Omphalocele and focal dermal hypoplasia.

A case of focal dermal hypoplasia associated with a giant omphalocele in a neonate is presented. The omphalocele was treated successfully by staged repair with Teflon mesh. The Teflon mesh was well incorporated into the fascial margins of the rectus muscle and the sac, and provided a stable abdominal wall for a long period of time. Focal dermal hypoplasia is a rare syndrome with widespread dysplasia of mesodermal and ectodermal tissues. Its most characteristic change in hypoplasia of the dermal connective tissue. From the experience of staged repair of omphalocele in this case, it seems that connective tissue reaction of the tissues is not deficient in this syndrome. This paper is the second case report of this syndrome with omphalocele in the world literature.