ABSTRACT
A family of five with the Wagner-Jansen Syndrome is presented including a father, his two sons and two daughters. In all eyes the vitreous cavity was optically empty with thick condensations behind the lens. The following fundus changes were noted: retinal pigmentation [3 eyes], lattice-like changes [3 eyes], sheathing of the retinal vessels [6 eyes], peripheral vascular abnormalities [2 eyes], white without pressure [2 eyes], snowflake deposits [6 eyes], retinal breaks without retinal detachment [3 eyes] and retinal detachment [3 eyes of 2 patients]. Myopia was found in 9 eyes and whitish punctate densities of the lens were found in all cases. The inheritance pattern was autosomal dominant. The main cause of blindness in this syndrome is retinal detachment that responds poorly to surgical procedures. The prophylactic treatment of retinal breaks is useful. The syndromes of Wagner, Jansen and Stickler are all associated with a combination of an optically empty vitreous cavity, sparse intravitreal membranes and strands, and lattice-like changes in the retina. Retinal detachment was not reported in the family described by Wagner but was described by Jansen in a pedigree with ocular finding but no systemic abnormalities. The distinctive vitreoretinal abnormalities, however, probably occur most frequency in families with various systemic abnormalities, most commonly referred to as the Stickler syndrome or hereditary arthro-ophthalmopathy