Total vertex craniopagus with crossed venous drainage: Case report of successful surgical separation

Case report: Twin boys joined at the head in a total vertex configuration were born in Egypt in June 2001. At 12 months, they were transported to Dallas for evaluation and eventual surgical separation. In Dallas, a large multidisciplinary team of health care providers from two pediatric hospitals was assembled to care for the boys. Extensive radio-graphic evaluation revealed that the twins had essentially separate, well-formed brains, each with regions of schizencephaly. Each child's left cerebral hemisphere drained most of the venous blood to the right jugular system of the other. A detailed assessment of the foreseeable risks of surgical separation was then estimated and presented to the parents, as well as to the ethics committee of the two institutions. The decision was then made to proceed with separation. Surgical planning included the construction of multiple polymer models, and the design and construction of customized head holders and an operating table. Prior to separation a series of preparatory operations were performed to expand the scalp, as well as the fascia lata for dural grafting. At the age of 28 months, the twins were successfully separated during a 33-h operation. No attempt was made to reconstruct the dural venous sinuses. Scalp closure was adequate, requiring a split-thickness skin graft on one boy. Outcome: Postoperatively each child demonstrated an incomplete right hemiparesis. One twin required cerebral spinal fluid shunting. Neither child had a CSF leak or a CSF infection. At 6 months follow-up, both boys are rapidly acquiring speech in both English and Arabic, motor function is improving, and both are progressing toward independent ambulation.