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Sclera
6th semester mbbs
Nischal Shrestha
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• Sclera forms posterior 5/6th opaque part of the external fibrous tunic of eyeball.
• Its whole outer surface is covered by Tenon’s capsule.
• In the anterior part , it is also covered by bulbar conjunctiva.
• Its inner surface lies in contact with choroid with a potential suprachoroidal space in between .
• In its anterior most part near limbus there is furrow which encloses the canal of schlemm.
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• Thickness: thin in children than adults. And females < males
• It is thickest posteriorly (1mm)and gradually becomes thin anteriorly.
• It is thinnest at the insertion of extraocular muscles (0.3 mm)
• Lamina cribrosa is a sieve-like sclera from which fibres of optic nerve pass.
• Sclera is thinnest at posterior to the attachment of superior rectus muscle.
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• Sclera is pierced by 3 sets of apertures (holes/gap):
1. Posterior aperture : situated around optic nerve and transmit long and short ciliary nerves and vessels.
2. Middle apertures (4 in number)are situated slightly posterior to the equator; through these pass the 4 vortex veins
(vena verticosae)
3. Anterior aperture are situated 3to4 mm away from limbus. Anterior ciliary vessels and branches from long ciliary
nerves pass through these.
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Microscopic structure
• Histologically, consists of 3 layers
1. Episcleral tissue: thin , dense vascularized layer of connective tissue which covers sclera proper. Fibroblasts ,
macrophages and lymphocytes are present here.
2. Sclera proper: avascular structure that contains collagen fibres.
3. Lamina fusca: innermost part which blends with suprachoroidal and supraciliary lamina of uveal tract. It is
brownish in color d/t presence of pigmented cells.
NERVE SUPPLY: supplied by branches from long ciliary nerves which pierce it 2-4 mm from limbus to form plexus.
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Inflammation of sclera
1. Episcleritis
2. scleritis
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Episcleritis
• Benign recurrent inflammation of episclera, involving the overlying Tenon’s capsule but not the underlying sclera.
• Affects young adults, women affected twice than men.
• Etiology :
idiopathic Systemic disease Hypersensitivity reaction infections
-Gout
-Psoriasis
-Rosacea
-Connective tissue
disorder
Endogenous tubercular
or streptococcal toxins
Herpes zoster virus
Syphilis
TB
Lyme disease
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Pathology
• There occurs localized lymphocytic infiltration of episcleral tissue associated with edema and congestion of overlying
Tenon’s capsule and conjunctiva.
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c/f
• Redness
• Mild ocular discomfort as gritty, burning or foreign body sensation
• Rarely mild photophobia and lacrimation
• Marked pain is absent
Symptoms
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Signs
• On examination, 2 clinical types of episcleritis may be recognized.
1. Simple episcleritis: characterized by sectorial (occasionally diffuse) inflammation of episclera. The engorged
episcleral vessels are large and run in radial direction beneath conjunctiva.
2. Nodular episcleritis: characterized by pink or purple flat nodule, usually situated 2-3 mm away from limbus. The
nodule is firm, tender ,can be moved separately from sclera and overlying conjunctiva also moves freely.
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Clinical course
• Limited course of 1o days to 3 weeks and resolves spontaneously.
• Recurrences are common.
• Rarely ,fleeting type of disease (episcleritis periodica) may occur. (fleeting= lasting for very short time)
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t/t
•
NSAIDs Artificial tears Cold compression Mild corticosteroid drops
Applied to closed lids
offer relief
Fluorometholone 2-3 hrly,
resolves it within a few days
0.5% carboxy
methyl cellulose
topical systemic
0.3%
ketorolac In recurrent cases,
e.g. indomethacin
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Scleritis
• Inflammation of the sclera proper.
• Comparatively serious disease which may cause visual impairment and even loss of eye if untreated.
• Incidence is much less than that of episcleritis.
• Usually occurs in elderly (40-70 years) involving female more than male.
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Etiology
• 50% cases are associated with some systemic diseases, most common being connective tissue diseases.
