Methylmalonic acidemia (MMA) is an autosomal recessive disorder and a metabolic disorder of organic acids in which the accumulation of methylmalonic acids causes various symptoms. MMA has several features related to acidosis, and the prevention of acidosis through nutritional management is necessary.
We performed the anesthetic management of a 7-year-old girl with MMA. She was diagnosed as having MMA during newborn screening, and she exhibited mental retardation and malnutrition. She was able to take nutrition orally, but she gradually became unable to eat because of vomiting ; she underwent a gastrostomy at the age of 2 years and 9 months old. Her kidney function gradually worsened, and a kidney transplantation was scheduled. Her oral hygiene was poor, and several calculus deposits were visible. Because the patient was noncooperative, periodontal treatment under general anesthesia was scheduled before the kidney transplantation.
Our anesthetic goal was to maintain her metabolism : in particular, we sought to minimize her required fasting time, and we gave her sugar, as appropriate, to prevent an increase in catabolism. The anesthesia was induced with sevoflurane, midazolam, propofol, fentanyl and rocuronium and was maintained with desflurane and remifentanil. We performed a blood analysis twice using samples collected from the right femoral artery before and after the operation ; the results showed that her metabolism remained under control during the operation. The operation was completed uneventfully ; because her respiration was good after extubation, she was transferred to the pediatric ward. She has since received a kidney transplantation using an organ from her father, as planned. This case shows that importance of managing a patient’s nutrition before and after operation.
