Ann Hepatobiliary Pancreat Surg.  2021 Aug;25(3):401-407. 10.14701/ahbps.2021.25.3.401.

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

Affiliations
  • 1Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 2Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Abstract

Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV combined with focal nodular hyperplasia (FNH) and hepatocellular adenoma. A 9-year-old girl who had been diagnosed with multiple FNH had CAPV. Her blood ammonia level was raised to 137 µg/dL. However, she did not complain of any symptoms. To treat CAPV and FNH, we decided to perform LDLT. The graft was a left liver graft from 39-year-old mother of the patient. Recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using interposition of an iliac vein homograft conduit to the superior mesenteric vein-splenic vein conf luence. The CAPV-associated congenital splenorenal shunt was securely ligated. The pathology report of the explant liver showed a 2 cm-sized hepatocellular adenoma and multiple FNH lesions measuring up to 7.1 cm. The patient recovered uneventfully from the LDLT operation. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, as CAPV patients can have various vascular anomalies, combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.

Keyword

Portal vein agenesis; Splenorenal shunt; Interposition graft; Iliac vein homograft; Liver tumor
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