The Role of Liver Transplantation for Congenital Extrahepatic Portosystemic Shunt

Abstract

Congenital extrahepatic portosystemic shunt (CEPS) is reported more frequently because of advances in imaging techniques. Liver transplantation (LT) is a therapeutic option, although the indications for LT are still controversial. This study reviewed 34 cases of LT for CEPS, including 30 cases reported in the English medical literature and the patients treated in our department, to collect the clinical data associated with LT. The median age at diagnosis and LT was 3.7 and 6.8 years, respectively. Hepatic encephalopathy, including persistent hyperammonemia, was the most common indication of LT. Pulmonary complications, including hepatopulmonary syndrome and pulmonary hypertension, were the second most common indications of LT, and those patients underwent LT soon after the diagnosis. Although a shunt directly draining into the inferior vena cava was the most common type and managed by a simple direct anastomosis of the portal vein at LT, some cases required the modification of the portal vein reconstruction, such as interposition. Thirty patients were alive with a median follow-up period of 18 months. LT for CEPS showed an excellent outcome. The development of pulmonary complications is an early indication for LT. Precise planning of portal vein reconstruction is required before LT.