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Adenotonsillectomy success for treating obstructive sleep apnea in children with Prader-Willi syndrome

Ross Rosen1, Jamil Hayden2, Abdul Saltagi2

  • 1Department of Otolaryngology-Head and Neck Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.

International Journal of Pediatric Otorhinolaryngology
|March 16, 2025
PubMed

Insights

Adenotonsillectomy is less successful for treating pediatric obstructive sleep apnea (OSA) in Prader-Willi syndrome (PWS) patients. Multidisciplinary care is recommended for PWS patients with OSA.

Area of Science:

  • Pediatric Genetics
  • Sleep Medicine
  • Otolaryngology

Background:

  • Prader-Willi syndrome (PWS) is a rare genetic disorder associated with an increased risk of pediatric obstructive sleep apnea (OSA).
  • Complex pathophysiology in PWS patients, including obesity and craniofacial abnormalities, can complicate OSA treatment.
  • Standard first-line treatment, adenotonsillectomy, may have limited success in this population.

Purpose of the Study:

  • To evaluate the effectiveness of adenotonsillectomy in treating pediatric obstructive sleep apnea (OSA) in patients with Prader-Willi syndrome (PWS).
  • To compare surgical success rates between PWS patients and a control group.
  • To identify medical interventions associated with increased OSA risk in PWS patients.

Main Methods:

  • A retrospective query of the TriNetX database identified pediatric patients (≤18 years) with PWS and OSA diagnoses.
  • Surgical interventions (adenotonsillectomy, tonsillectomy, adenoidectomy) were analyzed.
  • Primary endpoint was OSA diagnosis resolution at 6 months post-surgery; controls without PWS were used for comparison.

Main Results:

  • Of 2163 PWS patients, 1035 had OSA. Surgical success rate in PWS patients was 39.0%, significantly lower than controls (79.6%, p < 0.001).
  • Even when matched for demographics and obesity, success rates remained lower (36.8% vs. 82.1%, p < 0.001).
  • Growth hormone and testosterone use were associated with increased OSA risk (RR 1.43 and 1.39, respectively, p < 0.001).

Conclusions:

  • Adenotonsillectomy demonstrates significantly lower success rates for treating pediatric OSA in patients with PWS.
  • PWS patients with OSA may benefit from a multidisciplinary approach to manage their condition.
  • Further research is needed to establish optimal care practices for PWS patients with OSA.
Abstract

Keywords:
AdenotonsillectomyPrader-willi syndromeSleep apnea

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