Ophthalmology

For Elsevier
Commissioning Editor: Ellen Green
Project Development Manager: Helen Leng
Project Manager: Frances Affleck
Designer: George Ajayi
Ophthalmology
Jack J Kanski MD MS FRCS FRCOphth
Honorary Consultant Ophthalmic Surgeon
Prince Charles Eye Unit
King Edward VII Hospital
Windsor
UK

Brad Bowling FRCSEd (Ophth), FRCOphth

Specialist Registrar
Oxford Eye Hospital
Oxford
UK

EDINBURGH LONDON NEW YORK OXFORD PHILADELPHIA ST LOUIS SYDNEY TORONTO 2OO5
An imprint of Elsevier Limited

©Longman Group Ltd 1984, 1992

©Pearson Professional Limited 1997
©Harcourt Brace and Company Limited 1998
©Harcourt Publishers Limited 2001
©2005, Elsevier Limited. All rights reserved.

The right of Jack Kanski and Brad Bowling to be identified as authors of this work has been
asserted by them in accordance with the Copyright, Designs and Patents Act 1988.

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First published as Colour Aids Ophthalmology 1984

First Colour Guide edition 1992
Second Colour Guide edition 1997
Reprinted 1998
Reprinted 2001
First In Focus edition 2005

ISBN 0443100306

British Library Cataloguing in Publication Data

A catalogue record for this book is available from the British Library

Library of Congress Cataloging in Publication Data

A catalog record for this book is available from the Library of Congress

Note
Medical knowledge is constantly changing. Standard safety precautions must be followed, but
as new research and clinical experience broaden our knowledge, changes in treatment and
drug therapy may become necessary or appropriate. Readers are advised to check the most
current product information provided by the manufacturer of each drug to be administered
to verify the recommended dose, the method and duration of administration, and
contraindications. It is the responsibility of the practitioner, relying on experience and
knowledge of the patient, to determine dosages and the best treatment for each individual
patient. Neither the publisher nor the authors assumes any liability for any injury and/or
damage to persons or property arising from this publication.
The
publisher’s
policy is to use
paper manufactured
from s us tainable fores ts

Printed in China
 Acknowledgements

We are very grateful to the following colleagues for providing us with illustrations:
M Batterbury (Fig. for Question 28); Prof A Bird (Figs 50, 217, 218, 242, 243,
248); R Chopdar (Fig. for Question 101); Eye Academy (Fig. for Question 95);

A
J Federman (Fig. 229); R Marsh (Fig. 62); B Mathalone (Figs 67, 250, 258, 259);

c
k
C Migdal (Figs 70, 69, 237); A Mitchell (Fig. 56); P Morse (Figs 235, 265);

n
T Rahman (Figs 136, 200); A Ridgeway (Fig. 98); J Shilling (Fig. 132);

o
w
A Shun-Shin (Figs 44, 48, 125, 232, 245); D Spalton (Fig. 128); V Tanner (Fig. 43);

l
e
D Taylor (Fig. 167).

d
g
e
m
e
n
t
s
This pa ge inte ntiona lly le ft bla nk
 Contents

1. Blepharitis 2 26. Secondary open-angle

2. Non-neoplastic eyelid nodules 4 glaucomas 52
27. Primary angle-closure

C
3. Benign tumours of the eyelids 6

o
glaucoma 54

n
4. Premalignant and malignant

t
28. Secondary angle-closure

e
tumours of the eyelids 8

n
glaucomas 56

t
s
5. Ptosis 10
29. Developmental glaucomas 58
6. Entropion, ectropion and
trichiasis 12 30. Age-related cataract 60
7. Thyroid eye disease 14 31. Miscellaneous disorders of the
lens 62
8. Orbital tumours 16
32. Diabetic retinopathy 64
9. Miscellaneous orbital disease 18
33. Retinal vascular occlusion 66
10. Acute conjunctivitis 20
34. Miscellaneous retinopathies 68
11. Chronic conjunctivitis 22
35. Age-related macular
12. Conjunctival tumours 24 degeneration 70
13. Keratoconjunctivitis sicca and 36. Acquired maculopathies 72
cicatrizing conjunctivitis 26
37. Dystrophies of the fundus 74
14. Suppurative keratitis 28
38. Retinal detachment 76
15. Herpes simplex infection 30
39. Tumours of the uvea 78
16. Herpes zoster ophthalmicus 32
40. Tumours of the retina and optic
17. Corneal dystrophies 34 nerve head 80
18. Peripheral corneal ulceration 36 41. Acquired optic nerve
19. Disorders of corneal size and disorders 82
shape 38 42. Congenital optic disc
20. Episcleritis and scleritis 40 anomalies 84
21. Anterior uveitis 42 43. Childhood strabismus
(squint) 86
22. Posterior uveitis – infections 44
44. Third, fourth and sixth nerve
23. Non-infectious intermediate and palsies 88
posterior uveitis 46
45. Trauma 90
24. Idiopathic multifocal white dot
syndromes 48 Questions 92

25. Primary open-angle Answers 145

glaucoma 50 Index 169
 1 Blepharitis

Definition Very common chronic inflammation of the eyelid margins.

Classification Divided into anterior and posterior forms: the former may be
staphylococcal or seborrhoeic; a mixed picture is typical,
s
i
however.
t
i
r
a
h
Aetiology Causative factors:
p
e
l
• Staphylococcal: chronic infection of the bases of the
B
lashes – common in patients with eczema
• Seborrhoeic: usually associated with seborrhoeic
dermatitis – involves excess lipid production by eyelid
glands, converted to fatty acids by bacteria
• Posterior: dysfunction of the meibomian glands of the
posterior lid margins – common in patients with acne
rosacea.

Clinical Symptoms: usually worse in the morning, include grittiness,

features burning and redness, stickiness and crusting of the lids.
Signs
• Staphylococcal: dandruff-like scaling, mainly around the
eyelash bases (Fig. 1)
• Seborrhoeic: greasy debris around the lashes causing
them to adhere to one another (Fig. 2)
• Posterior: frothy tear film (Fig. 3) and plugging of the
meibomian gland orifices (Fig. 4)
All types usually manifest hyperaemia of the lid margins
(Fig. 5) and conjunctiva, and tear film instability.

Complications Corneal epitheliopathy, scarring, and marginal keratitis

(see Fig. 102); recurrent bacterial conjunctivitis, chalazia
(see Fig. 7) and styes (see Fig.10). Loss of lashes (madarosis)
and misdirection (trichiasis; Fig. 6).

Management • Lid margin hygiene using a weak solution of baby

shampoo
• Tear substitutes (e.g. hypromellose, carbomers)
• Antibiotic ointment (e.g. fusidic acid, chloramphenicol)
rubbed into the lid margins
• Systemic tetracycline.
2
 1

B
l
e
p
h
a
r
i
t
i
s
Fig. 1 Scales around eyelash bases in Fig. 2 Greasy adherent lashes, in
staphylococcal anterior blepharitis. seborrhoeic posterior blepharitis.

Fig. 3 Foam in the tear film. Fig. 4 Blocked meibomian gland orifices
in posterior blepharitis.

3
Fig. 5 Hyperaemia of lid margin. Fig. 6 Eyelash misdirection in long-
standing anterior blepharitis.
 2 Non- neoplastic eyelid nodules

Meibomian cyst (chalazion)

Definition A lesion consisting of lipogranulomatous inflammation
centred on a dysfunctional meibomian gland.
s
Clinical Extremely common, particularly in patients with posterior
e
ul
features blepharitis. A chronic, usually solitary, painless, firm swelling
d
in the tarsal plate (Fig. 7); can follow an acute meibomian
no
gland infection (see below). May be associated with a
d
secondary conjunctival granuloma (Fig. 8).
i
l
e
Management
y
Spontaneous resolution may occur, although usually only if
e
the lesion is small. Surgical incision and curettage is often
c
i
required.
t
s
a
Internal hordeolum (acute chalazion)
l
p
o
Definition An acute bacterial meibomian gland infection.
ne
Clinical
n-
An inflamed swelling within the tarsal plate which may be
features
o
associated with preseptal cellulitis (Fig. 9).
N
Management Topical antibiotic ointment and systemic antibiotics
(e.g. flucloxacillin) for preseptal cellulitis. Hot bathing may
promote discharge. Incision and curettage may be required
for a large abscess, or for a secondary chronic lesion.
External hordeolum (stye)
Definition A small abscess of an eyelash follicle.
Clinical An acute, painful inflamed swelling on the anterior lid
features margin, usually pointing through the skin (Fig. 10).
Management Removal of the associated lash, and hot bathing. Topical
antibiotic ointment. Large lesions may require incision.
Cysts of Zeis and Moll
Clinical A cyst of Zeis is a small, whitish, chronic, painless, opaque
features nodule on the lid margin (Fig. 11). A cyst of Moll is similar
but translucent.
Management Simple excision.
Molluscum contagiosum
Clinical Single or multiple, small, pale, waxy umbilicated nodules
features (Fig. 12), which may cause a secondary chronic ipsilateral
follicular conjunctivitis (see Fig. 55). These virally transmitted
lesions are common and more severe in AIDS patients.
4 Management Expression or cautery.
 2

N
o
n
-
n
e
o
p
l
a
Fig. 7 Large meibomian cyst. Fig. 8 Conjunctival granuloma secondary

s
to meibomian cyst.

t
i
c
e
y
e
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i
d
n
o
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u
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e
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Fig. 9 Internal hordeolum with preseptal Fig. 10 External hordeolum (stye).
cellulitis.

5
Fig. 11 Cyst of Zeis. Fig. 12 Lesions of molluscum contagiosum.
 3 Benign tumours of the eyelids

Squamous cell papilloma (viral wart)

Clinical The most common benign tumour of the eyelid which may
features be broad-based (sessile) or pedunculated (Fig. 13).
s
d
Management Simple excision, cautery or laser ablation.
i
l
e
Basal cell papilloma (seborrhoeic keratosis)
y
e
Clinical
he
This common tumour, usually found in the elderly, is a
features slowly-enlarging brownish papillary lesion with a greasy,
t
f
friable surface (Fig. 14).
o
s
Management Simple excision or curettage.
ur
o
Keratoacanthoma
um
Clinical An uncommon, fast-growing, firm, pinkish nodule that
t
n
features develops a keratin-filled crater (Fig. 15) and may be
g
mistaken for a malignancy. Remains static for several
ni
e
months before undergoing spontaneous involution.
B
Melanocytic naevus
Intradermal naevus An elevated lesion with variable
pigmentation. When located on the lid margin may be
associated with protruding lashes (Fig. 16). No malignant
potential.
Junctional naevus A flat, well circumscribed lesion with a
uniform brown colour, so-called because the naevus cells are
located at the junction of the dermis and epidermis. Low
malignant potential.
Compound naevus Usually elevated, with a homogeneous
tan to brown colour. Consists of both intradermal and
junctional components, the latter conferring a low
malignant potential.
Capillary haemangioma (strawberry naevus)
Clinical An irregular red lesion in an infant which may cause a
features mechanical ptosis and amblyopia (Fig. 17).
Management Local steroids if necessary, but frequently undergoes gradual
spontaneous involution.
Plexiform neurofibroma
Typically occurs in neurofibromatosis-1, characteristically
6 giving rise to an S-shaped lid margin and ptosis (Fig. 18).
 3

B
e
n
i
g
n
t
u
m
Fig. 13 Squamous cell papilloma (viral Fig. 14 Basal cell papilloma (seborrhoeic

o
wart). keratosis).

u
r
s
o
f
t
h
e
e
y
e
l
i
d
s
Fig. 15 Keratoacanthoma. Fig. 16 Intradermal naevus.

Fig. 17 Capillary haemangioma Fig. 18 Plexiform neurofibroma. 7

(strawberry naevus).
 4 Premalignant and malignant
tumours of the eyelids
Actinic (solar) keratosis
Clinical Although rare, this is the most common premalignant lid
features condition and is strongly associated with excessive sun
s
exposure in light-skinned individuals. It usually presents as a
d
i
persistent scaly plaque (Fig. 19), which must be biopsied.
l
e
y
e
Basal cell carcinoma
he
Basal cell carcinoma (BCC), the most common eyelid
t
f
malignancy, is locally invasive but does not metastasize.
o
About 50% involve the lower lid, 30% the medial canthal
s
ur
area.
o
um
Clinical Nodulo-ulcerative A ‘rodent ulcer’, with rolled
features hyperkeratotic edges and central granulation (Fig. 20),
t
nt
gradually enlarging over 1–2 years. A purely nodular
na
appearance is common (Fig. 21).
g
Sclerosing A flat indurated plaque with poorly demarcated
i
l
a
margins, often with loss of overlying lashes (Fig. 22).
m
nd
Squamous cell carcinoma
a
Clinical Squamous cell carcinoma (SCC) is much less common than
nt
features BCC. It grows more quickly and may metastasize. It may
na
arise de novo or from a premalignant condition such as
g
i
actinic keratosis (see above).
l
a
m
Nodular SCC Starts as a hyperkeratotic nodule or plaque
e
r
which later develops crusting fissures.
P
Ulcerative SCC Resembles a rodent ulcer (Fig. 23).

Sebaceous gland carcinoma

Clinical This is a rare but very aggressive tumour, which may
features originate in a meibomian or Zeis gland as a firm nodule
either on the lid margin or within the tarsal plate (Fig. 24),
when it may be mistaken for a chalazion (see Fig. 7).

Management • Surgical excision with a wide clearance margin is the

treatment of choice for most lid malignancies
• Radiotherapy in selected cases.

8
 4

P
r
e
m
a
l
i
g
n
a
n
Fig. 19 Actinic keratosis. Fig. 20 Rodent ulcer.

t
a
n
d
m
a
l
i
g
n
a
n
t
t
u
m
o
u
r
s
o
f
t
h
e
Fig. 21 Nodular basal cell carcinoma. Fig. 22 Sclerosing basal cell carcinoma.
e
y
e
l
i
d
s
Fig. 23 Ulcerative squamous cell Fig. 24 Sebaceous gland carcinoma. 9
carcinoma.
 5 Ptosis

Definition The upper eyelid rests at a lower position than normal.

Classification Neurogenic
• Third (oculomotor) nerve palsy (Fig. 25): must exclude
s
i
‘surgical’ cause (e.g. aneurysm, tumour) compressing nerve
s
o
• Horner’s syndrome (Fig. 26): congenital or acquired
t
P
dysfunction of the sympathetic autonomic pathway
• Marcus Gunn (‘jaw-winking’) syndrome: congenital,
uncommon.

Aponeurotic
• Involutional: age-related laxity of the levator aponeurosis
(Fig. 27)
• Postoperative: stretching of the aponeurosis during
surgery.

Mechanical
• Excessive lid weight: oedema, tumours (see Figs 17, 18),
redundant skin
• Cicatricial: reduced mobility from scarring of the upper
lid skin or conjunctiva.

Myogenic
• Simple congenital: unilateral or bilateral (Fig. 28)
• Blepharophimosis syndrome: rare, congenital bilateral
ptosis, associated with other eyelid and facial
abnormalities (Fig. 29)
• Acquired: myasthenia gravis, ocular myopathy, myotonic
dystrophy (Fig. 30).

Salient points • Third nerve palsy: pupillary involvement carries high index
of suspicion for ‘surgical’ aetiology. Misdirection of
regenerating nerve fibres may occur, resulting in ptosis or
lid retraction on eye movement
• Horner’s syndrome: ipsilateral miosis, heterochromia if
congenital, sweating decreased on affected side
dependent on location of lesion.

Management • Address any treatable cause (e.g. myasthenia gravis)

• Surgical options include levator or aponeurosis
strengthening and frontalis (brow) suspension.
10
 5

P
t
o
s
i
s
Fig. 26 Right third nerve palsy. Fig. 26 Left Horner’s syndrome.

Fig. 27 Severe bilateral involutional ptosis. Fig. 28 Severe left congenital ptosis.

Fig. 29 Blepharophimosis syndrome. Fig. 30 Bilateral ptosis in myotonic 11

dystrophy.
 6 Entropion, ectropion and
trichiasis
Entropion
Definition An inward-turning of the eyelid. If severe or prolonged may
cause corneal scarring (Fig. 31).
s
i
Classification • Involutional: most common form; results from age-
s
a
related changes in the lower lid
hi
• Cicatricial: most frequently secondary to scarring of the
c
i
r
upper palpebral conjunctiva, as in chronic trachoma
t
nd
(Fig. 32)
• Spastic: lower lid; caused by spasm of the orbicularis
a
muscle due to ocular irritation or essential
n
o
blepharospasm. Frequently temporary
i
p
• Congenital: very rare; only involves the lower lid. Caused
o
r
by hypertrophy of skin and orbicularis.
t
c
e
Management Surgical correction.
n,
o
i
Ectropion
p
o
r
Definition An outward-turning eyelid, virtually exclusively involving
nt
E
the lower lid. If severe and prolonged may cause
conjunctival keratinization (Fig. 33).
Classification • Involutional: the most common form; age-related tissue
laxity
• Cicatricial: scarring resulting from burns or surgery
(e.g. tumour resection; Fig. 34)
• Mechanical: excess lid weight (e.g. large tumour)
• Paralytic: facial nerve palsy; associated with incomplete
blinking and lid closure (Fig. 35)
• Congenital: may be part of the blepharophimosis
syndrome (see Fig. 29).
Management Surgical correction.

Trichiasis
Definition Inward misdirection of lashes, often secondary to acute or
chronic lid inflammation (e.g. blepharitis, trachoma).
Typically causes corneal irritation and sometimes scarring
(Fig. 36). Should be differentiated from distichiasis, in which
extra lashes (usually congenital) arise from meibomian
gland orifices.
Management Options include simple epilation, electrolysis, cryotherapy
12 and laser ablation.
 6

E
n
t
r
o
p
i
o
n
,
e
Fig. 31 Entropion of lower eyelid and Fig. 32 Cicatricial entropion.

c
corneal scarring.

t
r
o
p
i
o
n
a
n
d
t
r
i
c
h
i
a
s
i
s
Fig. 33 Ectropion and conjunctival Fig. 34 Cicatricial ectropion.
keratinization.

Fig. 35 Paralytic ectropian with poor lid Fig. 36 Trichiasis in trachoma. 13

closure.
 7 Thyroid eye disease

Aetiology Autoantibodies to both thyroid and orbital tissue cause

inflammation; the extraocular muscles are particularly
affected.
e
Clinical The clinical course involves an ‘active’ congestive stage,
s
a
features usually lasting 2–3 years, followed by a ‘quiescent’ stage in
e
s
which residual restriction of ocular movements may be the
i
d
chief feature.
e
y
e
Symptoms
d
• Redness
i
o
r
• Irritation
hy
• Aching
T
• Wide-eyed ‘staring’ appearance
• Double vision
• Decreased vision in severe cases.
Signs
• Fullness of the eyelids
• Conjunctival hyperaemia and chemosis (Fig. 37)
• Proptosis (exophthalmos): may be unilateral or bilateral
(Fig. 38). If severe, may prevent adequate lid closure with
resultant exposure keratopathy
• Lid retraction in primary position (Fig. 39), which
compounds the cosmetic effect of proptosis
• Lid lag in downgaze (von Graefe’s sign)
• Ophthalmoplegia due to inflammation early in the disease
and subsequently to fibrosis. The inferior and medial recti
are most frequently affected
• Choroidal folds (Fig. 40)
• Optic neuropathy is a sight-threatening complication
caused by compression of the optic nerve or its blood
supply by swollen orbital tissue, particularly extraocular
muscles. Useful tests include colour vision, visual acuity
and visual fields. There may be a relative afferent
pupillary defect and optic disc oedema.
Management • Tear substitutes and topical steroids for conjunctival and
corneal involvement
• Systemic steroids are usually reserved for optic neuropathy
• Surgery for proptosis (orbital decompression), diplopia
(muscle surgery) and eyelid retraction
• Radiotherapy may be appropriate for the congestive
14 phase, if severe.
 7

T
h
y
r
o
i
d
e
y
e
Fig. 37 Conjunctival hyperaemia and Fig. 38 Right proptosis.

d
i
chemosis.

s
e
a
s
e
Fig. 39 Bilateral lid retraction. Fig. 40 Choroidal folds.

15
 8 Orbital tumours

Capillary haemangioma
Clinical Presents in infancy with an anterior orbital swelling (Fig. 41),
features which may increase in size when crying. A similar eyelid skin
s
lesion, a ‘strawberry naevus’ (see Fig. 17), may also be present.
r
u
Management Steroids injected into the lesion or given systemically are
o
um
effective but the tumour often involutes spontaneously.
t
Cavernous haemangioma
l
a
t
Clinical
i
The most common benign orbital tumour in adults. Presents
b
r
features in young adults with painless axial proptosis of gradual onset.
O
Management Surgical excision.
Orbital varices
Clinical Dilated orbital veins. Presentation is at any age with:
features • Intermittent unilateral non-pulsatile proptosis
• Visible lesions in the eyelids or conjunctiva (Fig. 42)
• Acute orbital haemorrhage or thrombosis (less common).
Management Surgical excision may be required.
Rhabdomyosarcoma
Clinical This very rare but aggressive tumour typically presents at
features about the age of 7 years with progressive proptosis and a
palpable mass may be present (Fig. 43).
Management Incisional biopsy followed by radiotherapy and chemotherapy.
Neural tumours
Optic nerve glioma Presents in childhood with slowly
progressive proptosis and visual loss. The optic disc may be
swollen or pale (see Fig. 238). 25–50% of patients have
neurofibromatosis-1.
Optic nerve sheath meningioma Typically affects middle-
aged females and causes slowly progressive visual loss
followed later by proptosis (Fig. 44). The optic disc
frequently shows opticociliary shunt vessels (Fig. 45).
Management Options include observation, surgery and radiotherapy.
Dermoid cyst
Clinical Variable age at presentation, with proptosis and/or a
features palpable mass (Fig. 46) depending on site and size.
16
Management Excision, which must be complete.
 8

O
r
b
i
t
a
l
t
u
m
Fig. 41 Capillary haemangioma. Fig. 42 Orbital varices.

o
u
r
s
Fig. 43 Rhabdomyosarcoma. Fig. 44 Left proptosis due to optic nerve
sheath meningioma.

Fig. 45 Opticociliary shunt vessels. Fig. 46 Dermoid cyst. 17

 9 Miscellaneous orbital disease

Orbital cellulitis
Definition A potentially life-threatening acute bacterial infection of
the soft tissues of the orbit.
e
Aetiology
s
• Secondary to sinusitis (usually in children)
a
e
• Spread from infected adjacent structures (e.g. dacryocystitis)
s
i
• Following trauma and surgery.
d
l
Clinical
a
Symptoms Acute lid swelling and redness, pain and
t
i
features malaise.
b
r
o
Signs Reduced visual acuity, lid oedema and erythema
us
(Fig. 47), chemosis, proptosis, painful ophthalmoplegia and
o
optic disc swelling.
ne
a
Complications Intracranial infection, cavernous sinus thrombosis,
l
l
e
subperiosteal abscess, and blindness.
c
s
Management
i
• Intravenous antibiotics
M
• Orbital CT, principally to rule out an abscess
• Surgery for abscess drainage or sinus washout.
Idiopathic orbital inflammation (pseudotumour)
Definition Idiopathic inflammation of the soft tissues of the orbit.
Clinical Subacute onset of unilateral pain, lid oedema, chemosis,
features proptosis (Fig. 48), decreased vision and ophthalmoplegia.
Management Systemic steroids, radiotherapy or cytotoxic agents.
Carotid- cavernous fistula
Definition Indirect or direct arterial communication with the cavernous
sinus. Usually due to trauma or spontaneous arterial
rupture.
Clinical Headache, chemosis (Fig. 49), dilated episcleral vessels
features (Fig. 50), pulsatile proptosis with associated thrill and bruit,
ophthalmoplegia, raised intraocular pressure and retinal
vascular congestion and haemorrhages (Fig. 51).
Management Radiological intervention if appropriate.
Blow- out fracture of orbital floor
Clinical Periorbital oedema and ecchymosis, enophthalmos (Fig. 52),
features vertical diplopia, infraorbital nerve anaesthesia and
subcutaneous emphysema.
18
Management Surgery may be necessary in severe cases.
 9

M
i
s
c
e
l
l
a
n
e
o
Fig. 47 Orbital cellulitis. Fig. 48 Orbital pseudotumour.

u
s
o
r
b
i
t
a
l
d
i
s
e
a
s
e
Fig. 49 Severe chemosis due to direct Fig. 50 Dilated episcleral vessels due to
carotid–cavernous fistula. indirect carotid–cavernous fistula.

Fig. 51 Retinal haemorrhages and dilated Fig. 52 Right enophthalmos due to 19

veins in carotid–cavernous fistula. orbital floor blow-out fracture.
 10 Acute conjunctivitis

Acute allergic conjunctivitis

Acute onset of bilateral itching, lid oedema and chemosis
(Fig. 53), often in children after playing on grass in spring or
s
summer. Settles spontaneously within a few hours.
i
t
i
v
Seasonal allergic (hay fever) conjunctivitis
i
t
unc
Clinical Persistent itching, conjunctival hyperaemia and excess
features mucus production during the hay fever season.
nj
Management
o
Topical mast cell stabilizers and antihistamines usually
c
provide adequate symptomatic relief.
e
ut
Bacterial conjunctivitis
c
A
Clinical Subacute onset of bilateral, but frequently asymmetrical,
features redness, grittiness and a sticky discharge. Examination
shows conjunctival hyperaemia (Fig. 54) and mild papillary
changes. Visual acuity, corneas and pupils are normal.
Management Topical antibiotics (e.g. chloramphenicol, fusidic acid,
gentamicin) for about a week.
Adenoviral conjunctivitis
Clinical Acute onset of frequently bilateral grittiness and redness
features and a watery discharge. Examination shows a follicular
conjunctival reaction (Fig. 55), preauricular lymphadenopathy,
lid oedema, chemosis and subconjunctival haemorrhages
(Fig. 56). A punctate epithelial keratitis may progress to
persistent deeper infiltrates (Fig. 57). The condition is highly
contagious.
Management Topical lubricants; steroids may be considered for severe
keratitis.
Neonatal conjunctivitis
Definition A conjunctival inflammation occurring within the first
month of life.
Aetiology The timing of presentation may aid diagnosis.
• Chlamydia: the most common (Fig. 58); presents at
1–3 weeks
• Gonococcus: during first week
• Herpes simplex: 1–2 weeks
• Simple bacterial conjunctivitis: end of first week onwards.
Management • Ensure cornea is uninvolved
20 • Investigate and treat individual conditions as appropriate
(including maternal infection).
 10

A
c
u
t
e
c
o
n
j
u
Fig. 53 Acute allergic conjunctivitis. Fig. 54 Bacterial conjunctivitis.

n
c
t
i
v
i
t
i
s
Fig. 55 Follicular conjunctivitis in Fig. 56 Subconjunctival haemorrhages in
adenoviral infection. severe adenoviral conjunctivitis.

