Diseases of the Sclera
Dr. Aditya Prasad
MBBS, MD,FIAS,FIOS
Sklērós:hard
TOPICS
• ANATOMY AND PHYSIOLOGY
• INFLAMMATORY CONDITIONS
• STAPHYLOMA
• TUMOURS
• CONGENITAL ABNORMALITIES
Anatomy & Physiology
• Sclera is tough white colored opaque outer covering of
the eyeball, originated from neural crest & is composed
of collagen & elastin
• Composed of three ill-defined layer, namely the sclera
proper with episclera on the outside & lamina fusca
interiorly.
Blood Supply
• Pierced by anterior ciliary artery & episcleral veins
anteriorly(3-4 mm from limbus), & the vortex vein
(posterior to equator), posterior ciliary nerves & vessels
& the optic nerve posteriorly.
Inflammation
• Two forms of inflammation
―Superficial or episcleritis
―Deep or scleritis
Episcleritis
• It is a benign inflammatory involvment of deep
subconjunctival connective tissue, including superficial
scleral lamellae
• frequently affects both eyes
Aetiology
• Often regarded as an allergic reaction to endogenous
toxin
• Usually this condition is associated with collagenous
disease
• Usually history of rheumatoid arthritis is commonly
obtained
Symptoms
• Usually patient is young adults and frequently females
• Presents with an acute onset of redness
• Mild or no pain in both eyes
• No history of discharge
• Phenylephrine test: episcleral vessels blanch
Clinical types
1. Simple or diffuse episcleritis
2. Nodular episcleritis
Simple or diffuse episcleritis
• Patient have a sectoral or diffuse redness of one or both
eyes d/t engorgement of large episcleral vessels, which
runs radially beneath the conjunctiva.
• May have mild to moderate tenderness over the area of
episcleral injection
Nodular episcleritis
• Circumscribed nodule of dense leucocytic infiltration,
usually 2-3 mm from limbus
• It is hard, tender & immobile
• Mild pain , but usually there is feeling of discomfort &
tenderness on pressure and sometimes with neuralgia
(rare)
• More symptomatic & takes longer to resolve
• Site : temporal (usually)
NODULAR
EPISCLERITIS
Treatment (Episcleritis)
• Cold compression
• Mild cases : provide lubricating drops alone
• Moderate to severe case : Mild topical steroid (eg.
Fluorometholone) prescribed four times a day
• Rarely potent steroids are required
If topical fails…
• Oral NSAIDs may help along with topical NSAIDs provide
temporary benefit.
• Ibuprofen 400 mg orally 3-4 times.
• Aspirin 325-650 mg 3-4 times a day
• Indomethacin 200-400 mg 3-4 times.
SCLERITIS
• It is usually bilateral
• More frequently in women
• 50 % of this cases are associated with connective tissue
disorder
• Thorough investigations to rule out active systemic
diseases like PAN, RA, SLE, Reiter syndrome, Ankylosing
spondylitis, non-specific arteritis, Wegener
granulomatosis, Dermatomyositis, Polychondritis & Gout
Clinical classification of Scleritis
A Immune mediated
1) Anterior scleritis
• Non-necrotizing
i. Diffuse
ii. Nodular
• Necrotizing
• 1) with inflammation
• Vaso-occlusive
• Granulomatous
• Surgically induced
• 2) without inflammation (scleromalcia perforans)
2) Posterior scleritis
B Infectious scleritis
Symptoms
• Pain
• Redness with violaceous hue
• Watering
• Discharge
• Bluish or brownish
discoloration
• Diminution of vision
Signs
• Nodular scleritis :
• One or more hard purplish nodules usually near limbus
• May extend entirely around the cornea, in a ring
(annular scleritis)
• Diffuse scleritis :
• Hard whitish pin head sized nodules may develop in
inflamed zone (1 or more quadrants) and they disappear
without disintegrating
• Pink to purple in color
Necrotizing scleritis
• Associated with scleral necrosis, severe thinning &
melting in severe cases
• There are two forms : With inflammation or without
inflammation
Necrotising scleritis with inflammation
• Red , painful eye & progressive worsening conditions
• May be associated with anterior uveitis
• Usually a part of autoimmune disorder
NECROSIS
• Vasculitis: vascular sludging & occlusioninfarction
• Scleral thinning : ectatic uveal tissue shines through it
• Complications like : glaucoma, cataract, sclerosing
keratitis & peripheral corneal melting are common
Necrotising scleritis without inflammation
• Also known as scleromalacia perforans
• Occurs in seropositive rheumatoid arthritis
• Ischemia: yellowish patch of melting sclerasclera
absorbedpunched out area of thin sclera with underlying
visible uveal tissue
• Painless scleral thinning with melting in severe case
• Spontaneous perforation very rare
Posterior scleritis
• Inflammation with thickening of the posterior sclera may
start primarily posteriorly or may extent from anterior
scleritis
• Not usually associated with systemic disorders
• Clinical presentation is varied & the diagnosis is missed
• Features of associated structure’s inflammation:
Retinal detachment, Macular edema, proptosis, eom limitation
Clinical presentation
• Decreased vision
• Painful or painless
• Proptosis or restricted
ocular movement
• Fundoscopy :
—Disc oedema
— Macular oedema
—Choroidal folds
—Choroidal detachment
—Exudative retinal detachment.
