occur due to pupillary
block by the vitreous, especially if an
iridectomy has not been performed. If
viscoelastics used during surgery are not
completely removed (rarely used in the era of
ICCE and commonly used in modern
extracapsular surgery) this can lead to
transient rise in IOP in the postoperative
period.
6. Malignant glaucoma: This is due to a posterior
mis- direction of aqueous, which collects in the
posterior part of the vitreous cavity pushing
the vitreous for- ward. This will lead to
shallowing of the AC, pupillary block and rise in
IOP. Shallow AC is usually associated with wound
leak and decrease in IOP. When shallow AC is
associated with increase in IOP, malignant glau-
coma should be suspected.
 Chapter 28 Surgery for Cataract 441
7. Choroidal detachment: Persistent wound
leak and chronic uveitis can lead to exudation
into the supra- choroidal space leading to
bullous elevation of the choroid. In mild
cases choroidal folds will be seen as
radiating folds around the optic disk. When
bullous elevations occur they will be seen as
dark swellings through the pupil.
Management: If there is wound leak and
hypotony, re- suture the wound to correct the
hypotony. The uveal inflammation, if present,
should be controlled by sys- temic steroids.

Late Complications
1. Filtering cicatrix: If there is wound leak,
healing will be delayed leading to the
development of cystoid spaces at the incision
through which aqueous will pass into the
subconjunctival space. This will appear like
the filtering bleb produced by
trabeculectomy.
Management: The cystoid cicatrix should be
excised and resuturing is done.
2. Epithelial ingrowth: Epithelial down growth can
occur if the wound apposition is not proper
and/or associ- ated with iris prolapse.
Epithelial cells from the ocular surface will
grow inwards lining the posterior surface of
the cornea, angle and the anterior surface of
the iris. The epithelial down growth will be
seen as a translu- cent membrane on the
back of the cornea with a hori- zontal
advancing edge. The membrane blocking the
angle will lead to secondary angle closure
glaucoma. The eye will remain persistently
irritable. Sometimes the epithelial cells will
form cystic swelling inside the AC (Fig. 28.6).
3. Chronic uveitis: It can sometime follow cataract
surgery.
4. Secondary glaucoma: It can be pre-existing
open angle glaucoma or secondary glaucoma
due to secondary angle closure or as a
complication of chronic uveitis.

FIGURE 28.6: Postoperative implantation iris cyst

 Chapter 28 Surgery for Cataract 442

Secondary angle closure glaucoma may be size is smaller about 120° since only a smaller incision
due to: is required to remove the nucleus. A cystitome or a 26
• Peripheral anterior synechiae formation due to gauge needle (with its tip bent twice to fashion a
per- sistent postoperative shallow AC disposable cystitome) is used to make a circular
• Due to epithelial down growth opening on the anterior cap- sule. This opening can be
• Secondary to pupillary block with vitreous. a continuous curvilinear capsu- lorhexis (CCC) or can
opener capsulorhexis. Since ECCE require lot of
5. Cystoid macular edema (Irwin-Gauss Syndrome).
manipulations inside the AC, viscoelastics
This is the most common posterior segment
complication after cataract surgery. The
incidence is higher after ICCE than ECCE and
the incidence is higher if there is vitreous loss
after surgery. The release of prostaglan- dins and
vitreoretinal traction at the macula following
vitreous loss are the reasons. The CME will lead
to de- crease in visual acuity.
Treatment: Topical and systemic steroidal and
non- steroidal anti-inflammatory drugs.
6. Retinal detachment: The risk for development
of RD is higher after ICCE than ECCE, higher in
people with vitreous loss at the time of surgery
and also in people with high risk for RD like
high myopes.

EXTRACAPSULAR CATARACT
EXTRACTION

Extracapsular cataract extraction (ECCE) can be the

con- ventional type or manual small incision cataract
surgery (SICS).
The high rate of complications associated with
ICCE revived the interest in extracapsular
extraction.
Main advantage of ECCE over ICCE:
1. The ‘posterior capsule zonular complex’ retains
the normal anatomical compartments of the
eye. So the complications like vitreous loss and
its sequelae— CME and RD are comparatively
less after ECCE.
2. The incision required in ECCE is smaller
compared to ICCE and hence lesser the amount
of astigmatism.
3. There is the added advantage that the IOL can be
placed in the normal anatomical position behind
the iris sup- ported by the posterior capsule
and the zonules.

