Criswick-Schepens syndrome (familial exudative vitreoretinopathy). Study of a Colombian kindred.
Ophthalmoscopic evidence of Criswick-Schepens syndrome was found in nine of 22 members of a Colombian family. Histopathologic study of an affected eye enucleated because of neovascular glaucoma showed a focal, nodular zone of fibrovascular proliferation, necrosis, and acute inflammation within the temporal preequatorial retina associated with dense preretinal fibrous organization. Although the cause of the inflammation and cicatrization is unknown, such a nidus may explain some of the advanced clinical manifestations of the syndrome, including temporal dragging of the retina and falciform retinal fold.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003105:Colombia; D005123:Eye / Q000473:pathology; D005128:Eye Diseases / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D009887:Ophthalmoscopy; D010375:Pedigree; D012164:Retinal Diseases / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D013577:Syndrome; D014822:Vitreous Body