• Common conditions are:
1. Autoimmune collagen disorders, esp. rheumatoid arthritis, the most common association. About 0.5 % of pt. suffering from RA develop scleritis,
other disorders are Wegener’s granulomatosis, polyarteritis nodosa (PAN), SLE and ankylosing spondylitis.
2. Metabolic disorders like gout & thyrotoxicosis.
3. Some infections like herpes zoster ophthalmicus, chronic staph and strep infection
4. Granulomatosis diseases like TB, syphilis, leprosy , sarcoidosis
5. Irradiation, chemical burns, rosacea
6. Surgically induced scleritis (SIS), it occurs within 6 months postoperatively
7. Idiopathic. In many cases of scleritis, cause is unknown.
[nonpyogenic scleritis causes = TB, syphilis, leprosy]
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Pathology
• Histological changes are that of chronic granulomatous disorder.
• Fibrinoid necrosis, destruction of collagen together with infiltration of PMNs, lymphocytes, plasma cells &
macrophages.
• The granuloma is surrounded by multinucleated epithelioid giant cells
• vasculitis
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c/f
• Symptoms
- Pain :moderate to severe pain which is deep and boring type and wakes the patient early in morning . Ocular pain
radiates to jaw and temple.
- Redness may be localized or diffuse
- Photophobia and lacrimation
- Diminution of vision
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Signs
1. Non –necrotizing anterior diffuse scleritis:
- It is the commonest variety
- Widespread inflammation involving a quadrant (sector) or more of anterior
sclera
- Involved area is raised and pink to purple in color
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2. Non –necrotizing anterior nodular scleritis:
- Characterized by 1 or 2 hard, purplish elevated immovable scleral nodules (in
contrast to episcleritis , where it is movable), usually situated near limbus.
- Sometimes , nodules are arranged in a ring around limbus (annular scleritis)
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3. Anterior necrotizing scleritis with inflammation:
- It is acute severe form of scleritis
- Characterised by intense localized inflammation associated with areas of infarction d/t
vasculitis.
- The affected necrosed area is thinned out and sclera becomes transparent and ectatic with
uveal tissue shining through it.
- Usually associated with anterior uveitis.
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4. Anterior necrotizing scleritis w/o inflammation (scleromalacia perforans):
- Typically occurs in elderly females usually suffering from long standing RA.
- Characterised by development of yellowish patch of melting sclera (d/t obliteration of arterial supply)
- Melting sclera with overlying episclera and conjunctiva completely separates from surrounding normal sclera. 
becomes dead white eventually absorbs leaving behind it a large punched out area of thin sclera through which
uveal tissue shines.
- Spontaneous perforation is rare.
- t/t is ineffective!!!
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5. Posterior scleritis:
- Inflammation involving sclera behind equator
- The condition is frequently misdiagnosed
- Characterized by features of associated inflammation of adjacent structures, which include: exudative retinal
detachment, macular edema, proptosis and limitation of ocular movements.
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6. Infectious scleritis
• If purulent, suspect infectious
• Signs:
- Formation of fistula
- Painful nodules
- Conjunctival and scleral ulcers
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Complications
• Mostly with necrotizing
• Includes:
- Sclerosing keratitis
- Keratolysis
- Complicated cataract
- Secondary glaucoma
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t/t
•
Non-infectious
infectious
Antimicrobial both topical
and oral
Don’t give steroids
Surgical debridement
Non-necrotizing necrotizing
Topical steroid Topical steroid
Systemic
indomethacin 75mg
BD
Oral steroid
Scleral patch graft
Immunosuppressive like methotrexate or
cyclophosphamide in non responsive cases
No subconjunctival steroid
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Blue sclera
• Asymptomatic condition
• Generalized blue discoloration of sclera d/t thinning
• It is typically associated with osteogenesis imperfecta.
• Other causes are:
- Marfan’s syndrome
- Ehler –Danlos syndrome
- Buphthalmos
- High myopia
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Staphyloma
• Localised bulging of weak and thin outer tunic of eyeball (cornea or sclera), lined by uveal tissue which shines
through the thinned out fibrous coat.
