DISEASES OF SCLERA

Dr. Natasha Maria

 SCLERA-anatomy
•Tough white coloured opaque outer covering of
eye ball.

• Thickest posteriorly -1 mm

•Thinnest- just behind the extraocular muscles

insertion (0.3mm)
Histologically,

•Sclera - 3 layers

episcleral tissue

scleral proper

lamina fusca
 ANATOMY OF SCLERA
 Pierced anteriorly by:

-anterior ciliary arteries

-episcleral veins

 Pierced posteriorly by:

-vortex veins
-posterior ciliary nerves
-posterior ciliary vessels
-optic nerve
 LAYERS OF VESSELS IN EPISCLERA AND SCLERA

Conjunct Most Arteries- Easily moved on

superficial tortuous underlying structure.
ival Veins- straight Blanch with 10%
in
plexus conjunctiva phenylephrine.

Superfici Lies at the Vessels- In episcleritis, maximum

level of straight with congestion.
al radial Easily moved on
tenon’s
episcleral capsule configuaration underlying structure.
plexus Blanch with 10%
phenylephrine.

Deep Lies deep to Vessels- Scleritis-maximum

the tenon’s arranged in congestion.
vascular capsule & criscross Can’t be moved on
plexus directly over pattern underlying structure.
the sclera Does not blanch
with 10%
phenylephrine.
EPISCLERITIS
 Benign, idiopathic[90%], self
limited recuurent disorder
 Typically effects young to middle aged,
more in women.
 Systemic associations <1/3 cases
 Clinically two types:
1. Simple or diffuse episcleritis
2. Nodular episcleritis
 EPISCLERITIS
Symptoms
c/o ocular redness(for 10-21days).

 gritty,burning or foreign body

sensation, irritation.
Lacrimation,photophobia
Mostly in exposure zone of eye.
SIMPLE EPISCLERITIS

3/4th of all cases.

Sectoral/diffuse redness

Resolves spontaneously 1-
2wks
NODULAR EPISCLERITIS
Localised ,raised,congested nodules
 Seen in Interpalpebral region.
 More symptomatic
 Nodules consist of dense leucocytic
infiltration
Takes longer time to resolve.
DIFFERENTIAL DIAGNOSIS
Conjunctivitis-
superficial

Inflamed pinguecula

Phlytenulosis- mobile

Scleritis- deeper
 TREATMENT
• Mild- no treatment required.
• Artificial tear suplements
• Weak topical steroid QID for 1-2wks.
• Topical NSAIDS.
• Oral NSAIDS like T. Flurbiprofen 100mg.
(especially for prolonged remission).
EPISCLERITIS Vs SCLERITIS
EPISCLERITIS SCLERITIS

Redness, irritation. Severe boring pain.

No /minimal tenderness. Moderate to severe

tenderness.
Congested vessels-bright red, Congested vessels- purple
Moved easily with cotton bud. red, can’t be moved easily
with cotton bud.
Blanching of vessels with Blanching does not occur
10% phenylephrine. with 10% phenylephrine.
SCLERITIS

- Mostly B/L disease.

-More common in women

-Variable duration(months to years).

-Associated with connective tissue disease in

.
50% of cases..
Symptoms:

deep,boring pain, tender to palpate.

-whole eye/1 or 2 quadrants.

violaceous appearnce
Ocular manifestations:
. - on S/L-engorged conjunctival vessels.
-episclera-edematous & inflammed.

-scleral thickening/ scleral thinning.

-nodules (less circumscried than in
episcleritis) , necrosis.

- There maybe associated corneal and

uveal tissue involvement.
Autoimmune diseases associated with
scleritis
Ankylosing spondylitis
Rheumatoid arthitis
Wegners granulomatosis
SLE
Ulcerative colitis
 SCLERITIS

ANTERIOR POSTERIOR

NON NECROTISIN
NECROTISIN G
G
-WITH
-DIFFUSE INFLAMMATIO
-NODULAR N
-WITHOUT
INFLAMMATIO
N
ANTERIOR SCLERITIS
NON-NECROTISING:
1. DIFFUSE:
- benign
- wide spread inflammation
- sector/entire anterior sclera
- the area is raised & salmon pink to purple in
colour.
2.
NODULAR:

