Orthotopic liver transplantation in patients with biliary atresia and situs inversus

Situs inversus (SI) and the polysplenia syndrome (PS) occur relatively freq uently in patients with biliary atresia, the largest subgroup of pediatric liver transplantation patients. We present two cases of orthotopic liver tr ansplantation (OLT) in pediatric patients with SI. One had SI totalis, wher e a normal liver was placed in the left upper quadrant (LUQ) with the right lobe overlying the vertebral column. The second had PS and isolated SI of the liver, and a living-related left-lateral-segment graft was placed in th e LUQ. Although multiple, often unpredictable vascular and intestinal anoma lies occur frequently in association with SI, particularly in the setting o f the PS, these cases, as well as several others recently reported, reveal that these anomalies can be managed successfully by a variety of technical modifications of the standard OLT technique. Likewise, concerns about the p lacement of a situs solitus liver in the midline or LUQ position of a SI ab domen have proved to be unfounded.