Long-term outcome of liver transplantation for congenital extrahepatic portosystemic shunt.

Liver transplantation (LT) may have been viewed as the "last resort" for the treatment of congenital extrahepatic portosystemic shunt (CEPS) due to advancement of imaging and interventional radiology techniques. However, some patients still require LT, and criteria for LT are yet to be determined. We conducted a national survey of cases undergoing LT for CEPS between June 1998 and August 2018 and evaluated the clinical data and outcomes with a review of previously reported cases from the English-language medical literature. Twenty-six patients underwent LT in Japan at a median age at LT of 5.2 years old. The most common indications for LT were persistent hyperammonemia (62%) and liver nodule (50%), followed by pulmonary complications (38%). Pulmonary complications in all patients, including intrapulmonary shunt and pulmonary hypertension, were improved after LT. Regarding the 29 previously reported cases in the English-language literature, a liver nodule (49%), including hepatoblastoma and hepatocellular carcinoma, was the most common indication for LT, followed by pulmonary complications (34%). Twenty-five patients (96%) in our survey and 26 patients (90%) in the literature review were alive with a median follow-up period of 9.5 years and 1.6 years, respectively. Although LT has a limited role in management of CEPS, our study indicated that LT was safe as an alternative treatment for select cases with malignant tumor or pulmonary complications and those with complications related to new portosystemic collateral vessels after shunt closure, such as pulmonary hypertension or hepatopulmonary syndrome.