Conventional
Extracapsular Cataract
Extraction
Corneoscleral incision is put as in ICCE, but the
 Chapter 28 Surgery for Cataract 443
are injected into the AC after entering into the the patient.
AC. These viscoelastics helps to maintain the AC The valve like self-sealing wound consists of an
with the iris and the lens in the normal anatomical initial vertical incision on the sclera, sclerocorneal
position, as well as protect the corneal tunnel and again a vertical internal incision in the
endothelium. cornea to enter the
After capsulorhexis, about 0.1–0.5 mL balanced AC.Theexternalincisioncanbeofvariousshapes—straight,
salt solution is gently injected into the lens V shaped, frown or curved incision (Fig. 28.7) made
between the cor- tex and the nucleus, and also on the sclera 2–3 m behind the limbus. Its length
between the capsule and the cortex. This helps varies from 5.5 to 6.5 mm. The sclerocorneal tunnel
the separation of the nucleus from the cortex is dissected forward
and the cortex from the capsule (hydrodissection),
and allows easy removal of the nucleus and the
cortex. Af- ter hydrodissection the nucleus is eased
into the AC using a Synsky’s hook or the disposable
cystotome. The nucleus is delivered out of the eye
using a vectis attached to a syringe or an IV line.
After the removal of the nucleus, the cortex is
aspirated out using the ‘Simcoe irrigation aspiration
can- nula’ attached by an IV line to a bottle of
Ringer’s lactate or balanced salt solution. The
Simcoe cannula allows simulta- neous irrigation of
the fluid into the eye and the aspiration of the
cortex. This helps to maintain the AC and protect
the endothelial layer during these manipulations.
In the ab- sence of this, an aspiration cannula
attached to a 5 or 10 mL syringe containing fluid
can be used to irrigate and aspirate alternatively to
remove the cortex. The viscoelastics can be
injected as and when required to maintain the
AC. Once the entire cortex is removed, the
posterior capsule with the peripheral rim of
anterior capsule is left behind. The IOL is now
introduced into the AC. The posterior chamber IOL
can be put inside the capsular bag or kept in
front of the capsule with the haptics of the IOL
supported at the ciliary sulcus. After the insertion
of the IOL, all viscoelastics are re- moved by
irrigation and aspiration. The AC can be main-
tained with fluid or an air bubble (which will get
absorbed in a few days) and the wound is closed
with interrupted or continuous 10-0 nylon or 8-0
black silk.

Manual Small Incision Cataract

Surgery
Manual small incision cataract surgery is a very
popular and widely practiced surgery in
developing countries like India. The advantage of
this technique is that it is a small incision
sutureless surgery that can be done without any
expensive equipment like the phacoemulsification
unit. Careful construction of the entry wound
results in mini- mal induced astigmatism, early
visual rehabilitation and return to routine life for
 Chapter 28 Surgery for Cataract 444

Some surgeons use an AC maintainer introduced

into the AC through a small incision put in the lower
part of the cornea. It is connected by an IV line to a
bottle of balanced salt solution. With the self-sealing
valve-like incision the continuous irrigation through
the AC maintainer keeps the anterior segment
FIGURE 28.7: Incisions in small incision cataract surgery (SICS) structures in their natural position and maintains
— straight, frown or ‘V’ shaped normal IOP. The use of the AC maintain- er reduces
the need for viscoelastics during surgery. The steps
from this external incision until it reaches 1 mm into of SICS are given in Figures 28.8 A to P.
clear cornea. The AC is entered about 2 mm from
the limbus with a 3.2 m keratome by dipping it Advantages of SICS
toward center of the AC. Thus this incision has an
initial vertical component in the sclera, a horizontal 1. No suturing required.
tunnel through the sclera and cor- nea, and a final 2. Minimal induced astigmatism.
vertical component through the cornea to reach the 3. Marked low incidence of postoperative iris
AC. This valve-like incision is self-sealing, and the lip prolapse and hyphema.
of the wounds are kept in opposition by the normal 4. Better control of expulsive hemorrhage by
IOP and does not require any sutures. spontane- ous closure of the wound.
After the entry into the AC, the surgery is 5. Early ambulation of the patient from the day of
continued and completed as in conventional ECCE. sur- gery itself.
Since all manip- ulations have to be done through a 6. It can be done without any expensive equipments
smaller valvular inci- sion, there is a learning curve like phacoemulsification unit (Fig. 28.9).
to master this technique.

FIGURES 28.8A to D: Steps of cataract surgery (SICS). A. Eye exposed with speculum; B. Anterior chamber (AC) maintainer introduced
at lower limbus; C. Trypan blue dye injected under air bubble through the side-port incision; D. Capsulorhexis started.
 Chapter 28 Surgery for Cataract 445

FIGURES 28.8E to J: Steps of cataract surgery (SICS). E. Capsulorhexis continued; F. Limbus based conjunctival flap cut with scissors after
completion of capsulorhexis; G. Cautery applied to bleeding spots; H. Incision put with Bard-Parker (BP) blade; I. Tunnel incision put up
to clear cornea with crescent knife; J. Anterior chamber (AC) entered with keratome.
 Chapter 28 Surgery for Cataract 446

FIGURES 28.8K to P: Steps of cataract surgery (SICS). K. Nucleus delivered into anterior chamber (AC); L. Nucleus removed with
two instruments; M. Cortex aspirated with Simcoe cannula; N. Posterior chamber intraocular lens (PCIOL) introduced into the eye;
O. Intraocular lens (IOL) rotated with the dialer into place; P. IOL in position on posterior capsule.
 Chapter 28 Surgery for Cataract 447

surgeries and there is a steep learning curve. This can

lead to a high rate of complications, specific to these
keyhole cataract surgeries in the initial learning
phase, which tend to come down as the skill and
experience of the surgeon increases.

FIGURE 28.9: Console of phacoemulsification unit

Phacoemulsification
The introduction of phacoemulsification by Kelman
in 1967 revolutionized cataract surgery. With this
instrument the nucleus is fragmented to fine particles
by ultrasound energy and aspirated. In
phacoemulsification, irrigation, aspiration and
fragmentation of the lens nucleus takes place at
the tip of the phaco handpiece (Fig. 28.10), which
can be introduced into the eye through an incision
2.8 m in size. With modern phacoemulsifying
equipments, emulsification can be done through an
incision 1 mm in size, while irrigation and
manipulations of the nuclear fragments are done
through another small incision with a second
instrument (irrigating chopper). This is called mi-
croincision cataract surgery (MICS).
The three operations at the tip of the handpiece
are:
• Irrigation alone
• Simultaneous irrigation and aspiration
• Ultrasound fragmentation and aspiration. All these
op- erations are controlled by three positions of a foot
switch. The IOL that has to be introduced into the eye
through the small incision is also suitably modified—
foldable lenses or injectable lenses, or lenses preloaded
into an injector are
used, which can be introduced through the small
incision. The smaller incision means there is
hardly any in-
duced astigmatism and the patient can return to
normal
activities quickly.

