Anaesthesia and mucopolysaccharidoses

The airway problems encountered during anaesthesia in all children with mucopolysaccharidoses presenting for a surgical procedure from 1988 to September 1991 are reviewed. Thirty-four patients underwent 89 anaesthetics for 110 procedures. The results reveal a high incidence of airway problems. The overall incidence of difficult intubation was 25% and failed intubation 8%. In those children with Hurler's syndrome, the difficult intubation incidence was 54% and failed intubation incidence 23%. Other potential anaesthetic problems such as cardiac anomalies and obstructive sleep apnoea are also reviewed.