General Anesthesia for a Child with Methylmalonic Acidemia Undergoing Oral Care and Dental Treatment before Kidney Transplantation

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  • HANO Kazuhiro
    Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University
  • KATO Mizuki
    Department of Dental Anesthesiology, Kyushu University Hospital
  • MIYAJIMA Riho
    Department of Dental Anesthesiology, Kyushu University Hospital
  • KAMEYAMA Izumi
    Department of Anesthesiology, Iizuka Hospital
  • OSHIMA Yu
    Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University
  • TSUKAMOTO Masanori
    Department of Dental Anesthesiology, Kyushu University Hospital
  • YOKOYAMA Takeshi
    Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University Department of Dental Anesthesiology, Kyushu University Hospital

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  • メチルマロン酸血症患者における腎移植前歯周治療に対する全身麻酔経験

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<p>  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.</p><p>  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.</p><p>  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.</p>

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