Complications of ECCE
Many complications of ICCE like wound related
problems, expulsive hemorrhage, CME and RD are
less in SICS and phacoemulsification. But both SICS
and phacoemulsifica- tion surgeries involve a lot of
intraocular manipulations, which require a lot of skill
and precision from the surgeon. Phacoemulsification
is the most complex of all types of cataract
 Chapter 28 Surgery for Cataract 448
Intraoperative Complications
1. Posterior capsular rupture and vitreous loss
(dis- cussed earlier).
2. Nuclear drop into vitreous (an important
complica- tion of phaco, especially in the early
learning period).

Early Postoperative Complications

1. Wound leak and shallow AC. This can occur
if the sclera-corneal tunnel incision is not
properly made. This can be prevented by
suturing the wound.
2. Corneal edema occur in cases with large
and hard nucleus, which makes the nucleus
delivery difficult in SICS and increased
ultrasound fragmentation time in
phacoemulsification leading to corneal
burns.
3. Rise in IOP can occur in the immediate
postoperative period if the viscoelastics are
not completely removed from the AC.
4. IOL decentration (Fig. 28.11): This can occur if
some parts of the zonules are weak or
ruptured during sur- gical manipulations or if
there is a posterior capsular tear.
Management: The IOL may be repositioned and
fixed with 10-0 or 9-0 prolene suture to a
scleral wound or the iris. If the IOL is totally
unstable it may be removed and replaced
with a scleral fixated or AC IOL.
5. Postoperative uveitis can occur if the cortical
matter is not completely removed.
6. Endophthalmitis, which can be sterile or
infective (al- ready discussed).

Late Postoperative Complications

1. Pseudophakic bullous keratopathy: It can
occur due to endothelial decompensation due
to surgical trau- ma. Penetrating keratoplasty
is required to manage this problem (Fig.
28.12).
2. Posterior capsular opacification: The posterior
cap- sule tends to become translucent and
the vision will be decreased (Fig. 28.13).
This can be managed by posterior
capsulotomy using yttrium-aluminum garnet
(YAG) laser. This can be done as an OP
procedure. If the laser machine is

FIGURE 28.10: Phaco handpiece

 Chapter 28 Surgery for Cataract 449

FIGURE 28.11: Intraocular lens (IOL) decentration FIGURE 28.12: Pseudophakic bullous keratopathy

not available, surgical posterior capsulotomy has to

be done.
CATARACT SURGERY IN
3. Pupillary capture of the IOL: Part of the optic is CHILDREN
syndrome: This UGH syndrome is usually seen in
seen an- terior to the papillary margin. Usually eyes, in which AC IOL is placed. The haptic placed
caused by postop- erative iritis and there is a in the angle leads to chronic irritation and recurrent
higher incidence of this prob- lem in children uveitis, glaucoma and hyphema.
undergoing cataract surgery (Fig. 28.14). 8. CME and RD: The incidence is lower compared to
4. Glaucoma it may be a pre-existing primary ICCE.
open an- gle glaucoma or pupillary block
glaucoma or second- ary glaucoma due to
chronic uveitis.
5. Decentered IOL: A mild decentration in the
immedi- ate postoperative period may increase
as time passes:
a. Wind shield wiper syndrome: This happens
when the IOL is too small for the eyes
(usually a myopic eye) and the lens is
placed in the sulcus. The lens moves like a
wind shield wiper with each ocular
movement.
Management: Lens may be made more stable
with sutures or replace it with a larger
lens.
b. Sunset syndrome: Due to inferior zonular
dialysis the IOL slowly sinks toward the 6
O’clock merid- ian, so that the upper part of
the pupil is aphakic and the lower part is
phakic and the edge of the optic portion of
the IOL is seen through the pupil (refer Fig.
28.11).
6. Intraocular lens drop into the vitreous: This can
oc- cur if there is a zonular dialysis or a posterior
capsular tear, which was undetected at the time
of surgery and a posterior chamber IOL was
inserted or zonular rup- ture can occur later
due to some trauma.
Management: The IOL has to be removed by
vitrecto- my and a scleral fixated IOL or AC IOL
may be placed.
7. The uveitis glaucoma hyphema (UGH)
 Chapter 28 Surgery for Cataract 450
Problems of
Cataract
Surgery in
Children
The cataract and its surgery in children differ
from that done in an adult in many aspects:
1. The capsule of a pediatric lens is thicken and
more elastic. So a circular capsulorhexis is
often difficult. The capsulorhexis often tends to
extend to the periphery.
2. The lens does not have a hard nucleus and so
no pha- cofragmentation is required. The
whole lens matter can be removed by
irrigation and aspiration.
3. The epithelium living the posterior capsule
is more active and tends to multiply rapidly,
and hence there is invariably posterior
capsular opacification. To avoid this, a small
posterior capsulorhexis and anterior vit-
rectomy is done at the end of lens matter
aspiration to maintain a clear visual axis.
4. The sclera is less rigid than that of an adult
and so the incision, even if made in a self-
sealing valvular fash- ion, has to be sutured
to keep it watertight.
5. In children less than 1–1½ years of age,
cataract surgery alone is usually done and
a secondary IOL

FIGURE 28.13: Posterior capsular opacification

 Chapter 28 Surgery for Cataract 451

when the problems in IOL power calculation will be

lesser. Another advantage is that accommodation will
be retained.

