[Anesthetic management of a patient with congenital myopathy complicated with severely impaired pulmonary function]

A 17-year-old man, who had received a diagnosis of congenital myopathy, was scheduled for superficial temporal artery to middle cerebral artery anastomosis procedure. Preoperative respiratory examinations showed the breathing capacity of 450 ml because of a scoilosis, deformity of a thorax and severe muscular atrophy. Anesthesia was maintained with propofol, fentanyl, remifentanil and vecuronium to avoid malignant hyperthermia (MH). Endotracheal intubation was performed with a gum elastic bougie for difficult airway management (DAM). After intubation, we checked the positioning of the tracheal tube by a chest X ray and bronchofiber findings. During perioperative period, no symptom of MH and respiratory dysfunction was noticed. In the anesthesia for patients with congenital myopathy, deterioration of respiratoy function, prevention for MH, and possibility of DAM should be considered.