Abstract
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.
Résumé
Les patients atteints de mucopolysaccharidoses démontrent une atteinte progressive étendue de plusieurs organes et tissus pouvant avoir des implications importantes pour ľanesthésiste. Ces atteintes sont peu communes et peu ďanesthésistes soignent ces patients régulièrement même si quelques-uns de ces patients subissent plusieurs anesthésies afin ďaméliorer leur qualité de vie. Il y a relativement très peu ďarticles dans la littérature concernant ľexpérience anesthésique avec ces patients. On rapporte une étude rétrospective sur ľexpérience récente du Children’s Hospital of Eastern Ontario à Ottawa, Canada, sur 38 anesthésies sur neuf patients atteints de mucopolysaccharidoses spécialement les syndromes de Hunter, Hurler, Sanfilippo et Morquio. Ľétablissement et le maintien des voies aériennes représentent le problème le plus communément rencontré chez ces patients. Ľincidence totale des problèmes reliés aux voies aériennes était de 26 pour cent. Chez les patients atteints du syndrome de Hurler ou de Hunter ľincidence des problèmes reliés aux voies aériennes était de 53 pour cent.
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Herrick, I.A., Rhine, E.J. The mucopolysaccharidoses and anaesthesia: a report of clinical experience. Can J Anaesth 35, 67–73 (1988). https://doi.org/10.1007/BF03010548
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DOI: https://doi.org/10.1007/BF03010548