Outline Objectives
Episcleral and Sclera Anatomy
Discolorations of Episclera + Sclera
Inflammations of Episclera + Sclera
1. To discuss normal and abnormal findings of the sclera and episclera
2. Discuss inflammation of the sclera and episclera
ANATOMY: Episclera / Sclera
Episclera: more superficial, loose CT w. a rich blood supply from Ant.
Ciliary arteries
o Superficial vasculature
o Deep Plexus Vessels
2. Sclera proper (stroma): composed of dense fibrous tissue with
fibroblasts and ECM
o Avascular
3. Lamina fusca: a thin layer of melanocytes, connected to the choroid by
collagen fibre
Scleral Abnormalities = Changes in appearance • Episcleritis • Scleritis
Focal Scleral Discolouration
- Scleral Hyaline Plaques: AKA Senile Scleral Translucency
o Focal, scleral hyaline plaques, sharply defined,
ovoid; near insertion of H rectus muscles, harmless
o Seen in ageing, PX > 70 yrs
o Collagen fibres become thicker, less uniform w. inc
age, particularly in region of the muscle insertions
o Sclera = progressively thinned, colour contrasts between one part of
sclera + next
- Hyaline Plaques; Fibres become disrupted then ca deposition can occur,
leading to production of hyaline plaques
- Alc(k)aptonuria: hereditary metabolic problem (Phenyl-alanine &
Tyrosine metabolism) = leads to accumulation of homogentisic acid in
scleral collage
- Haemochromatosis rusty-brown colour (hereditary disease, excessive
absorption + accumulation of dietary iron), dry eye
- Systemic minocycline (tetracycline antibiotic): blue-grey paralimbal
discolouration
- Metallic FB- long-term rust spots
Diffuse Scleral Discolouration:
- Yellow: usually due to jaundice (bilirubin)
- Grey/black: ocular melanocytosis, or oculodermal melanocytosis (Naevus
of Ota)
- Blue: thinning + Transparency so that underlying uvea is visible; assoc.
with: osteogenesis imperfecta, Ehlers-Danlos syndrome, pseudoxanthoma
elasticum..
Blood Vessels & the Sclera:
- Sclera is avascular but is surrounded by blood vessels
- Conjvessels: most superficial plexus, arteries tortuous, veins straight
- Superficial episcleral vascular plexus: vessels straight, radial
configuration,
- Deep episcleral vascular plexus: maximal congestion seen in scleritis
- Episcleritis– H15.
o What: Inflamm. of the superficial episcleral tissue + does not
involve the deep episcleral tissue that overlies the sclera
Conj. + superficial episcleral vascular plexuses are displaced
anteriorly+ the deep underlying episcleral plexus is NOT
involved, remains flat
Normal thickness of the underlying sclera.
Contrast w. scleritis = max site of vascular congestion in deep
episcleral plexus, which is displaced anteriorly due to oedema
of underlying sclera.
o Normal episcleral vascular
supply:
Radial superficial episcleral
vascular plexus (ant. ciliary
arteries; anastomose w. post.
ciliary arteries)
Deep vascular plexus adjacent
to sclera
o = Mild, non-vision threatening,
inflammation of the episclera
o Sudden onset, eye becomes red + uncomfortable within the
hour; sensation of hotness, prickling, discomfort; mostly young
adults, possible F predilection
o Mild localised oc. discomfort (some may say pain) only, no
radiations to face or temple, no impact on VA
o Signs:
Redness: mild to fiery flush, often intrapalebral most often in
upper temporal or nasal quadrants- sectoral vs diffuse
Peak within 12 hrs • Can recur + also swap between eye
o 2 clinical types: 1. Simple 2. Nodular
~70% cases idiopathic; ~30% cases assoc. w. an underlying
disorder - autoimmune systemic conditions (e.g.
rheumatoid arthritis)
Seldom progresses to scleritis
- Simple episcleritis- H15.11
o Simple (diffuse or sectoral) episcleritis = most common form
(~75%), occur as intermittent bouts
o Mod- to-severe inflamm, often recurs at 1-3 mth intervals.