Fig. 57 Corneal infiltrates following Fig. 58 Mucopurulent discharge in 21

adenoviral conjunctivitis. neonatal chlamydial infection.
 11 Chronic conjunctivitis

Adult chlamydial conjunctivitis

Clinical Sexually transmitted infection presenting with subacute
features onset of typically unilateral mucopurulent discharge
s
associated with large conjunctival follicles (Fig. 59) and
i
t
preauricular lymphadenopathy.
i
v
i
t
Management
unc
• Laboratory testing to confirm diagnosis
• Referral for assessment by a genitourinary specialist
nj
• Treat with antibiotics (e.g. azithromycin).
o
c
Vernal keratoconjunctivitis (VKC)
c
ni
o
Clinical A bilateral chronic or recurrent allergic condition that starts
hr
features in childhood, usually in patients with atopy (e.g. asthma,
C
eczema and hay fever).
Symptoms include itching, watering, photophobia and a
stringy discharge. Papillary conjunctivitis particularly
involves the superior tarsus (Fig. 60) and sometimes
the limbus (Fig. 61). Corneal signs include punctate
epitheliopathy, ulceration, plaque formation (Fig. 62)
and scarring, occasionally in the form of a limbal ‘Cupid’s
bow’ (‘pseudogerontoxon’ – Fig. 63).
Management Topical mast cell stabilizers, antihistamines and topical
steroids.

Atopic keratoconjunctivitis
Clinical A rare condition typically affecting young men with atopic
features dermatitis (eczema). Similar to VKC but carries a worse
prognosis. The eyelids are thickened and crusted, and
associated staphylococcal blepharitis (see Fig. 1) is common.
Intense papillary conjunctivitis may lead to symblepharon
(see Fig. 75), and corneal complications can be severe.
Aggressive herpes simplex keratitis (see Fig. 86) and
microbial keratitis (see Figs 77–80) may occur.
Management Similar to VKC but frequently more resistant.

Giant papillary conjunctivitis

A foreign-body-associated conjunctivitis characterized by
giant papillae on the superior tarsus (Fig. 64). Causes
include contact lens wear, artificial eyes and protruding
22 sutures.
 11

C
h
r
Fig. 59 Large follicles in chlamydial Fig. 60 Papillary conjunctivitis.

o
conjunctivitis.

n
i
c
c
o
n
j
u
n
c
t
i
v
i
t
i
s
Fig. 61 Vernal limbitis. Fig. 62 Corneal plaque.

Fig. 63 Pseudogerontoxon. Fig. 64 Giant papillae. 23

 12 Conjunctival tumours

Papilloma
Pedunculated papilloma Caused by a papillomavirus.
Typically affects children and young adults and may be
s
multiple. Usually located on the palpebral conjunctiva,
r
u
fornix or caruncle.
o
um
Sessile papilloma Affects older patients. Invariably single
and unilateral (Fig. 65), located on the bulbar conjunctiva or
t
l
at the limbus.
a
v
i
t
Carcinoma
unc
Conjunctival and corneal intra-epithelial neoplasia (CCIN)
nj
A slightly elevated, fleshy vascular or gelatinous mobile
o
C
mass, most frequently juxtalimbal (Fig. 66), sometimes with
corneal extension. Low malignant potential.
Invasive carcinoma Similar in appearance but fixed to
underlying tissues.
Choristoma
Dermoid A rounded, white nodule typically located at the
limbus (Fig. 67).
Lipodermoid A soft yellow-white subconjunctival mass
usually found at the outer canthus.
Pigmented lesions
Conjunctival (racial) epithelial melanosis A very common
physiological pigmentation in dark-skinned individuals.
Conjunctival naevus An uncommon lesion presenting
during childhood or early adult life. Single, well-demarcated,
flat or slightly elevated, variably pigmented lesion, most
commonly at the limbus (Fig. 68); size/pigmentation may
increase at puberty.
Primary acquired melanosis (PAM) A rare condition
presenting in old age with uni- or multifocal slowly growing
patches of intraepithelial pigmentation. Some malignant
potential.
Melanoma A rare tumour accounting for 2% of all ocular
malignancies. Most frequently arises within PAM, as a
nodular lesion (Fig. 69). May arise from a pre-existing
24 naevus or, rarely, de novo, usually at the limbus (Fig. 70).
 12

C
o
n
j
u
n
c
t
i
v
a
Fig. 65 Sessile conjunctival papilloma. Fig. 66 Conjunctival and corneal

l
intraepithelial neoplasia.

t
u
m
o
u
r
s
Fig. 67 Limbal dermoid. Fig. 68 Conjunctival naevus.

Fig. 69 Melanoma arising within an area Fig. 70 Primary conjunctival melanoma. 25

of primary acquired melanosis.
 13 Keratoconjunctivitis sicca and
cicatrizing conjunctivitis
Keratoconjunctivitis sicca
Aetiology Keratoconjunctivitis sicca (KCS), or ‘dry eye’, is a very
common condition resulting from any process that affects
s
the production or evaporation of the tears. Age-related
i
t
lacrimal gland atrophy and autoimmune damage (Sjögren’s
i
v
i
syndrome) are common causes; secondary Sjögren’s
t
unc
syndrome is the association of KCS with a connective tissue
disorder (e.g. rheumatoid arthritis).
nj
o
Clinical Symptoms Include grittiness and redness, often worse in
c
features the evening.
ng
i
Signs
z
i
r
• Irregular patchy tear film with rapid break-up after blinking
t
a
• Thin marginal tear strip with mucous debris
c
i
c
• Dry conjunctiva and cornea stains with Rose Bengal (Fig. 71)
nd
• Corneal filaments (Fig. 72)
• Schirmer’s test measures the wetting of a strip of filter
a
a
paper: typically reduced.
c
c
Management
i
• Tear substitutes usually suffice if used regularly
s
• Punctal occlusion in severe cases.
s
i
t
i
v
Cicatrizing conjunctivitis
i
t
unc
Aetiology Bilateral conjunctival manifestation of immune-mediated
blistering mucocutaneous diseases; serious but rare.
nj
o
Cicatricial pemphigoid A chronic disease characterized by
c
o
skin and mucous membrane blister formation.
t
a
r
Stevens–Johnson syndrome An acute, severe but generally
e
K
self-limiting vasculitis most commonly caused by
hypersensitivity to certain drugs and infections. Characteristic
‘target’ skin lesions and oral mucosal lesions (Fig. 73) are seen.
Complications • Conjunctival scarring (Fig. 74) and shrinkage
• Adhesions between the bulbar and palpebral conjunctiva
(symblepharon, Fig. 75), and of the outer canthi
(ankyloblepharon)
• Dry eye
• Aberrant eyelash growth
• Cicatricial entropion
• Corneal keratinization (Fig. 76).
Management • Topical and systemic steroids
26 • Tear substitutes
• Surgery (mainly for lid deformity).
 13

K
e
r
a
t
o
c
o
n
j
u
Fig. 71 Dry conjunctiva and cornea Fig. 72 Corneal filaments.

n
stained with Rose Bengal.

c
t
i
v
i
t
i
s
s
i
c
c
a
a
n
d
c
i
c
a
t
r
i
z
i
n
g
c
o
n
Fig. 73 Haemorrhagic crusting of lips in Fig. 74 Conjunctival scarring in cicatricial
j
u
Stevens–Johnson syndrome. pemphigoid.
n
c
t
i
v
i
t
i
s
27
Fig. 75 Symblepharon. Fig. 76 Corneal keratinization in
cicatricial pemphigoid.
 14 Suppurative keratitis

Bacterial keratitis
Clinical This serious condition is usually associated with pre-existing
features corneal surface disease or contact lens wear.
s
i
Symptoms Subacute onset of unilateral pain, redness,
t
i
t
photophobia and blurred vision
a
r
e
k
Signs Circumcorneal injection, stromal infiltrate and an
e
overlying epithelial defect. A hypopyon may be present if
v
i
severe.
t
a
ur
Aetiology Staphylococcal and pneumococcal typically causes yellow-
p
white, oval suppuration surrounded by relatively clear
up
cornea (Figs 77, 78).
S
Pseudomonas causes irregular suppuration associated with
a mucopurulent discharge (Figs 79, 80). Very severe
infections may extend into the sclera.
Management Specimens for culture (corneal scrape, conjunctival swab,
contact lenses/case if available) followed by intensive
topical antibiotics (e.g. ofloxacin, fortified cefuroxime/
gentamicin combination).

Fungal keratitis
Clinical Filamentous Frequently preceded by ocular trauma
features involving vegetable matter. Characterized by greyish-white
ulceration with indistinct feathery margins and satellite
lesions (Fig. 81).
Candida Typically occurs in debilitated patients or those
with pre-existing surface disease; similar appearance to
bacterial keratitis.
Management Topical antifungal agents.

Acanthamoeba keratitis
Clinical Patients, usually contact lens wearers, typically experience
features pain disproportionate to the clinical signs. Early cases are
characterized by dendritiform epithelial lesions, radial
keratoneuritis and stromal keratitis followed by a central
ring abscess associated with variable epithelial breakdown
(Fig. 82).
28 Management Topical amoebicidal agents.
 14

S
u
p
p
u
r
a
t
i
v
e
Fig. 77 Staphylococcal keratitis. Fig. 78 Pneumococcal keratitis.

k
e
r
a
t
i
t
i
s
Fig. 79 Pseudomonas keratitis. Fig. 80 Advanced pseudomonas keratitis.

Fig. 81 Fungal keratitis. Fig. 82 Acanthamoeba keratitis. 29

 15 Herpes simplex infection

Primary infection
This is caused by direct transmission of virus through
infected secretions to a non-immune subject, usually a
n
child. It may cause follicular conjunctivitis (see Fig. 55),
o
blepharitis (Fig. 83) and epithelial keratitis.
i
t
c
e
nf
Recurrent epithelial keratitis
i
Aetiology
x
Invasion of the corneal epithelium by reactivated latent
e
virus; common.
l
p
m
Clinical Corneal sensation is diminished. A branching ‘dendritic’
i
s
features ulcer is characteristic, demonstrated well with fluorescein
s
e
staining (Fig. 84). A geographical (amoeboid) ulcer (Fig. 85)
p
r
develops from an enlarging dendritic lesion particularly
e
H
when the latter has inadvertently been treated with topical
steroids.
Management Topical antiviral agents (e.g. aciclovir, ganciclovir).

Stromal necrotic keratitis

Aetiology This uncommon condition is caused by direct viral invasion
and destruction of the corneal stroma.
Clinical Cheesy, necrotic stroma appearing similar to bacterial or
features fungal infection (Fig. 86), with associated anterior uveitis.
Complications include scarring (Fig. 87) and perforation.
Management • Treatment of any associated epithelial defect
• Topical steroids, to reduce stromal inflammation, with
antiviral and antibiotic cover.

Disciform keratitis
Aetiology Possibly a hypersensitivity reaction to herpes virus.
Clinical Subacute, usually painless blurring of vision which may be
features associated with haloes around lights. Examination shows
an area of epithelial and stromal oedema with associated
keratic precipitates (Fig. 88). Other findings include mild
iritis and a ring of infiltrates surrounding the lesion
(Wessely ring).
Management Topical steroids combined with antiviral cover. In some
30 cases, oral aciclovir may be considered to reduce recurrence.
 15

H
e
r
p
e
s
s
i
m
p
Fig. 83 Primary herpes simplex skin Fig. 84 Large dendritic ulcer stained with

l
e
lesions. fluorescein.

x
i
n
f
e
c
t
i
o
n
Fig. 85 Geographical herpetic ulcer Fig. 86 Stromal necrotic keratitis.
stained with fluorescein.

Fig. 87 Scarring complicating stromal Fig. 88 Disciform keratitis.

keratitis. 31
 16 Herpes zoster ophthalmicus

Aetiology Herpes zoster (shingles) is caused by the varicella zoster

virus.

Definition Approximately 15% of all cases of herpes zoster affect the

us
ophthalmic division of the trigeminal nerve (herpes zoster
c
i
ophthalmicus).
m
l
ha
Clinical Skin lesions
ht
features An initial maculopapular rash evolves to form vesicles (small
p
blisters), which subsequently burst to form crusting ulcers.
o
Periorbital oedema is common (Fig. 89). Postherpetic
r
e
neuralgia can be severe, chronic and very distressing.
t
s
o
z
Ocular complications
s
e
Ocular involvement is common when the rash involves the
p
r
side of the nose (Hutchinson’s sign).
e
H
• Conjunctivitis and episcleritis: very common and usually
transient
• Acute iritis: common and may give rise to elevation of
intraocular pressure and segmental iris atrophy (Fig. 90)
• Acute keratitis: punctate epithelial, filamentary (see
Fig. 72), microdendritic, nummular (Fig. 91) and disciform
(see Fig. 88)
• Chronic corneal lesions: mucus plaque keratitis, secondary
lipid keratopathy (Fig. 92) and neurotrophic keratitis,
which may lead to extreme thinning (Fig. 93) and
perforation
• Scleritis (Fig. 94): uncommon and frequently involves the
cornea (sclerokeratitis)
• Neuro-ophthalmological: optic neuritis and extraocular
muscle palsies.

Management • If administered early, systemic antiviral agents

(e.g. aciclovir, valaciclovir, famciclovir) curtail the rash
and reduce ocular complications and neuralgia
• Steroid–antibiotic preparations (e.g. hydrocortisone–fusidic
acid) applied to the skin may be beneficial prior to crust
separation
• Treatment of iritis and most acute corneal lesions is with
topical steroids.

32
 16

H
e
r
p
e
s
z
o
s
t
Fig. 89 Severe herpes zoster Fig. 90 Segmental iris atrophy following

e
r
ophthalmicus. herpes zoster iritis.

o
p
h
t
h
a
l
m
i
c
u
s
Fig. 91 Nummular keratitis. Fig. 92 Lipid keratopathy.

Fig. 93 Severe corneal thinning. Fig. 94 Scleral thinning due to previous 33

scleritis.
 17 Corneal dystrophies

Clinical Epithelial basement membrane (Cogan microcystic

features or map–dot–fingerprint) dystrophy
Common, sporadic and non-progressive. Presents in second
s
or third decade with recurrent corneal erosions
e
hi
characterized by grittiness, lacrimation and photophobia on
p
waking. Examination shows subtle bilateral dot-like, cystic
o
r
(Fig. 95) or linear epithelial lesions.
t
s
y
d
Reis–Bücklers and Thiel- Behnke dystrophies
l
a
ne
Presentation of these dominantly inherited dystrophies of
the Bowman layer, which underlies the corneal epithelium,
r
o
C
is during the first decade of life with recurrent corneal
erosions, followed later by variable blurring of vision.
Bilateral superficial reticular or honeycomb-pattern
opacities are seen (Fig. 96).

Lattice dystrophies
This group (types 1–3) of rare, dominantly inherited
dystrophies presents at varying ages with recurrent corneal
erosions. On examination a network of spidery lines involves
the anterior and mid stroma (Fig. 97), the precise
morphology being dependent on type.

Granular dystrophy
Rare, dominantly inherited, presenting during the first
decade with recurrent erosions. Discrete, crumb-like
granules are seen within the anterior stroma (Fig. 98).

Fuchs’ endothelial dystrophy

Presentation of this, usually sporadic condition, is in later
life with initially unilateral visual impairment. Examination
shows corneal oedema – ‘bullous keratopathy’ if severe
(Fig. 99). The fellow eye invariably shows characteristic
endothelial changes (cornea guttata).

Management • Varies according to type and severity of dystrophy

• Lubricants alone in mild cases
• Bandage contact lenses for severe erosions and painful
bullous keratopathy
34 • Corneal grafting (Fig. 100) for advanced disease.
 17

C
o
r
Fig. 95 Epithelial basement membrane Fig. 96 Honeycomb opacities in

n
Thiel–Behnke dystrophy.

e
dystrophy.

a
l
d
y
s
t
r
o
p
h
i
e
s
Fig. 97 Lattice dystrophy. Fig. 98 Granular dystrophy.

Fig. 99 Bullous keratopathy. Fig. 100 Corneal graft (penetrating

keratoplasty). 35
 18 Peripheral corneal ulceration

Dellen
Clinical An area of thinning secondary to local stromal dehydration,
features most frequently associated with an elevated limbal lesion
n
(Fig. 101).
o
i
Management Eliminate the cause and promote rehydration by patching
t
a
r
and lubricants.
e
c
ul
Marginal keratitis
l
a
ne
Aetiology Hypersensitivity to staphylococcal exotoxins. Very common
r
and frequently associated with chronic anterior blepharitis
o
c
(see Fig. 1).
l
a
Clinical Subepithelial infiltrate, separated from the limbus by clear
r
he
features cornea (Fig. 102), often with an overlying smaller area of
p
epithelial breakdown.
i
r
e
P
Management Short course of topical steroids (e.g. fluorometholone,
prednisolone 0.5%).

Rosacea keratitis
Clinical Occurs in about 5% of patients with acne rosacea. Inferior
features punctate epitheliopathy that may progress to subepithelial
infiltration and peripheral vascularization (Fig. 103).
Occasionally severe peripheral thinning and perforation may
occur.
Management • Topical steroids
• Systemic tetracycline or doxycycline.

Ulceration associated with rheumatoid arthritis

Clinical May be chronic, without inflammation (Fig. 104) or acute
features and associated with severe inflammation at the limbus
(Fig. 105).
Management • Lubricants
• Topical and systemic steroids
• Other systemic immunosuppressants.

Mooren’s ulcer
Clinical Very rare but serious, painful condition that may be
features unilateral or bilateral and may spread circumferentially or
36 centrally (Fig. 106).
 18

P
e
r
i
p
h
e
r
a
l
c
Fig. 101 Corneal dellen stained with Fig. 102 Marginal keratitis.

o
fluorescein.

r
n
e
a
l
u
l
c
e
r
a
t
i
o
n
Fig. 103 Rosacea keratitis. Fig. 104 Corneal melting in rheumatoid
arthritis.

Fig. 105 Acute corneal ulceration in Fig. 106 Advanced Mooren’s ulcer. 37
rheumatoid arthritis.
 19 Disorders of corneal size and
shape
Keratoconus
Clinical Fairly common cone-like bulging of the central cornea
features (Fig. 107), presenting during the second or third decades of
e
life with slowly progressive blurring of vision from irregular
p
astigmatism. Both eyes are affected in almost all cases,
ha
though often asymmetrically.
s
nd
Early signs Include abnormal ophthalmoscopy reflex,
a
irregular ‘scissors’ retinoscopy reflex and fine vertical lines
e
z
in the deep stroma (Vogt striae – Fig. 108).
i
s
l
Late signs Include iron deposits at the base of the cone
a
ne
(Fleischer ring), bulging of the lower lid in downgaze
r
(Munson sign – Fig. 109), central corneal oedema of sudden
o
c
onset (acute hydrops – Fig. 109) and scarring.
f
o
Systemic associations Include atopic dermatitis,
s
r
osteogenesis imperfecta; syndromes – Down’s, Turner’s,
e
d
Ehlers–Danlos, Marfan’s.
r
o
s
Management • Refractive correction with spectacles is initially adequate
i
D
• As progression occurs, correction with contact lenses
• Corneal grafting in advanced cases
• Supportive treatment for acute hydrops.

Keratoglobus
Very rare congenital bilateral thinning and protrusion of the
entire cornea (Fig. 110). Acute hydrops may occur in some
cases.

Megalocornea
Very rare, X-linked recessive condition with corneal diameters
over 13 mm (Fig. 111). May develop lens subluxation.

Microcornea
Corneal diameters of less than 10 mm.
Classification • True microcornea: globe of normal dimensions.
• Sclerocornea: ‘scleralization’ of the peripheral cornea
makes it appear small
• Microphthalmos: small variably malformed globe (Fig. 112)
• Nanophthalmos: severe hypermetropia because of short
38 axial length, but otherwise normal.
 19

D
i
s
o
r
d
e
r
s
o
f
Fig. 107 Keratoconus. Fig. 108 Vogt’s striae.

c
o
r
n
e
a
l
s
i
z
e
a
n
d
s
h
a
p
e
Fig. 109 Munson’s sign in an eye with Fig. 110 Keratoglobus.
acute hydrops.

Fig. 111 Megalocornea. Fig. 112 Left microphthalmos. 39

 20 Episcleritis and scleritis

Episcleritis
Clinical This very common and innocuous condition presents with
features unilateral redness and slight discomfort.
s
i
t
Classification • Nodular (Fig. 113)
i
r
e
• Diffuse (Fig. 114).
l
c
s
nd
Management Treatment is often not required. If necessary, topical steroids
and systemic non-steroidal anti-inflammatory agents.
a
s
i
t
Scleritis
i
r
e
l
Deeper inflammation than episcleritis, with more severe
c
s
i
symptoms and frequent systemic association.
p
E
Aetiology Often idiopathic, but may follow eye surgery or be
associated with herpes zoster or systemic disease such as
rheumatoid arthritis, Wegener’s granulomatosis and
polyarteritis nodosa.

Classification Anterior non-necrotizing The most common type may be

nodular (Fig. 115) or diffuse.
Anterior necrotizing with inflammation Is a rare and
painful condition characterized by local scleral injection and
necrosis (Fig. 116). May be complicated by keratitis and
anterior uveitis.
Scleromalacia perforans Is rare and typically occurs in
women with rheumatoid arthritis. It is characterized by
uninflamed patches of scleral necrosis that expose the
underlying uvea (Fig. 117). Spontaneous perforation is rare
but may occur as a result of trauma. No benefit has been
demonstrated from treatment.

Posterior scleritis
Clinical Uncommon and may be difficult to diagnose. Signs include
features proptosis, ophthalmoplegia, optic disc swelling, choroidal
folds (see Fig. 40) and uveal effusion (Fig. 118).

Management • Systemic non-steroidal anti-inflammatory agents

• Topical and/or systemic steroids
40 • Other immunosuppressive agents.
 20

E
p
i
s
c
l
e
r
i
t
i
s
Fig. 113 Nodular episcleritis. Fig. 114 Diffuse episcleritis.

a
n
d
s
c
l
e
r
i
t
i
s
Fig. 115 Anterior non-necrotizing nodular Fig. 116 Anterior necrotizing scleritis
scleritis. with inflammation.

41
Fig. 117 Scleromalacia perforans. Fig. 118 Uveal effusion in scleritis.
 21 Anterior uveitis

Aetiology Commonly idiopathic but numerous systemic associations/

causes:
• HLA-B27-associated: ankylosing spondylitis, Reiter’s
s
syndrome, psoriatic arthritis
i
t
• Juvenile idiopathic arthritis: especially high-risk if
i
e
uv
pauciarticular-onset and ANA-positive
• Inflammatory bowel disease: ulcerative colitis, Crohn’s
r
o
disease
i
r
e
• Non-infectious systemic diseases: sarcoidosis, Behçet’s
nt
disease, Vogt–Koyanagi–Harada syndrome
A
• Infections: herpes zoster and simplex, syphilis, tuberculosis
• Idiopathic: Fuchs’ uveitis syndrome.
Clinical Symptoms
features • Acute iritis: subacute onset of photophobia, redness, pain
and blurred vision; usually unilateral
• Chronic anterior uveitis: symptoms may initially be absent
or mild.
Signs
• Acute iritis: circumcorneal (ciliary) injection, small pupil,
fine keratic precipitates (‘KPs’ – Figs 119, 120), aqueous
flare (Fig. 120) and cells, white ‘fluid level’ (hypopyon –
Fig. 121) and fibrinous exudate in severe inflammation
• Chronic anterior uveitis: usually little or no injection, KPs
typically large (‘mutton fat’ in granulomatous
inflammation, e.g. sarcoidosis), aqueous flare (Fig. 120)
and cells (often fewer than in acute iritis), iris nodules
(in granulomatous).
Complications • Iris–lens adhesions (posterior synechiae – Fig. 122)
• Corneal calcium deposition (band keratopathy – Fig. 123)
• Cataract (Fig. 123)
• Glaucoma
• A blind, soft and shrunken globe (phthisis bulbi – Fig. 124)
in end stage chronic uveitis.
Management • Topical steroids and mydriatics are the mainstay of
treatment
• Periocular steroid injection
• Systemic steroids, immunosuppressive agents and
antibiotics for infections (e.g. tuberculosis, syphilis).
42
[Link]
 21

A
n
t
e
r
i
o
r
u
v
Fig. 119 Keratic precipitates in chronic Fig. 120 Aqueous flare and large keratic

e
i
anterior uveitis. precipitates.

t
i
s
Fig. 121 Hypopyon in severe acute Fig. 122 Adhesions between the lens and
anterior uveitis. iris (posterior synechiae).

