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