-pain, f/b sensation,

redness
-tenderness of
globe
- appearance of
scleral nodule.
SIGNS:
-single or multiple nodules,
-deep red to purple in colour,
-immobile, tender to palpation,
-s/l beam is displaced by scleral
nodule.
ANTERIOR NECROTISING SCLERITIS

WITH INFLAMMATION:
-presents with red, painful eye with progressive
worsening of symptoms.
-usually part of a systemic autoimmune disease.
-most severe, potential threat to vision loss.
-responds poorly to analgesics.
COMPLICATIONS

Staphyloma
Scleral thinning
Keratitis
Anterior uveitis
long standing cases
glaucoma
sec. cataract
macular
edema
Poor prognosis.
High incidence of
visual impairement
WITHOUT INFLAMMATION:
Also called as SCLEROMALASIA
PERFORANS.

women with long standing RA.

Minimum signs of inflammation, no pain
Progression – scleral
thinning(astigmatism), visible uveal tissue.
Raised IOP – Staphyloma formation.
Globe rupture with minimal trauma.
POSTERIOR SCLERITIS
Uncommon, often misdiagnosed.
Variable presentation
Pain, visual impairement
Guarded visual prognosis
Signs[external]
Lid edema, fullness
Proptosis, restriction of extraocular movements
May be associated with anterior scleritis.
POSTERIOR SCLERITIS
Signs[Internal]
Disc swelling
macular/paramacular edema
Choroidal folds
 exudative RD
Ring choriodal detchmement
Papilledema
Infectious scleritis
Consists of :
- Gumma – seen in tertiary syphilis, uncommon now.
- Tuberculosis
- Suppurative infections
• Tuberculosis and suppurative infections are mostly
secondary extension from adjoining tissues.

Early stages difficult to diagnose.

Scleritis with purulent exudates /infiltrates

 signs -Formation of fistulae,painful

nodules,conjunctival,scleral ulcers
Causative agents in suppurative
infectuous scleritis
pseudomas aeruginosa
Strept.pneumonia
Staph.aureus
INVESTIGATIONS
 ANTERIOR SCLERITIS: mainly clinical.
POSTERIOR SCLERITIS:
B-scan – T sign represents fluid in tenon’s
capsule.
- thickened posterior sclera.
CT ORBIT with contrast – RING sign
represents enhancement of sclera.
LABORATORY
TESTS:

-CBP, ESR
-Rh factor
-ANCA, ANA
-HLA – B27
- FTA-ABS
-Urine analysis
-Chest X RAY
TREATMENT
1. -oral NSAIDS(in diffuse & mild nodular
scleritis)
MEDICAL:
1. -topical steroidal agents
Indomethacin(50mg tid/75mg
bd)
-oral non steroidal agents
Piroxicam (20mg);
others Ibuprofen, Naproxane
given with
Systemic steroids – prednisolone
1- 1.5mg/kg/day for 6months to 1yr.

Pulse Intra venous Methyl

prednisolone
– 0.5-1gm in severe scleritis / day for
3 days.

Oral prednisolone is often required

Sub conjunctival steroid inj.: in
non necrotising cases.

Immunosupressive/
immunomodulator therapy – to
patients unresponsive to prednisolone &
necrotising cases.
Oral / subcutaneous Methotrexate –
7.5- 25mg weekly.
Azathioprine – 1.5-2mg/kg/day
Mycophenolate mofetil
Cyclosporine
TNF – alpha
inhibitors(Infliximab,
adalimumab)

Alkylating agents
(chlorambucil,
cyclophosphomide)
Staphyloma
Staphyloma is a clinical condition characterised by
ectasia of the outer coats(cornea, sclera or both) of
the eye with incarceration of the uveal tissue.
Can be classified as:

1. Anterior
2. Intercalary
3. Ciliary
4. Equatorial
5. Posterior
Anterior staphyloma
Tumors
Benign-
consists mostly of choristomas.

Choristomas – benign tumour like lesions owing to

the presence of normal tissue in abnormal location.
Eg- Limbal and epibulbar dermoids.

Malignant
Mostly secondaries from tumors like retinoblastoma
and malignant melanoma.