title: Anaesthetic management of a child with Freeman-sheldon syndrome undergoing spinal surgery.
abstract: Freeman-Sheldon syndrome, or distal arthrogryposis type 2A, is a rare congenital myopathy and dysplasia characterised by multiple contractures, abnormalities of the head and face, defective development of the hands and feet and skeletal malformations. The facial muscle contracture produces the typical 'whistling face' appearance. Anaesthetic issues include difficult intravenous access, difficult airway and postoperative pulmonary complications. Although an association with malignant hyperthermia has been suggested, this has not been confirmed. We report the management of a seven-year-old girl with Freeman-Sheldon syndrome undergoing anterior and posterior spinal surgery and describe a successful anaesthetic regimen based on a total intravenous anaesthesia technique with remifentanil and propofol without neuromuscular blocking agents. The child had an uneventful anaesthetic and postoperative course. We believe the presence of the myopathy warranted the use of a 'non-triggering' anaesthetic, as suxamethonium and volatile agents may be associated with significant complications such as muscle rigidity and rhabdomyolysis in myopathic patients, even in the absence of malignant hyperthermia.
authors: Department of Anaesthesia and Intensive Care, Saint-Joseph University, Hotel-Dieu de France Hospital, Beirut, Lebanon.
authors: F C
authors: FC
authors: Richa
authors: P H
authors: PH
authors: Yazbeck
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D000768:Anesthesia, General; D018686:Anesthetics, Intravenous; D001176:Arthrogryposis / Q000150:complications; D001794:Blood Pressure / Q000502:physiology; D002648:Child; D019465:Craniofacial Abnormalities / Q000150:complications*; D004569:Electroencephalography; D005260:Female; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D016343:Monitoring, Intraoperative; D019637:Orthopedic Procedures; D010149:Postoperative Pain / Q000175:diagnosis / Q000188:drug therapy; D010880:Piperidines; D015742:Propofol; D000077208:Remifentanil; D012121:Respiration, Artificial; D012600:Scoliosis / Q000150:complications / Q000601:surgery*; D013131:Spine / Q000601:surgery*; D013577:Syndrome
chemical_list: D018686:Anesthetics, Intravenous; D010880:Piperidines; D000077208:Remifentanil; D015742:Propofol