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Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader-Willi Syndrome

Shi-Bing Wong1,2, Mei-Chen Yang2,3, I-Shiang Tzeng4

  • 1Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City 23142, Taiwan.

Children (Basel, Switzerland)
|June 24, 2022
PubMed

Insights

Obstructive sleep apnea syndrome (OSAS) is common in Prader-Willi syndrome (PWS) patients and rarely resolves spontaneously. Management should focus on weight control and surgical candidate selection, not just watchful waiting.

Area of Science:

  • Pediatric Endocrinology
  • Sleep Medicine
  • Genetics

Background:

  • Obstructive sleep apnea syndrome (OSAS) is a frequent comorbidity in Prader-Willi syndrome (PWS).
  • OSAS in PWS can lead to significant health consequences.
  • Understanding OSAS progression in pediatric PWS is crucial for effective management.

Purpose of the Study:

  • To investigate the longitudinal progression of OSAS in pediatric patients with PWS.
  • To analyze changes in polysomnography parameters over time.
  • To identify factors associated with OSAS severity in this population.

Main Methods:

  • Observational study with longitudinal follow-up.
  • Annual polysomnography (PSG) in pediatric PWS patients without prior upper airway surgery.
  • Analysis of Body Mass Index (BMI), BMI z-score, sleep parameters, apnea indices (CAI, OAHI), and oxygen saturation.

Main Results:

  • 20 out of 22 pediatric PWS patients had OSAS at baseline.
  • Spontaneous resolution of OSAS was rare (2 patients over 4 years).
  • While BMI and BMI z-score increased, CAI and OAHI showed no significant change; OAHI correlated with BMI z-score and deletion genotype.

Conclusions:

  • OSAS is highly prevalent and persistent in pediatric PWS patients.
  • Watchful waiting is likely insufficient for OSAS management in PWS.
  • Weight management and careful surgical candidate selection are vital for treating OSAS in PWS.

Keywords:
Prader–Willi syndromeobstructive sleep apnea syndromepolysomnographysleep-disordered breathing

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