FIGURE 28.14: Pupillary capture of intraocular lens (IOL) with

posterior capsular opacification (PCO)

implantation is done later after the child has

become older. Till then spectacle correction or
contact lens is given. In children older than 1½
years, IOL implanta- tion can be done as a
primary procedure.
6. Postoperative complications:
a. The postoperative inflammation is often more
se- vere than in an adult and often require
systemic steroids to control it. Due to the
active prolifera- tion of the capsular
epithelium and the postop- erative
inflammation, there is a high incidence of IOL
displacement and pupillary capture.
b. There is also an increased risk of glaucoma in
the postoperative period. This may be a
coexisting congenital glaucoma associated
with congenital cataract or a secondary
glaucoma. So the patient has to be closely
watched for any rise in IOP.
c. The RD CME and corneal problems are rare
in children.

Time of Surgery
The risk of development of amblyopia is an important
fac- tor in deciding the time of surgery. Unilateral
cataracts are at the highest risk for developing
amblyopia and so a uni- lateral cataract, which is
sufficiently dense as to interfere with vision should
be removed as early as 6 weeks after birth or
should be ideally done before 10 weeks.
In older children with congenital cataract,
surgery should be done if it is interfering with
near vision and reading, even if the distant visual
acuity is fairly adequate. In bilateral cataracts with
fairly good vision and it is not interfering in studies
the surgery can be delayed, till the child is older
 Chapter 28 Surgery for Cataract 452
Most congenital cataracts are not progressive refraction.
and de- cision regarding surgery depends on Long-term follow-up for any change in refraction
whether it is prefer- ential to have clearer vision and treatment for amblyopia are essential for proper
with no accommodation than slightly poorer visual re- habilitation after congenital cataract
vision with retained accommodation. surgery.

The child’s eye tends to grow throughout the INTRAOCULAR LENS

first 8–10 years of life and the refractive power IMPLANTATION IN CHILDREN
also changes. The correct selection of IOL power
in children is very difficult. The measurement of
IOL power by biometry in small chil- dren is
difficult. The future change in refraction is often
variable.
If a child is made emmetropic with an IOL at
the age of 1 year, as the child grows there will
be gradual develop- ment of myopia and at the
age of 10 years the child may require -7D or -8D
spectacle connection to see normally.
So the IOL power selection depends on its
effect on vi- sion immediately after surgery and
also the power that is expected to be required in
adulthood. Some surgeons cal- culate and select
the IOL power depending on the power
expected to be required in adulthood. The child
will have to wear hypermetropic spectacle
correction, the power of which can be decreased
gradually till emmetropia is achieved in
adulthood. Some surgeons aim for emmetropia at
the time of IOL implantation and any future change
in re- fraction (usually myopia) will be corrected
with spectacles. This is done more in unilateral
cataracts to decrease the risk of amblyopia by
providing equally good vision in both eyes in early
childhood. Some surgeons prefer to take a midway.
Amblyopia management is important and should
be- gin immediately after surgery. Aphakic children
should be given spectacles or contact lens.
Extended wear contact lens can be given in
unilateral cataracts in small children and aphakic
spectacles are enough in bilateral cataracts. Since
the power changes rapidly in young children
frequent change of spectacles and contact lens
(CL) are required at
3–6 monthly intervals in children up to 2–3 years of age.
Part time occlusion of the better eye should
be done in all cases of unilateral cataract to
overcome amblyopia.
Even in children in whom IOL implantation
has been done it is essential to give adequate
spectacle connection, especially for near vision
and regular follow-up for any change in
 Chapter 28 Surgery for Cataract 453

lenses to come, which were supported

Intraocular Lenses
Brief History
Sir Harold Ridley did the first intraocular lens
implanta- tion in 1949. He was inspired by the fact
that polymethyl methacrylate (PMMA) windshield
fragments in the AC were well-tolerated by the
pilots of World War II. His lens was a biconvex disk
made of PMMA inserted in the pos- terior chamber
after ECCE. His idea had firm scientific basis, but
the ECCE at that time was a crude surgery, and IOL
was also heavy and had problems from its steriliza-
tion. This resulted in many complications and limited
the use of his lenses.
The surgeries at that time were mostly ICCE
and the need for better alternatives to aphakic
correction led to the development of several lenses,
which required fixation to the iris by suture through
loops, struts or holes. The AC IOL was the next
 Chapter 28 Surgery for Cataract 454
FIGURES 28.15A to E: Various models of
intraocular lenses (IOLs). A. Original Ridley
IOL first implanted by Sir Harold Ridley; B.
Iris fixated IOL designed by Fyodorov; C. Iris
supported IOL for intracapsular cataract
extraction (ICCE) with two opposing haptics
placed anterior and posterior to iris; D.
Modern anterior chamber IOL with flexible
four point angle support; E. Modern
posterior chamber IOL.

at the angle with rigid haptics. The exact fitting

of size of the lens to that of the AC was often a
problem. If the lens was too big this led to
tenderness of the eye and pupillary distortion
and UGH syndrome. If the lens was small for the
eye this can cause lens decentering, poor vision
and endo- thelial damage. Flexible loop AC IOLs
were subsequently developed, which lessened the
complications of AC IOLs. Closed flexible loops
were replaced with open flexible loops and
these types of lenses are still used today when
there is posterior capsular tear or for secondary
IOL im- plantation in aphakic patients. With the
dawn of the mod- ern era of ECCE, AC IOLs has
lost their importance and focus was shifted to
the development of posterior cham- ber IOLs.

