Wagner described 13 affected individuals in a three-generation pedigree with autosomal dominant inheritance, low myopia, fluid vitreous, cortical cataract, and variably affected dark adaptation. The cardinal features noted were the complete absence of the normal vitreal scaffolding and preretinal, equatorial, and avascular greyish-white membranes. Rhegmatogenous retinal detachment was not originally reported. There are no associated systemic features. Twenty-eight members of the original pedigree have been examined  and four patients had a history of a rhegmatogenous retinal detachment in one eye at a median age of 20 years and 55 % of patients older than 45 years had peripheral tractional retinal detachments. Chorioretinal atrophy and cataract increased with the patients' age. Several families with Wagner syndrome, including the original pedigree, have been linked to 5q14.3.
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