Prader Willi syndrome and obstructive sleep apnea: co-occurrence in the pediatric population

Abstract

Background: A high prevalence of obstructive sleep apnea (OSA) occurs in children with Prader-Willi syndrome (PWS). Yet, due in part to the relatively small samples previously used, the prevalence of OSA has varied greatly across studies. It is also unclear if factors such as age, gender, body mass index (BMI), or type of genetic imprinting are associated with increased risk for OSA among children with PWS.

Objectives: To evaluate the (a) prevalence of OSA, as well as narcolepsy, in pediatric populations diagnosed with PWS; (b) effects of age, gender, body mass index, and genetic imprinting on OSA severity; and (c) efficacy of adenotonsillectomy (AT) for decreasing OSA severity in this population.

Methods: All studies assessing OSA among children with PWS through August 2013 were identified using the PubMed/Medline, Psych Info, Cochrane library, and Google Scholar data bases.

Results: Fourteen studies of children diagnosed with PWS and who were assessed for OSA using polysomnography (PSG) met inclusion criteria (n = 224 children). The prevalence of OSA across studies was 79.91% (n = 179/224). Among youths with OSA, 53.07% had mild OSA, 22.35% moderate OSA, and 24.58% severe OSA. Narcolepsy was found to occur in 35.71% of children with PWS. Adenotonsillectomy was associated with improvement in OSA for most children with PWS. However, residual OSA was present in the majority of cases post-surgery.

Conclusion: This study confirms the high prevalence of OSA and narcolepsy among children with PWS. Screening for OSA and narcolepsy among children with PWS is recommended. In addition, while adenotonsillectomy was effective in reducing OSA for some children, alternative treatments may need to be considered, given the only moderate response rate.

Keywords: Prader-Willi syndrome; meta-analysis; narcolepsy; obstructive sleep apnea; sleep disordered breathing.

Figure 1. Scatterplot of the relation between apnea