Ann Liver Transplant.  2023 Nov;3(2):128-135. 10.52604/alt.23.0013.

Living donor liver transplantation with direct collateral portal vein anastomosis in a pediatric patient with congenital absence of the portal vein

• Namgoong JM
• Hwang S
• Park GC
• Lee DY
• Kim KM
• Oh SH

• KMID: 2560934
• DOI: http://doi.org/10.52604/alt.23.0013

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which the mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV with a portal collateral vein of cavernous transformation. A 12-year-old boy was diagnosed with CAPV at the age of 11 years. Portal hypertension with collateral vein formation was rapidly progressed, therefore, we decided to perform LDLT. The graft was a modified right liver graft from the 41-year-old father of the patient. The recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of the portal collateral vein. This portal collateral vein was gently manipulated and its branches were used to form a branch patch. A modified right liver graft was implanted with direct ligation of the coronary and splenorenal collateral veins. The patient recovered from the LDLT operation. The reconstructed portal vein was maintained well without hemodynamic abnormality. This patient has been doing well for 2 months after the LDLT. In conclusion, since CAPV patients show various types of portocaval shunt, individualized portal vein reconstruction should be performed after thorough anatomical assessment before and during the liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Modified right liver graft; Direct anastomosis; Esophageal varix