Drug-induced sleep endoscopy in children with Prader-Willi syndrome

Ming-Chin Lan1,2, Yen-Bin Hsu3,4, Ming-Ying Lan3,4

  • 1Department of Otolaryngology-Head and Neck Surgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan.

Insights

Children with Prader-Willi syndrome (PWS) and obstructive sleep apnea (OSA) often have tongue base collapse, which is linked to higher apnea-hypopnea index (AHI) values. Positional therapy may help manage this condition.

Area of Science:

  • Pediatric Sleep Medicine
  • Genetics and Rare Diseases
  • Otolaryngology

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder associated with a high prevalence of obesity and sleep-disordered breathing.
  • Obstructive sleep apnea (OSA) is a common and serious comorbidity in children with PWS, often requiring intervention.
  • Drug-induced sleep endoscopy (DISE) is crucial for identifying specific patterns of upper airway collapse in OSA.

Purpose of the Study:

  • To analyze drug-induced sleep endoscopy (DISE) findings in pediatric patients with Prader-Willi syndrome (PWS).
  • To correlate specific upper airway collapse patterns identified during DISE with the apnea-hypopnea index (AHI) and body mass index (BMI).
  • To investigate the relationship between multilevel airway obstruction and OSA severity in children with PWS.

Main Methods:

  • Nine children diagnosed with Prader-Willi syndrome (PWS) underwent drug-induced sleep endoscopy (DISE).
  • Airway collapse patterns were systematically classified using the VOTE (Velum, Oropharynx, Tongue base, Epiglottis) system.
  • Statistical analysis was performed to examine associations between VOTE findings, AHI, and BMI.

Main Results:

  • Multilevel airway obstruction was identified in the majority (66.6%) of children with PWS.
  • Velopharyngeal obstruction was universally present (100%), and all patients exhibited positional obstructive sleep apnea (OSA).
  • Anterior-posterior tongue base collapse was significantly associated with higher AHI values (P=0.016), while no significant association was found with BMI.

Conclusions:

  • Partial or complete anterior-posterior tongue base collapse is a key finding linked to increased OSA severity (higher AHI) in children with PWS.
  • Management strategies for PWS patients should prioritize addressing tongue base collapse.
  • Positional therapy presents a potential therapeutic option for PWS patients, possibly by mitigating tongue base collapse severity.
Abstract

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