Diabetic retinopathy in an adolescent with Prader-Labhart-Willi syndrome.

The Prader-Labhart-Willi syndrome is characterized by perinatal muscular hypotonia, short stature, mental retardation, hypogonadism (cryptorchidism in males), and severe, progressive obesity. Another feature of this syndrome is the development of glucose intolerance, followed by overt juvenile diabetes mellitus of the maturity onset type (Illig, Ischymi, and Vischer, 1974). Esotropia and amblyopia are also common (Dunn, 1968). The aetiology of this syndrome is not clear, but the existence of a hypothalamic lesion is assumed (Hetherington and Ranson, 1942; Larsson, 1954). The appearance of diabetic retinopathy in long-standing diabetes is well known but its association with this syndrome has not been reported.