Radical brachiocephalic artery transection is often performed for the treatment of tracheo-innominate artery fistula. Postoperatively, the blood flow to the right upper extremity is supplied through the left carotid artery, the Willis arterial ring, and the right subclavian artery. Therefore, brain circulation may decrease when blood flow to the right upper extremity is increased.

  We performed anesthesia for dental treatment in a 14-year-old girl with a history of radical brachiocephalic artery transection. She was 138 cm in height and 17.2 kg in weight, and she had undergone radical brachiocephalic artery transection and tracheal plasty for the treatment of tracheal stenosis at the age of 13 years.

  A pulse oximeter was applied to her right finger to evaluate oxygen saturation and blood flow to her right upper extremity continuously. A probe for the noninvasive monitoring of regional oxygen saturation (rSo₂) was also placed on her forehead to evaluate cerebral blood flow continuously. Anesthesia was induced with intravenous midazolam, propofol, atropine, remifentanil and rocuronium, and the patient was intubated using a spiral tube (ID, 5.0 mm) nasally. The anesthesia was maintained using 1%-1.5% isoflurane. The rSo₂ transiently decreased from 70% to 53% on the right side during the induction of anesthesia, but it recovered quickly and was maintained at around 70%-90% during the operation. The pulse wave in the right finger was continuously detected during anesthesia, and the Spo₂ was maintained at 97%-100%. The dental procedure was completed uneventfully. After emergence from the anesthesia, the movement of her right upper extremity was the same as before surgery.

  In the present case, isoflurane was used to prevent an epilepsy seizure. The noninvasive monitoring of regional oxygen saturation (rSo₂) was helpful for evaluating cerebral blood flow during anesthesia.

  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.