Anaesthesia for Children with Mucopolysaccharidoses

Abstract

The mucopolysaccharidoses are a group of inherited disorders of metabolism, with varying clinical manifestations. A number of them present anaesthetic difficulties. This paper presents a summary table of the syndromes and reviews our experience over ten years with patients with these diagnoses. The clinical presentations, anaesthetic management, and complications are described. The effect of age and diagnosis on airway difficulties was studied. There were 31 patients, 28 of whom required anaesthesia, on a total of 99 occasions, for 115 procedures. The patients with Hunter, Hurler and Maroteaux-Lamy syndromes had significantly more airway difficulties as they grew older, and compared with patients in this group with other syndromes. Patients with Hurler's syndrome may have coronary artery involvement and one patient was given fentanyl and pancuronium for this reason. He proved impossible to intubate and an emergency tracheostomy was performed.