A case of erosive vitreoretinopathy

ABSTRACT

Hereditary vitreoretinopathies are potentially blinding inherited disorders characterized by an abnormal-appearing vitreous gel and associated retinal changes. Four of these disorders, Stickler's syndrome, Wagner's disease, erosive vitreoretinopathy, and Goldmann-Favre syndrome, exhibit marked syneresis of the vitreous gel. Erosive vitreoretinopathy has associated retinal pigment epithelial changes, poor night vision, visual field defects, and abnormal electroretinographic findings; symptoms not found in Stickler's syndrome. A 36-year-old man with progressive visual loss and a visual field defect had no systemic disease. His vitreous cavity was liquefied. Vitreous strands and a cataract were found in both eyes. Pronounced RPE degeneration was found superotemporally in both eyes and a bullous rhegmatogenous retinal detachment in the left eye accompanied two retinal tears. His visual field showed a ring scotoma in both eyes and the ERG finding was abnormal. We report one case of erosive vitreoretinopathy with retinal pigment epithelial changes, rhegmatogenous retinal detachment, visual field defects, abnormal electroretinographic findings, marked vitreous syneresis and cataract. These symptoms are distinct from previously described entities.