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Tracheostomy in children with mucopolysaccharidosis: A systematic review

Francesca Galluzzi1, Werner Garavello2

  • 1Department of Otorhinolaryngology, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.

International Journal of Pediatric Otorhinolaryngology
|November 24, 2024
PubMed

Insights

Tracheostomy is an effective airway management for children with mucopolysaccharidosis (MPS), though challenging due to disease progression. A multidisciplinary approach is recommended to prevent complications in these patients.

Area of Science:

  • Pediatric Surgery
  • Rare Diseases
  • Respiratory Medicine

Background:

  • Children with mucopolysaccharidosis (MPS) often present with difficult airways, necessitating tracheostomy.
  • Surgical airway management in MPS patients poses unique challenges and risks.

Approach:

  • A systematic review adhering to PRISMA guidelines was performed.
  • Literature search conducted on Pubmed and Embase databases for studies on tracheostomy in pediatric MPS.
  • Quality appraisal utilized the Joanna Briggs Institute methodology.

Key Points:

  • Included 9 studies encompassing 34 pediatric MPS patients (mean age 11 years; 1.7 male/female ratio).
  • MPS II was the most prevalent type (16/34). Tracheostomy rates ranged from 3.5% to 15.5%.
  • Indications included airway obstruction, pre-surgical planning, and intubation/extubation failure. Common complications: granulations, infections, stenosis, secretions, tracheitis. Decannulation was rare.

Conclusions:

  • Tracheostomy effectively ensures airway patency in pediatric MPS patients, often proving permanent.
  • Management is complex due to MPS-related clinical features and disease progression.
  • A planned, multidisciplinary approach is crucial for minimizing complications.
Abstract

Keywords:
ChildrenMPSMucopolysaccharidosisObstructive sleep apneatracheostomy

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