Parasitic omphalopagus complicated by omphalocele and congenital heart disease.

Conjoined twins occur in approximately one of every 50,000 to 200,000 births. Atypical or parasitic conjoined twins result from the embryonic death of one twin, which leaves behind body parts vascularized by the primary twin. Omphalopagus refers to conjoined twins joined at the level of the umbilicus. The authors report a case of atypical omphalopagus involving an acardiac, acephalic, parasitic twin and a host twin with a large omphalocele, transposition of the great arteries, and aortic coarctation. The authors briefly review reported cases of parasitic omphalopagus and emphasize the role of intensive neonatal care, preoperative planning, and staged surgical intervention in the successful management of complicated variants.