43
Fig. 123 Band keratopathy and cataract. Fig. 124 Phthisis bulbi.
 22 Posterior uveitis – infections

Toxoplasmosis
Aetiology Reactivation of prenatal infection with the protozoan
Toxoplasma gondii.
ns
Clinical Subacute onset of unilateral vitreous floaters. Examination
o
features shows a moderate-severe vitritis associated with a
i
t
c
solitary focus of retinitis adjacent to an old scar (Fig. 125).
e
nf
Management Treatment of vision-threatening lesions is with antimicrobial
i
agents and systemic steroids. Special considerations apply
–
s
to immunocompromised patients.
i
t
i
e
Toxocariasis
uv
Aetiology Rare infection caused by the common intestinal worm of
r
o
dogs Toxocara canis.
i
r
e
Clinical • Posterior pole granuloma: presents with poor
t
s
o
features vision in one eye
P
• Peripheral granuloma: presents with distortion of the
macula or disc (Fig. 126) or tractional retinal detachment
(Fig. 127)
• Chronic endophthalmitis: presents with leukocoria (see
Fig. 232), strabismus or visual loss.
Management Vitrectomy may be considered for retinal detachment.
Cytomegalovirus (CMV) retinitis
Common in patients with AIDS, though less so with the
introduction of newer drug regimens.
Clinical Yellow-white areas of retinal necrosis associated with
features variable haemorrhage and mild vitritis (Fig. 128). The
lesions spread along the vascular arcades and may involve
the optic nerve head (Fig. 129).
Management Antiviral agents administered systemically, or intravitreally
by injection or a slow-release implant.
Histoplasmosis
Aetiology Infection with the fungus Histoplasma capsulatum.
Clinical Punched-out chorioretinal scars (‘histo spots’), peripapillary
features atrophy (Fig. 130) and macular choroidal neovascularization
(CNV); no associated vitritis.
Management Laser photocoagulation or surgery may be appropriate for
44 the CNV.
 22

P
o
s
t
e
r
i
o
r
Fig. 125 Active retinal toxoplasmosis. Fig. 126 Peripheral Toxocara granuloma.

u
v
e
i
t
i
s
–
i
n
f
e
c
t
i
o
n
s
Fig. 127 Tractional retinal detachment in Fig. 128 Cytomegalovirus retinitis.
ocular toxocariasis.

Fig. 129 Advanced cytomegalovirus Fig. 130 Ocular histoplasmosis.

retinitis with optic nerve involvement. 45
 23 Non- infectious intermediate
and posterior uveitis
Intermediate uveitis
Clinical Relatively common, often bilateral, chronic idiopathic
features inflammation of the pars plana (pars planitis) and retinal
s
periphery of children and young adults, presenting with
i
t
gradually increasing vitreous floaters. Examination shows
i
e
uv
vitritis (Fig. 131), often accompanied by exudate on the
inferior pars plana (‘snowbanking’).
r
o
i
r
Complications
e
• Chronic cystoid macular oedema (main cause of severe
t
s
visual loss)
o
p
• Cataract.
nd
Management Periocular depot steroid injection when visual acuity is
a
e
reduced by cystoid macular oedema.
t
a
i
d
Sarcoidosis
e
m
Clinical Common idiopathic disease characterized by multisystem
r
e
features granulomatous inflammation. Anterior uveitis is common
nt
(see page 42); fundus signs include periphlebitis (‘candlewax
i
s
drippings’ – Fig. 132), retinal, choroidal (Fig. 133) and optic
u
o
nerve granulomata (Fig. 134), and peripheral retinal
i
t
neovascularization.
c
e
nf
Management Systemic steroids.
i
n-
o
Behçet’s disease
N
Clinical Uncommon idiopathic multisystem disease characterized by
features aphthous oral ulceration, recurrent genital ulceration and
skin lesions. Ocular features include periphlebitis, which
may result in venous occlusion (Fig. 135), retinitis, macular
oedema and optic atrophy (Fig. 136).

Management Systemic steroids and immunosuppressive agents.

46
 23

N
o
n
-
i
n
f
e
c
t
i
Fig. 131 Severe vitritis in intermediate Fig. 132 Retinal vasculitis with

o
u
uveitis. ‘candlewax’ perivascular exudates.

s
i
n
t
e
r
m
e
d
i
a
t
e
a
n
d
p
o
s
t
e
r
i
o
Fig. 133 Choroidal sarcoid granulomas. Fig. 134 Optic nerve head sarcoid r
granuloma.
u
v
e
i
t
i
s
Fig. 135 Severe periphlebitis with venous Fig. 136 Optic atrophy in Behçet’s
occlusion in Behçet’s disease. disease. 47
[Link]
 24 Idiopathic multifocal white dot
syndromes
‘White dot syndromes’ is the term for a heterogeneous
group of rare inflammatory conditions of unknown aetiology
which involve the posterior segment. Examples are
presented below.
s
e
m
Birdshot retinochoroidopathy
o
r
nd
Clinical Rare, idiopathic, chronic bilateral condition typically
features
y
affecting middle-aged females who carry the genetic marker
s
HLA-A29. Early lesions consist of multiple, deep, flat,
t
o
creamy-yellow spots radiating outwards from the disc
d
(Fig. 137), associated with variable vasculitis and vitritis.
e
t
These evolve into circumscribed, atrophic white areas
hi
w
(Fig. 138). Cystoid macular oedema is very common; optic
atrophy and cataract may also develop.
l
a
c
o
Management Periocular and systemic steroids and immunosuppressive
f
i
t
agents may be beneficial but the visual prognosis is
ul
guarded.
m
c
hi
Acute multifocal posterior placoid pigment
t
epitheliopathy (AMPPPE)
a
p
o
Clinical Uncommon, idiopathic, acute bilateral condition typically
i
d
features affecting young adults. Deep, oval, cream-coloured lesions
I
(Fig. 139) develop initially, and on resolving leave variable
pigmented scars (Fig. 140).

Management The visual prognosis is usually excellent and no treatment

is required.

Serpiginous choroidopathy
Clinical Rare, idiopathic, chronic bilateral condition of older
features patients. Cream-coloured lesions at the posterior pole
(Fig. 141) evolve to leave atrophic areas (Fig. 142).

Management Steroids and immunosuppressive agents may be tried but

the visual prognosis is poor.

48
 24

I
d
i
o
p
a
t
Fig. 137 Active birdshot Fig. 138 Inactive birdshot

h
retinochoroidopathy. retinochoroidopathy.

i
c
m
u
l
t
i
f
o
c
a
l
w
h
i
t
e
d
o
t
s
y
n
d
r
o
m
Fig. 139 Active AMPPPE. Fig. 140 Inactive AMPPPE.
e
s
Fig. 141 Active serpiginous Fig. 142 Late scarring in serpiginous
choroidopathy. choroidopathy. 49
 25 Primary open- angle glaucoma

Definition Glaucoma is a progressive optic neuropathy in which the

major risk factor for visual loss is elevated intraocular
pressure (IOP – Fig. 143). In primary open-angle glaucoma
(POAG), the most common of the glaucomas, raised IOP
a
m
occurs secondarily to a gradual reduction in the drainage of
o
aqueous humour via the trabecular meshwork. Glaucoma
uc
can be present, however, in the presence of normal IOP
a
(‘normal-tension’ glaucoma).
l
g
e
l
Incidence POAG affects at least 1% of the population over the age of
ng
40 years. Both eyes are usually affected. The risk to siblings
a
of affected individuals is approximately 10%.
n-
e
p
Important risk • High IOP
o
factors
y
• Age
r
a
• Black race
m
• Family history
i
r
P
• Myopia.

Clinical Usually asymptomatic until significant loss of visual field

features has occurred. Frequently the condition is first suspected at a
routine eye examination.

Signs
• Elevated IOP (>21 mmHg)
• Glaucomatous optic nerve damage (Figs 144–146)
• Visual field loss (Fig. 147)
• Open anterior chamber angle on gonioscopy.

Management • Topical treatment (usually first-line) with beta-blockers,

prostaglandin derivatives, carbonic anhydrase inhibitors,
alpha-agonists and miotics
• Laser trabeculoplasty
• Filtration surgery, e.g. trabeculectomy (Fig. 148).

50
 25

P
r
i
m
a
r
y
o
p
e
Fig. 143 Measurement of intraocular Fig. 144 Normal optic disc.

n
-
pressure.

a
n
g
l
e
g
l
a
u
c
o
m
a
Fig. 145 Moderate cupping with inferior Fig. 146 Advanced cupping.
notching.

(a) (b)
Fig. 147 Visual field loss in a glaucoma
patient (a) left eye (b) right eye. Fig. 148 Conjunctival filtration bleb 51
following trabeculectomy.
 26 Secondary open- angle
glaucomas
Pigmentary glaucoma
Aetiology Blockage of aqueous outflow and secondary trabecular
meshwork damage is caused by pigment granules derived
s
from the iris.
a
m
Clinical Common: most patients are young myopic males.
o
features
uc
Signs
a
l
g
• Vertical strip of pigment granules on the corneal
e
endothelium (Krukenberg spindle – Fig. 149)
l
ng
• Spoke-like transillumination defects in the mid-peripheral
a
iris (Fig. 150)
n-
• Gonioscopy: wide anterior chamber angle with heavily
e
p
pigmented trabecular meshwork (Fig. 151)
o
• Other features: deep anterior chamber, pigment granules
y
r
on the iris surface
a
nd
• If no glaucomatous damage: ‘pigment dispersion syndrome’.
o
Management As for POAG.
c
e
S
Pseudoexfoliation glaucoma
Aetiology Blockage of aqueous outflow by pseudoexfoliative material.
Clinical Common: typically affects the elderly.
features
Signs
• Pseudoexfoliative material on anterior lens surface
(Fig. 152) and pupillary border (Fig. 153)
• Gonioscopy: trabecular hyperpigmentation and deposits
of pseudoexfoliative material.
Management As for POAG but may be more resistant to treatment.

Angle- recession glaucoma

Aetiology Blunt traumatic trabecular damage.
Clinical Uncommon. Uniocular elevation of IOP, usually months or
features years after the initial injury. Examination clues to the
diagnosis include signs of previous damage such as tears in
the iris sphincter or iridodialysis (see Fig. 264), a slightly
deeper anterior chamber than in the fellow eye, and a
recessed and irregular angle on gonioscopy (Fig. 154).
Management Treatment is initially medical although filtration surgery is
52 often required eventually.
[Link]
 26

S
e
c
o
n
d
a
r
y
o
Fig. 149 Krukenberg spindle. Fig. 150 Iris transillumination defects.

p
e
n
-
a
n
g
l
e
g
l
a
u
c
o
m
a
s
Fig. 151 Trabecular hyperpigmentation. Fig. 152 Pseudoexfoliative material on the
anterior lens capsule.

Fig. 153 Pseudoexfoliative material on Fig. 154 Severe angle recession.

the lens and pupil edge.
53
 27 Primary angle- closure
glaucoma
Definition Elevation of IOP as a result of obstruction of the anterior
chamber angle by the peripheral iris.

Aetiology The normal increase in size of the lens with ageing may lead
a
m
to narrowing of the anterior chamber angle. In an
o
anatomically predisposed eye this can progress to
uc
obstruction.
a
l
g
Risk factors • Increasing age (usually over 60)
e
ur
• Female gender
s
• Hypermetropia, shallow anterior chamber and narrow angle.
o
l
c
-
Clinical Subacute angle-closure Episodic transient blurring of
e
l
features
g
vision and haloes around lights associated with aching
n
and/or redness of the eye caused by intermittent angle
a
y
closure that resolves spontaneously.
r
a
m
Chronic angle-closure Gradual and insidious narrowing of
i
the angle without acute symptoms, usually with gradual
r
P
elevation of IOP.
Acute angle-closure Sudden onset of total angle occlusion
(Fig. 155) results in an acute and very severe increase in IOP
with corresponding visual loss, ocular pain and redness,
frequently accompanied by nausea and vomiting.

Signs of acute angle-closure glaucoma include:

• Very high IOP
• Circumcorneal (ciliary) injection
• Corneal oedema (Fig. 156)
• Fixed, mid-dilated, oval pupil (Fig. 157)
• Very shallow anterior chamber.
Signs following resolution of the acute attack:
• Iris atrophy (Fig. 158)
• Anterior lens opacity (Glaukomflecken – Fig. 159)

Management • Treatment of acute glaucoma is with systemic carbonic

anhydrase inhibitors, topical beta blockers, miotics alpha
agonists and steroids; systemic osmotic agents may also
be required
• Laser peripheral iridotomies (Fig. 160) once the cornea
has cleared.
54
 27

P
r
i
m
a
r
y
a
n
g
Fig. 155 Total angle occlusion by Fig. 156 Corneal oedema in acute angle-

l
e
peripheral iris. closure.

-
c
l
o
s
u
r
e
g
l
a
u
c
o
m
a
Fig. 157 Fixed mid-dilated pupil in acute Fig. 158 Iris atrophy following resolution
angle-closure. of acute angle-closure.

55
Fig. 159 Glaukomflecken. Fig. 160 Laser peripheral iridotomy.
 28 Secondary angle- closure
glaucomas
Neovascular glaucoma
Aetiology Chronic retinal ischaemia leads to neovascularization on the
iris (rubeosis iridis) and in the anterior chamber angle.
s
Contraction of fibrovascular tissue (peripheral anterior
a
m
synechiae – PAS) causes angle closure. Common causes are
o
ischaemic central retinal vein occlusion (see Fig. 196),
uc
typically about 3 months after the thrombosis, and severe
a
proliferative diabetic retinopathy.
l
g
e
ur
Clinical Pain, decreased vision, anterior segment congestion, a
s
features very high IOP with corneal oedema, and severe rubeosis
o
l
iridis (Figs 161, 162).
c
-
e
l
Management
ng
• Panretinal photocoagulation if the fundus is visible, or
peripheral retinal cryotherapy if not
a
y
• Topical atropine and steroids to decrease inflammation
r
a
• Filtration surgery (trabeculectomy with adjunctive
nd
antimetabolite, or drainage implant – Fig. 163)
o
• Partial ablation of the ciliary body with laser
c
e
(‘cyclodiode’).
S
Inflammatory angle- closure glaucomas
Aetiology Anterior uveitis may lead to angle-closure glaucoma via two
mechanisms:
• 360° posterior synechiae (seclusio pupillae) causes iris
bombé, a shallow anterior chamber and angle closure
• Without pupil block due to contraction of inflammatory
debris in the angle with consequent PAS formation.

Iridocorneal endothelial (ICE) syndrome

Aetiology Synechial angle closure by proliferation and contraction of
abnormal corneal endothelial cells.

Clinical Rare. Typically affects one eye of a middle-aged woman.

features Signs include iris atrophy (Figs 165, 166), iris naevus or
nodules and/or severe corneal endothelial changes.

Management A drainage implant is often required.

56
 28

S
e
c
o
n
d
a
r
y
a
Fig. 161 Rubeosis iridis with bleeding Fig. 162 Advanced rubeosis iridis.

n
g
from new vessels.

l
e
-
c
l
o
s
u
r
e
g
l
a
u
c
o
m
a
s
Fig. 163 Drainage implant. Fig. 164 Seclusio pupillae with shallow
anterior chamber.

57
Fig. 165 Iris atrophy in ICE syndrome. Fig. 166 Very advanced iris atrophy.
[Link]
 29 Developmental glaucomas

Primary congenital glaucoma

Incidence 1/10 000 live births; 65% are boys. Both eyes involved in
75% of cases, usually asymmetrically.
s
a
Clinical
m
• Large eye (buphthalmos – Fig. 167) if IOP becomes
o
features elevated before age 3 years
uc
• Breaks in Descemet’s membrane (Haab striae – Fig. 168)
a
• Anterior chamber angle anomaly.
l
g
l
a
Management • Incision of the trabecular meshwork (goniotomy)
nt
• Less commonly trabeculotomy or trabeculectomy.
e
m
p
Iridocorneal dysgenesis
o
l
e
Definition
v
A spectrum of rare congenital disorders involving the
e
D
anterior segment, sometimes associated with congenital or
childhood glaucoma.

Classification Axenfeld–Rieger syndrome Posterior embryotoxon

associated with extensive PAS, iris hypoplasia, holes,
ectropion uveae and displaced pupil (Fig. 169).
Peters anomaly Extremely rare condition characterized by a
corneal scar associated with iris or lens adhesions (Fig. 170).
Aniridia May be complete or partial (Fig. 171). Three
phenotypes:
• AN-1 (~66%): isolated ocular defect with autosomal
dominant inheritance
• AN-2 (~33%): sporadic; 30% chance of Wilms’ tumour by
age 5 years (Miller’s syndrome)
• AN-3 (1–2%): mental handicap and cerebellar ataxia
(Gillespie’s syndrome).

Phacomatoses
A group of conditions characterized by hamartomas in
multiple organs. Congenital glaucoma may occur in:
• Sturge–Weber syndrome (Fig. 172); glaucoma in 30% of
cases, due to either an angle anomaly or elevation of
episcleral venous pressure.
• Neurofibromatosis-1; glaucoma is uncommon.
58
 29

D
e
v
e
l
o
p
m
e
n
Fig. 167 Bilateral buphthalmos. Fig. 168 Haab striae.

t
a
l
g
l
a
u
c
o
m
a
s
Fig. 169 Axenfeld–Rieger syndrome. Fig. 170 Peters anomaly.

Fig. 171 Incomplete aniridia. Fig. 172 Naevus flammeus in

Sturge–Weber syndrome. 59
 30 Age- related cataract

Definition Cataract is an opacity of the natural crystalline lens. It is

an extremely common cause of visual impairment in older
patients.
t
c
Classification Subcapsular cataract May be anterior or posterior
a
r
(Fig. 173). Patients experience glare and typically have more
a
t
problems with reading than distance vision.
a
c
Nuclear cataract (‘nuclear sclerosis’) Consists of a central
d
e
darkening of the lens (Fig. 174). It may cause an increase in
t
a
myopia so that distance vision is affected more than near.
l
e
r
Cortical cataract – spoke-like opacities (Fig. 175).
-
e
g
A
Mature cataract – completely opaque lens with a white
pupil (Fig. 176).

Management Surgical extraction is the only treatment for cataract. The

majority of modern cataract surgery is now carried out by
means of phacoemulsification, in which the lens is removed
through a narrow incision using a small-bore ultrasonic
probe. The natural lens is replaced by an artificial
intraocular lens implant (IOL– Fig. 177). Under special
circumstances or where phacoemulsification equipment is
not available, ‘extracapsular’ extraction is carried out,
involving a larger corneal incision requiring sutures.

Complications • Posterior lens capsular opacification (Fig. 178) occurs

eventually in about 20% of cases; can be treated by YAG
laser capsulotomy
• Acute or chronic bacterial endophthalmitis is rare but
serious
• Expulsive (suprachoroidal) haemorrhage is a rare but
potentially disastrous operative complication
• Retinal detachment is rare; myopes are particularly at
risk.

60
 30

A
g
e
-
r
e
l
a
t
e
d
Fig. 173 Posterior subcapsular cataract. Fig. 174 Nuclear cataract (nuclear sclerosis).

c
a
t
a
r
a
c
t
Fig. 175 Cortical cataract seen against Fig. 176 Mature cataract.
the red reflex.

Fig. 177 Intraocular lens implant. Fig. 178 Severe posterior capsular 61
opacification.
 31 Miscellaneous disorders of the
lens
Secondary cataract
Aetiology • Trauma: penetrating, concussion, radiation and electric
shock
• Associated with ocular disease: chronic anterior uveitis
ns
(see Fig. 123), high myopia, acute angle-closure glaucoma
e
l
(see Fig. 159), hereditary retinal dystrophies
he
• Associated with systemic disease: diabetes, myotonic
t
dystrophy atopic dermatitis
f
o
• Drug-induced: most commonly systemic steroids.
s
r
e
Infantile cataract
d
r
o
Aetiology • Idiopathic sporadic: may be unilateral
s
i
d
• Hereditary: most frequently autosomal dominant
us
• Associated with other ocular malformations: persistent
o
hyperplastic primary vitreous, retinopathy of prematurity,
ne
aniridia (see Fig. 171), Peters anomaly (see Fig. 170)
a
• Embryopathies: intrauterine infections (e.g. rubella,
l
l
e
toxoplasmosis)
c
s
• Metabolic: galactokinase deficiency, galactosaemia and
i
M
hypocalcaemia
• Syndromes: Lowe, Down, Turner and cri du chat.
Clinical Appearance of infantile cataract is variable (Figs 179, 180).
features
Ectopia lentis
Definition Displacement of the lens; may be partial (subluxation) or
complete.
Aetiology • Trauma (Fig. 181)
• Familial ectopia lentis: may be associated with ectopic
pupil (Fig. 182)
• Associated with other ocular disorders: aniridia (see
Fig. 171) and buphthalmos (see Fig. 167)
• Syndromes: Marfan’s (Fig. 183) and Weill–Marchesani
• Metabolic: homocystinuria and hyperlysinaemia.
Abnormalities of lens size and shape
• Microphakia: small lens
• Microspherophakia: small spherical lens
• Coloboma: (Fig. 184) may be associated with colobomas
of the iris and choroid, and giant retinal tears
62 • Lenticonus: cone-shaped lens surface (e.g. anterior
lenticonus in Alport syndrome).
[Link]
 31

M
i
s
Fig. 179 Lamellar congenital cataract. Fig. 180 Punctate congenital lens opacities.

c
e
l
l
a
n
e
o
u
s
d
i
s
o
r
d
e
r
s
o
f
t
h
e
l
e
n
s
Fig. 181 Lens subluxation due to blunt Fig. 182 Congenital ectopic pupil with
trauma. associated lens subluxation.

Fig. 183 Upward lens subluxation in Fig. 184 Lens coloboma and opacity.
Marfan’s syndrome. 63
 32 Diabetic retinopathy

Incidence Diabetic retinopathy (DR) is the most common cause of

blindness in the working-age population. The incidence and
severity of DR are strongly related to duration of diabetes;
good control of blood glucose and hypertension are very
hy
important.
t
a
p
Clinical Background DR Microaneurysms, dot and blot
no
features haemorrhages and hard exudates (Figs 185, 186).
i
t
e
r
Preproliferative DR Cotton-wool spots, intraretinal
c
microvascular anomalies (IRMA), venous changes (beading,
i
t
e
looping and segmentation) and dark blot haemorrhages
b
(Fig. 187).
a
i
D
Proliferative diabetic retinopathy New vessel formation at
the optic disc (NVD – Fig. 188) or elsewhere on the retina
(NVE – Fig. 189). Severe visual loss may occur as a result of
vitreous haemorrhage or tractional retinal detachment due
to contraction of fibrovascular tissue (see Fig. 224).
Diabetic maculopathy Maculopathy is the most common
cause of visual impairment in patients with diabetes. Loss of
visual function is usually caused by oedema, typically
accompanied by exudates. Less commonly, the macula
becomes ischaemic, often with severe deterioration in
central vision.

Management • Regular review if treatment is not indicated, frequency

dependent on severity of DR
• Panretinal laser photocoagulation for proliferative DR
(Fig. 190)
• Grid or focal laser photocoagulation for macular oedema
fitting certain criteria (‘clinically significant macular
oedema’)
• Vitrectomy for persistent vitreous haemorrhage or
tractional retinal detachment involving the centre of the
macula.

64
 32

D
i
a
b
e
t
i
Fig. 185 Mild background diabetic Fig. 186 Severe background diabetic

c
retinopathy. retinopathy.

r
e
t
i
n
o
p
a
t
h
y
Fig. 187 Pre-proliferative diabetic Fig. 188 Severe NVD.
retinopathy.

Fig. 189 Severe NVE. Fig. 190 Laser scars following panretinal
photocoagulation. 65
 33 Retinal vascular occlusion

Retinal vein occlusion (RVO)

Aetiology Predisposing factors include increasing age, hypertension,
hyperviscosity, vasculitis, thrombophilic disorders and raised
n
IOP.
o
i
us
Clinical Presents with sudden mild to severe loss of vision in one
l
c
features eye. Acute signs include haemorrhages, cotton wool spots,
c
o
venous tortuosity, optic disc and retinal oedema.
r
a
ul
Classification • Branch RVO (Fig. 191): usually involves a retinal quadrant
c
• Hemiretinal vein occlusion (Fig. 192)
s
a
v
• Central RVO: ischaemic or non-ischaemic (Fig. 193).
l
na
Complications • Retinal neovascularization, especially in BRVO, is treated
i
t
with laser photocoagulation
e
R
• Macular oedema is treated with grid laser
photocoagulation to the macula in selected cases
• Neovascular glaucoma (see Fig. 161) in ischaemic CRVO.

Retinal artery occlusion (RAO)

Aetiology Embolization from a carotid or cardiac source, or vaso-
obliteration by atheroma or arteritis.

Clinical Acute loss of vision; may be permanent or transient

features (amaurosis fugax). Retinal pallor corresponding to the
involved area (central or branch) is seen (Fig. 194), and in
central RAO a ‘cherry red spot’ at the fovea is typically
present (Fig. 195). Segmentation of the arteriolar blood
column (‘cattle trucking’) may be seen. Later the arterioles
become attenuated and the optic disc pale.

Management • Urgent erythrocyte sedimentation rate (ESR) to exclude

giant cell arteritis and investigation of other risk factors
• Amaurosis fugax: aspirin; carotid endarterectomy for
severe stenosis
• Acute RAO may be relieved by lowering IOP by massage,
intravenous acetazolamide, anterior chamber
paracentesis.

66
 33

R
e
t
i
n
a
l
v
a
s
c
Fig. 191 Branch retinal vein occlusion. Fig. 192 Inferior hemiretinal vein

u
occlusion.

l
a
r
o
c
c
l
u
s
i
o
n
Fig. 193 Central retinal vein occlusion. Fig. 194 Branch retinal artery occlusion.

Fig. 195 Central retinal artery occlusion

with ‘cherry-red spot’.
67
[Link]
 34 Miscellaneous retinopathies

Hypertensive retinopathy
Classification • Grade 1: mild generalized arteriolar narrowing (Fig. 196)
• Grade 2: focal as well as marked generalized arteriolar
s
constriction (Fig. 197)
e
hi
• Grade 3: as Grade 2 plus retinal haemorrhages, cotton
t
wool spots and hard exudates (Fig. 198)
a
p
• Grade 4: as Grade 3 plus optic disc swelling (Fig. 199).
no
i
t
Sickle- cell retinopathy
e
r
us
Patients typically have SC or SThal disease.
o
ne
Classification • Stage 1: peripheral arteriolar occlusion
a
• Stage 2: peripheral arteriovenous anastomoses
l
l
e
• Stage 3: growth of extraretinal new vessels from
c
s
anastomoses
i
M
• Stage 4: vitreous haemorrhage (Fig. 200)
• Stage 5: tractional retinal detachment.

Management A high rate of spontaneous infarction of ischaemic areas

means that photocoagulation is rarely performed but
vitrectomy may be required for stage 4 and 5 disease.

