Retinal neovascularization in Potter's syndrome secondary to renal agenesis

Abnormalities of retinal vascularization were observed in a male stillborn at 33 weeks gestation with Potter's syndrome secondary to renal agenesis. Each eye had a persistent tunica vasculosa lentis and hyaloid artery as well as enlarged intraretinal vessels and a sharply demarcated avascular peripheral retina. Light microscopy revealed marked proliferation of capillaries and mesenchymal spindle cells of the retina. Electron microscopy of the retinal vascular endothelial cells revealed intact tight junctions with no fenestrations. Spindle cells had gap and intermediate junctions that appeared normal in number and morphology.