ABSTRACT
To the examining physician, the vitreous displays such an inert appearance that it is difficult to consider the possibility of serious pathologic activity occurring in its lowmetabolic-activity, low-cellularity, sticky fluid. However, about a decade after Jules Gonin discovered the role of retinal holes in causing retinal detachments, the first report of an inherited vitreoretinal degeneration was published (1). Wagner (1) described an autosomal dominant disease affecting the vitreous, retina, and lens in a Swiss family and named it hyaloideoretinal degeneration. Later, it became apparent that three different hereditary forms of hyaloideoretinal degenerations occur, namely, autosomal dominant, autosomal recessive, and sex-linked (2). Still later, the occasional association with extraocular abnormalities such as abnormalities of the joints and skeleton (3), cleft palate (4), and generalized connective tissue disease was found (5). In fact, the autosomal dominant isolated formofWagner (1) seemed to bemuch less common than the autosomal dominant formassociatedwith a generalized connective tissue disorder. In addition, several ‘‘new’’ forms of an isolated vitreoretinal degeneration were described, which did not fit into any of the known forms.
