Introduction Sleep abnormalities are recognized complications of Prader-Willi Syndrome (PWS). The prevalence of sleep disorders has varied across studies with a reported range of OSA from 44% to 100%.1 In prvious studies, a link has been suggested between the risk of OSA in those with PWS and body mass index (BMI), growth hormone treatment(GHT)2 and the genetic type. Therefore, we set to examine the incidence and management of OSA as well as the relationship with GHT in the Irish paediatric population.
Methods This is a retrospective chart review of patients with a diagnosis of PWS and OSA from 1999–2018. We looked at polysomnography (PSG) results, ENT evaluation and subsequent recommended interventions. We categorised the type of sleep disorder and intervention used. After which we followed up with a post intervention PSG examining efficiency of treatment. PSG results were interpreted by the Paediatric Respiratory physician at Tallaght University Hospital.
Results A total of 49 patients were identified in the 19 years period, 14 of which were excluded (1 died, 5 left the country, and 7 had limited data). Of the remaining 35 patients, twenty patients (57%) were diagnosed with moderate/severe OSA. Of those with moderate/severe, 15 of 20 (75%) were received GHT, of whom 5 (33.3%) developed a worsening of OSA. Eight of 35 patients (22.8%) patients had a normal PSG, of whom one (12.5%) developed OSA after initiating GHT. Seven (20%) patients experienced equivocal PSG results. Notably, four of 7 (57.1%) patients with equivocal polysomnogray results developed OSA, of whom one wasn’t on GHT(25%). Of the 35 patients adentonsillectomy was recommended in 6 (17.1%).
Conclusion In the paediatric Irish population with PWS, the overall incidence of OSA was 71.4%. Our study confirmed that all children with PWS who are being considered for growth hormone therapy should primarily undergo assessment for OSA by polysomnography and should be referred for management by ENT for possible adenotonsillectomy. It also shows that despite ENT intervention or growth hormone therapy PSG remains a much needed screening for OSA in children with PWS.
Sedky K, Bennett DS, Pumariega A. Prader willi syndrome and obstructive sleep apnea: co-occurrence in the pediatric population. J Clin Sleep Med. 2014;10(4):403–409. doi:10.5664/jcsm.3616.
Nixon GM, Rodda CP, Davey MJ. Longitudinal association between growth hormone therapy and obstructive sleep apnea in a child with Prader-Willi syndrome. J Clin Endocrinol Metab. 2011;96(1):29–33. doi:10.1210/jc.2010-1445.
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