Retinopathy of prematurity (ROP)

Definition Proliferative retinopathy affecting low-birth-weight preterm
infants exposed to high ambient levels of oxygen.

Classification • Stage 1: demarcation mark parallel to the ora serrata

• Stage 2: ridge with associated neovascular tufts
• Stage 3: fibrovascular proliferation from the ridge
(Fig. 201)
• Stage 4: subtotal tractional retinal detachment
• Stage 5: total retinal detachment
• ‘Plus’ disease: vitreous haze, posterior pole vascular
tortuosity
• Cicatricial ROP: complications due to fibrosis following
involution of acute disease.

Management • Structured screening of at-risk infants

• Treatment with peripheral retinal cryotherapy or laser
photocoagulation for ‘threshold’ disease.
68
 34

M
i
Fig. 196 Generalized arteriolar constriction Fig. 197 Focal arteriolar constriction in

s
c
in Grade 1 hypertensive retinopathy. Grade 2 hypertensive retinopathy.

e
l
l
a
n
e
o
u
s
r
e
t
i
n
o
p
a
t
h
i
e
s
Fig. 198 Haemorrhages, cotton wool spots Fig. 199 Grade 3 changes plus optic disc
and hard exudates in Grade 3 hypertensive swelling and a macular star in Grade 4
retinopathy. hypertensive retinopathy.

Fig. 200 Vitreous haemorrhage in Stage Fig. 201 Ridge with fibrovascular
4 sickle-cell retinopathy. proliferation in Stage 3 ROP.
69
 35 Age- related macular
degeneration
Incidence Age-related macular degeneration (AMD) is the most
common cause of legal blindness in industrialized societies.
Patients are typically over the age of 65 years; both eyes are
usually affected, frequently asymmetrically.
n
o
i
Clinical Hard drusen Small, round, discrete, yellow-white lesions,
t
a
r
features usually located at the macula (Fig. 202).
ne
e
Soft drusen Larger lesions with ill-defined edges (Fig. 203)
g
associated with exudative AMD.
e
d
r
Non-exudative (‘dry’) macular degeneration Atrophic and
a
ul
hyperplastic changes of the retinal pigment epithelium (RPE)
c
associated with slowly progressive degeneration of the
a
m
overlying neuroretina and underlying choriocapillaris
(Fig. 204).
d
e
t
Exudative (‘wet’) macular degeneration The ingrowth of
a
l
e
choroidal new vessels through Bruch membrane: choroidal
r
-
neovascularization (CNV). Presents with unilateral distortion
e
g
of central vision. On examination, an area of the macula is
A
elevated by subretinal fluid or blood (Fig. 205), often with
associated clumps of exudates (Fig. 206). The lesion evolves
in most cases to leave subretinal ‘disciform’ scarring with
permanent loss of central vision (Fig. 207).
RPE detachment (PED) Fluid elevates the RPE in a dome
configuration. This may progress to exudative AMD
described above or may occasionally settle spontaneously.
Polypoidal choroidal vasculopathy (PCV) This recently-
described form of AMD may masquerade as CNV. Its
behaviour, particularly the response to treatment, has yet to
be clearly defined.

Management • Often of negligible or temporary benefit and therefore

only used in selected cases
• Antioxidant vitamin and mineral supplements may retard
the progression of AMD
• Conventional laser may be effective at destroying CNV
that does not encroach on the central macula
• Photodynamic therapy (PDT) is a newer technique using a
laser to activate a light-sensitive dye preferentially taken
up by CNV.
70
 35

A
g
Fig. 202 Macular hard drusen. Fig. 203 Macular soft drusen.

e
-
r
e
l
a
t
e
d
m
a
c
u
l
a
r
d
e
g
e
n
e
r
a
t
i
o
n
Fig. 204 Early ‘dry’ macular degeneration. Fig. 205 Subretinal haemorrhage
associated with CNV.

Fig. 206 Intra- and subretinal exudation Fig. 207 Advanced disciform scarring.
associated with CNV. 71
 36 Acquired maculopathies

Macular hole
Clinical The visual deficit is often noticed by chance when one eye is
features closed. On examination a rounded punched-out area
s
measuring one-third of a disc diameter is seen at the fovea,
e
hi
surrounded by a grey halo of retinal elevation (Fig. 208).
t
Treatment in early cases is by vitrectomy and intraocular
a
p
gas.
o
ul
c
Myopic maculopathy
a
m
Clinical Presentation of this common disorder is with either
d
features unilateral metamorphopsia or impaired visual acuity. On
e
r
examination, a variety of changes may be seen: pigment
ui
q
proliferation (Fuchs spot – Fig. 209), atrophic maculopathy
c
A
(Fig. 210), breaks in the Bruch membrane (‘lacquer cracks’)
and macular haemorrhage.

Macular epiretinal membrane

Clinical This proliferation of glial cells and associated fibrous tissue
features across the retinal surface is often idiopathic but may
occur following retinal disease, surgery or trauma.
Presentation is with unilateral distortion and blurring of
gradual onset. An early membrane may be subtle
(‘cellophane maculopathy’) and manifest mild retinal
wrinkling and vascular tortuosity. As a membrane thickens
and contracts, the changes become more evident (‘macular
pucker’ – Fig. 211).

Angioid streaks
In this rare binocular condition, linear streaks are seen
radiating from the optic disc (Fig. 212). Vision can be
affected, particularly by CNV. About 50% of individuals with
angioid streaks have pseudoxanthoma elasticum.

Bull’s eye maculopathy

Central foveolar hyperpigmentation surrounded by a
depigmented zone encircled in turn by a hyperpigmented
ring (Fig. 213). Causes include antimalarial drugs, cone
dystrophy, Stargardt disease (see Fig. 215) and Batten
disease.
72
[Link]
 36

A
c
q
u
i
r
e
d
m
Fig. 208 Macular hole. Fig. 209 Myopic maculopathy: Fuchs spot

a
c
u
l
o
p
a
t
h
i
e
s
Fig. 210 Atrophic maculopathy. Fig. 211 Macular pucker.

Fig. 212 Angioid streaks. Fig. 213 Bull’s eye maculopathy. 73

 37 Dystrophies of the fundus

Retinitis pigmentosa
Presentation of this uncommon condition is during the
second decade of life with night blindness. Inheritance may
us
be autosomal dominant, recessive or X-linked. A large
und
number of genetic abnormalities have been found to cause
the clinical picture, the hallmarks of which are mid-
f
peripheral perivascular ‘bone-spicule’ pigmentation,
he
arteriolar attenuation and waxy disc pallor (Fig. 214).
t
Associated macular oedema and cataract are common. A
f
o
number of systemic syndromes are associated.
s
e
hi
Stargardt’s disease and fundus flavimaculatus
p
o
These two uncommon conditions represent variants of the
r
t
s
same underlying autosomal recessive disease. Presentation is
y
D
with central visual impairment: in early adulthood in
Stargardt’s and early middle age in fundus flavimaculatus. In
early Stargardt’s disease a ‘beaten-bronze’ macular lesion is
seen that slowly progresses to an atrophic lesion (Fig. 215).
In fundus flavimaculatus yellow-white flecks are scattered
throughout the posterior pole and mid-peripheral fundus
(Fig. 216), and maculopathy similar to Stargardt’s may
develop.

Best’s disease
In this rare dominantly inherited disorder the macular
appearance evolves over time from a juvenile egg yolk
(vitelliform) lesion (Fig. 217) to scarring and severe visual
loss in adult life.

Choroidal dystrophies
Choroideremia Very rare X-linked disorder presenting
during the first decade with night blindness characterized
by enlarging midretinal patches of chorioretinal atrophy
that progressively spread centrally but spare the macula till
late on (Fig. 218).
Gyrate atrophy Very rare recessively inherited inborn error
of metabolism presenting during the first decade with night
blindness characterized by coalescing midretinal patches of
chorioretinal atrophy (Fig. 219).
74
 37

D
y
s
t
r
o
p
h
i
e
s
Fig. 214 Retinitis pigmentosa. Fig. 215 Advanced Stargardt’s disease.

o
f
t
h
e
f
u
n
d
u
s
Fig. 216 Fundus flavimaculatus. Fig. 217 Vitelliform lesion in Best’s
disease.

Fig. 218 Advanced choroideremia. Fig. 219 Gyrate atrophy. 75

 38 Retinal detachment

Definition A separation of the neuroretina from the underlying retinal

pigment epithelium (RPE) by the accumulation of subretinal
fluid (SRF).
nt
Rhegmatogenous retinal detachment
e
hm
Aetiology A retinal tear (Fig. 220) develops as a result of vitreoretinal
c
traction on a weak area in the peripheral retina (e.g. lattice
a
degeneration – Fig. 221).
t
e
d
l
Clinical • Acute: mobile convex, slightly opaque, corrugated
na
features detached retina (Fig. 222) with break(s)
i
t
• Long-standing: retinal thinning, cysts, demarcation lines
e
R
(‘high water marks’), and fibrotic and immobile retina
(proliferative vitreoretinopathy – PVR – Fig. 223).

Management • Scleral buckling for uncomplicated cases

• Vitrectomy combined with intravitreal injection of gas or
silicone oil for complicated cases.

Tractional retinal detachment

Contraction of fibrous tissue, e.g. associated with
proliferative diabetic retinopathy, causes the retina to
detach without a break; on examination concave immobile
retina with shallow SRF is seen (Fig. 224). Treated by pars
plana vitrectomy.

Exudative retinal detachment

Aetiology The passage of fluid from the choroid into the subretinal
space occurs following breakdown of physiological barriers.
Causes include intraocular tumours, inflammation, severe
CNV, extensive laser photocoagulation and severe
hypertension.

Clinical Convex, very mobile retina with deep shifting fluid and
features absence of retinal breaks (Fig. 225). Treatment consists of
addressing the cause.

76
 38

R
e
t
i
n
a
l
d
e
t
a
Fig. 220 Large retinal tear. Fig. 221 Lattice degeneration.

c
h
m
e
n
t
Fig. 222 Acute superior rhegmatogenous Fig. 223 Total rhegmatogenous
retinal detachment. detachment with severe PVR.

Fig. 224 Tractional retinal detachment. Fig. 225 Exudative retinal detachment. 77
[Link]
 39 Tumours of the uvea

Choroidal naevus
Clinical This common benign tumour is a flat or slightly elevated,
features oval or round, slate-grey lesion, usually less than 3 mm
a
in diameter. Overlying drusen are often present (Fig. 226).
e
uv
Choroidal melanoma
he
Clinical This is the most common primary malignant intraocular
t
f
features tumour in adults. Examination shows a pigmented or
o
amelanotic subretinal mass associated with exudative
s
ur
retinal detachment (Fig. 227)
o
um
Management Options include enucleation, plaque or external beam
T
irradiation, laser photocoagulation or local resection.

Iris melanoma
Clinical A pigmented or non-pigmented inferior iris nodule
features (Fig. 228) which may be associated with pupillary distortion,
ectropion uveae and iris neovascularization.

Choroidal haemangioma
Clinical A very rare dome-shaped or placoid, orange-red lesion
features typically located at the posterior pole (Fig. 229) which may
be associated with secondary cystoid degeneration and
pigment mottling.

Metastatic carcinoma
Clinical Solitary or multiple, unilateral or bilateral, creamy-white,
features oval lesions with ill-defined borders, most commonly
located at the posterior pole (Fig. 230). Common primary
sites are the bronchus and breast.

Choroidal osteoma
Clinical This very rare tumour typically presents in a young female
features as a slightly elevated, orange-yellow lesion with well-
demarcated borders, located at the posterior pole (Fig. 231).
25% are eventually bilateral.

78
 39

T
u
m
o
u
Fig. 226 Choroidal naevus with overlying Fig. 227 Large choroidal melanoma with

r
s
drusen. exudative retinal detachment.

o
f
t
h
e
u
v
e
a
Fig. 228 Iris melanoma. Fig. 229 Choroidal haemangioma with
exudative retinal detachment.

Fig. 230 Three choroidal metastases. Fig. 231 Choroidal osteoma. 79

 40 Tumours of the retina and
optic nerve head
Retinoblastoma
Clinical The most common malignant ocular tumour in children.
features Bilateral in 30% of cases; 6% of patients have a positive
d
family history. Presents typically at about 18 months with a
a
white pupil (leukocoria – Fig. 232) and grows either into the
e
h
vitreous cavity (endophytic) or in the subretinal space
e
(exophytic).
v
r
ne
Management Options include external beam or plaque irradiation, laser
c
i
photocoagulation, cryotherapy, systemic chemotherapy, and
t
p
enucleation.
o
nd
Retinal astrocytoma
a
na
Clinical An uncommon, benign tumour that typically affects patients
i
features with tuberous sclerosis (Bourneville’s disease). Whitish,
t
e
round mass frequently situated near the optic nerve head
r
he
(Fig. 233). May be multiple and bilateral.
t
f
Retinal capillary haemangioma
o
s
Clinical
ur
Uncommon benign tumour. Round, orange-red lesion
o
features associated with dilated supplying and draining vessels
um
(Fig. 234). Frequently multiple, with both eyes affected in
T
50% of cases. 25% of patients have systemic lesions (von
Hippel–Lindau syndrome). Treatment involves laser
photocoagulation or cryotherapy.

Retinal cavernous haemangioma

Very rare congenital lesion characterized by grape-like
aneurysmal clusters (Fig. 235).

Retinal racemose haemangioma

A very rare congenital arteriovenous malformation
consisting of grossly dilated and tortuous vessels (Fig. 236).
A proportion of patients will have central nervous system
lesions (Wyburn–Mason syndrome).

Melanocytoma of the optic nerve head

Rare, benign black tumour with feathery edges (Fig. 237)
which usually occurs in pigmented races.
80
 40

T
u
m
o
u
r
s
o
f
Fig. 232 Retinoblastoma causing left Fig. 233 Retinal astrocytoma.

t
h
leukocoria.

e
r
e
t
i
n
a
a
n
d
o
p
t
i
c
n
e
r
v
e
h
e
a
d
Fig. 234 Retinal capillary haemangioma. Fig. 235 Retinal cavernous haemangioma.

Fig. 236 Retinal racemose haemangioma. Fig. 237 Melanocytoma of the optic nerve 81
head.
 41 Acquired optic nerve disorders

Optic neuritis
Definition Inflammation of the optic nerve, with a range of causes,
the most important being multiple sclerosis.
s
r
Clinical
e
Presents with subacute, usually unilateral, impairment of
d
features central vision that may be associated with pain, especially
r
o
on eye movement. The optic disc is usually normal
s
i
d
(retrobulbar neuritis) and occasionally swollen (papillitis).
e
Severe or recurrent attacks may lead to optic atrophy
v
r
(Fig. 238).
ne
c
Anterior ischaemic optic neuropathy
i
t
p
o
Definition Infarction of the optic nerve head.
d
e
Classification • Arteritic: associated with giant cell arteritis and has a
r
ui
poor prognosis
q
c
• Non-arteritic: associated with hypertension and
A
atherosclerosis.

Clinical Presents with severe acute unilateral visual loss (arteritic) or

features variable visual decrease associated with an altitudinal visual
field defect (non-arteritic). On examination, the optic disc is
pale and swollen. Splinter-shaped haemorrhages are
common (Fig. 239).
Management An immediate ESR is essential. The arteritic form is treated
with high-dose systemic steroids to protect the other eye.

Papilloedema
Definition Disc swelling caused by raised intracranial pressure.

Clinical Symptoms of raised intracranial pressure including

features headaches and nausea. Transient visual obscuration lasting
a few seconds are common but visual acuity is normal until
late.

Signs
• Early: hyperaemia with indistinct margins (Fig. 240)
• Established: obvious elevation, peripapillary haemorrhages
(Fig. 241) and cotton wool spots
• Long-standing: markedly elevated ‘champagne cork’
82 appearance (Fig. 242).
[Link]
 41

A
c
q
u
i
r
e
d
o
Fig. 238 Optic atrophy. Fig. 239 Anterior ischaemic optic

p
neuropathy.

t
i
c
n
e
r
v
e
d
i
s
o
r
d
e
r
s
Fig. 240 Early papilloedema. Fig. 241 Established papilloedema.

Fig. 242 Long-standing papilloedema. 83

 42 Congenital optic disc anomalies

Tilted disc
Clinical Oval optic disc with its vertical axis directed obliquely
features (Fig. 243) often associated with myopia and usually
s
bilateral. A stable upper temporal visual field defect that
e
i
fails to respect the vertical midline is frequent.
l
a
m
Optic disc drusen
no
a
Deposits of hyaline-like material within the optic nerve head
c
which are often bilateral and familial.
s
i
d
c
May mimic the appearance of optic disc swelling
i
t
(pseudopapilloedema). The optic nerve head is lumpy and
p
o
elevated, with no physiological cup. Emerging blood vessels
l
a
branch anomalously (Fig. 244). Visual field defects or
t
i
choroidal neovascularisation may occur.
n
e
ng
Myelinated nerve fibres
o
C
Persistent myelination of the retinal nerve fibres. Feathery
white patches which may be mistaken for papilloedema
when located around the optic disc (Fig. 245).

Optic disc pit

Usually unilateral dark, round or oval pit in a larger
than normal disc (Fig. 246). About 50% of eyes develop
a serous detachment of the macula.

Optic disc coloboma

Caused by defective closure of the fetal fissure. The optic
nerve head contains a large inferior excavation (Fig. 247).
Visual acuity is usually impaired and a superior visual field
defect is typical. May be associated with a variety of
congenital neurological and systemic anomalies.

Morning glory anomaly

The disc is enlarged and excavated with hyaloid remnants
within its base and blood vessels emerging radially
(Fig. 248). Vision may be severely impaired. Associated
congenital central nervous system and other midline defects
may be present.
84
 42

C
o
n
g
e
n
i
t
a
l
o
Fig. 243 Tilted disc. Fig. 244 Optic disc drusen.

p
t
i
c
d
i
s
c
a
n
o
m
a
l
i
e
s
Fig. 245 Myelinated nerve fibres. Fig. 246 Optic disc pit.

Fig. 247 Optic disc coloboma. Fig. 248 Morning glory anomaly. 85
 43 Childhood strabismus (squint)

Strabismus
Definition A misalignment of the eyes.

Esotropia (convergent squint)

)
nt
Clinical Infantile esotropia
ui
Presentation is in the first 6 months
q
features of life; should not be confused with pseudoesotropia
s
(
(Fig. 249). Constant large angle (Fig. 250), occasionally
us
inferior oblique overaction and nystagmus.
m
Accommodative esotropia
s
Convergent squint linked with
i
b
focussing, typically presenting at about 2.5 years. In some
a
r
cases, the eyes can be straightened by spectacle correction
t
s
of a hypermetropic refractive error.
d
o
Non-accommodative esotropia Includes sensory esotropia,
ho
caused by loss of vision in one eye impairing fusion, and
d
l
consecutive esotropia, occurring after surgery to correct
hi
C
exotropia.
Exotropia (divergent squint– Fig. 251)
Classification • Congenital
• Intermittent: in infants and older children
• Secondary: similar to esotropic counterpart
• Consecutive: following surgery for esotropia.

Duane’s syndrome
Clinical Uncommon congenital condition, bilateral in 20% of cases.
features Eyes are usually straight in the primary position but
abduction severely restricted (Fig. 252), with retraction of
the globe and narrowing of the palpebral fissure on
adduction (Fig. 253).

Brown’s syndrome
Clinical Rare congenital condition, bilateral in 10% of cases. Eyes
features straight in the primary position, but there is limited
elevation in adduction (Fig. 254).

Management of squint
• Ophthalmoscopy to exclude media opacity or fundus lesion
• Correction of significant refractive error
• Treatment of amblyopia (usually occlusion therapy)
86 • Extraocular muscle surgery, if appropriate.
 43

C
h
i
l
d
h
o
Fig. 249 Pseudoesotropia due to Fig. 250 Left infantile esotropia. Note

o
d
epicanthic folds. Note symmetrical corneal asymmetrical corneal reflexes.

s
reflexes.

t
r
a
b
i
s
m
u
s
(
s
q
u
i
n
t
)
Fig. 251 Right exotropia. Fig. 252 Left Duane’s syndrome;
attempted abduction of the left eye.

Fig. 253 Left Duane’s syndrome; note Fig. 254 Right Brown’s syndrome.
retraction of the left eye on adduction. 87
[Link]
 44 Third, fourth and sixth nerve
palsies
Clinical Third (oculomotor) nerve palsy
features
• Ptosis due to paralysis of the levator palpebrae superioris
• Divergence due to unopposed action of the lateral rectus
s
e
• Defective elevation due to paralysis of the superior rectus
i
s
and inferior oblique muscles (Fig. 255)
l
a
• Defective depression due to paralysis of the inferior
p
e
rectus (Fig. 256)
v
r
• Defective adduction due to paralysis of the medial rectus
ne
(Fig. 257)
h
• Intorsion on attempted downgaze (unopposed superior
t
x
oblique)
i
s
• Internal ophthalmoplegia – dilated, poorly reactive pupil
nd
and defective accommodation.
a
h
Fourth (trochlear) nerve palsy
t
ur
o
• Hyperdeviation (latent or manifest) in the primary
f
position; accentuated by ipsilateral head tilt: positive
,
d
Bielschowsky test (Fig. 258)
r
hi
• Defective depression in adduction (Fig. 259)
T
• Vertical diplopia, worse in downgaze

Sixth (abducens) nerve palsy

• Horizontal diplopia most marked in abduction
• Convergence in the primary position due to unopposed
medial rectus
• Limitation of abduction (Fig. 260).

Aetiology • Vascular disease (e.g. atherosclerosis, hypertension,

diabetes): isolated or as part of larger stroke
• Posterior communicating artery aneurysm: consider in 3rd
nerve palsy especially if painful, pupil involved and
absence of vascular risk factors
• Raised intracranial pressure: bilateral 6th nerve palsy
(false localizing sign)
• Trauma: especially bilateral 4th nerve palsy
• Cavernous sinus lesions (e.g. fistula, thrombosis): 4th and
6th nerve palsy
• Tumours (e.g. acoustic neuroma, nasopharyngeal
carcinoma): 6th nerve palsy.
88
 44

T
h
i
r
d
,
f
o
u
r
t
Fig. 255 Right third nerve palsy: failure Fig. 256 Right third nerve palsy: failure

h
of elevation. of depression.

a
n
d
s
i
x
t
h
n
e
r
v
e
p
a
l
s
i
e
s
Fig. 257 Right third nerve palsy: failure Fig. 258 Positive Bielschowsky test
of adduction. showing right hyperdeviation.

Fig. 259 Right fourth nerve palsy: failure Fig. 260 Left sixth nerve palsy: failure of 89
of depression in adduction. abduction.
 45 Trauma

Corneal abrasion
Clinical Very common and often caused by fingernails and plant
features stems. Pain is marked and associated with blepharospasm
a
and lacrimation. The epithelial defect, which stains with
m
fluorescein (Fig. 261), often heals within 24 hours. Some
u
patients subsequently develop recurrent corneal erosions.
a
r
T
Management Antibiotic ointment; padding may improve comfort in very
large abrasions but does not enhance healing.

Foreign body
Clinical • Subtarsal: usually scratches the superior cornea with
features blinking
• Corneal foreign body: typically ferrous and may be
associated with a surrounding rust ring (Fig. 262)
• Penetrating: commonly hammered metal fragments.
Complications include infection and cataract.

Blunt anterior segment trauma

• Subconjunctival haemorrhage and periocular ecchymosis
• Hyphaema (Fig. 263): re-bleeding may result in a severe
IOP rise with a risk of corneal blood staining
• Iris sphincter damage
• Iridodialysis (Fig. 264)
• Angle recession (see Fig. 154): risk of chronic glaucoma
• Lens damage: subluxation, dislocation (see Fig. 181) and
cataract formation
• Scleral rupture: usually caused by severe trauma.

Blunt posterior segment trauma

• Vitreous haemorrhage: may hide underlying damage;
ultrasound indicated
• Commotio retinae (Fig. 265): traumatic oedema
• Choroidal rupture (Fig. 266)
• Retinal tear: dialysis, equatorial or macular holes may
cause retinal detachment
• Avulsion of the optic nerve: in severe trauma.

90
 45

T
r
a
u
m
a
Fig. 261 Corneal abrasion stained with Fig. 262 Corneal foreign body with rust
fluorescein. ring.

Fig. 263 Hyphaema. Fig. 264 Iridodialysis.

Fig. 265 Commotio retinae. Fig. 266 Choroidal rupture. 91

 ? Questions
ns
o
i
t
s
ue
Q
1. This 15- year- old boy was born with the bilateral ocular abnormality
shown.
a. What is the diagnosis?
b. Where is the genetic mutation?
c. Some individuals with this condition develop an abdominal tumour. This
patient’s brother, mother and grandfather all have the eye abnormality
shown; what is the likelihood that the patient will develop the tumour?
d. What other ocular features may be present?

2. This patient is aged 76. The lesion shown has been present for at least a
year, its surface intermittently breaks down and crusts.
a. What is the likely diagnosis?
b. What are the different clinical types?
92 c. What is the treatment?
[Link]
 ?

Q
u
e
s
t
i
o
n
s
3. This 64- year- old woman recently underwent ocular surgery.
a. What was the surgical procedure?
b. What is the main indication for this operation?
c. What alternative therapy is available?
d. What adjunctive agents can be used if the risk of failure is high?

4. This patient has worn rigid contact lenses for 3 years.

a. Describe the signs.
b. What is the histology?
c. What may be the pathogenesis?
d. Give two other causes of this condition.

93
 5. This is a retinal capillary
haemangioma.
a. What is the name and inheritance
pattern of the phacomatosis
associated with this lesion?
b. Name three systemic lesions
? occurring in this phacomatosis.
c. What proportion of patients with a
s
solitary retinal capillary
on
haemangioma have systemic
i
involvement?
t
s
e
d. How do the retinal lesions threaten
u
Q
sight and how can they be treated?

6. A 64- year- old man was found by his optometrist to have this condition
in one eye.
a. What is the disorder shown?
b. Approximately what percentage of eyes with this condition develops
glaucoma?
c. In which geographical region is it particularly common?
d. Why would cataract surgery be associated with a higher risk of
complications?

94
 ?

