INFLAMMATORY DISEASES

Cytomegalovirus retinitis. Cytomegalovirus retinitis may cause focal full-thickness necrosis of the retina (bracket). Note the abrupt transition between the

normal retina and the area of retinitis. The characteristic histopathologic finding is the cytomegalo cell (inset), with eosinophilic intranuclear (arrowhead) and intracytoplasmic (arrow) viral inclusions.

Endogenous fungal endophthalmitis. The chorioretinitis in these cases is often posterior and multifocal. In this fungal chorioretinal abscess, there is full-thickness retinal inflammation with necrosis, intraand subretinal hemorrhage, focal retinal pigment epithelial detachment by inflammatory cells (arrows), and a dense infiltrate of acute and chronic inflammatory cells (including granulomatous inflammation) within the choroid. Special stains for fungi (Gomori’s methenamine silver) show numerous branching, septated fungal hyphae (inset) consistent with Aspergillus sp.

Sympathetic ophthalmia. In this condition, the choroid is often markedly thickened, with a dense infiltrate of chronic inflammatory cells including epithelioid cells (asterisks), multinucleated giant cells (arrowheads), and numerous lymphocytes. The chronic inflammatory reaction may also involve the ciliary vessels and nerves (arrow) and the retinal vasculature. Note the intact choriocapillaris beneath the retinal pigment epithelium.

Sympathetic ophthalmia. Granulomatous inflammation is characteristic of this ocular disease. A Dalen-Fuchs nodule is an aggregate of epithelioid cells and/or multinucleated giant cells (open arrows) interposed between the retinal pigment epithelium (arrows) and Bruch’s membrane (arrowheads). Note the dense infiltrate of histiocytes in the choroid (asterisk).

Ocular toxoplasmosis. In human immunodeficiency virus infection, Toxoplasma gondii may produce a necrotizing retinitis and granulomatous choroiditis. Note the extensive necrosis of the retina and retinal pigment epithelium, with loss of the normal lamellar architecture of the retina. A T gondii tissue cyst (arrow, inset) is present. Marked chronic granulomatous inflammation with epithelioid and multinucleated giant cells (arrowheads) are present in the choroid, with loss

of the choriocapillaris.

Vascular sheathing. In various local and systemic diseases (including central retinal vascular occlusion, sympathetic ophthalmia, collagen vascular disease, and sarcoidosis), inflammatory cells may surround or enter the retinal blood vessel walls. Note the chronic inflammatory cell infiltrate (arrow) within the wall of this branch retinal vein and the spillover of inflammatory cells onto the internal limiting membrane (arrowhead).

Child abuse (shaken baby syndrome). Ophthalmic examination in the live patient and pathologic examination of the eyes post mortem may be critical in establishing the diagnosis of child abuse. At times, the only pathologic findings at autopsy are observed in the eyes. Careful examination of the optic nerve sheath in cross section may reveal blood in the subdural and arachnoid spaces (arrow). Crater-like retinal folds (arrowhead) in the macular region are virtually pathognomonic of shaken baby syndrome and are often associated with sub-internal limiting membrane (ILM) hemorrhages. Scattered blot and sub-ILM hemorrhages (open arrow) may be observed in the midperipheral and peripheral retina.

Papilledema. Histologically, papilledema is characterized by lateral displacement of the juxtapapillary retina from the optic nerve head by swollen nerve fiber bundles (between brackets) and by thickening or swelling of the nerve fibers in the anterior aspect of the optic nerve head (asterisks). Note the associated intraretinal (arrow) and nerve fiber layer (arrowhead) hemorrhages and trace vitreous hemorrhage (open arrow) in this case of shaken baby syndrome. Similar pathologic findings may be observed in other causes of papilledema, including pseudotumor cerebri, central nervous system mass lesions, and malignant hypertension.

Sub-internal limiting membrane (ILM) hemorrhage. This type of retinal hemorrhage most often appears clinically as a large blot hemorrhage and occasionally as a boatshaped hemorrhage. Note the large amount of blood (asterisk) beneath the retinal ILM (arrows). A dense hemorrhage is also present in the outer plexiform layer

(O) in this case of shaken baby syndrome.

Traumatic retinopathy (pseudo-retinitis pigmentosa [RP]). Traumatic retinopathy and RP share similar histologic findings, including marked loss of the photoreceptor cell layer; focal gliosis (asterisk); pigment migration into the retina, often around blood vessels (arrows); variable retinal pigment epithelial attenuation, hypertrophy, and hyperplasia; and drusen formation (arrowhead). Note also the loss of the normal lamellar architecture of the retina and indistinct choriocapillaris.

Lattice degeneration of the retina. This common peripheral retinal degeneration is characterized histologically by liquefied vitreous (asterisk) over the area of retinal degeneration bordered by condensed vitreous (arrows). The inner retina is primarily affected. An inner lamellar hole with an operculum (beneath asterisk) and a thin outer retinal membrane (open arrow) can progress to an atrophic full-thickness hole.

Typical peripheral cystoid degeneration of the retina with focal retinoschisis. This peripheral retinal degeneration is almost universally present in autopsy and enucleated eyes of patients over 8 years old. It begins near the ora serrata with cysts in the outer plexiform and inner nuclear layers. These cysts may coalesce to form areas of retinoschisis (asterisk).

Operculated retinal tear. These tears in the peripheral retina carry a low risk for progression to retinal detachment. Note the rounded, gliotic margins of

the full-thickness retinal defect. Localized subretinal fluid (asterisk) and focal retinal pigment epithelial hyperplasia (arrowhead) are present. The inset shows an operculum with internal limiting membrane (arrow) and neuroglia, which was observed a short distance from the retinal hole in this photomicrograph.

Retinal detachment (RD) with proliferative vitreoretinopathy (PVR). Retinal detachment may be complicated by PVR. In PVR, preretinal and subretinal fibroglial and retinal pigment epithelial

proliferation may be observed. Note the open funnel, total RD with retinal folds, subretinal fibroglial strands (arrowheads), and a subretinal napkin-ring membrane (arrows). The subretinal napkin-ring membrane (inset) is composed of fibroglial tissue (asterisk) with hyperplastic retinal pigment epithelium (open arrow).

SELECTED REFERENCES

1.Eagle RC Jr. Eye Pathology: An Atlas and Basic Text.

Philadelphia: WB Saunders Co; 1999.

2.Sassani JW, ed. Ophthalmic Pathology With Clinical Correlations. Philadelphia: Lippincott-Raven Publishers; 1997.

3.Spencer WH, ed. Ophthalmic Pathology: An Atlas and Textbook. 4th ed. Philadelphia: WB Saunders Co; 1996.

4.Yanoff M, Fine BS. Ocular Pathology. Barcelona: MosbyWolfe; 1996.