Mitochondrial DNA depletion syndrome (MDS) is an autosomal recessive inherited disorder characterized by a reduction in mitochondrial DNA copy number in clinically affected tissues. We provided anesthetic management to a 14-year-old girl with MDS. She was diagnosed as having MDS based on genetic screening, and she had undergone a liver transplantation at the age of 14 years. She also had mental retardation and seizures. We were unable to provide proper treatment with her cooperation, so dental treatment under general anesthesia was scheduled.
Since it was important for this patient to maintain her metabolism and, in particular, to avoid hypoglycemia, glucose was administered appropriately to prevent increased catabolism. Anesthesia was induced with sevoflurane, midazolam, remifentanil and rocuronium and was maintained with isoflurane and remifentanil. Her blood glucose level was evaluated every 30 minutes during surgery, and glucose loading was adjusted as needed. Her metabolism remained under control during the anesthesia, and the operation was accomplished uneventfully. Her respiration condition was good after extubation, so she was moved to the pediatric ward.
