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Congenital Absence of the Portal Vein:Living Donor Liver Transplantation for Severe Hypoglycemia and Impaired Consciousness Shuji KAWAMOTO 1 , Yosuke MATSUO 1 , Tsuguhiro MATSUMOTO 1 , Akiko HIROTSU 1 , Kazuya HASHIMOTO 1 , Moritoki EGI 1 1Department of Anesthesia, Kyoto University Hospital Keyword: congenital absence of the portal vein , living donor liver transplantation , hepatic first-pass effect , hypoglycemia , hyperammonemia pp.573-576
Published Date 2024/8/10
DOI https://doi.org/10.18916/masui.2024080012
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 We experienced a rare pediatric case of a congenital portal vein defect, necessitating living donor liver transplantation due to recurrent episodes of severe hypoglycemia and altered consciousness caused by hyperammonemia. The portal vein serves as a conduit for portal blood flow to the liver, facilitating the important first-pass metabolism of various substances, including insulin and ammonia. Close management of ammonia and blood glucose levels was essential prior to transplantation. The severe hypoglycemic episodes persisted until donor liver reperfusion, requiring the patient to undergo continuous preoperative glucose infusion, including frequent glucose monitoring and adjustment of the amount of glucose to be infused intravenously.


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電子版ISSN 印刷版ISSN 0021-4892 克誠堂出版

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