• Types:
1. Anterior
2. Intercalary
3. Ciliary
4. Equatorial
5. Posterior
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Anterior staphyloma
• An ectasia of pseudocornea (the scar formed from organised exudates and fibrous tissue covered with epithelium)
which results after total sloughing of cornea, with iris plastered behind it is called anterior staphyloma.
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Intercalary staphyloma
• localised bulge in limbal area lined by root of iris
• Treatment consists of localised staphylectomy under heavy doses of oral steroids
8/14/2019 Nischal Shrestha 37
Ciliary staphyloma
• it is the bulge of weak sclera lined by ciliary body.
• It occurs about 2–3 mm away from the limbus
• Its common causes are thinning of sclera following perforating injury, scleritis and absolute glaucoma. ( episcleritis does
not cause this)
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Equatorial staphyloma
• It results due to bulge of sclera lined by the choroid in the equatorial region
• Its causes are scleritis and degeneration of sclera in pathological myopia
• Occurs more commonly at the regions of sclera which are perforated by vortex veins
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Posterior staphyloma
• It refers to bulge of weak sclera lined by the choroid behind the equator
• Here again the common causes are pathological myopia, posterior scleritis and perforating injuries.
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Sclera

  • 1. Sclera 6th semester mbbs Nischal Shrestha 8/14/2019 Nischal Shrestha 1
  • 5. • Sclera forms posterior 5/6th opaque part of the external fibrous tunic of eyeball. • Its whole outer surface is covered by Tenon’s capsule. • In the anterior part , it is also covered by bulbar conjunctiva. • Its inner surface lies in contact with choroid with a potential suprachoroidal space in between . • In its anterior most part near limbus there is furrow which encloses the canal of schlemm. 8/14/2019 Nischal Shrestha 5
  • 6. • Thickness: thin in children than adults. And females < males • It is thickest posteriorly (1mm)and gradually becomes thin anteriorly. • It is thinnest at the insertion of extraocular muscles (0.3 mm) • Lamina cribrosa is a sieve-like sclera from which fibres of optic nerve pass. • Sclera is thinnest at posterior to the attachment of superior rectus muscle. 8/14/2019 Nischal Shrestha 6
  • 7. • Sclera is pierced by 3 sets of apertures (holes/gap): 1. Posterior aperture : situated around optic nerve and transmit long and short ciliary nerves and vessels. 2. Middle apertures (4 in number)are situated slightly posterior to the equator; through these pass the 4 vortex veins (vena verticosae) 3. Anterior aperture are situated 3to4 mm away from limbus. Anterior ciliary vessels and branches from long ciliary nerves pass through these. 8/14/2019 Nischal Shrestha 7
  • 8. Microscopic structure • Histologically, consists of 3 layers 1. Episcleral tissue: thin , dense vascularized layer of connective tissue which covers sclera proper. Fibroblasts , macrophages and lymphocytes are present here. 2. Sclera proper: avascular structure that contains collagen fibres. 3. Lamina fusca: innermost part which blends with suprachoroidal and supraciliary lamina of uveal tract. It is brownish in color d/t presence of pigmented cells. NERVE SUPPLY: supplied by branches from long ciliary nerves which pierce it 2-4 mm from limbus to form plexus. 8/14/2019 Nischal Shrestha 8
  • 9. Inflammation of sclera 1. Episcleritis 2. scleritis 8/14/2019 Nischal Shrestha 9
  • 10. Episcleritis • Benign recurrent inflammation of episclera, involving the overlying Tenon’s capsule but not the underlying sclera. • Affects young adults, women affected twice than men. • Etiology : idiopathic Systemic disease Hypersensitivity reaction infections -Gout -Psoriasis -Rosacea -Connective tissue disorder Endogenous tubercular or streptococcal toxins Herpes zoster virus Syphilis TB Lyme disease 8/14/2019 Nischal Shrestha 10
