Clinicopathologic correlation of spontaneous retinal pigment epithelial tears with choroidal neovascular membranes in age-related macular degeneration.

BACKGROUND: A correlation of the clinical and histopathologic features of age-related macular degeneration (AMD), complicated by choroidal neovascular membrane (CNVM) with spontaneous tears of retinal pigment epithelial (RPE) detachment, has not been reported. METHODS: Two patients with vision loss from AMD and spontaneous pigment epithelial detachment tears, involving the fovea with serous or hemorrhagic neurosensory retinal detachments, were examined clinically. The surgically excised CNVM from each patient was examined with light microscopy. RESULTS: The authors present two patients with clinicopathologic correlation of the ophthalmoscopic, fluorescein angiographic, and pathologic findings of spontaneous pigment epithelial detachment tears. In the surgical specimens from these two eyes, the removed tissue, presumed to be the spontaneous RPE tear with CNVMs, demonstrated a fibrovascular membrane surrounding RPE with adjacent abnormally thickened basement membrane. The RPE-basement membrane complex was folded redundantly and scrolled beneath itself. CONCLUSION: The authors found RPE-basement membrane abnormalities and CNVMs associated with clinically evident spontaneous pigment epithelial detachment tears. These findings support the current theories of their pathogenesis.

kappa-Immunoglobulin corneal deposits associated with monoclonal gammopathy. Immunohistochemical and electron microscopic findings.

A 62-year-old man had a 1-year history of bilaterally decreased visual acuity associated with irregular, patchy, grayish-white stromal infiltrates involving both corneas. As visualized by light microscopy, the keratectomy specimen showed numerous homogeneous, eosinophilic deposits located mostly within keratocytes in the posterior one-third of the stroma. The deposits stained reddish-brown with Masson's trichrome but were periodic acid/Schiff-negative. As visualized by electron microscopy, the intracytoplasmic deposits were located within dilated cisternae of rough-surfaced endoplasmic reticulum of keratocytes and disclosed a 10-nm periodicity with a distinct lattice pattern. Immunoperoxidase strains confirmed that the deposits contained immunoglobulin (IgG-kappa). At 2 weeks following a histopathologic examination of the keratectomy specimen, serum protein analysis and immunoelectrophoresis with quantitation of immunoglobulins demonstrated an IgG-kappa monoclonal gammopathy. The abnormal corneal deposits may be the first clinical sign of a malignant lymphoproliferative process that may be associated with a monoclonal gammopathy.