Perioperative management of living‐donor liver transplantation for methylmalonic acidemia

Abstract

Methymalonic 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.

Anesthetic management of a patient with methylmalonic acidemia: a case report

“…Since hypoglycemia worsens metabolic acidosis by protein catabolism sthenia, glucose loading should be avoided. Preoperative fasting should be as short as possible; some studies have reported 6 h as an optimal fasting time [2]. If necessary, glucose may be administered during preoperative fasting.…”

“…In general, propofol should also not be administered to patients with MMA. One study reported that propofol use was associated with deterioration of metabolic failure during general anesthesia in a living liver transplantation [2]. Another study has suggested that propofol may be safe for continuous intravenous administration, which was used for sedation during procedures such as magnetic resonance imaging [5].…”

Successful perioperative management of living-donor liver transplantation for a patient with severe methylmalonic acidemia: a case report

“…The guidelines for diagnosis and management of MMAemia suggest that LT should be considered in patients with MMAemia who suffer frequent metabolic acidosis despite dietary and medical treatment [1]. A report on 14 cases of LT for MMAemia in a single institute in Japan showed that severe metabolic acidosis occurred in 3 cases at the start of the operation, and death occurred perioperatively in 2 of these 3 cases [4]. Thus, metabolic acidosis should be prevented during LT, even though the prognosis of MMAemia after LT has recently been improved [4].…”

“…A report on 14 cases of LT for MMAemia in a single institute in Japan showed that severe metabolic acidosis occurred in 3 cases at the start of the operation, and death occurred perioperatively in 2 of these 3 cases [4]. Thus, metabolic acidosis should be prevented during LT, even though the prognosis of MMAemia after LT has recently been improved [4]. In a patient with MMAemia, MMA accumulation can cause metabolic acidosis and secondary mitochondrial dysfunction.…”

“…Recently, liver transplantation (LT) has been performed as supportive therapy for children with severe MMAemia to improve MMA metabolism through the action of the transplanted healthy liver [3]. However, there are few reports on perioperative management of LT for MMAemia [4].…”