[Anesthetic management of a child with moyamoya disease combined with von Gierke's disease].

We report on a child with moyamoya disease combined with von Gierke's disease. A 7-year-old girl with von Gierke's disease had a stroke associated with moyamoya disease. She had had many episodes of hypoglycemia and severe metabolic acidosis before surgery. General anesthesia was induced with midazolam 3 mg and fentanyl 100 microg followed by rocuronium 12 mg. After tracheal intubation, anesthesia was maintained with sevoflurane 2.5% in 33% oxygen and 66% nitrous oxide. We used mainly mixture of saline and glucose as intraoperative fluid instead of acetated Ringer solution, and controlled administration of glucose according to blood glucose levels. The patient's plasma lactate levels and base excess during operation showed changes compared with those before operation, because sodium bicarbonate was used during the surgery. The duration of anesthesia was 374 minutes. The patient woke up and spontaneous respiration returned, and the trachea was extubated in the operating room. We were able to manage this case safely without any complications.