Q
u
e
s
t
i
o
n
7.

s
The parents of this 6- month- old baby are concerned because the eye has
watered persistently since birth.
a. What is the probable cause?
b. What conservative measures might be adopted?
c. At what age should more active intervention be considered, and what form
should this take?
d. What is the most important, but much less common, condition that ought to
be excluded in an infant with a watering eye?

8. This patient underwent a

trabeculectomy 5 days ago.
a. What is the condition shown?
b. What is the likely cause in this
context?
c. Clinically, how does the appearance
of this condition differ from that of
retinal detachment?

95
 9. This 56- year- old white man first
noticed this slowly enlarging brown
patch 6 months ago.
a. What is the diagnosis?
b. What are the two main histological
types?
? c. Is there any chance of malignant
transformation?
s
d. What are the other causes of diffuse
on
conjunctival pigmentation?
i
t
s
e
u
Q
10. This is the MRI scan of a 33- year- old woman who developed blurred
vision in the left eye a week ago.
a. What abnormality is shown, and what is the diagnosis?
b. What might be seen on ophthalmoscopy?
c. What other ocular symptoms might she experience during the current
episode?
d. What other ocular complications may be associated with this disease?

96
11. This is a 6- year- old child with
orbital cellulitis.
a. What are the life-threatening
complications?
b. List alternative diagnostic
possibilities.
c. What is the management? ?

Q
u
e
s
t
i
o
n
s
12. This is the fundus of a
36- year- old man who has had type 1
diabetes for 20 years.
a. What grade of diabetic retinopathy
is shown?
b. What are the signs of the other
grades of diabetic retinopathy?
c. What are the most important risk
factors for diabetic retinopathy?
d. List the other ocular complications
of diabetes.

97
[Link]
 13. This is the fundus of a 72-
year- old man with type 2 diabetes.
a. What does the fundus show?
b. Define the three categories of
‘clinically significant macular
oedema’ (CSMO).
? c. How is CSMO treated?
d. List factors conferring an adverse
s
prognosis.
on
i
t
s
e
u
Q
14. This is a choroidal naevus.
a. What clinical features might arouse
suspicion that a naevus may be a
small melanoma?
b. What treatment modalities are
available for melanoma?
c. What factors influence prognosis?

15. This is a 17- year- old boy with

epilepsy.
a. What are the facial lesions called?
b. What is the systemic condition in
which they are seen, and what is its
inheritance pattern?
c. What are the ophthalmic features of
the disease?

98
16. This is the fundal appearance of
a 70- year- old hypertensive woman.
a. What is the lesion?
b. What complications may occur?
c. What is the management?

Q
u
e
s
t
i
o
n
s
17. This is a glaucoma drainage device.
a. What are the indications for such a device?
b. What proportion of patients undergoing implantation achieves adequate
pressure control without additional medication?
c. What are the complications of implantation?

18. This is the bilateral fundal

appearance of a 25- year old man.
Visual acuity in the right eye is 6/36
and in the left 6/18.
a. Fluorescein angiography shows early
hypofluorescence and late
hyperfluorescence of the lesions.
What is the most likely diagnosis?
b. What other ocular findings may be
evident?
c. What systemic features may be
present?
d. What is the prognosis? 99
 19. This is the coronal CT scan of a
19- year- old man.
a. What is the abnormality
demonstrated?
b. What may be the cause?
c. What physical signs may be
? present?
d. What is the management?
s
on
i
t
s
e
u
Q
20. This is the fundal appearance in
a patient with Grönblad–Strandberg
syndrome.
a. What abnormality is shown?
b. What is the histopathology of the
lesions?
c. What is ‘peau d’orange’ in this
context?
d. What are the other systemic
associations?

100
 ?

Q
u
e
s
t
i
o
n
s
21. This is a 72- year- old patient with Parinaud’s (dorsal midbrain)
syndrome, showing failure of upgaze.
a. What are the other clinical signs of Parinaud’s syndrome?
b. What are the likely causes in a patient of this age?
c. What are the important causes in children?

22. The parents of this 18- month- old baby noticed this abnormality.
a. What is this sign called?
b. What is the most important cause to be considered in a child of this age?
c. List the other important causes.

101
 23. This shows herpes zoster
ophthalmicus.
a. Describe the evolution of the rash.
b. What is Hutchinson’s sign and what
is its significance?
c. What are the important ocular
? complications?
s
on
i
t
s
e
u
Q
24. This shows the histopathology of a skin lesion caused by a virus.
a. Describe the features and give the diagnosis.
b. What is the characteristic mode of ocular presentation of this condition?
c. Patients with what systemic disease characteristically develop multiple lesions
of this type?

102
[Link]
 ?

Q
u
e
s
t
i
o
n
25.

s
This is the optic disc of an 81- year- old man whose intraocular pressure
is 12.
a. What is the most likely diagnosis?
b. What systemic features may be present?
c. What are the characteristic visual field defects in this condition?

26. This patient is about to undergo

cataract surgery.
a. What is the morphological type of
this lens opacity?
b. Although corrected visual acuity is
6/9, symptoms are substantial. What
form are these likely to take?
c. The patient, who is aged only 46,
has asthma. What connection might
this have with the cataract?

27. This is the fundal appearance of

a 37- year- old Turk, who has
experienced recurrent mouth ulcers
and lower limb arthralgia.
a. What is the ocular diagnosis?
b. What is the probable systemic
diagnosis?
c. He has been treated previously for
anterior uveitis with hypopyon. Is
this significant?
d. What is the likely HLA association? 103
?
s
on
i
t
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e
u
Q
28. A few months ago, the parents of this 4- year- old child noticed that
one eye turns in towards the nose.
a. What points should be covered in the history?
b. How should the child be assessed initially?
c. What is the management of convergent squint presenting at this age?

29. This is the fundal appearance of

a 63- year- old woman who recently
became aware of a small missing
patch in the centre of the vision in
one eye.
a. What is the diagnosis?
b. What is the visual acuity likely to
be?
c. Can anything be done to improve
her vision?
d. What is the chance of the condition
affecting the other eye?

104
 ?

Q
u
e
s
t
i
o
n
s
30. This patient experienced the sudden onset of floaters.
a. What abnormality is shown?
b. How should it be treated?
c. What conditions predispose to the development of retinal detachment?

31. This patient has had a

photophobic, red and gritty eye for 2
days.
a. What is the diagnosis?
b. What key physical sign should be
checked prior to measuring the
intraocular pressure?
c. What is the differential diagnosis?
d. What is the treatment?

105
?
s
on
i
t
s
e
u
Q
32. The eyes of this 2- week- old baby have been very sticky for the last
week.
a. By definition, what is the diagnosis?
b. What are the causes, and when does each typically present?
c. What are the systemic manifestations of chlamydial infection in neonates?

33. This is the fluorescein

angiogram of a 70- year- old woman.
a. What lesion is demonstrated?
b. Given the patient’s age, what is the
likely cause?
c. What other underlying pathology
might be responsible?

106
 H e s s s c r e e n c h a r t N a m e N o

F i e l d o f l e f t e y e ( f i x i n g w i t h r i g h t e y e ) F i e l d o f r i g h t e y e ( f i x i n g w i t h l e f t e y e )

I n f . O b l .
S u p . R e c t . I n f . O b l . S u p . R e c t .

?
I n f . O b l . S u p . R e c t .
S u p . R e c t . I n f . O b l .

M e d . R e c t . M e d . R e c t .
L a t . R e c t . M e d . R e c t . M e d . R e c t . L a t . R e c t . L a t . R e c t .
L a t . R e c t .

N a s a l t e m p
t e m p

Q
u
I n f . R e c t S u p . O b l . S u p . O b l .
I n f . R e c t .

e
s
t
I n f . R e c t . I n f . R e c t .
S u p . O b l .
S u p . O b l .

i
o
n
s
G r e e n b e f o r e l e f t e y e G r e e n b e f o r e r i g h t e y e

D i a g n o s i s

34. This is the Hess chart of a 48- year- old patient with double vision.
a. What is the diagnosis?
b. Describe in detail the changes shown on the chart.

35. This is the fundal appearance of

a patient who lost vision in the eye
earlier in the day.
a. Describe the signs.
b. What is the diagnosis?
c. What imaging investigations might
be appropriate and why?
d. What are the other common causes
of sudden, profound, painless
unilateral loss of vision?

36. This patient’s optometrist was

concerned that this might be a
melanoma.
a. What is the diagnosis?
b. What are the characteristics that
support this?
c. Are you concerned regarding
systemic implications?
107
[Link]
 37. This patient has had increasing
difficulty with night vision
(nyctalopia) over the last few years.
a. What are the signs and what is the
diagnosis?
b. What other ocular features may be
? present?
c. What are the electrodiagnostic
s
findings?
on
d. What systemic disorders may be
i
associated with this condition?
t
s
e
u
Q
38. This patient has noticed that
she sweats much more on one side of
her face.
a. Describe the signs and give the
diagnosis.
b. What other ocular features may be
present?
c. What pharmacological tests might
be useful?

39. This is the cornea of an elderly patient with congenital syphilis.

a. What is the condition?
b. Rarely, an intrastromal corneal haemorrhage may occur. What is the
mechanism?
c. List the late systemic signs of congenital syphilis.
108
40. This patient has long, spindly
fingers (arachnodactyly) and a high-
arched palate.
a. What is the ocular sign?
b. What is the systemic diagnosis, and
what is its genetic basis?
c. What other systemic features may ?
be present?

Q
d. What other systemic conditions are

u
associated with this ocular finding?

e
s
t
i
o
n
s
41. This is the retinal appearance in a 56- year- old woman with birdshot
retinochoroidopathy.
a. What is the HLA association?
b. Is vitritis common in this condition?
c. What special investigation is useful when deciding to start treatment?
d. What are the most common causes of visual loss?
109
?
s
on
i
t
s
e
u
Q
42. This is the eye of a man in his twenties who recently contracted
urethritis. Reiter’s syndrome is suspected.
a. What other systemic features might he have?
b. What other ocular feature is characteristic?
c. What blood test will help to confirm the diagnosis?
d. What cardiac complication can occur?

43. This woman aged 40 has thyroid eye disease.

a. What are the symptoms of this condition?
b. Is thyroid eye disease more commonly associated with primary hypo- or
hyperthyroidism?
c. What is the least common ocular motility defect?
d. Orbital soft tissue swelling can sometimes lead to optic neuropathy. What
110 treatments are available?
 ?

Q
u
e
s
t
i
o
n
44.

s
This middle- aged woman has ocular cicatricial pemphigoid.
a. What are the typical cutaneous lesions?
b. What are the complications?
c. How is the active disease treated?

45. This is the bilateral optic disc

appearance of an obese 30- year- old
woman complaining of headache,
nausea and transient visual
obscurations.
a. What is the likely diagnosis?
b. What is the most important
complication?
c. What are the treatment options?

46. This patient underwent penetrating keratoplasty a year ago. For the
past month vision in the eye has been blurred as a result of graft failure.
a. Clinically, how does graft failure differ from rejection?
b. How is rejection treated?
c. List factors conferring a poor prognosis for corneal grafting. 111
?
s
on
i
t
s
e
47.
u
This patient has been working in the Middle East for several years.
Q
a. What is the lesion?
b. What are the indications for its removal?
c. What are the possible surgical techniques?

48. At a routine sight test, an

optometrist discovered this condition
(a fluorescein angiogram is shown) in
both eyes of an asymptomatic
hypermetropic patient.
a. What is the diagnosis?
b. What is the cause?
c. List other causes.

112
[Link]
 15 90 15
15 90 15
30 30
80 30 30
80

70
45 45 70
45 45
60
60

60 50 60
60 50 60
40
40

30
75 75 30
75 75
20
20

10
10
27 24 21 18 15 12 9 6 6 9 12 15 18 21 24 27 90

?
90 27 24 21 18 15 12 9 6 6 9 12 15 18 21 24 27 90
90
10
10

20
20
105 105 105 105
30

Q
30

40
40

u
120 120
50 120 120

e
50

s
60
60

t
135 135
70 135 135
70

i
o
80
150 150 80
150 150

n
165 90
165 90
165 165

s
49. These are the visual fields of a 50- year- old woman with a family
history of glaucoma but normal intraocular pressures.
a. Are the visual fields typically glaucomatous?
b. What other pathology might be suspected?
c. What other investigation is indicated?

50. This woman has chronic arthritis and complains of persistently gritty
eyes.
a. What is the systemic diagnosis?
b. Why are her eyes gritty?
c. What simple tests can help to confirm the diagnosis?
d. What other ocular complications may occur? 113
?
s
on
i
t
s
e
u
Q
51. This patient underwent removal of an acoustic neuroma 2 weeks ago.
a. What is the abnormality shown?
b. What ocular problems may develop?
c. What is the management?

52. This 57- year- old man has intraocular pressures of 26 mmHg in both
eyes.
a. Does he have glaucoma?
b. What percentage of patients with this intraocular pressure develop
glaucoma?
c. How will measuring the optic disc diameter help to determine if the nerve is
normal?
114 d. What other investigations may be useful?
 ?

Q
u
e
s
t
i
o
n
53.

s
This 9- year- old girl suffers from juvenile idiopathic arthritis (JIA).
a. What are the signs shown?
b. What are the ocular manifestations of JIA?
c. List three characteristics in JIA which are associated with a high risk of eye
problems.

54. This is the cornea of a patient who wears soft contact lenses. The eye
has been red and very uncomfortable for 24 hours.
a. What is the diagnosis?
b. What is the management?
c. List the causative microorganisms that do not grow on common culture
media but may constitute a serious threat to vision if untreated.
115
 55. (a) The patient is attempting to
look to the left; a fine nystagmus can
be seen in the left eye. (b) No
abnormality is seen on right gaze.
(c) Convergence is normal.
a. What is the diagnosis?
? b. Where is the lesion located? (a)
c. Give the most likely cause in this
s
patient.
on
d. List other causes.
i
t
s
e
u
Q
(b)

(c)

56. This 37- year- old man has

granulomatous iritis. Ophthalmoscopy
revealed moderate vitritis and
retinitis, as shown.
a. He has no risk factors for HIV
infection. What is the probable
diagnosis?
b. What investigations might help to
confirm the diagnosis?
c. What is the management?

57. This patient sustained an

ischaemic central retinal vein
occlusion 3 months ago.
a. What complication has occurred,
and what is the likely sequel?
b. List other common causes of the
condition shown.
c. How should this patient be
116 managed?
58. This elderly woman presented
to the general accident and
emergency department with
abdominal pain and nausea. The
casualty officer noticed that she had
a red eye.
a. What is the diagnosis? ?
b. What is the management?

Q
c. What are the other common causes

u
of a very painful and red eye?

e
s
t
i
o
n
s
59. This baby’s parents are concerned that this rapidly growing lesion may
damage the child’s eyesight.
a. What is the diagnosis?
b. Is the parents’ concern justified?
c. What is the most common form of treatment?
d. Does the lesion have any systemic implications?

117
[Link]
?
s
on
i
t
s
e
u
Q
60. Bilateral ptosis is shown.
a. What are the muscles responsible for elevating the upper lid, and what is
their innervation?
b. What are the causes of ptosis?
c. What are the causes of pseudoptosis?

61. This infant’s parents are extremely worried by this lesion.

a. What is the diagnosis?
b. What is its pathological categorization?
c. When might treatment be indicated?
d. Are there any systemic implications?

118
 ?

Q
u
e
s
t
i
o
n
s
62. This child is attempting to look up and to the right.
a. Describe the motility abnormality and give the diagnosis, assuming eye
movements are normal in other positions of gaze.
b. What is the management?
c. Most cases are congenital but what are the causes of an acquired lesion of
this type?

63. This elderly patient suddenly

lost vision in one eye.
Ophthalmoscopy revealed a pale,
swollen optic disc.
a. What questions are essential in a
patient presenting with sudden loss
of vision and this disc appearance?
b. What urgent blood tests are
indicated?
c. What further investigation is
necessary?

64. This child also has ptosis and

progressive bilateral extraocular
muscle weakness.
a. What is the diagnosis?
b. What is the underlying genetic
abnormality?
c. In addition to genetic analysis, what
investigations might be helpful in
confirmation of the diagnosis?
119
?
s
on
i
t
s
e
u
Q
65. This 78- year- old man has bilateral corneal arcus as well as this
condition in both eyes.
a. What is the diagnosis?
b. How is sight threatened?
c. What is the treatment?

66. This 60- year- old woman complains of watering of the eye for the last
6 months.
a. What abnormality is shown?
b. What further investigations should be performed to exclude other causes of
the watering?
c. Assuming the abnormality above is responsible for the symptoms, what are
the treatment options?
120
 ?

Q
u
e
s
t
i
o
n
s
67. This child was born with the condition shown.
a. Describe the signs; what is the likely diagnosis?
b. What other signs might be present in this condition?
c. How can the diagnosis be confirmed?

68. This 33- year- old man complains of mild ocular irritation for the last 3
days.
a. What is the diagnosis?
b. What features suggest that this is not a bacterial keratitis?
c. What is the treatment?

121
?
s
on
i
t
s
e
u
69.
Q
This 15- year- old boy was struck in the eye by a tennis ball.
a. What is the sign shown?
b. What is the most important complication of this condition?
c. What management should be instituted?

70. This patient was poked in the eye by a baby earlier in the day.
a. What is this lesion?
b. What are the symptoms?
c. What is the management?

122
[Link]
 ?

Q
u
e
s
t
i
o
71.

n
This 50- year- old suddenly developed a droopy eyelid and double vision

s
associated with ipsilateral frontal headache.
a. What is the diagnosis?
b. What underlying condition must be urgently excluded?
c. What clinical features are important when considering the cause?

72. This patient recently underwent

retinal detachment surgery.
a. The complication of retinal
detachment shown is the most
common cause of failure of surgical
repair. What is it?
b. List the signs of the condition.
c. What form of surgery is likely to be
used to attempt re-attachment?

73. This healthy 35- year- old

woman presented with the sudden
onset of floaters.
a. What is shown?
b. As there is no useful view of the
fundus, what investigation should
be performed?
c. List the causes of the condition
shown.

123
 74. This middle- aged patient
complains of gradually worsening
distortion of vision.
a. What abnormality is shown?
b. What is its histopathological basis?
c. What are the causes?
?
s
on
i
75.
t
These are
s
e
instrumentation used
u
Q
to perform pars plana
vitrectomy (PPV).
a. Name the
instruments.
b. List the important
indications for PPV.

76. This patient has grey- white round and polygonal opacities in the
superficial cornea, densest centrally and forming a honeycomb pattern in
places; the diagnosis is Thiel–Behnke corneal dystrophy (Bowman layer
dystrophy type 2).
a. What is the typical clinical presentation of this condition?
b. Describe the histology.
c. What is the inheritance pattern and where is the gene?
d. What is the other dystrophy affecting the Bowman layer and how do the two
124 differ clinically?
 ?

Q
u
e
s
t
i
o
n
s
77. This boy with a congenital ocular motility disorder is attempting right
gaze.
a. Describe the signs and give the diagnosis.
b. How would he be categorized in the Huber classification of this disorder?
c. Excluding other motility signs, what are the ocular associations of this
condition?

78. Vision is very poor in this eye;

the fellow eye is normal.
a. Describe the signs and give the
diagnosis.
b. What is the incidence of subsequent
serous retinal detachment?
c. What are the systemic implications?

125
 90 90
63.00
120 60 Na s a l Na s a l 120 60
60.77

58.54
150 30 150 30

56.31

54.08
180 00 180 00

51.85
?
49.62
210 330 210 330
s
47.38
on
45.15
Ta ng OD Ta ng OS
i
240 300 240 300
t
s
270 270
42.92
e
Axis Dis t P wr Ra d Z Axis Dis t P wr Ra d Z
u
000 0.00 49.93 6.76 0.00 000 0.00 56.84 5.94 0.00 40.69
Q
KS: 50.35D @ 130 KS : 59.88D @ 080
KF: 50.87D @ 170 38.46
KF: 46.11D @ 040
KD: 4.24D KD: 9.01D
36.23
S im K/Avg (1.60) S im K/Avg (1.60)
34.00

EH Re l

79. This investigation was used to assess the cornea of a 23- year- old
woman with increasing astigmatism.
a. What was the investigation employed?
b. What is represented by the colour coding used?
c. Is the cornea normal according to the scan?

80. This patient complains of drooping eyelids worse towards the end of
the day together with intermittent double vision.
a. What underlying systemic disease may be present?
b. What other eye signs would reinforce the putative diagnosis?
c. What blood test can be useful in helping to confirm the diagnosis?
126 d. What pharmacological test might be indicated?
 ?

Q
u
e
s
t
i
o
n
81.

s
This patient sustained a severe bilateral chemical injury 3 years ago.
a. What is the device shown?
b. What are the indications for surgery of this type?
c. What are the complications?

82. This man has noticed worsening vision in the right eye over the past
few months. Diffuse stellate keratic precipitates are present on slit lamp
examination.
a. Describe the signs and give the diagnosis.
b. Besides that shown, what other sight-threatening complications might occur?
c. How is the condition treated? 127
[Link]
?
s
on
i
t
s
e
u
Q
83. The vision in this pseudophakic eye has gradually been getting worse
for several months.
a. Describe the complication of cataract surgery shown.
b. What other symptoms is the patient likely to be experiencing?
c. What is the treatment?
d. What are the complications of the treatment?

84. This patient’s eye has been gritty and red for several weeks.
a. What is the cause of the symptoms?
b. What is the pathogenesis of the involutional form of this condition?
128 c. What is the treatment?
85. This 81- year- old woman has
severe ‘wet’ age- related macular
degeneration in her other eye. Her
optometrist was concerned about a
recent fall in the vision of the eye
shown above.
a. What is the condition shown? ?
b. If there is clinical suspicion of

Q
choroidal neovascularization (CNV),

u
what investigation should be

e
s
performed?

t
i
c. Is there anything that can be done

o
n
to reduce the risk of CNV in this

s
eye?

86. This elderly patient has a

dome- shaped macular elevation
corresponding to the abnormality
shown above.
a. What is the investigation shown?
b. What is the lesion?
c. What is the pathogenesis?
d. How might it be treated?

87. This 16- year- old girl has been

unwell for several weeks. A physician
identified this fundal appearance.
a. Describe the lesions. What are they
called?
b. Leukaemia is a possible underlying
diagnosis. List others.
c. How else might leukaemia manifest
in the eyes?

129
 88. This patient has oculocutaneous
albinism with white hair and very
pale skin.
a. Is he likely to be tyrosinase-negative
or -positive?
b. How would visual-evoked potential
? analysis help in the assessment of
this patient?
s
c. Name two systemic syndromes
on
associated with this condition.
i
d. Could this patient have either of
t
s
e
these syndromes?
u
Q
89. This was an incidental finding
in a 30- year- old man.
a. Describe the lesion. What is the
most likely cause?
b. At what age do such lesions tend to
reactivate in immunocompetent
individuals?
c. What diagnostic tests are available?

90. This patient recently underwent

cataract surgery complicated by
vitreous loss.
a. What is shown?
b. What is meant by ‘vitreous loss’?
c. What are the postoperative
complications of vitreous loss?

130
 ?

Q
u
e
s
t
i
o
91.

n
Retinal detachment surgery involving placement of an explant was

s
carried out on this patient’s eye 1 year ago.
a. What complication has occurred?
b. Could removal of the explant precipitate re-detachment?
c. How should the explant be removed?

92. This patient presented with a sore red eye.

a. What sign is present?
b. The patient has had no previous eye problems and is otherwise healthy. Is
systemic investigation indicated?
c. What screening investigations might be carried out? 131
?
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on
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Q
93. This patient has a systemic disorder associated with multiple fractures
and deafness.
a. What is the systemic disorder?
b. What is the pathological basis of the abnormality shown?
c. What other systemic diseases are associated with this sign?

94. This patient has approximately

10 dioptres of myopia.
a. What is the pathological process
shown?
b. List other ocular associations of
high myopia.
c. Is this patient’s ocular axial length
likely to be nearer 20 mm or
30 mm?

95. This myopic

patient is undergoing
refractive surgery.
a. What is the
procedure?
b. Can this procedure
also be used to
correct hypermetropia
or astigmatism?
c. What complications
may occur?
132
[Link]
 ?

Q
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96.

s
This 30- year- old woman has been aware of this triangular red patch
and mild aching in the eye for 3 days.
a. What is the diagnosis?
b. Classify this condition.
c. Should systemic investigations be performed?
d. What is the treatment?

97. While shaving, this patient noticed the appearance shown.

a. What is the diagnosis?
b. What symptoms may be present?
c. What investigations are required?

133
 98. The visual acuity in this
30- year- old man’s right eye has
deteriorated over the last couple of
days.
a. What is the likely diagnosis?
b. What is the visual acuity likely to
? be?
c. If there is any doubt regarding the
s
diagnosis, what investigation is
on
appropriate?
i
t
s
e
u
Q
99. This child’s left eye turns outward when he is tired, day- dreaming or
exposed to bright light.
a. What is the diagnosis?
b. What are the clinical categories of this form of strabismus?
c. How is it managed?

134
100. This child is undergoing
occlusion therapy for amblyopia.
a. What is amblyopia?
b. What are the causes?
c. What is the usual age limit for
useful treatment of amblyopia?
?

Q
u
e
s
t
i
o
n
s
101. This young woman has been undergoing psychiatric investigation; one
of her doctors noticed this ocular sign.
a. What is the sign called?
b. What is the histology?
c. What is the underlying diagnosis?
d. What is the other common ocular sign?
e. What are the other major systemic abnormalities?
135
?
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on
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Q
102. This elderly patient takes a drug for cardiac arrhythmia.
a. Describe and name the corneal appearance.
b. What drug is the patient likely to be taking?
c. What proportion of patients on the cardiac drug develop this?
d. What other drugs, and what disease, can give a similar appearance?

