Ординатура / Офтальмология / Английские материалы / Ocular Pathology_6th edition_Yanoff, Sassani_2009

4.Di use anterior scleritis is one of the very few severely painful eye conditions.

The boring pain may be localized to the eye or generalized, usually in the distribution of the second and third branches of the trigeminal nerve.

5.As in all forms of scleritis, scleral edema and inflammation are present.

a.The diagnostic features di erentiating it from episcleritis are the outward displacement of the deep vascular network of the episclera and the typical blue-red color.

b.A small area or the whole anterior segment may be involved.

B.Nodular

1.Nodular anterior scleritis is most prevalent in both women and men from the fourth to sixth decades, but in women a noticeable peak occurs in the sixth decade.

Nodular scleritis can be considered of intermediate severity between diffuse and necrotizing disease.

2.Approximately half of the patients have bilateral involvement.

3.The pain is as described in di use anterior scleritis.

4.The nodule, unlike the one in nodular episcleritis, is deep red, totally immobile, and quite separate from the overlying congested episcleral tissues.

Rarely, biopsy of such nodule may be diagnostic of sarcoidosis.

C.Necrotizing—with inflammation (most severe form of scleritis)

1.Necrotizing anterior scleritis with inflammation mostly occurs in women.

2.Approximately half of the patients have bilateral involvement.

3.The pain is as described for the di use form except that it is the most severe type of ocular pain.

4.It is the most destructive form of scleritis, with over

60% of eyes experiencing complications other than scleral thinning and 40% losing visual acuity.

a.The patients may present with severe edema and acute congestion (brawny scleritis) or a patch of avascular episcleral tissue overlying or adjacent to an area of scleral edema.

b.In some cases, the inflammation remains localized to one small area and may result in almost total loss of scleral tissue from that area.

c.Most often, the inflammation starts in one area and then spreads circumferentially around the globe until the whole of the anterior segment is involved.

D.Necrotizing—without inflammation (scleromalacia perforans)

1.Necrotizing anterior scleritis without inflammation mostly a icts women.

2.Approximately half of the patients have bilateral involvement.

3.Patients rarely complain of pain in scleromalacia perforans and present without subjective symptoms.

4.A grayish or yellowish patch on the sclera, without inflammation, may progress to complete dissolution of sclera and episclera, covered by a thin layer of conjunctiva.

II.Posterior scleritis

A.Posterior scleritis and anterior scleritis are usually associated, and occur most frequently in women in their sixth decade.

Approximately 30% of patients who have a posterior scleritis have an associated systemic disease, such as various types of vasculitis, autoimmune disease, and lymphoma.

B.Most patients have unilateral involvement.

C.The pain is as described for di use anterior scleritis.

D.Proptosis, exudative detachment, and other fundus changes such as optic disc edema may be seen in addition to anterior scleritis.

E.Posterior scleritis in a nodular configuration may simulate choroidal neoplasm.

Ultrasonography is most helpful in the diagnosis.

III.Complications

A.A decrease in visual acuity (14%) may result from keratitis, cataract, anterior uveitis, or posterior uveitis.

B.Keratitis (29%)

1.Di use anterior scleritis

a.Localized stromal keratitis

b.Localized sclerosing keratitis

2.Nodular anterior scleritis

a.Acute stromal keratitis

b.Sclerosing keratitis

c.Corneal gutter

3.Necrotizing scleritis

a.Sclerosing keratitis

b.Keratolysis

C.Corneal vascularization (9%)

D.Cataract (7%)

E.Uveitis (30%)

F.Glaucoma (12%)

G.Scleral thinning and scleral defects (perforation of the globe is rare except after subconjunctival steroid injection)

1. Spontaneous rupture of a posterior staphyloma has

been reported.

IV. Associated systemic diseases

A.Almost half of the patients with scleritis have a known associated systemic disease, approximately 15% of which represent connective tissue diseases. Almost 80% of the associated systemic diseases are known prior to the onset of the initial scleritis.

Scleromalacia perforans is associated with long-standing rheumatoid arthritis in approximately 46% of patients. The connective tissue diseases are most prevalent in necrotizing anterior scleritis with inflammation. Twenty-one percent of patients with necrotizing anterior scleritis with inflammation, which is probably the malignant phase of systemic connective tissue disease, die within 8 years of diagnosis.

B.Other associated systemic diseases include hypersensitivity disorders (e.g., erythema nodosum, asthma, erythema multiforme, contact dermatitis, Wegener’s granulomatosis; Fig. 8.59, and see p. 184 in Chapter 6), polychondritis, Goodpasture’s syndrome, granulomatous conditions (e.g., tuberculosis, syphilis), viral and bacterial infection (e.g., herpes zoster, HSV, Pseudomonas), porphyria, and metabolic disorders (e.g., gout).

C.Systemic diseases, such as leukemia, may mimic scleritis.

V.Histology—the basic lesion is a granulomatous inflammation surrounding abnormal scleral collagen.

A.Vasculitis with fibrinoid necrosis and neutrophil invasion of the vessel wall are present in 75% of scleral and 52% of conjunctival specimens.

Vascular immunodeposits are present in 93% of scleral and 79% of conjunctival specimens.

B.In the conjunctiva, there are increased T cells of all types, macrophages, and B cells.

C.In the sclera, increased T cells of all types and macrophages are seen.

D.Increased HLA-DR expression is markedly increased in both conjunctiva and sclera.

TUMORS

Fibromas

See discussion of mesenchymal tumors in subsection Primary Orbital Tumors, Chapter 14.

Nodular Fasciitis

See discussion of mesenchymal tumors in subsection Primary

Orbital Tumors, Chapter 14.

Hemangiomas

See discussion of mesenchymal tumors in subsection Primary Orbital Tumors, Chapter 14.

Neurofibromas

See discussion of mesenchymal tumors in subsection Primary Orbital Tumors, Chapter 14.

Contiguous Tumors

Conjunctival Tumors

II. Uveal malignant melanoma

Episcleral Osseous Choristoma and Episcleral

Osseocartilaginous Choristoma

I.The tumor (Fig. 8.60) is typically present between the lateral and upper recti.

II.It is symptomless, is present at birth, and characteristically contains bone.

III.Histologically, normal-appearing bone is seen in the abnormal episcleral location.

IV. The di erential diagnosis includes classical limbal dermoids, epithelial inclusion cysts, prolapsed orbital fat, papillomas, dermolipomas, and complex choristomas.

Bone formation occurs through the condensation of mesenchyme in two ways: (1) membranous bone forms from mesenchymal condensation directly without first forming cartilage (e.g., many skull bones and intraocular ossification); and (2) bone forms from mesenchymal formation of cartilaginous template (e.g., ribs)— both types of bone formation occur in episcleral osseous choristoma and episcleral osseocartilaginous choristoma.

A

C

Ectopic Lacrimal Gland

See p. 543 in Chapter 14.

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