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Drug-Induced Sleep Endoscopy (DISE) with Target Controlled Infusion (TCI) and Bispectral Analysis in Obstructive Sleep Apnea
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Obstructive sleep disorders in Prader-Willi syndrome: The role of surgery and growth hormone

Michael A DeMarcantonio1, David H Darrow, Eric Gyuricsko

  • 1Eastern Virginia Medical School, Norfolk, VA, USA.

International Journal of Pediatric Otorhinolaryngology
|October 1, 2010
PubMed

Insights

Upper airway surgery alone is often insufficient for resolving obstructive sleep apnea (OSA) in Prader-Willi syndrome (PWS) patients. Pre-treatment polysomnography and combined therapies like tonsillectomy and CPAP are recommended for PWS with OSA.

Area of Science:

  • Pediatric Surgery
  • Sleep Medicine
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is associated with muscle hypotonia and obesity, leading to a high incidence of obstructive sleep apnea (OSA).
  • Growth hormone therapy in PWS patients has raised concerns regarding potential exacerbation of OSA and sudden death.
  • Pre-treatment evaluation for OSA and consideration of adenotonsillectomy before growth hormone therapy are suggested for PWS patients.

Purpose of the Study:

  • To evaluate the effectiveness and safety of surgical interventions for obstructive sleep apnea (OSA) in patients with Prader-Willi syndrome (PWS).

Main Methods:

  • Retrospective review of PWS patients who underwent adenoidectomy or adenotonsillectomy between 2001 and 2009.
  • Pre-operative and post-operative polysomnography (PSG) was performed to assess the apnea-hypopnea index (AHI) and oxygen saturation.
  • Analysis of changes in BMI, AHI, and oxygen saturation levels post-surgery.

Main Results:

  • Five PWS patients were identified, undergoing various upper airway surgeries.
  • A decrease in median AHI from 16.4 to 4.4 was observed, but without statistical significance (p=0.274).
  • Improvements in mean and nadir oxygen saturation were noted but did not reach statistical significance; no intra-operative complications occurred.

Conclusions:

  • Upper airway surgery alone is often insufficient for complete resolution of OSA in PWS patients.
  • Children with PWS considered for growth hormone therapy require pre-treatment OSA assessment via polysomnography.
  • Management of OSA in PWS may necessitate tonsillectomy and/or CPAP, with reassessment prior to hormonal therapy.
Abstract

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