VITREOUS HEMORRHAGE AS AN OPHTHALMIC COMPLICATION OF GALACTOSEMIA

A major complication of galactosemia is cataracts. This is usually con sidered to be the sole ophthalmic feature of this disorder. However, w e have encountered vitreous hemorrhage, a very rare ophthalmic finding , in five neonates with galactosemia and have found four probable addi tional cases in the literature. All of these infants had severe neonat al manifestations of galactosemia and were discovered to have vitreous hemorrhage by ophthalmologic examination initiated by the observation of clouding of the eye or on a routine basis. The infants lost most o r all vision from the affected eye. Retinal abnormalities were present in the involved eyes of the five neonates of whom we have direct know ledge. Thus we believe that retinal hemorrhage is the most likely sour ce of the vitreous hemorrhage and that the coagulopathy associated wit h neonatal disease in galactosemia leads to vitreous hemorrhage. Promp t recognition and therapy for the coagulopathy would likely prevent vi treous hemorrhage in galactosemia.