o Episodes usually last 7-10 days, most resolve after 2 to 3 weeks
Simple sectoral episcleritis
Vs. simple diffuse episcleritis
- Nodular episcleritis – H15.2
o Typically first noted on waking, redness over next 2-3 days in same
location
Translucent nodule (elevated lesion) centrally within inflamed
area; localised granulomatous inflammation; intrapalpebral
Prolonged attacks of inflammation, typically more
discomfort/pain than simple episcleritis; self-limiting
Some px w. nodular episcleritis have associated systemic
disease
o Less common than simple episcleritis
- Episcleritis MX:
o Similar for simple + nodular episcleritis; More often indicated in
nodular
o If condition is mild, no tx necessary–
Episcleritis is not sight threatening
o Tx sx present– (Cool) Oc lubricants for relief of irritation, cool
compresses
o Weak topical steroid for 1-2 weeks: Fluorometholone, lotoprednol
o Topical NSAIDs: Ketorolac
o Oral NSAIDs for frequent recurrent attacks
Typically for px with known systemic association
- Scleritis– H15.0: = Heterogeneous group of diseases w. scleral
inflammation
o Causes:
Local or systemic infections or
Immune mediated diseases
o Usually presents with severe boring pain/ache;
May involve eye, orbit (retrobulbar),
Radiates to face, temple, jaw
Exacerbated by eye movements, worse at night, wakes
patient early morning
o Often not responsive to mild analgesics or topical therapy
o Scleritis =vision threatening, must be distinguished from other
causes of red eye
o Inflammation in deeper tissues, may be ‘violaceous’ (purple) c.f red
(more superficial vascular plexus) episcleritis; scleral oedema
o Anatomical Classification:
A. Anterior scleritis
(Inflammation ant. to EOM
insertion) (98%) –H15.01
Non-necrotizing (85%):
diffuse or nodular
Necrotizing (13%): with or
without inflammation
o (Scleromalacia
perforans (v. rare)
B. Posterior scleritis
(inflamm. post. to EOM
insertion) (2%)– H15.03
Diffuse or nodular (10 to 20% cases
o A: diffuse B: nodular C: necrotising with inflammation D:
scleromalacia perforans E: posterior – ultrasound
- E.g: Diffuse Anterior Non-Necrotizing Scleritis
o Presentation:
Similar to episcleritis but more severe
Oc redness (generalised or localised), few days later aching +
radiating pain
More common F, ~50’s
Signs:
Vascular congestion + dilatation with oedema
As oedema resolves, may appear slight grey/blue,
Scleral translucency (collagen fibre rearrangement)
Commonly recurs; can last up to 6 yrs often with ~18
mth recurrence
o Relatively benign - Does not progress to necrosis
o Widespread scleral and episcleral injection
- Nodular Ant. Non-necroitisng scleritis
o Presentation:
Insidious onset of pain, oc redness, tender globe,
nodule appears
Often previous hx of HZO
o Signs: Single or Multiple nodules; 3-4 mm from limbus,
intrapalpebral
Deeper blue-red colour compared to episcleral nodules,
immobile
As inflammation decreases, sclera becomes translucent
Commonly recurs; can last up to 6 yrs, 18 month recurrences
>10% will develop necrotizing disease if not tvd early
o May resemble nodular episcleritis; more serious than diffuse scleritis
o Scleral nodule cannot be moved over underlying tissue; note slit
beam displaced by nodule
- Anterior Necrotizing Scleritis w. Inflammation
o Presentation:
> 60 age, bilateral ~60%
Gradual nset of Pain; persistent aching, radiating
Wakes px, poor response to analgesia
If not tx-ed early, aggressive + vision/eye threatening
o Signs:
Nodular scleritis w. deep vascular congestion
Scleral thinning due to necrosis, ‘blue’ choroid visible
Progressive scleral thinning and extension of necrosis around
globe
o =Specific types of necrotising disease:
Vaso-occlusive: characterised by areas of
congestion that coalesce, become avascular
(ischaemic) + necrotic
Granulomatous necrotising scleritis: injection in
adjacent cornea, extends posteriorly; raised, irregular,
oedematous sclera; may also involve ciliary body,
Trab meshwork
Surgically-induced scleritis: induced by various
types of surgery (e.g. after cataract surgery, post-
vitrectomy; post-pterygium; usually localised); can be
associated with infection
Painful + Most severe type
o Corneal complications:
Acute infiltrative stromal keratitis: localised or diffuse
Sclerosing keratitis: chronic thinning + opacification of
peripheral cornea to resemble sclera
Peripheral ulcerative keratitis (PUK): Progressive melting
+ Ulceration of cornea (more common in necrotising disease)
o Uveitis and Scleritis:
All types of scleritis can be assoc. w. uveitis (mild or
severe);
Ant. Uveitis = seen in up to 40% in eyes w. scleritis; mostly
mild disease.....
o Staphyloma = protrusion of any part of the eye; e.g. a staphyloma
of the sclera.
