Retinal involvement in familial exudative vitreoretinopathy

Abstract

34 cases of familial exudative vitreoretinopathy were seen during a 40-month period ending April 1981. There were 30 cases from 11 families and 4 sporadic cases. The familial cases showed the mode of inheritance compatible with the autosomal-dominant inheritance. Ophthalmoscopy and super-wide panoramic fluorescein angiography revealed characteristic retinal vascular abnormalities in all cases, embracing supernumerous vascular branchings, arteriovenous shunt formation in the extreme periphery, generalized hyperpermeability of vessels and incomplete arteriovenous interdigitating pattern. Vitreoretinal adhesion was present in the peripheral temporal retina in all cases. An avascular zone wider than 1 disc diameter was noted in the majority of cases in the extreme temporal periphery. A V-shaped retinal degeneration was seen along the temporal meridian in 18 cases (48%). Retinal detachment was present in 19 eyes of 14 cases, and it was rhegmatogenous in 11 and traction-induced in 8 eyes. Retinal exudates and total posterior vitreous detachment was seen only occasionally.