Diseases of sclera

Diseases of sclera

1. DISEASES OF SCLERA Dr. Nikhil Gotmare

2. Anatomy

3. A n a t o m y of sclera It forms the p osterior opaque 5/6 th part of the external fibrous coat of the eyeball Eye has three coats: Outer – Fibrous Middle – Vascular Inner - Nervous

4. O uter surface - covered by Tenon's capsule A nterior part - covered by bulbar conjunctiva

5. Its inner surface lies in contact with choroid with a potential suprachoroidal space in between

6. In its anterior most part near limbus there is furrow which encloses the Schlemm’s canal

7. Thickness of sclera T hinner - children and in females T hickest - posteriorly (1mm) , gradually becomes thin when traced anteriorly Thinnest - insertion of extraocular muscles (0.3 mm)

8. Apertures of sclera Anterio r - Anterior ciliary vessels, nerves and lymphatics Middle four vortex veins Posterior - Short ciliary vessels and nerves Long ciliary arteries Optic nerve

9. Anterio r - Anterior ciliary vessels, nerves and lymphatics

10. Middle four vortex veins Posterior - Short ciliary vessels and nerves Long ciliary arteries Optic nerve

11. Layers of sclera S clera Episclera Sclera proper Lamina fus ca T hin, dense vasculari z ed layer of connective tissue  fibroblasts, macrophages and lymphocytes A vascular structure  dense bundles of collagen fibres I nnermost blends with suprachoroidal and supraciliary laminae of the uveal tract. brownish in colour  p resence of pigmented cells

12. S taphylomas L ocalised bulging of weak and thin outer tunic of the eyeball (cornea or sclera) L ined by uveal tissue which shines through the thinned out fibrous coat. {‘ Staphylo -’ bunch of grapes}

13. C lassification posterior Anterior Intercalary Ciliary Equatorial

14. Anterior staphyloma Ass. With ectasia of cornea & iris Due to perforating corneal ulcer & injury

15. Intercalary staphyloma healing of a perforating injury or a peripheral corneal ulcer to ectasia of weak scar tissue formed at the limbus localised bulge in limbal area lined by root of iris {‘Intercalary’ - Occurring between differentiated tissues}

16. Ciliary staphyloma B ulge of weak sclera lined by ciliary body A bout 2-3 mm away from the limbus T hinning of sclera following perforating injury, scleritis and absolute glaucoma

17. Equatorial staphyloma B ulge of sclera lined by the choroid in the equatorial region at the regions of sclera which are perforated by vortex veins. C auses - S cleritis and degeneration of sclera in pathological myopia {‘Equator - 14mm behind limbus}

18. Posterior staphyloma B ulge of weak sclera lined by the choroid behind the equator C ommon causes are pathological myopia , posterior scleritis and perforating injuries.

19. Diagnosis  O phthalmoscopy The area is excavated with retinal vessels dipping in it

20. Pathological myopia

21. Blue sclera M arked , generalised blue discolouration of sclera due to thinning

22. Causes of Blue sclera Mnemonic- { H O P E S are too b ig } H - High myopia , H urlers and H ealed scleritis O - O steogenesis imperfecta - O culodermal melanosis P - P seudoxanthoma elasticum E - E hlers -Danlos syndrome S – S cleritis , S taphyloma, S cleromalacia perforans Ar e - M ar fan's syndrome Too – Tu rner’s B ig - B uphthalmos

23. Inflammations of sclera Episcleritis (superficial) Scleritis (deep)

24. Anterior scleritis (98%) Posterior scleritis (2%) Necrotizing scleritis with inflammation Non-necrotizing scleritis (85%) (a) Diffuse (b) Nodular Inflammations of sclera EPISCLERITIS (SUPERFICIAL) SCLERITIS (DEEP) Simple episcleritis Nodular episcleritis Immune mediated Infectious Necrotizing scleritis w ithout inflammation ( S cleromalacia perforans )

25. Epis cleritis B enign , recurrent inflammation of the episclera , involving the overlying Tenon's capsule but not the underlying sclera Common Usually idiopathic Females > males Middle age EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

26. Simple episcleritis A ccounts for 75% of cases T endency to recur (60%) Features often peak within 24 hours , gradually fading over the next few days EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

27. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious Nodular episcleritis Less acute onset and a more prolonged course Red eye typically first noted on waking , over next 2-3 days area of redness enlarges and becomes more uncomfortable Tender red vascular nodule (Interpalpebral area) Underlying flat anterior scleral surface deep beam is not displaced above the scleral surface

28. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious T r e a tme n t If mild – No treatment, cold compress or refrigerated artificial tears Weak t opical corticosteroid eyedrops , four times a day 1-2 weeks Topical NSAID is an alternative, though maybe less effective Oral NSAID is occasionally required

29. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious SCLERITIS Oede ma and cellular infiltration of the entire thickness of sclera

30. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious Immune mediated scleritis Most common type Much less common than episcleritis Spectrum – from trivial and self limiting disease to a necrotizing process that can involve adjacent tissue and threaten vision

31. Non-necrotizing Diffuse Nodular superficial displacement of the entire beam EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

32. Non-necrotizing EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious Female > males 5 th decade Redness – bluish hue, vessels do not blanch with 10% phenylephrine, immobile Pain may radiate to face and temple, discomfort wakes patient in early hours of morning When edema/inflammation subsides affected area takes grey/bluish appearance Duration- avg - 6 years with frequent reccurences decreasing after first 18months Long term visual prognosis is good

33. Necrotizing scleritis EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious ○ Vaso-occlusive commonly associated with rheumatoid arthritis Isolated patches of scleral oedema with overlying non-perfused episclera and conjunctiva P atches coalesce, and if unchecked rapidly proceed to scleral necrosis ○ Granulomatous may occur in conjunction with conditions such as granulomatosis or polyarteritis nodosa Within 24 hours, the sclera, episclera , conjunctiva and adjacent cornea become irregularly raised and oedematous

34. ○ Surgically induced scleritis typically starts within 3 weeks of a procedure - strabismus repair, trabeculectomy and scleral buckling, and excision of pterygium with adjunctive mitomycin C. The necrotizing process starts at the site of surgery and extends outwards, but tends to remain localized to one sector. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

35. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious Scleromalacia perforans 5% of scleritis P rogressive scleral thinning without inflammation A ffects elderly women with longstanding rheumatoid arthritis (but has also been described in association with other systemic disorders) Symptoms Mild non-specific irritation pain is absent and vision unaffected Signs ○ Necrotic scleral plaques near the limbus without vascular congestion ○ Coalescence and enlargement of necrotic areas. ○ Slow progression of scleral thinning with exposure of underlying uvea

36. P osterior scleritis S clera behind the equator frequently misdiagnosed Choroidal folds at posterior pole, oriented horizontally Exudative RD EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

37. Fluid in the Tenon space may give a characteristic ‘T’ sign , the stem of the T being formed by the optic nerve and the cross bar by the fluid-containing gap EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious

38. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious Investigations Laboratory: ESR, CRP, ANA, C-ANCA, P-ANCA, CCP, syphilis serology, Lyme serology, hepatitis B surface antigen and antiphospholipid antibodies. Investigation for tuberculosis, sarcoidosis or ankylosing spondylitis Radiological imaging: Chest, sinus, joint and other imaging may be indicated in the investigation of a range of conditions such as tuberculosis, sarcoidosis, Churg–Strauss syndrome, Wegener granulomatosis, ankylosing spondylitis and other condition

39. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious • Topical steroids - do not affect the natural history of the scleral inflammation, but may relieve symptoms and oedema in non-necrotizing disease • Systemic NSAIDs should be used alone only in non-necrotizing disease • Periocular steroid injections may be used in non-necrotizing disease but their effects are usually transient; [ contraindicated in necrotizing scleritis] • Systemic steroids (e.g. prednisolone is 1–1.5 mg/kg/day) are used when NSAIDs are inappropriate or inadequate (necrotizing disease). Intravenous methylprednisolone may be used for emergent cases. • Immunosuppressives and/or biological blockers - if control is incomplete with steroids alone, as a steroid-sparing measure cyclophosphamide, azathioprine, methotrexate, ciclosporin, tacrolimus and others; in necrotizing disease, rituximab may be particularly effective. Treatment of immune-mediated scleritis

40. EPISCLERITIS Simple episcleritis Nodular episcleritis SCLERITIS Immune mediated Anterior - Non-necrotising - Necrotising with inflammation - Necrotising without inflammation ( scleromalacia perforans ) Posterior Infectious R are B ut may present diagnostic difficulty as the initial clinical features are similar to those of immune-mediated disease Causes • Herpes zoster is the most common infective cause. • Tuberculous scleritis is rare and difficult to diagnose. • Leprosy. • Syphilis. • Lyme disease. • Other causes include fungi ( Fig. 8.14D ), Pseudomonas aeruginosa and Nocardia. Treatment Once the infective agent has been identified, specific antimicrobial therapy initiated Topical and systemic steroids may also be used to reduce the inflammatory reaction If appropriate, surgical debridement - to debulk a focus of infection and facilitates the penetration of antibiotics Infectious scleritis

41. Thank you