Temporoparietal craniopagus. Case report and review of the literature.

A case of craniopagus twins joined in the temporoparietal area is presented, along with a review of the literature on craniopagus. A large area of brain was shared between the neurologically normal infants, with defects in the scalp, skull, and dura. The twins were separated in a three-step procedure. First, areas of shared brain were divided and separated with silicone sheets. The second procedure consisted of the insertion of scalp expanders to allow primary skin closure. In the third procedure complete separation was performed which was complicated by severe hypotension in one infant that was due to dural sinus hemorrhage. Cerebrospinal fluid leak was the most difficult problem encountered in the postoperative period; this was treated with lumboperitoneal and ventriculoperitoneal shunts. After 2 years, one twin is neurologically normal; the other is severely developmentally delayed, possibly related to the severe hypotension experienced during the third procedure. A review of the literature on craniopagus is presented. Analysis of data in the literature suggests that the area involved in the craniopagus as well as the venous connections are closely related to survival following separation of craniopagus twins.