Marfan-like syndrome with lens involvement. Hyaloideoretinal degeneration with anterior chamber angle, facial, dental, and skeletal anomalies

Abstract

An autosomal dominant syndrome with Marfan-like features was found in a black pedigree. Eye findings included cataract, lens colobomas, dislocated lenses, myopia, hyaloideoretinal degeneration, and abnormalities of the anterior chamber angles. Facial and dental anomalies included slightly underdeveloped bridge of the nose, protruding maxilla, and dental malocclusions. Selected members of this pedigree showed dolichestenomelia or arachnodactyly or both. Good visual prognosis resulted after (1) cataract or dislocated lens surgery and (2) prophylactic therapy of retinal holes and degeneration.