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

Jung-Man Namgoong; Shin Hwang; Gil-Chun Park; Hyunhee Kwon; Kyung Mo Kim; Seak Hee Oh

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자료유형: 학술저널

저자정보: Jung-Man Namgoong (University of Ulsan College of Medicine) Shin Hwang (University of Ulsan College of Medicine) Gil-Chun Park (University of Ulsan College of Medicine) Hyunhee Kwon (University of Ulsan College of Medicine) Kyung Mo Kim (University of Ulsan College of Medicine) Seak Hee Oh (University of Ulsan College of Medicine)

저널정보: 한국간담췌외과학회 Annals of Hepato-Biliary-Pancreatic Surgery 한국간담췌외과학회지 제25권 제3호

발행연도: 2021.8

수록면: 401 - 407 (7page)

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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 confluence. 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.

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

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