Ann Liver Transplant.  2022 Nov;2(2):144-150. 10.52604/alt.22.0019.

Living donor liver transplantation in a pediatric patient having intrahepatic portocaval shunt with congenital absence of the intrahepatic portal vein

• Namgoong JM
• Hwang S
• Park GC
• Ha S
• Gang S
• Park J
• Oh SH

• KMID: 2560910
• DOI: http://doi.org/10.52604/alt.22.0019

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into systemic circulation. We report the case of a pediatric living donor liver transplantation (LDLT) for CAPV with an intrahepatic portosystemic shunt. A 3-year-old boy was diagnosed with CAPV at the age of 1 year. There was no evidence of portal hypertension due to complete diversion of the portal blood flow into the retrohepatic inferior vena cava. The patient suffered from hepatic encephalopathy; therefore, we decided to perform LDLT. The graft was the left liver from the 31-year-old mother of the patient. The recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using a branch patch of the native portal vein. The patient recovered uneventfully from the LDLT. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, the features of CAPV and portocaval shunt may vary among CAPV patients; thus, portal vein reconstruction should be customized according to anatomical variations.

Keyword

Portal vein agenesis; Portocaval shunt; Portosystemic shunt; Living donor liver transplantation; Hyperammonemia