Ann Liver Transplant.  2022 May;2(1):69-77. 10.52604/alt.22.0015.

Living donor liver transplantation with proximal splenic vein ligation in a pediatric patient with congenital absence of the portal vein

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

• KMID: 2560884
• DOI: http://doi.org/10.52604/alt.22.0015

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 prominent splenorenal shunt. A 6-year-old boy was diagnosed with CAPV at the age of 1 year. Portal hypertension with splenomegaly was progressed, therefore, we decided to perform LDLT. The graft was a left liver graft from the 31-year-old mother of the patient. The recipient hepatectomy was performed according to the standard procedures of pediatric LDLT. Portal vein reconstruction was performed using the interposition of an iliac vein homograft conduit to the enlarged collateral vein at the common bile duct wall. The proximal splenic vein was securely ligated to control the splenorenal shunt. The patient recovered uneventfully from the LDLT operation. The reconstructed portal vein was well maintained without any hemodynamic abnormalities. In conclusion, because CAPV patients can have several vascular anomalies, such combined vascular anomalies should be thoroughly assessed before and during the liver transplantation operation. Individualized portal vein reconstruction with homograft vein interposition combined with intraoperative cine-portogram is an effective technique with satisfactory results.

Keyword

Portal vein agenesis; Splenorenal shunt; Interposition graft; Iliac vein homograft; Portogram