SEPARATION OF OMPHALOPAGUS TWINS - UNIQUE RECONSTRUCTION USING SYNGENEIC CRYOPRESERVED TISSUE

Omphalopagus twin girls were admitted for evaluation of possible separ ation and repair at age 7 days. Prenatal sonograph ic diagnosis occurr ed late in the third trimester and was followed by cesarean section de livery shortly thereafter. Results of extensive evaluation over the ne xt 7 days including x-rays, computed tomography and ultrasound scan of the head and torso, and cardiac catheterization showed: the gastroint estinal tracts were separate and normal, the livers were joined but ha d separate biliary and vascular systems, and the hearts were separate with vastly different anatomy and function. One twin (twin A) had a no rmal heart with a small insignificant VSD. Twin B had a single ventric le, an incompetent A-V valve, stenotic pulmonic valve, ASD, PDA, and c ongenital heart block. Hemodynamic support of twin B was almost entire ly from twin A. The vascular communications between the two consisted of a major connection between the internal mammary arteries and large arterial and venous connections traversing the joined livers. Because of continued deterioration of twin B, separation was undertaken at age 15 days. The separation included dividing the liver and the multiple large vascular connections. Two teams then reconstructed each twin sep arately. Twin B began showing signs of cardiac decompensation shortly after separation in spite of placement of a pacemaker, pulmonary arter y banding, and ligation of the PDA. Cardiac function rapidly deteriora ted and she died. Tissue from her chest wall was cryopreserved and pla ced in the tissue bank. Twin A underwent closure of her abdomen, and r eceived a temporary bovine pericardial patch over the chest defect. Sh e subsequently underwent placement of a graft of twin B's rib cage to bridge the bony chest defect and skin flap closure. She is presently t aking a normal diet and thriving at home at age 18 months. The use of cryopreserved tissue from a syngeneic source provides a unique method of reconstruction in this situation. Copyright (C) 1998 by W.B. Saunde rs Company.