Congenital ventral hernia in association with focal dermal hypoplasia.

Focal dermal hypoplasia is a rare, X-linked dominant syndrome characterized by dysplasia of the skin, skeleton, and central nervous system. We report an infant who was born with severe focal dermal hypoplasia and an epigastric hernia. Operative timing and approach to abdominal wall defects in the presence of severe cutaneous dysplasia are discussed.