重度の低血糖と意識障害を呈した先天性門脈欠損症患児に対する生体肝移植術の麻酔経験

Congenital Absence of the Portal Vein：Living Donor Liver Transplantation for Severe Hypoglycemia and Impaired Consciousness

要旨

　高インスリン血症と高アンモニア血症のために肝移植を施行した小児先天性門脈欠損症症例を経験した。肝初回通過効果喪失のため，術前よりアンモニア値と血糖値の管理に留意する必要があった。重度の低血糖発作により，術前から持続ブドウ糖投与を継続し，綿密な血糖測定を行いながら，ブドウ糖投与量の調整が必要であった。

　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.