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Airway malacia in children with achondroplasia

Kimberly E Dessoffy1, Peggy Modaff, Richard M Pauli

  • 1Department of Pediatrics, University of Wisconsin-Madison, Madison, Wisconsin.

American Journal of Medical Genetics. Part A
|December 7, 2013
PubMed

Insights

Airway malacia (abnormal airway softening) is common in children with achondroplasia, leading to more breathing problems. This condition significantly increases the need for interventions like oxygen and surgery.

Area of Science:

  • Pediatric Pulmonology
  • Genetics
  • Respiratory Medicine

Background:

  • Achondroplasia is a genetic disorder associated with significant respiratory complications.
  • Airway malacia, a condition characterized by abnormal softening of the airway, is a potential contributor to these respiratory issues.

Purpose of the Study:

  • To determine the frequency of airway malacia in infants and young children diagnosed with achondroplasia.
  • To evaluate the impact of airway malacia on the respiratory health of children with achondroplasia.

Main Methods:

  • Retrospective chart review of 236 patients with achondroplasia seen between 1985 and 2012.
  • Analysis included clinical examinations, polysomnography, and airway visualization data.
  • Comparison of patients with and without evidence of airway malacia.

Main Results:

  • Airway malacia was identified in 5.5% (13/236) of children with achondroplasia, predominantly affecting the lower airway.
  • Patients with airway malacia showed a higher incidence of obstructive sleep apnea, oxygen use, and need for airway surgeries, including tracheostomy.
  • The frequency of airway malacia in this cohort appears higher than general population estimates.

Conclusions:

  • Airway malacia is a significant finding in children with achondroplasia, contributing to respiratory morbidity.
  • Early identification and management of airway malacia are crucial for addressing complex breathing abnormalities in this population.
  • The presence of airway malacia may necessitate more aggressive and invasive treatment strategies.

Keywords:
achondroplasiaairway obstructionbone dysplasiasbronchomalacialaryngomalaciamalacianatural historyrespiratory problemstracheomalacia

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