103. This otherwise healthy 55- year- old man has developed increasing
astigmatism over several months in both eyes. After an Internet search he
wonders whether he has keratoconus.
a. Regardless of the corneal appearance, do you think the patient’s diagnosis is
correct?
b. What is the significance of the precise location of the changes shown?
c. What is the likely diagnosis?
136
104. This 40- year- old woman
complains that her left eye is
gradually getting larger. CT of the
orbit showed a well- circumscribed
oval lesion within the muscle cone.
a. Bearing in mind the CT finding,
what is the sign shown? ?
b. This lesion is the most common

Q
benign orbital tumour in adults;

u
what is the diagnosis?

e
s
c. What other ocular signs might be

t
i
present?

o
n
d. What is the treatment?

s
105. Ophthalmological review has been requested by a physician, who is
uncertain if this young adult’s optic discs are swollen.
a. What is the investigation shown?
b. What abnormality is demonstrated?
c. What other investigations could be performed?

137
[Link]
?
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on
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u
Q
106. This eye of a 35- year- old woman has glaucoma. The fellow eye is
normal.
a. What is the likely diagnosis?
b. What are the other forms of this condition?
c. What proportion of patients develop glaucoma?

107. This patient has congenital dental and facial anomalies as well as this
condition, which affects both eyes.
a. Describe the signs.
b. What is the diagnosis?
c. What is the genetic basis of this condition?
d. What proportion of patients develop glaucoma, and at what age?

138
 ?

Q
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108.

s
Cataract surgery was carried out on this eye 2 days earlier.
a. Describe the signs.
b. What is the probable diagnosis?
c. What is the management?

109. A 29- year- old man was found to have this appearance at a routine
refraction.
a. What is the sign called?
b. What is the likely diagnosis?
c. What is the probable refractive error?
d. What proportion of patients with this condition are likely to develop
glaucoma?

139
?
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Q
110. Persistent irritation, redness and stickiness, worse in the morning,
have led this patient’s general practitioner to refer him to an
ophthalmologist.
a. What signs are evident?
b. What is the diagnosis?
c. What is the treatment?

111. This young man was involved in a car accident; he was not wearing a
seatbelt.
a. What injury is shown?
b. How should this be managed?

140
 ?

Q
u
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s
t
i
o
112. This patient with glaucoma described episodic blurring of vision

n
s
associated with rainbows around lights; the episodes ceased following a laser
procedure.
a. What form of laser treatment has been carried out?
b. What forms of glaucoma can present with these symptoms?
c. What other uses are there for the type of laser used to treated this patient?

113. This is a symmetrically bilateral appearance in a 51- year- old patient

with slowly deteriorating vision.
a. What is the diagnosis?
b. Are recurrent erosions common in this disorder?
c. Will the patient require penetrating keratoplasty?
d. What are the other forms of this disorder?

141
 114. This is the fundus of an
8- week- old baby.
a. What is the diagnosis?
b. Describe the different stages of this
disease.
c. What are the criteria for the
? decision to treat this condition?
d. What form will treatment take?
s
on
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u
Q
115. This is the fundus appearance
in both eyes of a 40- year- old man
with severe headaches.
a. What clinical test is indicated?
b. Why are the macular exudates
distributed in the fashion shown?
c. What other fundus signs might be
seen in this condition?

116. This is the iris appearance in a 34- year- old man.

a. What is the sign?
b. What is the probable underlying systemic disease?
c. What are the other ophthalmic features of the systemic disorder?
142 d. What is the inheritance pattern, and where is the gene locus?
[Link]
 T e s t d u r a t i o n : 0 1 : 3 3 m i n

T o t a l d e v i a t i o n

R i g h t e y e

F i x a t i o n e r r o r s : 0 / 3

F a l s e p o s i t i v e e r r o r s : 1 / 5

3 0 °
?

Q
u
P r o b a b i l i t y

e
s
≥

t
P 5 % P < 5 %

i
o
T e s t d u r a t i o n : 0 1 : 1 5 m i n

n
s
P < 2 % P < 1 %

T o t a l d e v i a t i o n

L e f t e y e

3 0 °

F i x a t i o n e r r o r s : 1 / 3

F a l s e p o s i t i v e e r r o r s : 1 / 5

117. This patient has been referred to the eye clinic by her optician.
a. What is the investigation shown?
b. What is the principle behind the test?
c. What are its main advantages?

118. This elderly Indian patient has had these whitish corneal nodules for
many years.
a. What are the lesions?
b. What is likely to be the underlying eye disease responsible?
c. What is the treatment? 143
?
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on
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Q
119. This painless lump has been present for several months.
a. What is the diagnosis and pathogenesis?
b. What is the corresponding conjunctival lesion?
c. What forms of treatment are available?

120. The patient’s general

practitioner was concerned about this
fundal appearance.
a. What is the diagnosis?
b. What are the ocular associations?
c. What is the pathogenesis?
d. What is the population prevalence?

144
 Answers

1. a. Aniridia.
b. Usually in the PAX6 gene on chromosome 11p13.
c. Probably the same as the general population. Aniridia is classified
into AN-1 (65% of patients) with autosomal dominant inheritance

A
n
and no systemic implications, AN-2 (33%), a sporadic form carrying a

s
w
30% risk of Wilms’ tumour (Miller’s syndrome), and AN-3 or

e
Gillespie’s syndrome with autosomal recessive inheritance, associated

r
s
with neurological problems.
d. Nystagmus, glaucoma, cataract, lens subluxation, foveal and optic
nerve hypoplasia and corneal opacity.

2. a. Basal cell carcinoma (BCC).

b. Nodulo-ulcerative (‘rodent ulcer’) and sclerosing types.
c. Surgical excision, including a wide (4 mm) margin of clinically normal
tissue.

3. a. Trabeculectomy, a form of filtering surgery.

b. Glaucoma.
c. • Topical prostaglandin derivatives, beta-blockers, alpha agonists,
carbonic anhydrase inhibitors and miotics.
• Systemic carbonic anhydrase inhibitors.
• Laser trabeculoplasty, cyclodiode and peripheral iridotomy in
narrow-angle glaucoma.
• Non-penetrating drainage surgery (e.g. viscocanalostomy) and
valve implants (e.g. Molteno).
d. 5-fluorouracil and mitomycin C.

4. a. There are multiple large, highly vascular, cobblestone-like conjunctival

projections.
b. Hyperplastic conjunctival epithelium surrounds a vascular core and a
cellular chronic inflammatory infiltrate (lymphocytes, plasma cells,
eosinophils).
c. • Mechanical irritation by a poorly fitting lens.
• Allergy to lens material or cleaning solutions.
• Immune reaction to lens deposits.
d. Severe chronic allergic conjunctivitis and a protruding suture.

5. a. Von Hippel–Lindau syndrome (VHL).

b. CNS haemangioblastoma, renal carcinoma, phaeochromocytoma,
polycythaemia, and renal, pancreatic and other abdominal cysts.
c. Approximately 50%. Virtually all patients with multiple retinal
tumours will have VHL. 145
 d. Most commonly by retinal exudation and occasionally by vitreous
haemorrhage and epiretinal membrane formation. The lesions can be
treated with laser photocoagulation, cryotherapy or plaque
radiotherapy (brachytherapy).

6. a. Pseudoexfoliation.
b. The cumulative risk of developing glaucoma in eyes with
pseudoexfoliation is about 5% at 5 years and 15% at 10 years,
although this is much higher if the fellow eye already has
s
pseudoexfoliative glaucoma.
r
e
c. Scandinavia.
w
d. The pupil frequently dilates poorly, making access to the lens difficult.
s
n
A
The lens capsule and zonules are fragile, leading to an increased risk
of capsular rupture and vitreous loss. Postoperatively, corneal
decompensation and capsular opacification are more common, as is
late dislocation of the implant.

7. a. Failure of canalization of the nasolacrimal duct.

b. Daily lacrimal sac massage.
c. Probing of the lacrimal system should be considered only at 12
months; dacryocystorhinostomy may be necessary in resistant cases.
d. Congenital glaucoma.

8. a. Choroidal detachment.
b. Overfiltration with associated low intraocular pressure.
c. A choroidal detachment appears as a smooth, brown convex elevation
that does not involve the posterior pole, being limited by the exit
points of the vortex veins. The ora serrata does not limit anterior fluid
spread, and thus may be visible without indentation.

9. a. Primary acquired melanosis (PAM).

b. PAM with and without atypia, the latter a proliferation of normal
melanocytes within the basal epithelial layer, the former shows large
melanocytes with prominent nucleoli and pagetoid spread.
c. About 50% within 5 years in PAM with atypia.
d. Racial epithelial melanosis and congenital melanocytosis (in contrast
to PAM, lies deep to the conjunctiva and cannot be moved over the
sclera).

10. a. Periventricular demyelinated plaques, their long axes aligned with the
ventricular margins; the patient has optic neuritis associated with
multiple sclerosis.
b. The optic disc may be normal (retrobulbar neuritis) or, less commonly,
swollen and hyperaemic (papillitis). There may be pallor of the
contralateral optic disc, usually temporally. Retinal vascular sheathing
is sometimes present.
c. Pain, particularly on upgaze, headache, tenderness of the eye,
146 decreased colour vision and visual field loss.
 d. Ocular motor cranial nerve palsies, internuclear ophthalmoplegia,
nystagmus; rarely, intermediate uveitis and peripheral retinal
vasculitis.

11. a. Meningitis, brain abscess and cavernous sinus thrombosis.

b. Preseptal cellulitis, rhabdomyosarcoma and orbital inflammation
associated with retinoblastoma and leukaemia.
c. Conjunctival and nasal swabs, intravenous antibiotics and monitoring
of optic nerve function. Urgent orbital CT scan to exclude periosteal

A
abscess should be considered. Involve otorhinolaryngological

n
colleagues because sinus washout may be required.

s
w
e
12.

r
a. Proliferative diabetic retinopathy (DR) with disc neovascularization.

s
b. Background DR – exudates and dot and blot haemorrhages;
preproliferative DR – deep round haemorrhages, cotton wool spots
and venous irregularity; advanced DR – vitreous haemorrhage and
tractional retinal detachment.
c. Duration of diabetes, poor control, hypertension, renal impairment
and obesity. Hyperlipidaemia and smoking may also be important.
Pregnancy is sometimes associated with rapid deterioration.
d. Cataract, unstable refraction, ocular motor nerve palsies, neovascular
glaucoma, asteroid hyalosis, papillopathy, iris transillumination
defects and orbital mucormycosis.

13. a. Severe background diabetic retinopathy.

b. • Retinal thickening within 500 µm of the centre of the macula.
• Exudates within 500 µm of the centre of the macula if associated
with retinal thickening.
• Retinal thickening within one disc diameter of the centre of the
macula if greater than one disc area in size.
c. With focal argon laser photocoagulation to points of leakage (usually
microaneurysms) or grid laser to diffusely leaking areas. Innovative
modalities include intravitreal steroids, systemic pharmaceutical
agents and vitrectomy.
d. Hard exudates involving the fovea, significant ischaemia, diffuse
rather than focal oedema, cystoid macular oedema and poor visual
acuity at presentation.

14. a. Visual symptoms, especially photopsia. Large lesion size: diameter

>5 mm, thickness >1 mm, orange pigment overlying the lesion,
associated subretinal fluid and documented increase in size after
puberty.
b. Largely dependent on patient profile (especially age), tumour size and
location and preferences of the patient and surgeon. Modalities
available for the primary tumour include radioactive plaque
application (brachytherapy), external beam radiotherapy, local
resection, enucleation, stereotactic radiosurgery using the gamma
knife and diode laser transpupillary thermotherapy (‘TTT’). 147
[Link]
 c. Histological type, the presence of chromosomal abnormalities in
tumour cells, tumour size, patient age, the presence or absence of
extrascleral extension and tumour location (anterior worse than
posterior).

15. a. Adenoma sebaceum.

b. Tuberous sclerosis, also known as Bourneville’s disease, which is
inherited in an autosomal dominant pattern.
c. Retinal astrocytomas are present in about 50%. Other rarer features
s
include the ocular sequelae of raised intracranial pressure
r
e
(papilloedema, optic atrophy and sixth nerve paresis) and
w
hypopigmented retinal and iris spots.
s
n
A
16. a. A retinal artery macroaneurysm.
b. Frequently, these lesions thrombose and undergo spontaneous
involution. However, chronic leakage may cause retinal oedema, with
the risk of macular involvement. Haemorrhage may occur if the
macroaneurysm ruptures.
c. Laser may be applied to a vision-threatening lesion, otherwise
observation is appropriate.

17. a. Artificial drainage shunts are used when the success rate of
trabeculectomy is low. Examples include neovascular, traumatic and
certain developmental glaucomas, severe conjunctival scarring and
uncontrolled glaucoma despite previous trabeculectomy with
adjunctive antimetabolite.
b. Only about one-third in the longer term.
c. Overdrainage (with shallow/flat anterior chamber), cataract, corneal
decompensation, tube retraction or erosion through the conjunctiva,
endophthalmitis and extraocular muscle imbalance.

18. a. Acute multifocal posterior placoid pigment epitheliopathy (AMPPPE).

b. Iritis and vitritis, and sometimes optic disc oedema and retinal
periphlebitis.
c. An influenza-like illness and erythema nodosum.
d. The prognosis is good, 80% of eyes regaining 6/12 or better.

19. a. The scan shows a right orbital floor ‘blowout’ fracture.

b. Severe blunt trauma.
c. Periocular swelling and bruising, subcutaneous air (‘surgical
emphysema’), enophthalmos, paraesthesia in the infraorbital nerve
distribution, limitation of vertical eye movement and damage to the
globe.
d. Systemic antibiotics (as this is a compound fracture), avoidance of
blowing the nose, and surgical repair if indicated, usually for larger
defects and soft tissue entrapment; timing of surgery is controversial.

20. a. Angioid streaks; the combination of these and pseudoxanthoma

148 elasticum is known as Grönblad–Strandberg syndrome.
 b. Angioid streaks consist of dehiscences in the elastic layer of the
Bruch membrane due to progressive degeneration and deposition of
calcium with secondary changes in the retinal pigment epithelium
and choriocapillaris.
c. Peau d’orange (‘orange skin’), refers to a pigmented mottling,
particularly evident temporal to the macula, seen in eyes with angioid
streaks.
d. Ehlers–Danlos syndrome type 6, Paget’s disease and possibly certain
haemoglobinopathies.

A
n
21. a. Light–near dissociation of pupillary reactions, lid retraction (Collier’s

s
w
sign), convergence–retraction nystagmus (elicited with an optokinetic

e
r
nystagmus drum), paresis (or spasm) of convergence and

s
accommodation. Downgaze is typically normal. The eyes will be
straight in the primary position of gaze in the absence of additional
motility abnormality.
b. Stroke or tumour involving the midbrain and posterior fossa aneurysm.
c. Sylvian aqueduct stenosis, pinealoma and meningitis.

22. a. Leukocoria: ‘white pupil’.

b. Retinoblastoma, the most common malignant intraocular tumour in
children; 60% of cases present with leukoria and both eyes are
affected in 30% of patients.
c. Persistent hyperplastic primary vitreous, Coats’ disease, advanced
retinopathy of prematurity and toxocariasis.

23. a. An initial maculopapular rash progresses to form vesicles and

subsequently crusting ulcers; associated periorbital oedema is typical.
b. This refers to skin lesions on the side of the tip of the nose and is
associated with an increased risk of ocular complications.
c. Acute iritis, keratitis (epithelial, nummular, disciform and
neurotrophic), scleritis, optic neuritis and ocular motor nerve palsies.

24. a. A well-defined nodule with a central crater. The base consists of

proliferating squamous cells with eosinophilic cytoplasmic inclusions
that enlarge as they progress towards the large central crater,
degenerating with loss of their nuclei in the process. The diagnosis is
molluscum contagiosum.
b. With a persistent follicular conjunctivitis, caused by continual
shedding of viral particles from a lesion on the lid margin.
c. Acquired immune deficiency syndrome (AIDS).

25. a. Normal-tension glaucoma because the disc shows glaucomatous

cupping.
b. A range of vascular phenomena have been reported: Raynaud’s
phenomenon, migraine, nocturnal systemic hypotension, reduced
blood flow velocity of the ophthalmic and ciliary arteries,
paraproteinaemia and the presence of serum autoantibodies. 149
 c. Paracentral scotoma, nasal step, arcuate defect, ring scotoma and
end-stage with a small residual central island.

26. a. Posterior subcapsular (PSC).

b. Symptoms out of proportion to the corrected visual acuity such as
substantial glare, vision worse in bright light or when encountering
car headlights at night, and difficulty with reading vision, caused by
pupillary constriction enhancing the effect of the opacity.
c. The patient may have taken systemic steroids, which promote the
s
formation of PSC cataract.
r
e
w
27. a. Superotemporal branch retinal vein occlusion.
s
n
A
b. Behçet’s disease. A retinal venous occlusion is very uncommon in a
patient of this age without an underlying predisposing condition.
Behçet’s affects the venous circulation more than the arterial.
c. Yes; anterior uveitis, often with a hypopyon, is one of the major
diagnostic criteria for Behçet’s, along with recurrent aphthous oral
ulceration.
d. HLA-B51.

28. a. Besides age of onset and variability of the squint, family history, birth
history and general health.
b. Visual acuity, stereopsis, confirm the presence of a deviation (i.e.
exclude pseudoesotropia), measurement of the deviation, checking
the ocular movements (particularly to exclude a sixth nerve palsy),
refraction and ocular examination (a squint can be the presenting
sign of pathology such as retinoblastoma and congenital cataract).
c. Typically, a hypermetropic refractive error will be present. Spectacle
correction will usually partially or completely control the squint, and
the child should be reviewed after several weeks.

29. a. Age-related macular hole.

b. Typically this will be 6/60, though eccentric fixation may permit up to
6/9.
c. Vitrectomy, usually accompanied by peeling of the internal limiting
membrane surrounding the hole, and intraocular gas injection,
facilitates closure of the hole and visual improvement in most
patients. Best results are achieved with holes present for less than
a year.
d. Up to 15% at 5 years.

30. a. A U-shaped retinal tear.

b. Laser photocoagulation with two rows of confluent burns. If the tear
is very peripheral, cryotherapy may be easier to apply.
c. Myopia, lattice degeneration, aphakia and pseudophakia (particularly
when complicated by vitreous loss), trauma (old or recent), and family
history of retinal detachment, especially when associated with
150 Stickler’s syndrome.
31. a. Dendritic ulcer due to herpes simplex keratitis (HSK).
b. Corneal sensation is typically reduced in HSK and must be checked
before the instillation of local anaesthetic.
c. Healing corneal abrasion is probably the most common source of
diagnostic difficulty; other causes of a dendritic appearance include
herpes zoster keratitis, the epitheliopathy of contact lens wear,
acanthamoeba keratitis and topical medication toxicity.
d. Topical aciclovir, ganciclovir or trifluorothymidine. Debridement is
used in some circumstances. Long-term oral aciclovir can be used to

A
reduce the frequency of recurrence if necessary.

n
s
w
32. a. Neonatal conjunctivitis or ‘ophthalmia neonatorum’ – conjunctivitis

e
r
occurring within the first neonatal month.

s
b. • Chemical conjunctivitis: within the first postnatal week is usually
due to antiseptic agents although in the past commonly due to
silver nitrate gonococcus prophylaxis.
• Gonococcus: within the first week.
• Chlamydia: 1–3 weeks
• Herpes simplex: 1–2 weeks
• Staphylococci and other bacteria: end of first week onwards.
c. Infection may involve the lungs (pneumonitis), nasopharynx, middle
ear, vagina and rectum.

33. a. Although a series of frames is ideally required to ascertain the diagnosis,

this almost certainly shows a choroidal neovascular membrane.
b. The ‘wet’ form of age-related macular degeneration.
c. Other, less common, causes include myopia, punctate inner
choroidopathy, traumatic choroidal rupture, angioid streaks,
inappropriate retinal laser photocoagulation and optic disc drusen.

34. a. Right superior oblique palsy.

b. The smaller area is enclosed by the chart for the right eye, suggesting
that this is the abnormal side. The right chart is centred at a higher
level than the left, showing that this is the higher eye in the primary
position. In addition to the primary underaction of the right superior
oblique, there is overaction (due to contracture with time) of the
unopposed ipsilateral inferior oblique, overaction of the contralateral
inferior rectus yoke muscle (due to Hering’s law of equal innervation),
and underaction of the contralateral superior rectus (due to Hering’s
law, as the contracted right inferior oblique requires less innervation).

35. a. The retina appears pale and oedematous, except at the centre of the
macula where the contrast gives the appearance of a ‘cherry-red
spot’. The arterioles are attenuated.
b. Central retinal artery occlusion.
c. Carotid artery assessment by ultrasound (duplex), MRI or
conventional angiography, and cardiac imaging, in order to exclude
an embolic source. 151
 d. Vitreous haemorrhage, branch retinal artery occlusion, retinal venous
occlusion, macular haemorrhage (usually due to macular
degeneration), anterior ischaemic optic neuropathy and optic
neuritis.

36. a. The ‘typical’ form of congenital hypertrophy of the retinal pigment

epithelium (CHRPE).
b. The lesion is solitary, flat, dark-grey to black, round, with a
hypopigmented rim and hypopigmented lacunae.
s
c. The ‘typical’ form of CHRPE does not carry any systemic implications,
r
e
in contrast to the ‘atypical’ form (spindle-shaped or oval, multiple,
w
bilateral, hypopigmentation at one margin), which is associated with
s
n
A
familial adenomatous polyposis, Gardner’s syndrome and Turcot’s
syndrome.

37. a. Mid-peripheral retinal pigmentary deposits in a bone-spicule pattern,

waxy disc pallor and arteriolar attenuation, characteristic of retinitis
pigmentosa.
b. Relatively common features include: mid-periphery annular scotomas,
maculopathy (cystoid oedema, atrophy), posterior subcapsular
cataracts, myopia and open-angle glaucoma (3%).
c. Reduced scotopic (and later photopic) responses in ERG, abnormal
EOG, prolonged dark adaptation.
d. Important conditions include Bassen–Kornzweig syndrome, Refsum’s
syndrome, Usher’s syndrome, Kearns–Sayre syndrome and
Bardet–Biedl syndrome.

38. a. Slight right ptosis and miosis. The diagnosis is Horner’s syndrome. The
patient is actually experiencing reduced sweating on the abnormal
side, as the lesion is proximal to the splitting of the sudomotor fibres
to travel along the external carotid.
b. Slight elevation of the lower eyelid, heterochromia, relative hypotony,
conjunctival hyperaemia and hyperaccommodation.
c. Cocaine 4% dilates a normal pupil due to prevention of noradrenaline
(norepinephrine) re-uptake, but not a Horner’s pupil.
Hydroxyamphetamine 1% causes release of noradrenaline
(norepinephrine) from a functioning third-order sympathetic neurone
and so will dilate the pupil in first- or second-order lesions as well as
the normal pupil. Adrenaline (epinephrine) 0.1% dilates the pupil of a
third-order lesion due to denervation hypersensitivity.

39. a. A feathery deep stromal scar and ghost vessels characteristic of

interstitial keratitis.
b. The ghost vessels may refill and, rarely, bleed if the inflammation
reactivates.
c. Bony features include sabre tibiae, saddle-shaped nose, frontal
bossing, prognathism, high-arched palate, dental malformations
152 (Hutchinson’s teeth and Moon’s molars) and sensorineural deafness.
[Link]
40. a. The lens is subluxated upwards.
b. Marfan’s syndrome, associated with a mutation of the fibrillin gene
on chromosome 15.
c. Tall, but with arm span greater than height, muscular
underdevelopment, hernias, aortic dilatation and dissection, heart
valve disease, striae and fragility of the skin.
d. Homocystinuria, Weill–Marchesani syndrome, hyperlysinaemia,
sulphite oxidase deficiency, Ehlers–Danlos syndrome and Stickler’s
syndrome.

A
n
41.

s
a. 90% or more of patients are positive for HLA-A29.

w
b. Yes, moderate vitritis is typical.

e
r
c. Electroretinography (ERG), which provides a baseline measure of

s
retinal function.
d. Optic atrophy and chronic cystoid macular oedema.

42. a. Arthritis (especially knees and ankles), plantar fasciitis, Achilles

tendonitis, keratoderma blenorrhagica, mouth ulceration, balanitis,
epididymitis, orchitis and prostatitis.
b. Acute anterior uveitis and occasionally punctate epithelial keratitis.
c. HLA-typing will be positive for HLA-B27 in 70% of cases.
d. Aortic incompetence.

43. a. Ocular discomfort, double vision, reduced vision and cosmetic

problems.
b. The incidence is far higher in hyperthyroidism (thyrotoxicosis) than
hypothyroidism (myxoedema). However, patients can be clinically and
biochemically euthyroid.
c. Failure of adduction.
d. Systemic steroids, orbital radiotherapy and surgical decompression.

44. a. Recurrent, non-scarring blisters and sparse localized erythematous

plaques.
b. Symblepharon (adhesions between bulbar and palpebral conjunctiva),
ankyloblepharon (adhesions between the lids at the outer canthus)
and severe dry eye. Advanced disease can involve severe secondary
corneal changes, which may cause blindness.
c. Topical lubricants, steroids and antibiotics where appropriate;
subconjunctival mitomycin C; protective contact lenses; systemic
options include steroids, dapsone, intravenous immunoglobulins and
cytotoxic agents.

45. a. Idiopathic intracranial hypertension, although CT or MRI brain scan is

required to rule out a space-occupying lesion.
b. Blindness may result from secondary optic atrophy due to chronic
papilloedema.
c. Diuretics, systemic steroids, optic nerve fenestration and
lumboperitoneal shunt. 153
 46. a. In pure graft failure, which occurs because of endothelial
decompensation, the eye is quiet, whereas rejection involves an
inflammatory element (although this may be mild). Specific signs of
epithelial (discrete subepithelial infiltrates – Krachmer’s spots) or
endothelial (keratic precipitates, oedema, more marked inflammation)
rejection will be evident. Failure is often a consequence of rejection.
b. The mainstay of treatment is topical steroids, although periocular
injection or systemic immunosuppression may be necessary.
c. Eyelid disease, tear film dysfunction, anterior segment inflammation,
s
r
corneal vascularization or thinning and uncontrolled glaucoma.
e
w
s
47. a. A pterygium, an area of degenerative fibrovascular tissue encroaching
n
A
on the cornea most commonly seen in hot, dry climates.
b. Visual deterioration (due to astigmatism or visual axis obstruction),
substantial irritation and cosmesis.
c. Simple removal is associated with a high rate of recurrence.
Conjunctival patch autografting, irradiation and antimitotic agent
application are more effective.

