肥大型心筋症とてんかんを伴ったヌーナン症候群患者の全身麻酔経験

Noonan syndrome is characterized by distinctive clinical features, including cardiovascular anomalies, mental delay and skeletal abnormalities. Orofacially, patients with Noonan syndrome have distinctive facial features that include ptosis of the eyelids, hypertelorism, and a low junction of the ears. Epilepsy is an occasional complication of Noonan syndrome. We describe the anesthetic management of a 15-year-old girl with Noonan syndrome. She had hypertrophic obstructive cardiomyopathy(HCM), mental delay, epilepsy and severe scoliosis;she was scheduled to undergo dental treatment under general anesthesia. She had experienced daily hypoxia symptoms because of HCM as well as severe scoliosis. Our anesthetic goal was to provide an adequate preload and afterload. Anesthesia was induced with midazolam, propofol, fentanyl and rocuronium and was maintained with isoflurane and remifentanil. Intraoperative monitoring included noninvasive hemodynamic monitoring and direct monitoring of the arterial pressure. During surgery, we observed a unique tongue movement. The operation was completed uneventfully. After extubation, the stable spontaneous respiration of room air was achieved. However, she experienced epileptic symptoms during which her eyes were open and rolled back into her head, she gradually fell asleep, and her breathing was shallow and slow. She became hypoxic, but she recovered with the administration of positive-pressure ventilation. However, her SpO₂ decreased to 84%-88% upon spontaneous breathing only. To prevent hypoxia, respiratory support with non-invasive positive-pressure ventilation was provided for 40 minutes. Her spontaneous respiration recovered after she awakened from the epileptic symptoms. This case suggests that considerable attention is needed not just for the distinctive complications of Noonan syndrome, but for other possible complications such as epilepsy.