piperidines and Myasthenic-Syndromes--Congenital

Congenital myasthenic syndromes (CMS) are heterogeneous disorders of neurotransmission caused by genetic mutations of neuromuscular junction molecules. We report anesthetic management of a CMS patient who was a 14-year-old boy with endplate acetylcholinesterase deficiency. The patient used noninvasive positive pressure ventilation (NPPV) at night. He underwent a corrective maneuver for severe scoliosis under general anesthesia. General anesthesia was maintained using propofol and remifentanil. Intraoperative mechanical ventilation remained stable. Extubation was performed on the next day and NPPV was started. Several hours later, he complained of a stomachache and intense abdominal bloating. Computed tomography revealed a massive amount of air in the stomach and intestine. He recovered from abdominal bloating the next day without treatment for decompression. Lung-thoracic compliance has been reported to decrease immediately after a corrective maneuver for scoliosis patients. In our case, we suspected a relative increase of abdominal compliance to lung-thoracic compliance as a cause of intense abdominal bloating by air injection from NPPV with his daily setting. In CMS, symptoms, therapy and contraindicated drugs vary according to the location of dysfunction. Therefore, anesthetic management according to each genotype should be designed to avoid drugs that could either trigger or worsen CMS. Intensive respiratory care is advisable after surgery.

Topics: Adolescent; Air; Anesthesia, General; Humans; Intestines; Lung Compliance; Male; Myasthenic Syndromes, Congenital; Perioperative Care; Piperidines; Positive-Pressure Respiration; Postoperative Complications; Propofol; Remifentanil; Respiration, Artificial; Scoliosis; Stomach