Posterior Chamber Intraocular lenses

The earlier versions were three piece lenses with
prolene haptics and the lenses were of
planoconvex design. This
 Chapter 28 Surgery for Cataract 455

was soon followed by single piece biconvex lenses. patient. Multifocal lenses are developed for
Many modifications were made in the shape of the overcoming this problem. In the first models, the
posterior sur- face and edge of the lenses to decrease central portion of the lens was designed for near
the development of posterior capsular vision and the outer portion for distant vision. The
opacification. main disadvantage of these earlier de- signs was
poor distant vision in bright light when the pu- pil
Special Purpose Lenses constricts in bright light. Modern multifocal lenses are
of two types—diffractive or refractive type. The
Scleral-fixated lenses: Lenses with eyelets in the diffractive lenses have a series of blended diffractive
haptics were introduced to be used in aphakic zones, which fo- cus light from distant, intermediate
patients as scleral- fixated lenses by passing and near objects on the retina. These lenses give
sutures through the eyelets. good distant and near vision, but the intermediate
Aniridia lenses: Another type of special lens is lenses vision is not so good.
with opaque flanges to be used in aniridia or iris The refractive type has five zones, which give
coloboma. fairly good distant, intermediate and near vision. The
Foldable lenses: The development of foldable lenses main ad- vantage of multifocal lenses is the less
was the next major step in the evolution of IOLs. Most dependence on spectacles, and hence most suitable
foldable lenses are manufactured from silicone or for ladies who are un- willing to wear glasses and do
acrylic material. The foldable lenses allow insertion of not do much driving. The disadvantage of multifocal
the lens through the small incision made for lenses is reduction in contrast sensitivity and visual
phacoemulsification. acuity, and patients may experience some glare and
The acrylic lenses can be hydrophobic or colored halos. These lenses are best toler- ated if
hydrophilic. Hydrophobic lenses have less water implanted bilaterally and there is a short learning
content and are thin- ner, but they can produce phase to adjust to the new visual sensations.
greater reaction in eyes prone to develop uveitis. Researchers are going on to improve the
Hydrophilic lenses are more biocom- patible and quality of multifocal lenses. The latest in this
better tolerated by eyes with less chance for series is the accom- modative IOL. The lens optic
uveitis, but the chances of development of posterior has supportive flanges with hinges, which allow
cap- sular opacification is higher. the lens to move forward on attempting
Most IOLs contain chromophores that give UV accommodation and backwards on relaxing
pro- tection. Some lenses contain blue light filters accommodation.
also, which lessen the damage to the retina. Adjustable IOL: It is another one in this series. The
Aspheric optics: Some IOLs have aspheric optic, refrac- tive power can be adjusted with UV light
which overcome the spherical aberration and after implanta- tion done 1 week after surgery. The
improve the qual- ity of vision especially in UV light causes polym- erization of the molecules
medium intensity lighting. with precise correction of the spherical and
astigmatic power.
Heparin-coated lenses: This coating reduces the
adhesion of inflammatory cells and such lenses are Toric IOLs: These they are lenses with a cylindrical
useful in eyes with uveitis undergoing surgery. correc- tion also, used in patients with high
astigmatic error. The axis of the cylinder is also
Multifocal lenses: The main disadvantage of IOLs marked on the lens and the lens has to be
com- pared to natural lens is that they are of fixed positioned in the capsular bag with the axis in the
focal length and give clear vision only at a specific correct meridian.
distance. Usually, when the IOL power is Lot of research is going on to improve the
calculated, surgeons aim for cor- rection of distant quality of vi- sion with the lenses and we can
vision and suitable spectacles are given for expect lenses with near perfect vision in the
correction of near vision, irrespective of the age of market soon.
the
 Chapter 28 Surgery for Cataract 456

Glaucoma
Surgery
2
9
Girija Devi PS, Thomas George T

prostaglandins are generally not used

PREPARATION OF PATIENT preoperatively as they cause uveal
inflammation).
Preparation of patient is common to all intraocular
surgi- cal procedures.

Preoperative Preparations
1. Preoperatively one has to rule out/control
systemic diseases like diabetes mellitus,
hypertension, heart disease, pulmonary disease
and allergies to drugs as with any surgical
procedure.
2. Local foci of infection would predispose to
postop- erative endophthalmitis and need to
be looked out for and treated, especially chronic
dacryocystitis by syringing the nasolacrimal
ducts. If there is a dacryo- cystitis it has to be
dealt with by a dacryocystorhinos-
tomy/dacryocystectomy surgery. Hordeolum
inter- num/externum as well as conjunctivitis if
present, need treatment.
3. Complete blood count screen for systemic
infections that can spread to the eye
compromised by surgical wound, by a
bacteremia (e.g. osteomyelitis, dental caries,
etc.). Erythrocyte sedimentation rate is a gen-
eral screening for inflammation in the body,
some of which can predispose to severe
postoperative inflam- mation in the eye (e.g.
rheumatoid arthritis).
4. Most centers would cover the surgery with
periopera- tive broad-spectrum systemic
antibiotics (e.g. a fluo- roquinolone started 1
day prior and continued for 2 days after
surgery). The patient is put on preoperative
topical antibiotics and anti-inflammatory drops.
5. To avoid sudden decompression during surgery,
the intraocular pressure (IOP) is reduced
preoperatively by medical therapy (with
acetazolamide tablets). If required, topical
antiglaucoma medications and in- travenous
mannitol can be given (pilocarpine and
 6. On the day of surgery, the eye to be
operated is pre- pared by cleaning with
povidone-iodine solution (5%) instilled in both
eyes and periocular skin surface wipe (7.5%
or 10%).
7. Local anesthesia is given in the form of Chapter 28 Surgery for Cataract 457
regional block (peribulbar injection of 2%
lidocaine and bupivacaine) for anesthesia
of eye with akinesia of muscles of ocular
motility and orbicularis oculi muscles.
After the operative procedure the eye dressed
for about 6–12 hours.

