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Anaesthesia and mucopolysaccharidoses. A review of airway problems in children
R W Walker1, M Darowski, P Morris
1Royal Manchester Children's Hospital, Pendlebury.
Insights
Children with mucopolysaccharidoses (MPS) face significant anesthesia risks. Difficult intubation occurred in 25% of MPS patients, with Hurler
Area of Science:
- Anesthesiology
- Pediatrics
- Genetics
Background:
- Mucopolysaccharidoses (MPS) are rare genetic disorders.
- Airway complications are a known risk during anesthesia in MPS patients.
- Previous data on anesthetic challenges in pediatric MPS patients is limited.
Purpose of the Study:
- To review airway problems during anesthesia in children with mucopolysaccharidoses.
- To determine the incidence of difficult and failed intubation in this population.
- To highlight other potential anesthetic concerns in pediatric MPS patients.
Main Methods:
- Retrospective review of anesthetic records.
- Data collected from 1988 to September 1991.
- Analysis of 89 anesthetics for 110 procedures in 34 pediatric patients with MPS.
Main Results:
- High incidence of airway problems observed.
- Overall difficult intubation rate was 25%; failed intubation rate was 8%.
- In Hurler's syndrome, difficult intubation was 54% and failed intubation 23%.
Conclusions:
- Anesthesia in children with MPS presents substantial airway challenges.
- Specific syndromes like Hurler's syndrome carry higher intubation risks.
- Careful anesthetic planning is crucial for managing airway and associated comorbidities in MPS patients.
Abstract:
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.