Macular hole and retinal detachment in Best's disease

The authors report a family with Best's vitelliform macular dystrophy in which one member developed a macular hole and retinal detachment. The retinal detachment was repaired by resultant extensive rhegmatogenous pars plana vitrectomy, fluid-air exchange, and photocoagulation. Examination of the vitreous specimen disclosed numerous large macrophages with abundant cytoplasm distended by lipofuscin.