Paediatric airway management in mucopolysaccharidosis 1: a retrospective case review.

Context: Airway management in children suffering from mucopolysaccharidosis 1 (Hurler syndrome) remains challenging despite advances in both treatment and airway management techniques.

Objectives: Forty-one anaesthetic charts following ten children over a 6-year period (2004-2010) were reviewed with emphasis on airway problems.

Results: All children had early stem cell transplantation at the age of 2 years or earlier. Mean (SD) age was 5 (4.3) years. Mask ventilation was difficult in five of 41 (12%) anaesthetics or in three of ten children. There were 29 intubations. Direct laryngoscopy was described as difficult (Cormack and Lehane ≥3) on 11 occasions in five of ten children. There were three of 26 (12%) failed intubations with direct laryngoscopy. These situations were resolved by a fibre-optic procedure, by laryngeal mask airway (LMA) insertion or by use of a videolaryngoscope. A laryngeal mask airway was used 11 times to avoid invasive airway management and once when direct laryngoscopy was impossible.

Conclusion: The airway management of children with mucopolysaccharidosis 1 remains critical, despite advances in both treatment and airway management techniques. Problems did not seem to increase as children grew older. We assume that technical improvements such as standardised use of the laryngeal mask airway or attached tube channel videolaryngoscopes as well as a stem cell transplantation treatment of the disease helped the management of older children with mucopolysaccharidosis 1.