Anaesthesia for children with mucopolysaccharidoses

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C Moores1, J G Rogers, I M McKenzie

  • 1Department of Anaesthesia, Royal Children's Hospital, Melbourne, Victoria.

Anaesthesia and Intensive Care
|August 1, 1996
PubMed

Insights

Mucopolysaccharidoses (MPS) are inherited metabolic disorders often causing anesthetic challenges. Airway difficulties significantly increase with age in patients with Hunter, Hurler, and Maroteaux-Lamy syndromes.

Area of Science:

  • Biochemistry
  • Genetics
  • Anesthesiology

Background:

  • Mucopolysaccharidoses (MPS) are inherited metabolic disorders.
  • These conditions present diverse clinical manifestations.
  • Certain MPS syndromes pose significant anesthetic challenges.

Purpose of the Study:

  • To summarize MPS syndromes and their anesthetic implications.
  • To review a decade of anesthetic management for MPS patients.
  • To analyze the impact of age and specific diagnoses on airway difficulties.

Main Methods:

  • Retrospective review of 10 years of anesthetic records for 31 MPS patients.
  • Analysis of 99 anesthetic occasions for 115 procedures.
  • Correlation of age and diagnosis with airway management complications.

Main Results:

  • 28 out of 31 patients required anesthesia.
  • Patients with Hunter, Hurler, and Maroteaux-Lamy syndromes experienced increased airway difficulties with age.
  • One patient with Hurler's syndrome, potentially having coronary artery involvement, required emergency tracheostomy due to intubation failure.

Conclusions:

  • Anesthetic management in MPS requires careful consideration of specific syndromes.
  • Age is a significant factor in the progression of airway difficulties in certain MPS types.
  • Early recognition and preparedness for potential airway complications are crucial for patient safety.

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