48. a. Choroidal folds.

b. Idiopathic, because of bilateral involvement, lack of symptoms and
the presence of hypermetropia.
c. Orbital disease (e.g. thyroid, tumours) and ocular causes (e.g.
hypotony, choroidal tumours, posterior scleritis, chronic papilloedema
and scleral buckling).

49. a. No. The defects involve the temporal field of each eye; by definition,
a bitemporal hemianopia.
b. Chiasmal compression by a space-occupying lesion such as a pituitary
adenoma needs to be excluded urgently. ‘Tilted’ optic discs, relatively
common in myopes, can also give a similar field pattern, although the
defects typically cross the midline.
c. MRI of the brain and orbits, to include the optic chiasm.

50. a. The hands show features typical of rheumatoid arthritis.

b. She almost certainly has dry eyes: keratoconjunctivitis sicca, i.e.
secondary Sjögren’s syndrome.
c. Slit-lamp assessment of the tear film may show debris, froth, an
irregular or thin marginal meniscus and a short break-up time. Other
tests include staining the cornea and conjunctiva with rose Bengal
and fluorescein, and the Schirmer test.
d. Keratitis (which can be severe, with melting and perforation), and
scleritis. Acquired Brown’s syndrome is very rare.

51. a. A right lower motor neurone facial nerve palsy.

b. Exposure keratopathy secondary to poor eyelid closure; cosmetic
154 implications may also be substantial.
 c. Topical lubricants may suffice in mild cases. Botulinum-induced ptosis
is useful in recovering palsies but surgery will be necessary for
profound permanent deficit (e.g. tarsorrhaphy, lid shortening and
nerve grafting).

52. a. Probably not, as the optic discs appear normal.

b. Approximately 10% at 5 years.
c. If the disc is unusually small, the physiological cup size might be
considerably smaller than that shown.

A
d. Visual fields, to exclude glaucomatous changes and act as a baseline

n
for monitoring purposes; pachymetry for central corneal thickness, as

s
w
the measured intraocular pressure is higher than the true level in eyes

e
r
with thick corneas; and baseline optic disc imaging, preferably with

s
computed parameter analysis.

53. a. Corneal calcium deposition in an interpalpebral distribution (band

keratopathy).
b. Some children with JIA develop a chronic anterior uveitis, which can
lead to secondary glaucoma, band keratopathy and cataract. The eye
typically remains white and comfortable despite active inflammation
so that the diagnosis may be overlooked.
c. Early-onset pauciarticular disease, the presence of serum antinuclear
antibodies, and HLA-DR5 positivity.

54. a. Contact lens-related keratitis, which clinically appears to be

bacterial.
b. Corneal scraping for microscopy, culture and antibiotic sensitivity
testing, followed by intensive topical broad-spectrum antibiotics.
Admission to hospital is usually advisable. A mydriatic may improve
comfort and prevent posterior synechiae formation. Oral antibiotics
are sometimes used for ulcers near the limbus.
c. Acanthamoeba, fungi, atypical mycobacteria and herpes simplex.

55. a. Right internuclear ophthalmoplegia (INO).

b. The right medial longitudinal fasciculus (MLF); the side of the lesion
in the MLF corresponds to the eye that fails to adduct (the right eye
in 55a).
c. Multiple sclerosis.
d. Stroke, tumours, trauma, encephalitis, drugs and hydrocephalus.

56. a. Acute retinal necrosis; in this age group, varicella is the likely cause.
b. Polymerase chain reaction (PCR) analysis of aqueous and vitreous for
viral DNA.
c. Intravenous aciclovir for 10 days then orally for 3 months; systemic
steroids 24 hours after commencement of antiviral therapy.

57. a. Rubeosis iridis, which develops in about 50% of ischaemic CRVOs and
often results in neovascular glaucoma. 155
 b. Diabetes, central retinal artery occlusion, carotid obstructive disease
and chronic intraocular inflammation.
c. Urgent panretinal photocoagulation. If this fails to induce regression
prior to irreversible pressure elevation, cyclodiode laser or drainage
surgery can be carried out. The visual prognosis is very poor.

58. a. The cornea is cloudy the anterior chamber shallow and the pupil
dilated. The diagnosis is acute angle-closure glaucoma, in which the
abdominal symptoms may occasionally be so severe as to constitute
s
the main focus. Intraocular pressure measurement will confirm the
r
e
diagnosis.
w
b. Admission to hospital, intravenous acetazolamide and topical agents to
s
n
A
lower the intraocular pressure, followed by laser iridotomy to both eyes.
c. Acute iritis, corneal abrasion, keratitis and scleritis.

59. a. Capillary haemangioma or ‘strawberry naevus’.

b. Yes, because the lesion may induce amblyopia as a result of
associated ptosis or strabismus.
c. Intralesional steroid injection. However, most strawberry naevi
constitute only a cosmetic problem and can be left alone in
anticipation of spontaneous resolution by the age of 7 years.
d. Very rarely, capillary haemangioma is part of a wider syndrome –
Maffucci’s (multiple systemic haemangiomata and bone lesions) or
Kasabach–Merritt (haematological abnormalities).

60. a. Levator palpebrae superioris and Müller’s muscle; the former is

supplied by the superior division of the oculomotor nerve and the
latter by the sympathetic component of the autonomic nervous
system.
b. • Neurogenic: third nerve palsy, Horner’s syndrome, Marcus Gunn’s
(‘jaw-winking’) syndrome.
• Aponeurotic: involutional (age-related), postoperative.
• Mechanical: oedema, tumours, heavy redundant skin, cicatricial.
• Myogenic: simple congenital, blepharophimosis syndrome,
myasthenia gravis, ocular myopathy, myotonic dystrophy.
c. • Proptosis of the contralateral eye
• Eyelid: redundant skin overhanging lid margin (dermatochalasis)
• Globe: microphthalmos, phthisis
• Eye muscles: vertical muscle imbalance
• Orbit: enophthalmos.

61. a. A limbal dermoid.

b. A choristoma, a congenital growth of normal tissue at an abnormal
location.
c. For visual interference, irritation, or cosmesis.
d. Limbal dermoids are usually an isolated abnormality but may on
occasion be part of Goldenhar’s syndrome, Treacher Collins’ syndrome
156 or the naevus sebaceous syndrome of Jadassohn.
62. a. Failure of elevation of the left eye in adduction, consistent with a left
Brown’s syndrome.
b. As with all squints, refraction, media and fundus examination and
treatment of amblyopia are important. Surgery is needed only if there
is significant deviation in the primary position, diplopia or an
anomalous head posture.
c. Inflammation (e.g. rheumatoid arthritis), trauma and superior oblique
surgery.

A
63.

n
a. Giant cell arteritis should be suspected and enquiry made concerning

s
headache, scalp tenderness, jaw claudication, muscle girdle

w
e
(particularly neck) aching, malaise, loss of weight and specifically

r
s
previous diagnosis of polymyalgia rheumatica.
b. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP);
however, the diagnosis is not excluded by a low result nor
categorically confirmed by a high one.
c. Temporal artery biopsy. Systemic (usually intravenous) steroids must
be commenced immediately in this situation and should not be
delayed while biopsy is awaited.

64. a. Kearns–Sayre syndrome, which is characterized by pigmentary

retinopathy and chronic progressive external ophthalmoplegia. The
child may also have ataxia, deafness, diabetes and other systemic
abnormalities.
b. Mitochondrial DNA deletions.
c. ECG shows cardiac conduction defects, CSF is abnormal and muscle
biopsy shows the ‘ragged red fibre’ pattern.

65. a. Iridoschisis, a very rare gradual splitting of the iris into anterior and
posterior lamellae with disintegration of the anterior layer.
b. IOP elevation. It is postulated that episodic angle-closure glaucoma
associated with very high IOP leads to the iris changes, rather than
the iris changes precipitating the initiating event.
c. Laser peripheral iridotomy, followed by medical treatment as
appropriate.

66. a. Stenosis of the lower eyelid punctum. There is also a degree of medial
ectropion resulting in the punctum pointing away from the lacus
lacrimalis.
b. Further assessment falls into two categories: exclusion of any cause
of ocular irritation, particularly ‘paradoxical’ lacrimation due to dry
eye, and obstruction elsewhere in the lacrimal drainage system. Basic
assessment of the lacrimal canaliculi and nasolacrimal duct involves
syringing with saline.
c. Simple dilation of the punctum or incisional (‘one-’ or ‘two-snip’)
enlargement of the canaliculus, combined if necessary with ectropion
repair. 157
[Link]
 67. a. The right cornea is larger than the left, although the left also appears
enlarged. The most likely diagnosis is primary congenital glaucoma,
although other causes of large corneas, large eyes and secondary
infantile glaucoma must be excluded.
b. Breaks in Descemet’s membrane (‘Haab striae’ when healed), an
abnormal anterior chamber angle and optic disc cupping.
c. Examination under general anaesthesia will be necessary to facilitate
gonioscopy, measurement of intraocular pressure and corneal
diameter.
s
r
e
68.
w
a. Marginal keratitis.
s
b. It is not always possible to be certain but the overall clinical picture
n
A
should be considered. Important points include: the relatively mild
symptoms, the absence of predisposing factors such as contact lens
wear, the distribution of the infiltrate in a band parallel to the limbus
but separated by a clear zone. Typically the infiltrate is much larger
than any associated epithelial defect. Chronic anterior blepharitis is
often present.
c. A short course of weak topical steroid (e.g. fluorometholone).

69. a. A hyphaema, a common complication of moderate to severe blunt

trauma; blood in the anterior chamber which has settled to form a
‘fluid level’.
b. The major risk is of a further bleed with resultant elevation of
intraocular pressure, which can potentially lead to permanent corneal
bloodstaining. Possible damage to anterior and posterior segment
structures must also be assessed.
c. Activity is severely restricted, sometimes with hospital admission. The
patient is initially reviewed daily. Other measures may include
immobilization of the pupil with atropine, administration of an oral
antifibrinolytic agent and treatment of anterior uveitis or raised
pressure.

70. a. A corneal abrasion.

b. Typically, the symptoms of an abrasion are severe: marked grittiness,
redness, lacrimation and photophobia. Spontaneous recurrence of the
epithelial defect is common (recurrent erosion syndrome).
c. Antibiotic ointment, cycloplegia to reduce discomfort, and oral
analgesia; firm padding of the eye may improve comfort in a large
abrasion but probably does not speed resolution. A bandage contact
lens is sometimes used.

71. a. Isolated left third (oculomotor) cranial nerve palsy.

b. A compressive lesion, particularly posterior communicating artery
aneurysm.
c. Clinical features increasing the level of suspicion of a compressive
158 lesion include pupillary involvement, headache or ocular pain,
 younger patient age and absence of known vascular risk factors such
as diabetes. A careful history and examination for additional
neurological features is essential, followed by CNS imaging if
appropriate. There is a wide range of causes of third nerve palsy; 25%
are idiopathic.

72. a. Proliferative vitreoretinopathy (PVR).

b. Vitreous haze and condensation; pigment cells on the inferior retina;
rolling of the edges of retinal breaks; wrinkling of the inner retinal

A
surface; full-thickness retinal folds; subretinal strands.

n
c. Pars plana vitrectomy, if necessary using silicone oil tamponade and

s
w
peeling, segmentation or delamination of epiretinal membranes.

e
r
s
73. a. Vitreous haemorrhage.
b. B-scan ultrasonography, principally to exclude retinal detachment.
c. Examples include proliferative retinopathies (diabetic, retinal vein
occlusion, Eales’s disease, retinopathy of prematurity, sickle-cell
disease), posterior vitreous detachment and retinal tears,
‘breakthrough’ bleeding from choroidal neovascularization, and
trauma.

74. a. A macular epiretinal membrane. A dense membrane is usually referred

to as ‘macular pucker’ and a thinner membrane as in ‘cellophane
maculopathy’.
b. Membranes consist of retinal glial cells proliferating at the
vitreoretinal interface, having reached the retinal surface via breaks
in the internal limiting membrane.
c. Retinal surgery and photocoagulation, trauma, inflammation and
retinal vascular disease.

75. a. Infusion cannula (lower right), light pipe and vitreous cutter.
b. • Rhegmatogenous retinal detachment associated with the
following: significant PVR, media opacity precluding adequate
retinal view (vitreous haemorrhage, posterior lens capsular
opacity), giant retinal tears and posterior breaks including macular
holes.
• Tractional retinal detachment, usually diabetic or traumatic.
• Persistent vitreous haemorrhage.
• Removal of intraocular foreign body.
• Severe endophthalmitis.
• Age-related macular hole.
• Macular degeneration: excision of choroidal neovascular
membranes, macular translocation.

76. a. With recurrent erosions at the end of the first decade.

b. Irregular bands of collagen, staining blue with Masson trichrome,
replace the Bowman layer which may even be absent.
c. Autosomal dominant; the gene is located at 10q24. 159
 d. Reis–Bücklers dystrophy (Bowman layer type 1), in which the
honeycomb pattern assumed by the opacities is considerably less
prominent.

77. a. Normal right abduction but limited left abduction associated with
retraction of the globe and narrowing of the palpebral fissure. The
diagnosis is left Duane’s retraction syndrome.
b. This patient can be classified as Huber type 1:
• Huber type 1: limited abduction
s
• Huber type 2: limited adduction
r
e
w
• Huber type 3: limited abduction and adduction.
s
c. Anisometropia, coloboma, microphthalmos and heterochromia.
n
A
78. a. The disc is enlarged and is surrounded by a hypopigmented ring. The
emerging blood vessels manifest a radial pattern. The excavation is
funnel-shaped and contains a central core of whitish glial tissue. The
diagnosis is morning glory anomaly.
b. Approximately 30%.
c. These are uncommon: frontonasal dysplasia, which includes facial and
midline neurological abnormalities, and neurofibromatosis type 2.

79. a. Computed corneal topographic imaging: ‘corneal topography’ or

mapping.
b. The contour of the cornea at differing locations; colours at the red
end of the scale are used to represent steep corneal regions, with the
flattest areas shown as blue.
c. The contours demonstrated are consistent with keratoconus. In the
right eye the cone is located inferiorly with dioptric values at the
apex reaching 56 D; in the left the cone is central with values up to
63 D.

80. a. Myasthenia gravis.

b. Fatigue on sustained upgaze with worsening of ptosis. Cogan’s lid
twitch – overshoot on moving from depression to primary position
in myasthenia. Ice test – reduced ptosis after ice pack applied for
2 minutes.
c. Acetylcholine receptor antibody levels.
d. Edrophonium (an anticholinesterase) testing: significant temporary
improvement in ptosis and/or diplopia following intravenous
administration.

81. a. A keratoprosthesis or artificial corneal implant.

b. When the cornea is severely damaged but the retina and optic nerve
retain good function and conventional corneal grafting has an
extremely poor prognosis.
c. Endophthalmitis, glaucoma, retroprosthesis membrane formation and
160 extrusion.
82. a. Right cataract and heterochromia iridis. These findings, together with
the stellate keratic precipitates, suggest Fuchs’ uveitis syndrome.
b. Glaucoma, and occasionally severe vitreous opacities.
c. Topical steroids are not beneficial and may promote glaucoma and
cataract. Severe vitreous opacities may benefit from periocular steroid
injection.

83. a. Significant posterior capsular opacification.

b. Glare.

A
c. Nd:YAG laser capsulotomy.

n
s
d. Retinal detachment, cystoid macular oedema, lens dislocation,

w
e
intraocular pressure elevation (usually transient) and visually

r
s
inconsequential pitting of the implant.

84. a. A lower lid entropion.

b. Age-related horizontal lid laxity, weakening of the effect of the lower
lid retractors and over-riding of the pretarsal by the preseptal
orbicularis oculi with associated prolapse of orbital fat.
c. Surgical options depend on the presence or absence of horizontal lid
laxity and include simple everting sutures, the horizontal lid-splitting
Wies procedure, inferior retractor tightening (Jones procedure) and
horizontal lid shortening.

85. a. Numerous large drusen.

b. Fundus fluorescein angiography would demonstrate CNV and aid in
deciding whether treatment is indicated and what form this should
take.
c. A specific antioxidant vitamin and mineral supplement regimen
reduces the risk of CNV in patients fitting certain criteria, including
the situation described.

86. a. Indocyanine green angiography (ICG); the wavelengths used penetrate

blood and pigment more effectively than fluorescein angiography and
ICG is therefore superior for investigating retinal pathology in some
circumstances.
b. This is a late phase ICG of a pigment epithelial detachment (PED).
c. A thickened degenerate Bruch membrane may impede the transit of
fluid from the retinal pigment epithelium (RPE) to the choroid.
d. There is no treatment for PEDs; laser photocoagulation may cause
tearing of the RPE. Spontaneous resolution sometimes occurs.

87. a. Several large haemorrhages with white centres (Roth spots) which
usually represent fibrin emboli occluding ruptured blood vessels.
b. This patient requires urgent investigation. Other causes include
anaemia, bacterial endocarditis, diabetes and dysproteinaemia.
c. Other fundus changes include cotton wool spots, haemorrhages,
peripheral retinal neovascularization, a ‘leopard spot’ fundus and 161
 infiltration of the optic nerve head. Elsewhere in the eye,
subconjunctival haemorrhage, orbital involvement, iritis,
pseudohypopyon and hyphaema may occur.

88. a. Given the marked iris transillumination and the extremely pale skin
and hair, tyrosinase-negative.
b. Patients with tyrosinase-negative oculocutaneous albinism have a
decreased number of crossing nerve fibres at the optic chiasm, as well
as other central visual pathway anomalies.
s
c. Chediak–Higashi syndrome (white-cell-related immunodeficiency) and
r
e
Hermansky–Pudlak syndrome (a lysosomal storage disease with
w
platelet dysfunction).
s
n
A
d. No, because only tyrosinase-positive albinism is associated with these
syndromes.

89. a. An area of chorioretinal atrophy associated with retinal pigment

epithelial hyperplasia. The most common cause of this appearance is
old congenital toxoplasmosis.
b. Usually in the teens to thirties. In the immunocompromised, however,
reactivation of congenitally acquired infection or acquisition of new
infection commonly occurs at any age.
c. Serology for toxoplasma antibodies but the titre, however, does not
correlate with activity. Polymerase chain reaction (PCR) DNA testing
of a vitreous biopsy sample may be diagnostic.

90. a. A fluorescein angiogram showing the petalloid hyperfluorescent

pattern characteristic of cystoid macular oedema.
b. The prolapse of vitreous gel into the anterior segment from its
physiological location in the posterior segment, usually by inadvertent
rupture of the posterior capsule or zonules.
c. Increased risk of cystoid macular oedema, retinal detachment,
endophthalmitis, uveitis, glaucoma, corneal decompensation and lens
implant dislocation.

91. a. A silicone explant, placed during the surgery, has eroded through the
conjunctiva.
b. At this stage, it should be safe to remove the explant without
precipitating re-detachment. Removal in the first few months,
however, carries a 5–10% risk.
c. Under sterile conditions: if very loose, the explant can simply be
lifted gently out of its position and any visible sutures trimmed; if
firmly attached, conjunctival dissection and suture release is
necessary.

92. a. Large (‘mutton fat’) keratic precipitates characteristic of

granulomatous uveitis.
b. Yes. Granulomatous uveitis should always be investigated, except in
162 the presence of a previously established systemic illness.
[Link]
 c. Where appropriate, initial screening might include a full blood count,
ESR, serum angiotensin-converting enzyme, syphilis serology and
chest X-ray (e.g. tuberculosis, sarcoidosis).

93. a. Osteogenesis imperfecta. Types 1 and 2 are associated with blue

sclera.
b. The blue appearance is caused by a collagen abnormality leading to
relative scleral transparency.
c. The more important include Ehlers–Danlos syndrome (usually type 6,

A
although blue sclera has been reported in other types),

n
pseudoxanthoma elasticum (dominant type 2), and Turner’s

s
w
syndrome.

e
r
s
94. a. Extensive chorioretinal atrophy, a form of myopic maculopathy.
b. Other macular changes include choroidal neovascularization (CNV),
haemorrhage without CNV, lacquer cracks (which predispose to CNV),
macular hole and pigment proliferation (Fuchs spot). Highly myopic
eyes have a predisposition to retinal detachment, cataract and
glaucoma.
c. Nearer 30 mm.

95. a. Laser in-situ keratomileusis (LASIK), in which a hinged flap as shown

is cut from the corneal surface, excimer laser ablation is performed
on the exposed stroma and the flap is replaced.
b. Yes, but only to a limited extent: approximately 4 D of hypermetropia
and 5 D of astigmatism.
c. Operative buttonholing of the flap and corneal perforation;
postoperative infection, sterile diffuse lamellar keratitis (DLK or ‘sands
of the Sahara’), flap dislocation or wrinkling, persistent epithelial
defects and epithelial ingrowth under the flap.

96. a. Episcleritis (strictly simple sectorial episcleritis, the commonest type).

b. Simple or nodular; the former may be sectorial or diffuse.
c. In contrast to scleritis, systemic investigation is not appropriate
because the prevalence of an associated systemic disease is extremely
low.
d. Mild cases are self-limiting and require no treatment. Topical steroids
or oral non-steroidal anti-inflammatory drugs are sometimes needed
in severe or persistent cases.

97. a. A spontaneous subconjunctival haemorrhage (SCH).

b. Mild discomfort.
c. Blood pressure should be checked, because spontaneous SCH is twice
as common in patients with hypertension as those without. No
further investigation is necessary, even if bleeding should recur at the
same site. If both eyes are involved or there is other cause for
suspicion of a bleeding diathesis, further assessment might be
considered. 163
 98. a. A localized detachment of the neurosensory retina at the macula –
central serous retinopathy.
b. Approximately 6/12, characteristically improved by a weak ‘plus’
(convex) lens.
c. Fundus fluorescein angiography will usually demonstrate an ‘ink-blot’
or ‘smoke-stack’ pattern of increasing hyperfluorescence.

99. a. Intermittent exotropia.

b. Basic exotropia, in which the angle is the same for near and
s
distance; convergence weakness, where the deviation is greater for
r
e
w
near; divergence excess, with a greater deviation for distance.
s
c. As with all squints, media and fundus examination followed by
n
A
refraction is the initial step. Spectacle correction of myopia may
stimulate accommodation such that the squint is controlled.
Orthoptic treatment is sometimes effective but surgery is frequently
necessary.

100. a. Defective visual acuity persisting after correction of refractive error

and removal of visual pathway pathology, if present.
b. Amblyopia commonly occurs in association with strabismus, when the
image from the deviating eye is suppressed by the brain during the
critical developmental period. It can also be caused by anisometropia
(difference in refraction between the eyes), stimulus deprivation
(cataract) and ametropia (bilateral high refractive error).
c. This varies according to parent and child motivation and other
factors, but is probably around 7–8 years in strabismic amblyopia and
up to 12 years for anisometropic.

101. a. A Kayser–Fleischer ring.

b. Deposition of copper granules in the peripheral region of Descemet’s
membrane.
c. Wilson’s disease, an abnormality of copper metabolism caused by a
deficiency of caeruloplasmin.
d. Anterior capsular ‘sunflower’ cataract.
e. Liver disease and basal ganglia dysfunction

102. a. The cornea shows a branching, whorled pattern with its centre
located inferior to the pupil, consisting of very fine deposits within
the epithelium: vortex keratopathy or cornea verticillata.
b. Amiodarone.
c. Virtually all patients receiving amiodarone develop corneal changes.
d. Other drugs include chloroquine and hydroxychloroquine. Fabry’s
disease, a glycolipidosis, is also associated with vortex keratopathy.

103. a. No. Keratoconus is extremely unlikely to present at this age.

b. Involvement specifically of the peripheral rather than central cornea
leads the ophthalmologist towards a number of defined conditions
164 having a predilection for this site.
 c. Taking account of the patient’s gender, absence of known systemic
disease, absence of inflammation, involvement of both eyes and the
appearance of the cornea, this is likely to be Terrien’s marginal
degeneration. This condition is an uncommon idiopathic painless
thinning of the peripheral cornea; the main visual effect is
astigmatism.

104. a. Axial proptosis.

b. Cavernous haemangioma. 70% of these occur in women and the

A
majority are located within the muscle cone.

n
s
c. Optic disc swelling or pallor, choroidal folds and ocular motility

w
disturbance.

e
r
d. Surgical excision; the tumour is usually well encapsulated.

s
105. a. B-scan ultrasonography, which produces a two-dimensional section
of the tissue scanned. A-scan ultrasonography produces a one-
dimensional scan and is principally used in biometric calculation of
intraocular lens implant power.
b. A hyperdense signal is evident at the optic nerve head, consistent with
optic disc drusen, a cause of pseudopapilloedema.
c. Fluorescein angiography will demonstrate autofluorescence of
superficial disc drusen and absence of leakage that would occur in
true disc swelling. CT scanning will show any calcification of the
drusen.

106. a. Progressive iris atrophy, a variant of iridocorneal endothelial (ICE)

syndrome, characterized by a proliferation of abnormal corneal
endothelial cells across the anterior chamber angle and iris.
b. Iris naevus (Cogan–Reese) syndrome, characterized by a diffuse iris
naevus with associated iris atrophy in about 50% of cases, and
Chandler’s syndrome, in which corneal changes predominate.
c. Approximately 50%.

107. a. A displaced pupil (corectopia), full-thickness iris defects

(pseudopolycoria) and cataract.
b. Rieger’s syndrome, a variant of the Axenfeld–Rieger syndrome.
c. Inheritance is autosomal dominant; genes implicated include FOXC1
on chromosome 6p25, and PITX2 on chromosome 4q25.
d. About 50%, usually during childhood or early adulthood.

108. a. A large hypopyon.

b. Bacterial endophthalmitis.
c. Initially, vitreous biopsy and intravitreal antibiotics. The benefit of
topical, periocular injection and systemic antibiotics is not clear, but
are often used. Systemic steroids are often given to reduce
inflammation. Vitrectomy is of benefit only if vision is reduced to
‘light perception’. 165
 109. a. A Krukenberg spindle.
b. Pigment dispersion syndrome.
c. Most patients with this condition are myopic.
d. Up to 50% of patients may eventually develop glaucoma.