• Infectious scleritis
Rare compared to non infectious scleritis
Scleritis with purulent exudates, fistula formation,scleral ulcers
Diagnosis
• Careful history with thorough systemic examination and
based on clinical judgment go for investigation
Investigation
• Full blood count
• Rheumatoid factor (RF)
• Mantoux test
• Antinuclear antibody (ANA)
• Antineutrophil cytoplasmic antibody (ANCA)
• Veneral disease research lab (VDRL)
• Fluorescent treponemal antibody absorption test (FTA-ABS)
• Serum uric acid estimation
• Treponema pallidum immobilization (TPI test)
• X-ray : chest & sacroiliac joint
• Overall immunological survey for tissue antibodies
Treatment
• Oral NSAIDs
• Oral prednisolone 1mg/kg per day then to be tapered to 20
mg per day over subsequent 2-3 weeks .
• Immunosuppresive agent : cyclophosphamide, methotrexate,
cyclosporin or azathioprine.
• H2 receptor blockers – ranitidine, famotidine twice daily .
• In case of any infectious cause treat with appropriate topical &
systemic antimicrobial agents
Treatment- Necrotizing scleritis
• Systemic steroids
• Immunosuppresives
• Abundant lubrication in scleromalacia perforans.
• If in risk of perforation – perform scleral patching.
Treatment- Posterior scleritis
• NSAIDs
• Steroids
• Immunosuppresive therapy
• IV methyprednisolone as pulse therapy and helps in
reducing prolonged oral steroid intake side-effects
Not to be done…
• Biopsy
• Subconjunctival injections
STAPHYLOMA
• Clinical condition characterised by an ectasia of outer
coats of the eye with an incarceration of uveal tissue
• Cause : Inflammatory or degenerative disease which
causes weakening of the globe and are associated with
raised IOP & ultimately contribute to development of the
staphyloma
*ECTASIA –dilatation or distension of tissue
*Incarceration - confined
Types of staphyloma
1. Anterior
2. Intercalary
3. Ciliary
4. Equatorial
5. Posterior
Anterior Staphyloma
• Partial or total depending on whether part or whole of the
cornea is affected
• Cause : Sloughing corneal ulcer ( most common cause)
Intercalary Staphyloma
• Located at the limbus & is lined by root of the iris & the
anterior most part of ciliary body
• Causes :
— Perforating injury of peripheral cornea
—Marginal corneal ulcer
—Anterior scleritis
—Scleromalacia perforans
—Complicated cataract surgery with poor apposition
—Secondary glaucoma.
Ciliary Staphyloma
• Affects the ciliary zone .
• Causes :
—Glaucoma
—Scleritis
—Trauma
Equatorial Staphyloma
• Location : 14 mm behind the limbus.
• Causes :
—Scleritis
—Degenerative myopia
—Chronic uncontrolled glaucoma
Posterior Staphyloma
• Affects the posterior pole of the eye
• Cause : Degenerative high axial myopia
Diagnosis
• Fundoscopy
• B-scan ultrasonography
• Indirect Ophthalmoscopy : cresenteric shadow in macular
region
• Pale colored retina at the site of staphyloma due to
degenerative changes.
Treatment for Staphyloma
• Treating inflammatory condition
• Prevention of secondary glaucoma
• Repair with corneal & scleral graft
• Staphylectomy
• Keratoplasty
• Enucleation
TUMOURS
Choristomas
• Benign tumour-like lesions owing to the presence of normal
tissue in an abnormal location.
• Involves episclera more commonly than sclera
• Usually congenital
• May increase in size & prominance with age.
• Limbal & epibulbar dermoids
Goldenhar syndrome
• Oculo-auriculo-vertebral syndrome
Malignant tumors
• Primary tumors : Rare
• Secondarily : retinoblastoma & malignant melanoma
• Tumors outside the eyeball : SCC, malignant melanoma.
BLUE SCLERA
• Ocular disease : Keratoconus & Keratoglobus
• Associated disease
—Osteogenesis imperfecta (fragilitas ossium)
—Ehlers-Danlos syndrome
—Marfan syndrome
•Thank you
