Ann Liver Transplant.  2021 Nov;1(2):165-173. 10.52604/alt.21.0021.

Emergency living donor liver transplantation under extracorporeal membrane oxygenation in an infant with biliary atresiapolysplenia syndrome

• Namgoong JM
• Hwang S
• Park GC
• Kwon H
• Kim KM
• Oh SH

• KMID: 2560868
• DOI: http://doi.org/10.52604/alt.21.0021

Abstract

Biliary atresia-polysplenia syndrome (BAPS) is diagnosed in a small number of patients with biliary atresia (BA). We present a case of emergency living donor liver transplantation (LT) successfully performed in an infant with BAPS undergoing extracorporeal membrane oxygenation. The recipient was a 10-month-old boy who did not undergo Kasai portoenterostomy due to rapid progression of liver cirrhosis. Co-existing malformations included heterotopic inferior vena cava without hepatic communication, direct hepatic vein drainage into the right atrium, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. Patient condition deteriorated rapidly after pulmonary hemorrhage, and thus emergency living donor LT was performed after starting veno-venous extracorporeal membrane oxygenation (ECMO) with a pediatric end-stage liver disease score of 32. A left lateral section graft obtained from his father showed a graft-to-recipient weight ratio of 3.2%. The recipient surgery was performed according to standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Multiple portal collateral veins were ligated and intraoperative portography was performed to secure portal vein inflow. The patient was weaned off ECMO and ventilator were weaned off on 17 days and 65 days respectively after transplantation. The patient stayed at the intensive care unit for 3 months before and after transplantation. Our pediatric patient with BAPS manifested various anatomical malformations. Successful LT requires comprehensive preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring.

Keyword

Preduodenal portal vein; Inferior vena cava; Intestinal malrotation; Vascular complication; Pediatric transplantation