  • 11. Pathology • There occurs localized lymphocytic infiltration of episcleral tissue associated with edema and congestion of overlying Tenon’s capsule and conjunctiva. 8/14/2019 Nischal Shrestha 11
  • 12. c/f • Redness • Mild ocular discomfort as gritty, burning or foreign body sensation • Rarely mild photophobia and lacrimation • Marked pain is absent Symptoms 8/14/2019 Nischal Shrestha 12
  • 13. Signs • On examination, 2 clinical types of episcleritis may be recognized. 1. Simple episcleritis: characterized by sectorial (occasionally diffuse) inflammation of episclera. The engorged episcleral vessels are large and run in radial direction beneath conjunctiva. 2. Nodular episcleritis: characterized by pink or purple flat nodule, usually situated 2-3 mm away from limbus. The nodule is firm, tender ,can be moved separately from sclera and overlying conjunctiva also moves freely. 8/14/2019 Nischal Shrestha 13
  • 15. Clinical course • Limited course of 1o days to 3 weeks and resolves spontaneously. • Recurrences are common. • Rarely ,fleeting type of disease (episcleritis periodica) may occur. (fleeting= lasting for very short time) 8/14/2019 Nischal Shrestha 15
  • 16. t/t • NSAIDs Artificial tears Cold compression Mild corticosteroid drops Applied to closed lids offer relief Fluorometholone 2-3 hrly, resolves it within a few days 0.5% carboxy methyl cellulose topical systemic 0.3% ketorolac In recurrent cases, e.g. indomethacin 8/14/2019 Nischal Shrestha 16
  • 18. Scleritis • Inflammation of the sclera proper. • Comparatively serious disease which may cause visual impairment and even loss of eye if untreated. • Incidence is much less than that of episcleritis. • Usually occurs in elderly (40-70 years) involving female more than male. 8/14/2019 Nischal Shrestha 18
  • 19. Etiology • 50% cases are associated with some systemic diseases, most common being connective tissue diseases. • Common conditions are: 1. Autoimmune collagen disorders, esp. rheumatoid arthritis, the most common association. About 0.5 % of pt. suffering from RA develop scleritis, other disorders are Wegener’s granulomatosis, polyarteritis nodosa (PAN), SLE and ankylosing spondylitis. 2. Metabolic disorders like gout & thyrotoxicosis. 3. Some infections like herpes zoster ophthalmicus, chronic staph and strep infection 4. Granulomatosis diseases like TB, syphilis, leprosy , sarcoidosis 5. Irradiation, chemical burns, rosacea 6. Surgically induced scleritis (SIS), it occurs within 6 months postoperatively 7. Idiopathic. In many cases of scleritis, cause is unknown. [nonpyogenic scleritis causes = TB, syphilis, leprosy] 8/14/2019 Nischal Shrestha 19
  • 20. Pathology • Histological changes are that of chronic granulomatous disorder. • Fibrinoid necrosis, destruction of collagen together with infiltration of PMNs, lymphocytes, plasma cells & macrophages. • The granuloma is surrounded by multinucleated epithelioid giant cells • vasculitis 8/14/2019 Nischal Shrestha 20
  • 22. c/f • Symptoms - Pain :moderate to severe pain which is deep and boring type and wakes the patient early in morning . Ocular pain radiates to jaw and temple. - Redness may be localized or diffuse - Photophobia and lacrimation - Diminution of vision 8/14/2019 Nischal Shrestha 22
  • 23. Signs 1. Non –necrotizing anterior diffuse scleritis: - It is the commonest variety - Widespread inflammation involving a quadrant (sector) or more of anterior sclera - Involved area is raised and pink to purple in color 8/14/2019 Nischal Shrestha 23
  • 24. 2. Non –necrotizing anterior nodular scleritis: - Characterized by 1 or 2 hard, purplish elevated immovable scleral nodules (in contrast to episcleritis , where it is movable), usually situated near limbus. - Sometimes , nodules are arranged in a ring around limbus (annular scleritis) 8/14/2019 Nischal Shrestha 24