Postoperative Preparations
Topical steroids and antibiotic drops are instituted
and ta- pered over 6–8 weeks.

Surgical peripheral iridectomy also creates a SURGICAL PERIPHERAL

peripheral iris defect that bypasses the pupil in a IRIDECTOMY
pupillary block to aqueous flow.

Indications
1. Nowadays surgical peripheral iridectomy
done as part of other surgeries, e.g.
trabeculectomy, cataract surgery with
vitreous loss and when using anterior
chamber (AC) intraocular lenses.
2. Sometimes, the iris is too thick or
edematous due to inflammation that laser
iridotomy is not possible. Then surgical
iridectomy is done.
3. Non-glaucoma indications are foreign bodies
on the iris, tumors of the iris, for optical
purposes (optical iridectomy used to be
done in the past in central sta- tionary
opacities, usually in the lower nasal segment
to facilitate reading), etc.
 Chapter 29 Glaucoma Surgery 440

Procedure FIGURE 29.1: Pathway for drainage of aqueous after

trabeculectomy
The pupil should not be dilated. All precautions for an
intra- ocular surgery are to be taken. After
preparation and anes- thesia the eye is draped and
opened with a speculum. A su- perior rectus tendon
bridle suture is taken to expose more of the superior
corneoscleral junction or limbus. A 3–4 mm beveled
incision is made in the cornea close to the limbus.
The peripheral iris is grasped with a fine-toothed
forceps (e.g. Lims’ forceps) and cut with scissors
(e.g. de Weckers’ scissors). This creates a
triangular opening in the iris at the periphery with
the apex toward the pupil. The corneal wound is
sutured with 10-0 nylon suture. Subconjunctival
injection of steroid antibiotic can be
administered.

TRABECULECTOMY
Trabeculectomy is the most commonly done
antiglauco- ma surgery.
In the past, the full thickness filtration
procedures were done like iridencleisis, trephining,
thermal scleros- tomy, etc. A filtering surgery done
with a partial thickness scleral flap, e.g.
trabeculectomy gives some resistance to aqueous
outflow and helps to maintain the IOP at physi-
ological level.

Principle
Here an alternate pathway for aqueous drainage is
cre- ated from the AC to the subconjunctival space
bypassing the pupil (as peripheral iridectomy is
part of the proce- dure) and the trabecular
meshwork via the ostium cre- ated (Figs 29.1 to
29.3). Aqueous from the subconjuncti- val space gets
absorbed by the episcleral veins. It is done in
almost all types of glaucoma where medical
therapy is inadequate to control IOP.
 Chapter 29 Glaucoma Surgery 441
scleral flap decides the resistance to outflow
postoperatively). The peripheral iris under the
trabeculectomy ostium is grasped with a fine-
toothed forceps and cut with scissors to create a
pe- ripheral iridectomy. Now the scleral flap is
reposited and

FIGURE 29.2: A post-trabeculectomy eye

FIGURE 29.3: Ultrasound biomicroscopic image of a post-

trabeculectomy eye to show normal tissues. 1. Conjunctiva;
2. Cornea; 3. Iris; 4. Sclera; 5. Anterior lens capsule; 6.
Ciliary body.

Procedure
The pupil should not be dilated. All precautions for
an in- traocular surgery are to be taken. After
preparation and anesthesia, the eye is draped
and opened with a specu- lum. A superior rectus
tendon bridle suture is taken to expose more of
the superior corneoscleral junction or limbus. A
slow-controlled 20 G paracentesis (stab incision
through clear cornea into the AC) is made to
decompress the AC. A limited peritomy
(conjunctiva disinserted at the limbus) is done in
one of the superior quadrants (this is a fornix-
based conjunctival flap). Some surgeons may pre-
fer a limbus-based flap, where the conjunctiva is
opened near the superior rectus tendon and
dissected anteriorly up to the limbus. The Tenon’s
capsule is opened to expose the episclera below.
Wet field bipolar diathermy or cau- tery is done
in this exposed area of episclera. A triangular or
rectangular flap 3–4 mm wide is marked out
and dis- sected from posterior to anterior with a
Bard-Parker (BP) knife. This scleral flap (Fig. 29.4)
is one-fourth to one-third thickness of sclera. The
dissection is carried till the limbus, so that clear
cornea is visible under the flap. The flap is not cut
at the limbal end (i.e. it is left hinged at the
limbus). Now a rectangular window is made in
the sclera bed un- der the flap anterior to the
scleral spur with a sharp blade or a
trabeculectomy punch (this full thickness window
is the trabeculectomy ostium into the AC and the
size of the same in comparison to the overlying
 Chapter 29 Glaucoma Surgery 442