110. a. Thickening, reddening and telangiectasia of the lid margin skin, with
crusting and scaling around the lashes, particularly at the bases.
b. This patient has anterior blepharitis, predominantly of the
staphylococcal type.
s
r
c. An approximate ascending hierarchy of treatment: rigorous daily lid
e
w
hygiene regimen, topical lubricants, lid margin antibiotic ointment,
s
intermittent systemic antibiotics (e.g. tetracycline, doxycycline) and
n
A
weak topical steroids.

111. a. A corneoscleral penetrating injury with a flat anterior chamber.

b. Ideally, CT scanning should be carried out to exclude an intraocular or
intraorbital foreign body. Primary wound repair should be undertaken
within a few hours with the intention of restoring the integrity of the
eye wall and preventing infection; a careful search for occult wounds
should be carried out as part of the procedure. Antibiotics are
administered as appropriate. It should be remembered that systemic
stabilization and management of any life-threatening injuries has
priority.

112. a. Nd:YAG (neodymium: yttrium–aluminium–garnet) iridotomy.

b. A sudden and severe spike in intraocular pressure sufficient to cause
corneal oedema can be caused by intermittent subacute primary
angle-closure (including plateau iris syndrome), Posner–Schlossmann
syndrome, pigment dispersion syndrome, herpetic keratouveitis and
occasionally pseudoexfoliative glaucoma.
c. Capsulotomy, disruption of vitreous strands in the anterior chamber
and disruption of the posterior hyaloid face to disperse premacular
blood.

113. a. Lattice dystrophy type 3 or 3a; the former is probably of autosomal

recessive inheritance and the latter autosomal dominant.
b. No.
c. This is necessary in most cases to improve vision.
d. Type 1 (Biber–Haab–Dimmer), which presents in the first decade with
recurrent erosions, and Type 2 (Meretoja’s syndrome), a systemic
amyloidosis that presents in middle age with facial palsy and corneal
involvement.

114. a. Retinopathy of prematurity.

b. Stage 1: Demarcation line at the border of vascularized and avascular
retina
166 Stage 2: Ridge at the same site
 Stage 3: Extraretinal fibrovascular proliferation (shown in the
illustration)
Stage 4: Subtotal retinal detachment
Stage 5: Total retinal detachment
Plus disease: Posterior pole vascular engorgement, vitreous haze, poor
mydriasis.
c. Threshold disease, consisting of five contiguous or eight non-
contiguous clock-hours of stage 3 together with ‘plus’ disease.
Surgery may be carried out for stage 4 or 5.

A
d. Laser or cryotherapy to the avascular area.

n
s
w
115. a. Measurement of the blood pressure because the signs are highly

e
r
suggestive of severe hypertensive retinopathy.

s
b. Because the deposits are located in the retinal layer of Henle and
follow the fibre arrangement accordingly.
c. Exudative retinal detachment (particularly in toxaemia of pregnancy),
Elschnig spots (focal choroidal infarcts), and Siegrist streaks, (flecks
along choroidal vessels indicating fibrinoid necrosis).

116. a. Lisch nodules.

b. Neurofibromatosis-1.
c. Eyelid neurofibroma, glaucoma, optic nerve glioma, ectropion uveae,
prominent corneal nerves, choroidal naevi and melanomas, retinal
astrocytomas, and spheno-orbital encephalocele.
d. Autosomal dominant with irregular penetrance and variable
expressivity; the gene locus is on chromosome 17q11.

117. a. A printout of frequency-doubling perimetry (commonly referred to as

frequency-doubling technology, FDT).
b. FDT specifically targets the magnocellular ganglion cells, which are
damaged early on in the course of glaucoma. It does this by utilizing
the illusion of frequency doubling produced by a low spatial
frequency grating undergoing high temporal frequency reversal.
c. It is much quicker and easier to perform than conventional
automated perimetry.

118. a. Salzmann’s nodular degeneration.

b. Trachoma is the likely cause in this patient.
c. Often, no treatment is required. If the eyes are irritable, topical
lubricants can be used. Superficial keratectomy or lamellar
keratoplasty may be necessary in severe cases.

119. a. A chalazion, which is caused by a granulomatous inflammatory

reaction to accumulated secretions in a blocked meibomian gland.
b. A conjunctival granuloma.
c. Chalazia may resolve spontaneously. Persistent lesions are usually
treated by curettage and, rarely, by local steroid injection. Topical or 167
[Link]
 systemic antibiotics may be required for an acutely inflamed or
infected lesion.

120. a. Myelinated nerve fibres.

b. An enlarged blind spot may be present with a peripapillary lesion, and
peripheral lesions may give corresponding field defects. Myopia and
amblyopia may be associated, and symptomatic macular involvement
has been reported. Rarely, associated retinal vascular abnormalities
can be present.
s
c. Myelinated fibres may represent a choristoma resulting from ectopic
r
e
oligodendrocytes.
w
d. About 1%.
s
n
A
168
 Index

Page numbers in italics refer to questions Basal cell papilloma, 6, 7

and corresponding answes Behçet’s disease, 46, 47, 103, 150
Best’s disease, 74, 75
Biber–Haab–Dimmer degeneration, 141, 166

I
A Bielschowsky test, 88, 89

n
d
Birdshot retinochoroidopathy, 48, 49, 109,

e
Abducens nerve palsy, 88. 89 153

x
Abrasion, corneal, 90, 91, 122, 158 Blepharitis, 2–3, 140, 166
Acanthamoeba keratitis, 28, 29 Blepharophimosis syndrome, 10, 11
Acetazolamide, 156 Blow-out fracture, 18, 19, 100, 148
Acquired maculopathies, 72–73 Blue sclera, 131, 163
Actinic keratosis, 8, 9 Blunt trauma, 90, 91, 121, 158
Acute allergic conjunctivitis, 20, 21 Bournville’s disease, 80, 98, 148
Acute multifocal posterior placoid pigment Bowman layer dystrophies, 34, 35, 124,
epitheliopathy (AMPPPE), 48, 49, 159–160
99, 148 Brown’s syndrome, 86, 87, 112:154,
Adenoma sebaceum, 98, 148 118:157
Adenoviral conjunctivitis, 20, 21 Bull’s eye maculopathy, 72, 73
Age-related macular degeneration, 70–71, Buphthalmos, 58, 59
106:151, 128:161
AIDS, molluscum contagiosum in, 4, 102,
149 C
Albinism, 129, 162
Amaurosis fugax, 66 Candidal keratitis, 28
Amblyopia, 86, 135, 164 ‘Candlewax drippings’, 46, 47
Amiodarone, corneal deposits in, 136, 164 Capillary haemangioma
Angioid streaks, 72, 73, 100, 148–149 eyelid, 6, 7, 117, 156
Angle-recession glaucoma, 52, 53 orbit, 16, 17
Aniridia, 58, 59, 92, 145 retina, 80, 81, 94, 145–146
Ankyloblepharon, 26, 153 Capsular opacification, 60, 61
Antioxidants, 70, 128, 161 Carotid-cavernous fistula, 18, 19
Antiviral agents, 30, 32, 105:151, 115:155 Cataract
Artificial corneal implant age-related, 60–61, 103, 150
(keratoprosthesis), 126, 160 infantile, 62, 63
Astigmatism secondary, 62
correction, 132, 163 Cataract surgery
Astrocytoma, retinal, 80, 81, 98, 148 complications, 127:161, 130:162,
Atopic keratoconjunctivitis, 22 139:165
Axenfeld–Rieger syndrome, 58, 59, 138, 165 methods, 60, 61
Cavernous haemangioma
orbit, 16, 137, 165
B retina, 80, 81
Cellulitis, orbital, 18, 19, 97, 147
Bacterial conjunctivitis, 20, 21 Central serous retinopathy, 134, 164
Bacterial endophthalmitis, 60, 139, 165 Chalazion (meibomian cyst), 4, 5, 144,
Bacterial keratitis, 28, 29 167–168
Band keratopathy, 42, 43, 114, 155 Chandler’s syndrome, 138, 165 169
Basal cell carcinoma, 8, 9, 92, 145 Chediak–Higashi syndrome, 129, 162
 Cherry-red spot, 66, 67, 107, 151 size and shape disorders, 38–39
Chlamydial conjunctivitis, 20, 21, 22 suppurative keratitis, 28–29, 114, 155
Choristoma, conjunctival, 24, 25, 118, 156 topographic mapping, 125, 160
Choroid ulceration, 36–37
detachment, 95, 146 verticillata, 136, 164
dystrophies, 74, 75 Cotton-wool spots, 64, 65, 66, 69
folds, 112, 154 ‘Cupid’s bow’ (pseudogerontoxon), 22, 23
haemangioma, 78, 79 Cyst of Moll, 4, 5
melanoma, 78, 79, 98, 147–148 Cystoid macular oedema, 130, 162
metastatic carcinoma, 78, 79 Cyst of Zeis, 4, 5
x
naevus, 78, 79, 98 Cytomegalovirus retinitis, 44, 45
e
neovascularization (CNV), 70, 71,
d
n
106:151, 128:161
I
osteoma, 78, 79 D
rupture, 90, 91
Choroideremia, 74, 75 Dacryocystorhinostomy, 95, 146
Choroidopathy, serpiginous, 48, 49 Dellen, corneal, 36, 37
Cicatricial pemphigoid, 26, 27, 110, 153 Dermoid, limbal, 24, 25, 118, 156
Cicatrizing conjunctivitis, 26, 27 Dermoid cyst, 16, 17
Clinically significant macular oedema Diabetic maculopathy, 64, 65
(CSMO), 64, 98, 147 Diabetic retinopathy, 64–65, 97:147,
Cogan microcystic dystrophy, 34, 35 98:147
Cogan–Reese syndrome, 138, 165 Disciform keratitis, 30, 31
Cogan’s lid twitch, 126, 160 Distichiasis, 12
Collier’s sign, 149 Drusen
Coloboma macular, 70, 71, 128, 161
lens, 62, 63 optic disc, 84, 85, 137, 165
optic disc, 84, 85 Dry eye, 26, 27
Commotio retinae, 90, 91 Duane’s syndrome, 86, 87, 124, 160
Computed corneal topographic imaging,
125, 160
Congenital hypertrophy of the retinal E
pigment epithelium (CHRPE), 107,
152 Ectopia lentis, 62, 63
Congenital syphilis, 108, 152 Ectropion, 12, 13, 120, 157
Conjunctival and corneal intra-epithelial Eczema of the eyelids, 3
neoplasia (CCIN), 24, 25 Edrophonium test, 126, 160
Conjunctival tumours, 24–25, 96:146, Electroretinography, 153
118:156–157 Elschnig spots, 167
Conjunctivitis Entropion, 12, 13, 128, 161
acute, 20–21, 106, 151 Episcleritis, 40, 41, 133, 163
chronic, 22–23, 93, 145 Esotropia, 86, 87, 104, 150
cicatrizing, 26, 27 Exotropia, 86, 87, 134, 164
Cornea External hordeolum (stye), 4, 5
abrasion, 90, 91, 122, 158 Exudative retinal detachment, 76, 77
dystrophies, 34–35, 124, 159–160 Eyelid
epithelial basement membrane benign tumours, 6–7, 117, 156
dystrophy, 34, 35 ectropion, 12, 13, 120, 157
foreign body trauma, 90, 91 entropion, 12, 13, 128, 161
graft, 34, 35, 111, 154 non-neoplastic lumps, 4–5, 102:149,
herpes simplex infection, 30–31, 105, 144:168
151 premalignant and malignant tumours,
170 implant, 126, 160 8–9, 92, 145
pannus, 22, 23 see also Blepharitis; Ptosis
 in hypertensive retinopathy, 68, 69
F retinal, in diabetic retinopathy, 64, 65
in retinal vein occlusion, 66, 67
Fabry’s disease, 136, 164 spontaneous subconjunctival, 133, 163
Foreign body trauma, 90, 91 vitreous, 123, 159
Fourth nerve palsy, 88, 89 Hay fever conjunctivitis, 20
Frequency-doubling technology (FDT), 143, Hering’s law, 151
167 Hermansky–Pudlak syndrome, 129, 162
Fuchs’ endothelial dystrophy, 34, 35 Herpes simplex infection, 20, 30–31, 105,
Fuchs’ spot, 72, 73 151
Fuchs’ uveitis syndrome, 42, 127, 161

I
Herpes zoster ophthalmicus, 32–33,

n
Fundal dystrophies, 74–75, 107, 152

d
102:149, 115:155

e
Fundus flavimaculatus, 74, 75 Hess chart, 106, 151

x
Fungal keratitis, 28, 29 Histoplasmosis, 44, 45
Horner’s syndrome, 10, 11, 107, 152
Hutchinson’s sign, 32, 102, 149
G Hypermetropia correction, 132, 163
Hypertensive retinopathy, 68, 69, 142, 167
Giant cell arteritis, 82, 119, 157 Hyphaema, 90, 91, 121, 158
Giant papillary conjunctivitis, 22, 23, 93, 145
Gillespie’s syndrome, 58, 145
Glaucoma I
angle-closure
primary, 54–55, 116, 156 Ice test, 126, 160
secondary, 56–57, 138, 165 Idiopathic intracranial hypertension, 111,
congenital, 58, 59, 120, 158 153
developmental, 58–59, 120, 158 Idiopathic orbital inflammation
drainage device, 56, 57, 99, 148 (pseudotumour), 18, 19
neovascular, 56 Indocyanine green angiography, 128, 161
normal-tension, 50, 103:149-150, Infantile cataract, 62, 63
112:154 Infective keratitis, 28–29, 114, 155
open-angle Internal hordeolum (acute chalazion), 4, 5
pigmentary, 52, 53 Internuclear ophthalmoplegia, 115, 155
primary, 50–51, 93, 145 Intraocular lens implant (IOL), 60, 61
pseudoexfoliation, 52, 53, 94, 146 Iridocorneal dysgenesis, 58, 59, 92, 145
secondary, 52–53, 94:146, 139:166 Iridocorneal endothelial (ICE) syndrome, 56,
Glaukomflecken, 54, 55 57, 138, 165
Granular dystrophy, 34, 35 Iridodialysis, 90, 91
Granulomatous uveitis, 42, 43, 131, Iridoschisis, 119, 157
162–163 Iris
Grönblad–Strandberg syndrome, 100, 148 atrophy, 56, 57
Gyrate atrophy, 74, 75 melanoma, 78, 79
see also Aniridia
Iris naevus syndrome, 138, 165
H Iritis, 42, 56
Ischaemic central retinal vein occlusion,
Haab striae, 58, 59, 120, 158 66, 67, 116, 156
Haemangioma Ischaemic optic neuropathy, 82, 83, 119,
choroidal, 78, 79 157
retinal, 80, 81, 94, 145–146
see also Capillary haemangioma;
Cavernous haemangioma J
Haemorrhage 171
anterior chamber (hyphaema), 90, 91 Juvenile idiopathic arthritis, 42, 114, 155
 Maculopathy
K acquired, 72–73, 100:149, 104:150,
123:159, 132:163
Kasabach–Merritt syndrome, 117, 156 diabetic, 64, 65
Kayser–Fleischer ring, 135, 164 Maffucci’s syndrome, 117, 156
Kearns–Sayre syndrome, 119, 157 Map–dot–fingerprint dystrophy, 34, 35
Keratic precipitates, 42, 43, 131, 162–163 Marcus Gunn syndrome, 10, 11
Keratitis Marfan’s syndrome, 62, 63, 108, 153
contact lens-related, 28, 29, 114, 155 Megalocornea, 38, 39
fungal, 28, 29 Meibomian gland
herpes simplex, 30–31, 105, 151
x
cyst (chalazion), 4, 5, 144, 167–168
e
herpes zoster, 32, 33, 102:149 dysfunction in blepharitis, 2, 3
d
interstitial, 108, 152
n
Melanocytic naevus, 6, 7
I
marginal, 36, 37, 121, 158 Melanocytoma, optic nerve head, 80, 81
pseudomonas, 28, 29 Melanoma
rosacea, 36, 37 choroidal, 78, 79, 98, 147–148
suppurative, 28–29, 114, 155 conjunctival, 24, 25
Keratoacanthoma, 6, 7 iris, 78, 79
Keratoconjunctivitis Melanosis, conjunctival, 24, 96, 146
atopic, 22 Meretoja’s syndrome, 141, 166
sicca, 26, 27, 112, 154 Microcornea, 38, 39
vernal, 22, 23 Miller’s syndrome, 58, 92, 145
Keratoconus, 38, 39, 125:160, 136:164–165 Molluscum contagiosum, 4, 5, 102, 149
Keratoglobus, 38, 39 Mooren’s ulcer, 36, 37
Keratoprosthesis, 126, 160 Morning glory anomaly, 84, 85, 125, 160
Keratosis, actinic, 8, 9 Multiple sclerosis, 82, 96:146-147,
Krukenberg spindle, 52, 53, 139, 166 115:155
Munson sign, 38, 39
‘Mutton fat’ keratic precipitates, 42, 131,
L 163
Myasthenia gravis, 10, 126, 160
Lacrimal system probing, 95, 146 Myelinated nerve fibres, 144, 168
Laser in-situ keratomileusis (LASIK), 132, Myopia correction, 132, 163
163 Myopic maculopathy, 72, 73, 132, 163
Lattice dystrophies, 34, 35, 141, 166 Myotonic dystrophy, ptosis, 11
Lens disorders, 62–63
see also Cataracts
Leukaemia, 129, 161 N
Leukocoria, 80, 81, 101, 149
Limbal dermoid, 24, 25, 118, 156 Naevus
Lipodermoid, 24 choroidal, 78, 79, 98
Lisch nodules, 142, 167 conjunctival, 24, 25
melanocytic, 6, 7
Nasolacrimal duct, congenital obstruction,
M 95, 146
Nd:YAG iridotomy, 141, 166
Macular degeneration see Age-related Neonatal conjunctivitis, 20, 21, 106, 151
macular degeneration Neovascular glaucoma, 56
Macular epiretinal membrane, 72, 73, 123, Nerve palsies, 88–89, 106:151, 113:155,
159 122:158–159
Macular hole, 72, 73, 104, 150 Neural tumours, 16, 17
Macular oedema Neurofibromatosis-1, 58, 142, 167
172 clinically significant, 64, 98, 147 Nuclear sclerosis, 60, 61
cystoid, 130, 162 Nystagmus, 115, 155
[Link]
 Primary acquired melanosis, 24, 25, 96,
O 146
Primary congenital glaucoma, 58, 59, 120,
Oculocutaneous albinism, 129, 162 158
Oculomotor nerve palsy, 88, 89, 122, Proliferative vitreoretinopathy, 76, 77, 123,
158–159 159
Optic atrophy, 82, 83 Pseudoesotropia, 86, 87
Optic disc Pseudoexanthoma elasticum, 72, 100, 148
congenital anomalies, 84–85, 125, 160 Pseudoexfoliative glaucoma, 52, 53, 94,
normal, 51 146
Optic nerve glioma, 16

I
Pseudogerontoxon, 22, 23

n
Optic nerve head

d
Pseudomonas keratitis, 28, 29

e
drusen, 84, 85, 137, 165 Pseudopapilloedema, 84, 137, 165

x
infarction, 82, 83, 119, 157 Pseudoptosis, 117, 156
melanocytoma, 80, 81 Pseudotumour (idiopathic orbital
Optic nerve sheath meningioma, 16, 17 inflammation), 18, 19
Optic neuritis, 82, 83, 96, 146–147 Pterygium, 111, 154
Orbital cellulitis, 18, 19, 97, 147 Ptosis, 10–11, 117, 156
Orbital floor fracture, 18, 19, 100, 148 Punctum
Orbital pseudotumour, 18, 19 stenosis of, 120, 157
Orbital tumours, 16–17, 137, 165
Orbital varices, 16, 17
Osteogenesis imperfecta, 131, 163 R
Osteoma, choroidal, 78, 79
Racial epithelial melanosis, 24, 96, 146
Recurrent erosion syndrome, 90, 122, 158
P Reis–Bücklers dystrophy, 34, 124, 160
Reiter’s syndrome, 42, 109, 153
Papillary conjunctivitis, 22, 23 Retina
Papilloedema, 82, 83 acute necrosis, 32, 115, 155
Parinaud’s (dorsal midbrain) syndrome, 101, astrocytoma, 80, 81, 98, 148
149 haemangioma, 80, 81, 94, 145–146
Pars plana vitrectomy, 76, 123, 159 haemorrhages, see Haemorrhages,
Peau d’orange, 100, 149 retina
Penetrating keratoplasty (corneal graft), 34, lattice degeneration, 76, 77
35, 111, 154 retinoblastoma, 80, 81, 101, 149
Periphlebitis, 46, 47 tear formation, 90, 105, 150
Peters anomaly, 58 trauma, 90, 91
Phacomatoses, 58, 59, 94, 145–146 Retinal artery
Photodynamic therapy, 70 macroaneurysm, 99, 148
Phthisis bulbi, 42, 43 occlusion, 66, 67, 107, 151–152
Pigmentary glaucoma, 52, 53 Retinal detachment, 76–77, 105, 150
Pigment dispersion syndrome, 52, 139, 166 exudative, 76, 77
Pigment epithelial detachment (PED), 70, rhegmatogenous, 76, 77
128, 161 surgical complications, 130, 162
Pilocarpine, 156 tractional, 76, 77
Pit, optic disc, 84, 85 Retinal vein occlusion, 66, 67, 103:150,
Plexiform neurofibroma, 6, 7 116:156
Pneumococcal keratitis, 28, 29 Retinitis
Polypoidal choroidal vasculopathy (PCV), 70 cytomegalovirus, 44, 45
Posterior lens capsular opacification, 60, Retinitis pigmentosa, 74, 75, 107, 152
61, 127, 161 Retinoblastoma, 80, 81, 101, 149
Premature infants, retinopathy, 68, 69, 142, Retinopathy 173
167 central serous, 134, 164
 Retinopathy (cont’d) Sturge–Weber syndrome, 58, 59
diabetic, 64–65, 97, 98, 147 Stye, 4, 5
hypertensive, 68, 69, 142, 167 Subcapsular cataract, 60, 61, 103, 150
of prematurity, 68, 69, 142, 166–167 Subconjunctival haemorrhage, 133,
sickle-cell, 68, 69 163–164
Rhabdomyosarcoma, 16, 17 Superior oblique palsy, 88, 89
Rhegmatogenous retinal detachment, 76, Suppurative keratitis, 28–29, 114, 155
77 Symblepharon, 26, 27, 153
Rheumatoid arthritis Syphilis, congenital, 108, 152
corneal ulceration, 36, 37
x
keratoconjunctivitis sicca, 26, 112, 154
e
scleromalacia perforans, 40, 41 T
d
n
Rieger’s syndrome, 138, 165
I
Rodent ulcer, 8, 9 Terrien’s marginal degeneration, 136,
Rosacea keratitis, 36, 37 164–165
Roth spots, 129, 161 Thiel–Behnke dystrophy, 34, 35, 124,
Rubeosis iridis, 56, 57, 116, 155–156 159–160
Third nerve palsy, 88, 89, 122, 158–159
Thyroid eye disease, 14–15, 110, 153
S Toxocariasis, 44, 45
Toxoplasmosis, 44, 45, 129, 162
Salzmann’s nodular degeneration, 143, 167 Trabeculectomy, 50, 51, 58, 93, 145
Sarcoidosis, 46, 47 Trachoma, 143, 167
Schirmer’s test, 26 Tractional retinal detachment, 76, 77
Sclera, blue, 131, 163 Trauma
Scleritis, 40, 41 blunt, 90, 91, 121, 158
Scleromalacia perforans, 40, 41 corneal abrasion, 90, 91, 122, 158
Seasonal allergic conjunctivitis, 20 foreign body, 90, 91
Sebaceous gland carcinoma, 8, 9 orbital floor fracture, 18, 19, 100, 148
Seborrhoeic keratosis, 6, 7 penetrating, 90, 140, 166
Serpiginous choroidopathy, 48, 49 Trichiasis, 12, 13
Siegrist streaks, 167 Tuberous sclerosis, 80, 98, 148
Silicone explant, retinal surgery, 130, 162 Tumours
Sixth nerve palsy, 88, 89 conjunctiva, 24–25, 96:146,
Sjögren’s syndrome, 26, 112, 154 118:156–157
Solar (actinic) keratosis, 8, 9 eyelid, 6–9, 92:145, 117:156
Spring catarrh, 22, 23 optic nerve head, 80, 81
Squamous cell carcinoma, 8, 9 orbit, 16–17, 137, 165
Squamous papilloma, 6, 7 retina see Retina
Squint see Strabismus, childhood uvea, 78–79, 98, 147–148
Staphylococcal infections
blepharitis, 2, 140, 166
keratitis, 28, 29 U
Stargardt’s disease, 74, 75
Stevens–Johnson syndrome, 26, 27 Ultrasonography, 137, 165
Strabismus, childhood Uveal effusion, 40, 41
amblyopia, 86, 135, 164 Uveal tumours, 78–79, 98, 147–148
Brown’s syndrome, 86, 87, 112:154, Uveitis
118:157 anterior, 42–43, 114:155, 127:161,
Duane’s syndrome, 86, 87, 124, 160 131:163
esotropia, 86, 87, 104, 150 intermediate, 46, 47
exotropia, 86, 87, 134, 164 posterior
174 Strawberry naevus, 6, 7, 117, 156 infectious, 44–45, 129, 162
Stromal necrotic keratitis, 30, 31 non-infectious, 46
 Vogt striae, 38, 39
V Von Hippel–Lindau syndrome, 80, 94, 145
Vortex keratopathy, 136, 164
Varicella see Herpes zoster ophthalmicus
Varices, orbital, 16, 17
Vernal keratoconjunctivitis, 22, 23
Viral wart, eyelid, 6, 7 W
Vitamin and mineral supplements, 70, 128,
161 White dot syndromes, 48–49, 99:148,
Vitrectomy, 68, 76, 104:150, 123:159 109:153
Vitritis, 46, 47 Wilms’ tumour, 58, 92, 145

I
n
Vitreous haemorrhage, 123, 159 Wilson’s disease, 135, 164

d
e
Vitreous ‘loss’, 130, 162 Wyburn-Mason syndrome, 80

x
175