Diseases of Sclera

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Diseases of Sclera

• Copy of lecture taken by
• Dr Kavita Kumar
• Associate Professor
• Department of Ophthalmology
• Gandhi Medical College
• Bhopal

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Special features of Sclera

• Avascular
• Dense fibrous tissue
• Lack of reaction to insult
• Two types of inflammation - episcleritis and
  scleritis

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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

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Symptoms and Signs

• Young females
• Acute redness
• Mild pain
• No discharge
• Two types - simple or diffuse
• - nodular

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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

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Nodular Episcleritis

• Features
• Circumscribed nodule as large as lentil
• 2-3 mm away from limbus
• Hard tender immovable
• Conjunctiva moving freely over it
• Traversed by deeper episcleral vessels hence
  purple in colour not bright red
• Tenderness, discomfort and sometimes neuralgia

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Nodular Episcleritis

• Features
• Longer time to resolve
• Can pass on to scleritis
• Temporal location
• Chronic course and strong tendency to recur

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Clinical Photograph of Episcleritis
From Dr Sanjay Shrivastavas collection
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Clinical Photograph of Episcleritis(under
treatment)
From Dr Sanjay Shrivastavas collection
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Nodular Episcleritis

• Fleeting attacks and frequently repeated called
  EPISCLERITS PERIODICA FUGAX
• Heals and leaves a slate coloured scar with
  conjunctiva adhered to it
• Cornea and uveal tract are rarely involved

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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

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Scleritis

• Extends more deeply
• Deep lymphocytic infiltration deep with in the
  scleral tissue
• Bilateral, rarer, more in females

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Scleritis

• 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

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Clinical Photograph of Scleritis
From Dr Sanjay Shrivastavas collection
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Clinical Photograph of Scleritis
From Dr Sanjay Shrivastavas collection
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Clinical classification of scleritis

• A. Anterior scleritis
• Nodular
• Diffuse
• Necrotising - with inflammation
  - without inflammation
• B. Posterior scleritis

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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

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Diffuse scleritis

• Hard whitish nodule pin head size with inflamed
  surrounding zone
• Disappear without disintegration

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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

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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

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Necrotizing scleritis

• Scleral necrosis
• Severe thinning
• Melting of sclera
• Two types - with inflammation
• - without inflammation

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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

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Necrotizing scleritis without inflammation-scler
omalacia perforans

• Patients with seropositive rheumatoid arthritis
• Painless scleral thinning and melting
• Cause is ischemia

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Posterior scleritis

• Inflammation with thickening of posterior sclera
• Primary or secondary extension of anterior
  scleritis
• Not associated with systemic disease
• Usually no symptoms

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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

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Diagnosis episcleritis / scleritis

• Full blood count
• RA factor
• Mantoux test
• ANA
• Anti neutrophill cytoplasmic antibody

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Diagnosis episcleritis / scleritis

• VDRL
• Serum uric acid
• X-ray chest, sacro iliac joint
• LE cells
• Full immunological survey for tissue antibodies

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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

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Posterior scleritis

• Same as ant scleritis
• IV Methylprednisolone as pulse therapy
• Local steroids ineffective
• SUBCONJUNCTIVAL INJECTIONS CONTRAINDICATED
• Infectious diseases are treated with appropriate
  antibiotics

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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

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TYPES

• Anterior
• Intercalary
• Ciliary
• Equatorial
• Posterior

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Anterior staphyloma

• Partial or total
• Mostly after sloughed cornea and pseudocornea
  formation
• AC becomes flat with secondary glaucoma
• Iris is incarcerated in anterior staphyloma

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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

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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

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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

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Posterior staphyloma

• Posterior pole of eye lined by choroid
• Degenerative high myopia
• Detected by fundoscopy and B- scan
  ultrasonography

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Treatment

• Treat the cause
• Small local excision with corneo-scleral graft
• Large unsightly blind eyes are enucleated and
  replaced with implant