Ann Liver Transplant.  2023 Nov;3(2):118-127. 10.52604/alt.23.0016.

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

• Namgoong JM
• Hwang S
• Park GC
• Kang S
• Kim KM
• Oh SH

• KMID: 2560933
• DOI: http://doi.org/10.52604/alt.23.0016

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 3-year-old boy was diagnosed with CAPV at the age of 2 years. Portal hypertension with collateral vein formation was progressed, thus we decided to perform LDLT. The graft was a left lateral section graft from the 35-year-old father of the patient. Recipient hepatectomy was performed according to the standard procedures of pediatric LDLT with isolation of two pericholedochal collateral veins. These collateral veins were unified and integrated with an iliac vein homograft using a modified patch-conduit venoplasty. The left lateral section graft was implanted with direct ligation of the coronary and splenorenal collateral veins. However, luminal thrombus was formed within the reconstructed portal vein conduit, thus the conduit was removed and graft portal vein was directly anastomosed with the pericholedochal collateral veins. The patient recovered from the LDLT operation, but the reconstructed portal vein was showed marked anastomotic stenosis. At 40 days after LDLT, percutaneous transhepatic balloon angioplasty was performed and the anastomotic stenosis was expanded. This patient has been doing well for 3 months after the LDLT. In conclusion, given the diverse presentations of portocaval shunt in CAPV patients, it is imperative to tailor the portal vein reconstruction approach based on a comprehensive anatomical assessment both prior to and during liver transplantation operation.

Keyword

Portal vein agenesis; Portocaval shunt; Left lateral section graft; Direct anastomosis; Percutaneous transhepatic angioplasty