Persistent tunica vasculosa lentis as a sign of congenital myotonic dystrophy.

We describe the ocular findings in eight patients with congenital myotonic dystrophy in the newborn period. While three infants had normal findings, five infants had evidence of persistent tunica vasculosa lentis (TVL) at gestational ages ranging from 34 to 40 weeks. Atrophy of vessels on the anterior lens capsule is normally complete by 34 weeks gestation and its persistence should suggest congenital infection or congenital myotonic dystrophy. Diagnosis of myotonic dystrophy has serious implications for the baby and the mother, in whom the condition may have not been recognized earlier.