• Chiaki Baba, Mureo Kasahara, Yasuhiro Kogure, Shugo Kasuya, Sukeyuki Ito, Takako Tamura, Akinari Fukuda, Reiko Horikawa, and Yasuyuki Suzuki.
• Department of Anesthesia and Intensive care, National Center for Child Health and Development, Tokyo, Japan.
• Paediatr Anaesth. 2016 Jul 1; 26 (7): 694-702.

AbstractMethymalonic acidemia (MMA) is a hereditary metabolic disorder characterized by a defect of the methylmalonyl-CoA mutase that breaks down propionate. The efficacy of liver transplantation for MMA was recently reported. However, the anesthetic management of liver transplant for MMA is not clear. The aim of this article is to describe an anesthetic management algorithm of liver transplant for MMA by reviewing our cases of liver transplant for MMA. Fourteen patients received a liver transplant; three cases showed metabolic decompensation during the transplant and two of the patients died. In the two patients who expired, propofol was used for maintenance anesthesia and preoperative continuous hemodiafiltration was used to reduce plasma methylmalonic acid level in one case, and to control severe metabolic decompensation before transplant for the other case. Their renal function was also worse than others and they were already experiencing metabolic decompensation before induction of anesthesia. Based on our experience of these 14 cases, we have established an anesthetic algorithm for patients with MMA undergoing liver transplant or other procedures. There are three important points in our experience: propofol should be avoided, dextrose infusion therapy should be continued to prevent metabolic decompensation, and liver transplant or other procedures should be avoided during metabolic decompensation.© 2016 John Wiley & Sons Ltd.

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