Diseases of Sclera
Special features of Sclera
• Avascular
• Dense fibrous tissue
• Lack of reaction to insult
• Two types of inflammation - episcleritis and scleritis
Episcleritis
• Benign inflammatory affection of deep subconjunctival connective tissue and superficial scleral lamellae
• Mostly bilateral
• Dense lymphocytic infiltration
• Reaction to endogenous toxin
• Association with rhuematoid arthritis
Symptoms and Signs
• Young females
• Acute redness
• Mild pain
• No discharge
• Two types - simple or diffuse
- nodular
Simple /diffuse episcleritis
• Sectorial or diffuse redness
• One or both eyes
• Mild to moderate tenderness over the area of redness
• Engorgement of large episcleral vessels which run in radial direction beneath the conjunctiva
Nodular Episcleritis
Treatment
• Mild to moderate- weak topical steroids
• Fluorometholone eye drops 4 times a day and lubricating drops
• Severe form - stronger steroids as prednisolone acetate eye drops 4-6 times a day
• Non steroidal anti inflammatory drugs like-ibuprofen 400 mg thrice daily or indomethacin can be given
Scleritis
• Extends more deeply
• Deep lymphocytic infiltration deep with in the scleral tissue
• Bilateral, rarer, more in females
•
• Associated with connective tissue disorder in 50% cases like - polyarteritis nodosa, SLE, reiters syndrome ankylosing spondylitis, wegners grannulomatosis, dermatomyositis, polychondritis, gout, herpes zoster ophthalmicus,syphilis
• Recent ocular surgery as cataract or RD surgery
A. Anterior scleritis
– Nodular
– Diffuse
– Necrotising - with inflammation - without inflammation
B. Posterior scleritis
Nodular scleritis
• One or more nodules
• Less circumscribed than episcleritis
• First dark red or bluish later becomes purple and semitransparent like porcelain
• All around cornea-annular scleritis –grave prognosis
Diffuse scleritis
• Hard whitish nodule pin head size with inflamed surrounding zone
• Disappear without disintegration
Clinical features of Scleritis
• Cornea and uveal tract are always involved as contrast to episcleritis
• Some iritis more often cyclitis and ant. Choroiditis
• No ulceration
• Dark purple weak cicatrix-ciliary staphyloma
• Secondary glaucoma common
Sclerosing keratitis
• Extends to cornea
• Opacity develops at the margin of cornea adjoining scleritis
• Tongue shaped, rounded apex towards center of cornea
• No corneal vascularisation or ulceration
• Pupillary area is usually spared
• Keratolysis is a serious complication
Necrotizing scleritis
• Scleral necrosis
• Severe thinning
• Melting of sclera
• Two types - with inflammation
- without inflammation
Necrotizing scleritis with inflammation
• Red ,painful eye, worsening of symptoms
• Associated with ant uveitis
• Autoimmune disorder
• Complications-glaucom, cataract, sclerosing keratitis, scleral melting are common
• Five year survival of patients at this stage of autoimmune disorder is 25%
Necrotizing scleritis without inflammation-
scleromalacia perforans
• Patients with seropositive rheumatoid arthritis
• Painless scleral thinning and melting
• Cause is ischemia
Posterior scleritis
• Inflammation with thickening of posterior sclera
• Primary or secondary extension of anterior scleritis
• Not associated with systemic disease
• Usually no symptoms
Symptoms and Signs
• Decreased vision
• With or without pain
• Proptosis
• Restricted ocular movements
• Post vitritis, disc edema ,macular edema, choroidal detachment exudative retinal detachment
• B-scan and CT scan shows thickened sclera
Diagnosis – episcleritis / scleritis
• Full blood count
• RA factor
• Mantoux test
• ANA
• Anti neutrophill cytoplasmic antibody
• VDRL
• Serum uric acid
• X-ray chest, sacro iliac joint
• LE cells
• Full immunological survey for tissue antibodies
Treatment
• Diffuse and nodular scleritis- NSAID
• Prednisolone -1mg /kg single morning dose
Tapered to 20 mg over 2-3 weeks
• H2 receptor blockers
• Necrotizing scleritis - additional immunosuppressant are recommended
• Abundant lubricant
• Scleral patch graft may be needed if risk of perforation
Posterior scleritis
• Same as ant scleritis
• IV Methylprednisolone as pulse therapy
• Local steroids ineffective
• SUBCONJUNCTIVAL INJECTIONS CONTRAINDICATED
• Infectious diseases are treated with appropriate antibiotics
Staphyloma
• Staphyloma is a clinical condition characterised by an ectasia of the outer coats (cornea,or sclera or both) with an incarceration of uveal tissue
• Two factors work - weakening of the outer wall and raised IOP
TYPES
• Anterior
• Intercalary
• Ciliary
• Equatorial
• Posterior
Anterior staphyloma
• Partial or total
• Mostly after sloughed cornea and pseudocornea formation
• AC becomes flat with secondary glaucoma
• Iris is incarcerated in anterior staphyloma
Intercalary staphyloma
• Limbus
• Root of iris and anterior most part of ciliary body
• Externally from limbus to 2mm behind
• Caused by - perforating injury at peripheral cornea involving limbus, marginal corneal ulcers, anterior scleritis, scleromalacia perforans, complicated cataract surgery with wound dehisence, secondary glaucoma
Ciliary staphyloma
• Affects ciliary zone - upto 8 mm behind the limbus
• Scleral ectasia with incarceration of ciliary body
• Caused by - developmental glaucoma, end stage of primary or sec glaucoma, scleritis, trauma to ciliary region of eye
Equatorial staphyloma
• Equatorial region of eye with incarceration of choriod
• 14 mm behind the limbus weak area due to passage of venae vorticosae
• Caused by scleritis , chronic uncontrolled glaucoma, degenerative myopia
Posterior staphyloma
• Posterior pole of eye lined by choroid
• Degenerative high myopia
• Detected by fundoscopy and B- scan ultrasonography
Treatment
• Treat the cause
• Small –local excision with corneo-scleral graft
• Large unsightly blind eyes are enucleated and replaced with implant
Dr Kavita Kumar
Associate Professor
Department of Ophthalmology
Gandhi Medical College
Bhopal