[Anesthesia in a patient with congenital nemaline type myopathy].

We report a 28-year-old female with known nemaline myopathy who underwent general anesthesia for osteosynthesis of a femoral neck fracture. Preoperative investigations including blood count, urea, serum electrolytes, prothrombin time, partial thromboplastin time, and electrocardiogram were all normal. Pulmonary function studies showed a restrictive defect with normal blood gases. The chest radiograph showed severe scoliosis. Because of the complications reported in nemaline myopathy, e.g. aspiration, hypoventilation, and the unknown predisposition for the development of malignant hyperthermia, we performed "trigger-free" general intubation anesthesia. Anesthesia was induced with propofol 100 mg i.v., nitrous oxide 66%, and oxygen 34%. The cardiac rate, oropharyngeal temperature, and oxygen saturation were recorded continuously. Blood pressure was measured every 5 min. Muscle relaxation was monitored using train-of-four (TOF) stimulation. Atracurium was given intravenously in 5-mg doses until the TOF-ratio was zero, then the patient was intubated and anesthesia maintained by continuous infusion of propofol 0.43 mg/kg per hour. Pulmonary ventilation using a Dräger Sulla 808 was adjusted to keep end-expiratory pCO2 between 32 and 34 mmHg. Fentanyl 0.2 mg was administrated at the beginning of the operation. Fifty minutes after the last bolus of atracurium the TOF-ratio was greater than 75%. At the beginning of skin suturing the propofol infusion was stopped; a few minutes later spontaneous respiration ensued. After extubation emergence was rapid and the patient was fully oriented and awake.(ABSTRACT TRUNCATED AT 250 WORDS)