The mucopolysaccharidoses and anaesthesia: a report of clinical experience

Patients with the mucopolysaccharidoses show widespread, progressive involvement and derangement of many organs and tissues which can have profound implications for the anaesthetist. These disorders are uncommon and few anaesthetists care for these patients on a regular basis although individual patients often undergo multiple anaesthetics for procedures intended to improve their quality of life. There is a relative paucity of literature dealing with clinical anaesthetic experience with these patients. We report a retrospective review of the recent experience at the Children's Hospital of Eastern Ontario in Ottawa, Canada, administering 38 anaesthetics to nine patients with several of the mucopolysaccharidoses; specifically the Hunter, Hurler, Sanfilippo and Morquio syndromes. The establishment and maintenance of an adequate airway represents the most commonly encountered anaesthetic-related problem in these patients. We found an overall incidence of airway-related problems of 26 per cent. In patients with the Hurler or Hunter syndromes the incidence of airway-related problems was 53 per cent.