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Paediatric airway management in mucopolysaccharidosis 1: a retrospective case review

Wilhlem A Osthaus1, Thomas Harendza, Lars H Witt

  • 1Department of Anaesthesiology and Intensive Care Medicine, Hannover Medical School, Carl Neuberg Strasse 1, Hannover, Germany.

European Journal of Anaesthesiology
|February 21, 2012
PubMed

Insights

Airway management in children with mucopolysaccharidosis 1 (Hurler syndrome) is challenging. Advances like stem cell transplantation and techniques such as laryngeal mask airway (LMA) use improve outcomes.

Area of Science:

  • Pediatric Anesthesiology
  • Rare Genetic Disorders
  • Airway Management

Background:

  • Mucopolysaccharidosis 1 (Hurler syndrome) presents significant airway management challenges in pediatric patients.
  • Despite advancements, maintaining a patent airway in these children remains a critical concern.

Purpose of the Study:

  • To review airway management challenges and outcomes in children with mucopolysaccharidosis 1.
  • To evaluate the impact of early stem cell transplantation and evolving airway techniques.

Main Methods:

  • Retrospective review of 41 anesthetic charts from ten children over a 6-year period (2004-2010).
  • Emphasis on documenting airway difficulties, intubation success rates, and management strategies.

Main Results:

  • Mask ventilation was difficult in 12% of anesthetics.
  • Direct laryngoscopy was difficult (Cormack and Lehane ≥3) in 50% of children, with a 12% failure rate.
  • Fiber-optic intubation, laryngeal mask airway (LMA), or videolaryngoscopy successfully managed difficult airways.

Conclusions:

  • Airway management in mucopolysaccharidosis 1 remains critical but manageable with advanced techniques.
  • Early stem cell transplantation and standardized use of devices like LMAs and videolaryngoscopes aid in managing older children.
  • Airway issues did not appear to worsen with age in this cohort.
Abstract

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