The sclera is the dense outer layer of the eyeball. It can develop two types of inflammation - episcleritis and scleritis. Episcleritis is more common and self-limiting, often affecting young adults, while scleritis is more severe and can be associated with underlying systemic diseases. Scleritis presents with redness and swelling of the sclera and is classified as anterior or posterior depending on location. Imaging like ultrasound and CT are useful for diagnosing posterior scleritis. Treatment involves topical or oral steroids, with immunosuppressants used for severe or resistant cases. Complications can include scleral thinning and staphylomas if left untreated.

1. Sclera
Abdelmonem Hamed
Professor of Ophthalmology
Benha University

2. Special features of Sclera
• Avascular
• Dense fibrous tissue
• Lack of reaction to
insult
• Two types of
inflammation -
episcleritis and
scleritis

3. EPISCLERITIS AND SCLERITIS
1. Episcleritis
• Simple
• Nodular
2. Anterior scleritis
• Non-necrotizing diffuse
• Non-necrotizing nodular
• Necrotizing with inflammation
• Necrotizing without inflammation
( scleromalacia perforans )
3. Posterior scleritis

4. Applied anatomy of vascular coats
Scleritis
• Maximal congestion of
deep vascular plexus
• Slight congestion of
episcleral vessels
• Maximal congestion
of episcleral vessels
EpiscleritisNormal
• Radial superficial episcleral
vessels
• Deep vascular plexus
adjacent to sclera

5. Simple episcleritis
• Common, self-limiting, & recurrent
• Typically affects young adults
Treatment
• Seldom associated with a systemic disorder
Simple sectorial episcleritis Simple diffuse episcleritis
• Topical steroids
• Systemic Steroids if not responding to topical treatment

6. Nodular episcleritis
• Less common than simple episcleritis
• May take longer to resolve
• Treatment - similar to simple episcleritis
Localized nodule which can be moved over sclera

7. Causes of Scleritis:
1. Rheumatoid arthritis
• Wegener granulomatosis
• Polyteritis nodosa
• Systemic lupus erythematosus
2. Connective tissue disorders
3. Miscellaneous
• Relapsing polychondritis
• Herpes zoster ophthalmicus
• Surgically induced

8. Diffuse anterior non-necrotizing scleritis
• Widespread scleral and episcleral injection
• Relatively benign - does not progress to necrosis
• Oral steroids if unresponsive
Treatment
• Oral NSAIDs

9. Nodular anterior non-necrotizing scleritis
Scleral nodule cannot be moved over
underlying tissue
More serious than diffuse scleritis
On examination resembles
nodular episcleritis
Treatment - similar to diffuse non-necrotizing scleritis

10. Anterior necrotizing scleritis with inflammation
Progression
• Painful and most severe type
• Complications - uveitis, keratitis, cataract and glaucoma
Treatment
• Oral steroids
• Immunosuppressive agents (cyclosporin)
• Combined intravenous steroids and cyclophosphamide if unresponsive
Scleral necrosis and
visibility of uvea
Spread and coalescence
of necrosis
Avascular patches

11. • Asymptomatic and untreatable
• Associated with rheumatoid arthritis
Progressive scleral thinning with exposure of underlying uvea
Anterior necrotizing scleritis with inflammation
(sclerokeratitis)

12. Anterior necrotizing scleritis:
without inflammation
(Scleromalacia perforans)
 Asymptomatic
 Mainly in females with
longstanding RhA
 Large areas of uvea eventually
exposed
 Spontaneous perforation rare
 No effective treatment

13. Posterior scleritis
Signs
• About 20% of all cases of scleritis
• About 30% of patients have systemic disease
Choroidal folds Subretinal exudation
Proptosis and
ophthalmoplegia
Disc swelling Exudative retinal
detachment
Ring choroidal
detachment
• Decreased vision
• With or without pain

14. Differential Diagnoses
1. Allergic Conjunctivitis
2. Atopic Keratoconjunctivitis
3. Bacterial Conjunctivitis
4. Bacterial Keratitis
5. Epidemic Keratoconjunctivitis
6. Fungal Keratitis
7. Giant Papillary Conjunctivitis
8. Acute Angle-Closure Glaucoma
9. Herpes Simplex Virus Keratitis
10. Interstitial Keratitis
11. Keratoconjunctivitis Sicca
12. Superior Limbic Keratoconjunctivitis
13. Toxoplasmosis
14. Viral Conjunctivitis

15. Imaging in posterior scleritis
Ultrasound
a - Thickening of posterior scler
(flat tire sign)
b -Fluid in Tenon space (‘T’ sign)
Axial CT
Posterior scleral thickening
a
b
a

16. Treatment of necrotizing scleritis
 Oral prednisone 1mg/kg/day Or Pulsed IV
Methylprednisolone (solu-medrol 1 gm)
 Monitor pain in first 2-3 days
 Taper dose of steroids to response
 Immunosuppressives
 cyclosporine in steroid resistant cases
 Young people systemic NSAI.

17. 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

18. TYPES
• Anterior
• Intercalary
• Ciliary
• Equatorial
• Posterior

19. Anterior staphyloma
• Partial or total
• Mostly after sloughed
cornea
• AC becomes flat with
secondary glaucoma
• Iris is incarcerated in
anterior staphyloma

20. Intercalary staphyloma
• Limbus
• Root of iris and anterior most part of
ciliary body
• Externally from limbus to 2mm behind
• Caused by:
1. perforating injury at peripheral cornea
involving limbus,
2. marginal corneal ulcers,
3. anterior scleritis,
4. scleromalacia perforans,
5. complicated cataract surgery with
wound dehisence, secondary glaucoma

21. Ciliary staphyloma
• Affects ciliary zone - upto 8 mm
behind the limbus
• Scleral ectasia with
incarceration of ciliary body
• Caused by:
1. developmental glaucoma,
2. end stage of primary or sec
glaucoma,
3. scleritis,
4. trauma to ciliary region of eye

22. 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:
1. scleritis ,
2. chronic uncontrolled
glaucoma,
3. degenerative myopia

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

24. Treatment
• Treat the cause
• Small –local excision with
corneo-scleral graft
(tectonic graft)
• Large unsightly blind
eyes are enucleated and
replaced with implant

25. BLUE SCLERA
1. In children.
2. In high myopia.
3. In Buphthalmos.
4. Over a staphyloma.
5. Osteogenesis imperfecta
a syndrome of :
a) Deafness (otosclerosis).
b) Fragility of bones.
c) Blue sclera.