- Scleromalacia Perforans (v rare) – H15.05:
o Necrotising scleritis w.o. inflammation; very rare
o Typically affects elderly women w. long standing RA
o Presents w. slight non-specific irritation, painless, vision not usually
affected (may get blurring due to astigmatism assoc. w. scleral
thinning)
o Obliterative vascular disease of deep episcleral vascular plexus,
slowly progressing
o Thin, white sclera; usually no corneal involvement except for limited
peripheral thinning
- Posterior Scleritis:
o May present with reduced vision or without
pain
o Unilateral or bilateral (~35%)
o Involves sclera posterior to insertion of RM; - requires
imaging to detect:
E.g. B-scan ultrasound (disc oedema, retinal detachment);
CT scan (thickened posterior sclera >2mm);
MRI
o Age of onset often >40 yrs;
~30% of those >50 yrs =is assoc. w. Systemic Disease
o Signs:
Exudative RD ~25% cases
Choroidal folds, uveal effusion
Disc oedema (/swelling)
o NOT to be confused w.:
Choroidal tumours, orbital
cellulitis, retinal detachment
with central serous
retinopathy….
Choroidal folds + Disc oedema
often occur with orbital tumours,
papilloedema, thyroid eye
disease, hypotony
- Scleritis + Systemic Diseases
~40-50% cases aw. Systemic Infectious Cause of Scleritis
inflammatory/immune mediated diseases (uncommon)
(e.g. vasculitic autoimmune, connective Most common causes:
tissue diseases): - 1. Bacterial: TB, Leprosy,
- 1. Rheumatoid arthritis**
Staph/Strep, syphilis,
- 2. AAV (ANCA Associated
Vasculitis) syndromes:
pseudomonas, Lyme disease
granulomatosis with polyangiitis (Borrelia)
(GPA) previously Wegener's - 2. Fungal: Aspergillus
granulomatosis), [Microscopic - 3. Viral: Herpes zoster
Polyangiitis (MPA), and Churg (varicella zoster), Epstein Barr,
Strauss Syndrome (CSS)] coxsackie
- 3. Polyarteritis nodosa - 4. Ancanthomoeba
(vasculitis) - 5. Toxoplasmosis
- 4. Systemic lupus
erythematosus (SLE)
(autoimmune)
- 5. Miscellaneous (herpes
zoster/simplex, syphilis, Bechet’s,
sarcoidoisis……)
o Investigations
Blood tests:
FBC (full blood count) =
ESR (erythrocyte sedimentation ratio)
CRP (C-reactive protein)
Rheumatoid factor
ANCA = Anti-neutrophil cytoplasmic antibodies;
Lysosomal components of neutrophils and monocytes)
ANA (anti-nuclear antibodies), anti-ds DNA
Urea + Electrolytes
Check BP
Other tests – depends on HX & Exam eg. chest X-ray
- Scleritis: MX
o = URGENT REFERRAL IN OPTOMETRIC PRACTICE
o For management, know:
Type + extent of disease; complications; underlying systemic
or local cause
Post. or necrotising = VERY URGENT compared to ant. non-
necrotising
Systemic NSAIDS for non-necrotising disease
Corticosteroids: systemic, periocular + subconjunctival
injections; IV Steroids (e.g. triamcinolone acetonide);
immunosuppressives (consider side-effects)
Surgical interventions for necrotising scleritis
- Scleritis vs Episcleritis;
Oc complications (in general) statistically more frequent in px w.
scleritis than in px with episcleritis – These include:
o Decrease in vision,
o Ant. Uveitis
o PUK
o Ocular hypertension.....
o PX with scleritis = older than PX with episcleritis
o Scleritis more likely aw. systemic disease
- DDX:
o Episcleritis vs Scleritis
o Anterior Scleritis:
Red/Purple eye, Pain/tenderness, Photophobia • Purplish
sclera • Oedema or thinning of sclera (loss of collagen)
Uveitis/PUK • No change with 2.5% phenylephrine
o Episcleritis: Red/pink eye • Not usually painful • Less
photophobia • No scleral changes usually present • Some vessel
blanching with 2.5% phenylephrine
Design of metboalism:
Metabolism: pathways – involve a eries of steps – which each step carried
out by an enzyme
o Metabolism incorporates all the chemical reactions that occur in a
cell / organism.
