Sclera

Anatomy of The Sclera

Composed primarily of type I collagen and proteoglycans Avascular, execpt :
superficial vesels of the episclera intrascleral vascular plexus at posterior of the limbus

Anteriorly, consists of dense vascular connective tissue

Hypersensitivity Reaction and Ocular Disease

Type I ( Anaphylactic or Atopic Reactions)
An antigen binds with two adjacent IgE ---> release of histamine and other mediator

Type II (Cytotoxic Hypersensitivity)

Interaction of immunoglobines with antigens --> cell lysis by complement activation Killer cells are involved in antibodydependent cell cytotoxity

 Type III (Immune-Complex Reactions)

follows the deposition of antigen-antibody complexes in ocular tissue with complement activation and neutrophil recruitment

Type IV (Delayed Hypersensitivity)

Antigens inter act with receptors on the surface of T lymphocytes and result in the release of lymphokines

Hypersensitivity Reactions and Ocular Disease

Type I II III IV Ocular Disease Allergic conjungtivitis Atopic keratoconjungtivitis & dermatitis Ocular cicatrical pemphigoid Mooren ulcer Scleritis Contact dermatitis Phlyctenulosis

Inflammation of the Sclera

Episcleritis
benign inflammatory of the deep subconjungtival connective tissues occasionally fleeting, frequently repeated (episcleritis periodica fugax)

 E/ : allergic reaction to an endogenous toxin Clinical finding :

Dense lymphatic infiltration of the subconjunctival and episcleral tissues

 Little or no pain, usually there is a feeling of discomfort and tenderness on pressure, often severe neuralgia Treatment :
corticosteroid drops/ointment NSAID drops

 Scleritis
bilateral, occurred most frequently in women more rare than episcleritis may extend entirely round the cornea, forming a very serious condition known as annular scleritis sometimes extend to cornea causing slerosing keratitis E/: immune-mediated vasculitis that inflames and destroys the sclera associated with systemic disease :
SLE, polyarteritis nodosa

 Clinical presentation
The swelling is at first dark red or bluish, later it becomes purple and semi transparent Onset usually gradual over several days
Subtypes And Prevalence of Scleritis
Location Anterior sclera Subtype Diffuse scleritis Nodular scleritis Necrotizing scleritis with inflammation without inflammation Prevalence 40% 44% 14% (10%) (4%) 2%

Posterior sclera

 Diffuse or nodular anterior scleritis

characterized by scleral edema and redness zone scleral nodule is a deep red to purple colour, immobile, and separated from the overlying episcleral tissue which is elevated by the nodule

 Necrotizing scleritis
the most destructive form a localized patch of inflammation with the edge of lesion more inflamed than the center sclera becomes blue-gray appearance and an altered deep episcleral blood vessel pattern

 Necrotizing scleritis without signs of inflammation (scleromalacia perforans)

in patiens with long-standing rheumatoid arthritis (55% of cases) signs are minimal and generally no pain sclera thins and the underlying dark uveal tissue become visible --> uvea is covered with only thin connective tissue and conjunctiva

 Posterior scleritis

occur in isolation or concomitant with anterior scleritis signs are pain, tenderness, proptosis, visual loss and restricted motility, retraction of the lower eyelid caused by infiltration of muscle in the region of posterior scleritis pain maybe reffered to other other parts of head --> D/ can be missed

Complications of scleritis
Complication are frequent and include :
peripheral keratitis (37%) uveitis (30%) cataract (7%) glaucoma (18%) scleral thinning (33%)

anterior uveitis as spillover phenomenon : anterior scleritis posterior uveitis : ant and post scleritis

Management of Scleritis
In mild cases of diffuse anterior & nodular scleritis : topical corticosteroid NSAID (indomethacine, naproxen, diclofenac, etc) are also effective Oral and high dose IV steroid may be effective in necroting sleritis sclerokeratitis

Congenital Anomalies of Sclera

Blue Sclera
Related to generalized thinning with increased visibility of underlying uvea Syndromes associated with :
Osteogenesis imperfecta type I Ehlers-Danlos syndrome type VI (EDS VI) EDS VI with normal lysyl hydroxylase

 Clinical findings : All three syndrome share each other Management : regular hearing evaluation after adolescence

Degenerative Process of the Sclera

Senile Plaques
E/:
scleral reigidity decrease of scleral hydration decrease of mucopolysaccharide

Accompanied by subconjungtival deposition of fat The plaques do not elicit inflammation and rarely extrude