7. Increased risk of cataract formation.

FIGURE 29.4: Ultrasound biomicroscopic image shown in Figure

with specific features of trabeculectomy marked out. The
peripheral iridectomy allows aqueous to bypass the pupillary
block. Flow through the trabeculectomy ostium under the
sclera flap takes aqueous to the subconjunctival space,
bypassing the trabecular meshwork resistance also. 1. Scleral
flap; 2. Subconjunctival bleb; 3. Trabeculectomy ostium; 4.
Peripheral iridectomy.

the edges sutured loosely with 10–0 nylon

interrupted sutures. The conjunctiva is reposited and
sutured to the limbus with 10-0 nylon interrupted
sutures. The AC is re- formed via the paracentesis
with balanced salt solution and a small air bubble.
Subconjunctival injection of ste- roid and antibiotic
can be administered.

Measures to Increase
Aqueous Drainage
Postoperative subconjunctival fibrosis is one of the
main reasons for failure of trabeculectomy. This can
be reduced by the following methods:
1. Preoperative use of antimitotic agents
subconjunc- tivally (mitomycin C and sometimes
5-fluorouracil, especially).
2. Releasable sutures.
3. Laser suture: Lysis may be used to control
aqueous drainage and corresponding IOP in
the immediate postoperative period.

Steps of Trabeculectomy
The steps of trabeculectomy are shown in Figures
29.5 to 29.13.

Complications
Complications of filtering surgery include the
following:
1. Hypotony due to excessive drainage.
2. Hyphema.
3. Suprachoroidal hemorrhage or effusions.
4. Blebitis/Endophthalmitis.
5. Encapsulation of the bleb with resultant transient
IOP elevation.
6. Loss of one or more lines of visual acuity.
 Chapter 29 Glaucoma Surgery 443
Vision loss may be a serious complication after
trab- eculectomy, especially in patients with
advanced optic disk damage, with severe and
ongoing unexplained loss (‘snuff-out’)
experienced by as many as 2% of patients.

Drainage Implant
Indications
When chances of failure are high as in failure of
repeated trabeculectomy or neovascular
glaucoma.

FIGURE 29.5: Conjunctival flap to be dissected is measured

with calipers

FIGURE 29.6: Fornix-based conjunctival flap dissected

FIGURE 29.7: Lamellar scleral flap to be dissected is

measured with calipers
 Chapter 29 Glaucoma Surgery 444

FIGURE 29.8: Incision put with Bard-Parker (BP) knife FIGURE 29.9: Incision completed

FIGURE 29.10: Lamellar flap dissected with crescent knife FIGURE 29.11: Lamellar scleral flap dissected and lifted up

FIGURE 29.12: A rectangular block of trabecular tissue

FIGURE 29.13: Scleral flap sutured after an iridectomy is done
is removed exposing the underlying iris
 Chapter 29 Glaucoma Surgery 445

Method suture, allowing flow to initiate 4–6 weeks

The implant has a silicon drainage plate with a postoperative once some conjunctival wound
flexible plastic tube attached to it. remodeling has taken place, thereby reducing the
The tip of the tube is placed in the AC to shunt risk of immediate postopera- tive hypotony (Fig.
aque- ous to an equatorial reservoir and then 29.14).
posteriorly to be absorbed in the subconjunctival Ahmed and Krupin implants: These have one way
space. valves, which are designed to maintain pressure
After creating a conjunctival flap the drainage above 8 mm Hg. These implants may reduce the
plate is fixed with sutures between two recti muscles risk of hypotony, a com- plication of non-valved
8–10 mm be- hind the limbus. After the plate is shunts in the early postoperative period (Fig.
attached to the globe, the tube is laid across the 29.15).
cornea and cut with a sharp scis- sors to create a Molteno implant: It consists of a silicone drainage
beveled edge with the opening toward the cornea. tube, which is connected to 1 or 2 acrylic plates that
The tube should extend approximately 2.5–3 mm into are sutured to the sclera (Fig. 29.16).
the AC to minimize the risk of tube-cornea touch or
retraction out of the AC. A 23-gauge needle is used CYCLODIALYSIS
to cre- ate a track through which the tube is inserted
into the AC just anterior and parallel to the iris. The
tube may be se- cured to the sclera a few Here the ciliary body is surgically detached from the
millimeters anterior to the plate with 7-0 or 8-0 scleral spur using a special instrument called
Vicryl suture. This suture helps to stabilize the tube cyclodialysis spatula. After entering the AC, the tip of
and should not be tight; otherwise, it will restrict flow this instrument is swept across the opposite angle to
in valved devices. separate the ciliary body from the scleral spur. This
The tube is covered to prevent its erosion opens a channel to the suprachoroidal space from the
through the conjunctiva. Preserved sclera or AC for egress of aqueous. Hypotony often re- sults and
cornea can be used for this purpose. After the results are unpredictable. In the past, it was com-
patch graft has been placed, the conjunctiva and monly used for management of aphakic
Tenon’s layers are pulled over the plate, tube and glaucoma.
patch graft, and sutured into place with 8-0 Vic- ryl
suture. If patch graft is not available the procedure CYCLODESTRUCTIVE
can be done under a lamellar scleral flap. The needle
track and tube entry are done under this flap. The
PROCEDURES
flap is then su- tured with 10-0 nylon sutures. Indications
Aqueous drains through the tube to the area
below the plate, which acts as a reservoir from where Indication is to reduce pain in an eye with little or
episcleral veins absorb the aqueous. no use- ful vision.
These can be used in eyes with vision also
when all other means fail, but then one is cautious
Types of Implants and small areas are treated (say 1 quadrant) and its
Baerveldt implant: It is available with larger plates effect observed for 3–6 weeks before more
with increased reservoir size. The Seton (tube) treatment is done.
connected to the reservoir, usually is tied off with
an absorbable Principle
Principle of all these procedures is to reduce
inflow of aqueous by destruction of ciliary processes.
Options are:
 Chapter 29 Glaucoma Surgery 446

