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Original article
Longitudinal evaluation of sleep disordered breathing in infants with Prader-Willi syndrome
  1. Abdullah Khayat1,2,
  2. Indra Narang1,2,
  3. Saadoun Bin-Hasan1,2,
  4. Reshma Amin1,2,
  5. Suhail Al-Saleh1,2
  1. 1 University of Toronto, Toronto, Ontario, Canada
  2. 2 Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada
  1. Correspondence to Dr Suhail Al-Saleh, Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada; suhail.al-saleh{at}sickkids.ca

Abstract

Objective To evaluate the course of sleep disordered breathing (SDB) in infants with Prader-Willi syndrome (PWS).

Design Retrospective longitudinal observational study.

Setting Sleep laboratory at The Hospital for Sick Children, Toronto, Canada.

Patients Infants with PWS.

Main outcome measures The natural history of SDB in infants with PWS within 2 years from baseline assessment.

Results We identified 28 (12 male) infants with PWS who had a baseline polysomnography (PSG) at a median age (interquartile (IQR)) of 0.9 (0.5, 1.1) years. The median central apnoea index (CAI) at baseline was 6.6 events/hour (IQR 2.6, 12.1). Of these, 15/28 (53%) infants with PWS were diagnosed with significant central sleep apnoea (CSA) (CAI≥5 events/hour). Median age (IQR) at follow-up PSG was 2.1 (1.5, 2.6) years. The median CAI improved from 6.6 to 2.3 events/hour (p<0.0001). Only four infants with PWS had persistent CSA at the time of the follow-up PSG. Furthermore, three infants with PWS were diagnosed with mild-to-moderate obstructive sleep apnoea (OSA) that has improved at follow-up studies whereas two patients with PWS with no evidence of OSA at baseline were diagnosed with severe OSA on the follow-up PSG requiring adenotonsillectomy. The overall median obstructive apnoea–hypopnoea index was similar between baseline and follow-up studies (0.6 and 0.8, respectively, p=0.91).

Conclusions CSA is prevalent in infants with PWS but usually improves with age. However, these patients continue to require ongoing PSG surveillance because some infants will have persistent CSA and others are at risk of developing OSA.

  • Central sleep apnea
  • Obstructive sleep apnea
  • Sleep disordered breathing
  • Prader-Willi Syndrome.
  • infants

https://doi.org/10.1136/archdischild-2016-311298

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    Footnotes

    • Contributors AK contributed substantially as a first author by designing the study, collecting data, reviewing the cases, reviewing the statistical analyses, writing the manuscript and approval of the final version of the manuscript. IN contributed substantially as a second author by reviewing of the cases, writing the manuscript, revising the manuscript critically and approval of the final version of the manuscript. SB-H contributed substantially as a third author by detailed data collection and review of the cases and revising the manuscript as well as approval of the final version of the manuscript. RA contributed substantially as a forth author by reviewing statistical results and revising the manuscript critically and approval of the final version of the manuscript. SA-S contributed substantially as a senior author by designing the study, detailed review of the data and statistical results, writing the manuscript and approval of the final version of the manuscript.

    • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Ethics approval The Hospital for Sick Children’s Research Ethics Board.

    • Provenance and peer review Not commissioned; externally peer reviewed.