  • 25. 3. Anterior necrotizing scleritis with inflammation: - It is acute severe form of scleritis - Characterised by intense localized inflammation associated with areas of infarction d/t vasculitis. - The affected necrosed area is thinned out and sclera becomes transparent and ectatic with uveal tissue shining through it. - Usually associated with anterior uveitis. 8/14/2019 Nischal Shrestha 25
  • 26. 4. Anterior necrotizing scleritis w/o inflammation (scleromalacia perforans): - Typically occurs in elderly females usually suffering from long standing RA. - Characterised by development of yellowish patch of melting sclera (d/t obliteration of arterial supply) - Melting sclera with overlying episclera and conjunctiva completely separates from surrounding normal sclera.  becomes dead white eventually absorbs leaving behind it a large punched out area of thin sclera through which uveal tissue shines. - Spontaneous perforation is rare. - t/t is ineffective!!! 8/14/2019 Nischal Shrestha 26
  • 28. 5. Posterior scleritis: - Inflammation involving sclera behind equator - The condition is frequently misdiagnosed - Characterized by features of associated inflammation of adjacent structures, which include: exudative retinal detachment, macular edema, proptosis and limitation of ocular movements. 8/14/2019 Nischal Shrestha 28
  • 29. 6. Infectious scleritis • If purulent, suspect infectious • Signs: - Formation of fistula - Painful nodules - Conjunctival and scleral ulcers 8/14/2019 Nischal Shrestha 29
  • 30. Complications • Mostly with necrotizing • Includes: - Sclerosing keratitis - Keratolysis - Complicated cataract - Secondary glaucoma 8/14/2019 Nischal Shrestha 30
  • 32. t/t • Non-infectious infectious Antimicrobial both topical and oral Don’t give steroids Surgical debridement Non-necrotizing necrotizing Topical steroid Topical steroid Systemic indomethacin 75mg BD Oral steroid Scleral patch graft Immunosuppressive like methotrexate or cyclophosphamide in non responsive cases No subconjunctival steroid 8/14/2019 Nischal Shrestha 32
  • 33. Blue sclera • Asymptomatic condition • Generalized blue discoloration of sclera d/t thinning • It is typically associated with osteogenesis imperfecta. • Other causes are: - Marfan’s syndrome - Ehler –Danlos syndrome - Buphthalmos - High myopia 8/14/2019 Nischal Shrestha 33
  • 34. Staphyloma • Localised bulging of weak and thin outer tunic of eyeball (cornea or sclera), lined by uveal tissue which shines through the thinned out fibrous coat. • Types: 1. Anterior 2. Intercalary 3. Ciliary 4. Equatorial 5. Posterior 8/14/2019 Nischal Shrestha 34
  • 36. Anterior staphyloma • An ectasia of pseudocornea (the scar formed from organised exudates and fibrous tissue covered with epithelium) which results after total sloughing of cornea, with iris plastered behind it is called anterior staphyloma. 8/14/2019 Nischal Shrestha 36
  • 37. Intercalary staphyloma • localised bulge in limbal area lined by root of iris • Treatment consists of localised staphylectomy under heavy doses of oral steroids 8/14/2019 Nischal Shrestha 37
  • 38. Ciliary staphyloma • it is the bulge of weak sclera lined by ciliary body. • It occurs about 2–3 mm away from the limbus • Its common causes are thinning of sclera following perforating injury, scleritis and absolute glaucoma. ( episcleritis does not cause this) 8/14/2019 Nischal Shrestha 38
  • 39. Equatorial staphyloma • It results due to bulge of sclera lined by the choroid in the equatorial region • Its causes are scleritis and degeneration of sclera in pathological myopia • Occurs more commonly at the regions of sclera which are perforated by vortex veins 8/14/2019 Nischal Shrestha 39
  • 40. Posterior staphyloma • It refers to bulge of weak sclera lined by the choroid behind the equator • Here again the common causes are pathological myopia, posterior scleritis and perforating injuries. 8/14/2019 Nischal Shrestha 40