[Anesthetic management for a child with pompe disease]

Pompe or glycogen storage disease type II is a genetic disorder affecting the cardiac and skeletal muscle. A 4-year-old boy with this disease was scheduled to undergo an orthopedic operation for clubbed foot. He had cardiomyopathy and skeletal muscle weakness; but his cardiac function was normalized by the long-term enzyme replacement therapy. General anesthesia was slowly induced with oxygen, nitrous oxide, and sevoflurane, and tracheal intubation was achieved without any muscle relaxants. In combination with a caudal blockade with 6 ml of 0.375% ropivacaine, general anesthesia was successfully maintained with oxygen, nitrous oxide, and sevoflurane. We did not use muscle relaxants to avoid prolonged respiratory depression. The perioperative course was uneventful and no complication was observed.