CLINICAL COURSE OF OCULAR COMPLICATIONS OF ARACHNODACTYLY

Abstract

IT IS the general belief that the dislocations of the lens and the rigid iris seen in arachnodactyly are nonprogressive, congenital defects. Observations on 21 patients over periods of five to fourteen years and on 25 others for shorter periods have convinced me that many are true abiotrophies. While some patients complain only of reduced visual acuity, others show progressive dislocation of the lens, with uveal degeneration ending in detachment of the retina. Knowledge is far from complete, and only a single eye of this type has been examined in the laboratory. This article is offered in the hope of attracting other case reports. The disease is inherited as a dominant in many families and as a recessive in others. A parent with normal eyes and some of the skeletal defects may transmit the disease in complete form, and the same is true of a parent with normal skeleton and