Technical Considerations in Liver Transplantation for Biliary Atresia With Situs Inversus

In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. We evaluated the surgical procedures and the outcomes in our patients and in the previously reported patients undergoing LT for BA with SI. Between November 2005 and October 2018, 235 children underwent LT with an overall 10-year graft survival of 94.7%. Of these, 6 (2.6%) patients received LT for BA with SI. Living donor liver transplantation (LDLT) was applied in all patients. Vascular anomalies at our center included the absence of the inferior vena cava (IVC; n = 5), a preduodenal portal vein (PV; n = 4), and an aberrant hepatic artery (HA; n = 1). Hepatic vein (HV) outflow obstruction occurred in 1 patient with an absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in 1 patient with a preduodenal PV at 5 months after LDLT. The overall survival of our patients was 80.0% at 3 years. In the literature, 49 patients with LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction (2.0%), PV complications (10.2%), and HA thrombosis (4.1%). All PV complications occurred in LDLT patients with a preduodenal PV (5/23; 21.7%). In conclusion, the detailed assessment of the surgical procedures and the systematic literature review demonstrated excellent outcomes after LT for BA with SI. Liver recipients with a preduodenal PV are exposed to a higher risk for vascular complications after a very challenging LT procedure with overall good outcomes.