FIGURE 29.14: Baerveldt implant FIGURE 29.15: Ahmed glaucoma valve

 Chapter 29 Glaucoma Surgery 447

Goniotomy
The prerequisite for this procedure is a reasonably
clear cornea for visualization of the angle (quite a
few of the

FIGURE 29.16: Molteno implant

1. Cyclocryotherapy: Here a CryoProbe, which

freezes the ciliary body transsclerally, is used.
It achieves
–80°C at its tip by the Joule Thomson effect
(sudden expansion of a compressed gas).
2. Cyclophotocoagulation: It can be done by
direct vi- sualization of ciliary processes during
surgery with endolaser probes (surgically) or
through the pupil in aphakic eyes. The lasers
used are the same as in reti- nal
photocoagulation, e.g. Argon, frequency doubled
Nd:YAG 532 nm and infrared diode 810 nm.
Cyclo- photocoagulation can be achieved
transsclerally with infrared lasers like the
continuous wave Nd:YAG ther- mal laser (1,064
nm wavelength) and infrared diode 810 nm.
This requires a fiberoptic contact probe.
3. Cyclodiathermy: Destruction of ciliary
processes by diathermy probe (needle).
4. Cycloanemization: Rarely done nowadays,
but it was considered in past. Surgically deprive
ciliary body of its blood.

RETROBULBAR
ALCOHOL INJECTION

In an absolute glaucoma, where the eye is blind and

pain- ful, one can inject 4 cc of absolute alcohol in
the retrobul- bar space. It would cause severe
inflammation, but de- stroy sensory nerves to give
pain relief. Often the muscles of ocular motility and
the levator palpebrae superioris are also paralyzed as
well making for an unsightly side effect.

SURGERY FOR CONGENITAL

GLAUCOMA
 Chapter 29 Glaucoma Surgery 448
patients with congenital glaucoma have opacities
and cor- neal edema, which would not allow for
this procedure).
Under general anesthesia the angle of the AC is
visual- ized with a surgical gonioscope like the
Barkan’s lens and through a paracentesis the
opposite angle is approached with the knife (e.g.
Swan goniotomy knife). The anterior portion of
the trabecular meshwork is incised circumfer-
entially to allow the trabecular fibers and iris to fall
back, and relieve resistance to outflow into the
canal of Sch- lemm (Figs 29.17 and 29.18).

Trabeculotomy
Principle
Trabeculotomy is an ab externo procedure
where the canal of Schlemm is dissected into a
probe passed into the same and rotated into
the AC to disrupt the trabec- ular meshwork to
establish connection with the canal (Figs
29.19A to G).

FIGURE 29.17: Goniotomy involves incising the anterior

trabecular meshwork under direct gonioscopic visualization,
allowing the trabecular meshwork and iris to fall back. This
relieves resistance to aqueous flow into the canal of Schlemm.

FIGURE 29.18: In trabeculotomy, the canal of Schlemm is

identified externally and a probe passed through it and swung
into the AC creating a tear in the trabecular meshwork. There
is a guide rod parallel to the probe seen outside the eye to let
the surgeon know the position of the probe. This allows the
trabecular meshwork and iris to fall back and relieves
resistance to aqueous flow into the canal of Schlemm.
 Chapter 29 Glaucoma Surgery 449

FIGURES 29.19A to G: Trabeculotomy. A. A partial thickness

scleral flap is raised; B. Under the scleral flap a radial incision
is made and deepened till Schlemm’s canal is opened (arrow);
C. Harms trabeculotome. Note the two parallel rods, the lower
probe and the higher guide rod; D. The trabeculotome is
inserted into the Schlemm’s canal; E. Once inserted fully the
direction of the probe is judged with the direction of guide
rod; F. Now the probe is rotated into the anterior chamber (AC);
G. The probe is inserted and rotated into AC on the left-hand
side also.
 Chapter 29 Glaucoma Surgery 446

Method lemm. During the rotation the surgeon knows the

After a limited peritomy, a partial thickness scleral probe orientation based on the direction of the
flap is raised. At the posterior end of the blue guide rod on the probe (otherwise one can easily
limbus a radial incision is placed straddling the damage nearby tissues like Descemet’s
scleral spur. This is deep- ened till the canal of membrane, iris, lens and ciliary body). The same is
done on the left-hand side also and the scleral flap
Schlemm is opened (a drop of aque- ous from the
and conjunctiva are closed with sutures.
AC or blood from the episcleral veins would reflux
Postoperatively the child is kept on close follow-up
often). Now, Harms trabeculotomy probe is passed
for life. All congenital glaucoma cases need lifelong
into the right-hand side segment of the Schlemm’s
follow- up. The glaucoma may go out of control even
canal and rotated into the AC to tear open the
very late and may require medical therapy (and if it
trabecular mesh- work and establish
fails resurgery with a trabeculectomy or drainage
communication to the canal of Sch- surgery added on).
Chapter 29 Glaucoma Surgery 447