EROSIVE VITREORETINOPATHY - A NEW CLINICAL ENTITY

Purpose: Vitreoretinopathies are disorders characterized by an abnorma l vitreous gel structure and associated retinal changes. The authors r eport a pedigree with vitreous changes characteristic of the vitreoret inopathies, but with retinal pigment epithelial changes, electroretino graphic abnormalities, and a clinical course distinct from previously described entities. Methods: Twenty-six family members were examined. Complete ophthalmologic examinations, electroretinography, and perimet ry were performed on patients who were at genetic risk for the disease . Particular attention was given to vitreous morphology and examinatio n of the retina and retinal pigment epithelium (RPE). Results: Fifteen individuals affected with an autosomal dominant vitreoretinal degener ation were identified. The disease is characterized by nyctalopia, pro gressive visual field loss, marked vitreous syneresis, progressive RPE atrophy, and combined traction-rhegmatogenous retinal detachments (11 patients). Thinning or ''erosion'' of the RPE in younger patients per mits increased visualization of the choroidal vessels. In advanced con ditions, equatorial areas are seen that appear clinically devoid of RP E, with extensive posterior atrophy in older patients. Diffuse rod-con e dysfunction is demonstrated by electroretinography. High myopia, epi physeal dysplasia, orofacial anomalies, and systemic manifestations ch aracteristic of other vitreoretinopathies are not present. Conclusion: The authors describe an entity clinically distinct from other vitreor etinopathies. The disease is characterized by pronounced vitreous abno rmalities, complicated retinal detachments, and a progressive pigmenta ry retinopathy. The most unusual and constant feature is the progressi ve change in RPE with concurrent visual field constriction and electro retinographic abnormalities. Because the RPE initially seems normal an d progressively thins or ''erodes'' in the equatorial periphery, the d escriptive name ''erosive'' vitreoretinopathy is proposed.