Sleep disorders and their management in children with Ehlers-Danlos syndrome referred to sleep clinics

Keren Armoni Domany, Sumalee Hantragool, David F. Smith, Yuanfang Xu, Monir Hossain, Narong Simakajornboon

Research output: Contribution to journalArticlepeer-review

Abstract

Study Objectives: The nature of sleep disorders in children with Ehlers-Danlos syndrome (EDS) is unknown. We aimed to describe the type, the management, and the short-term outcome of sleep disorders in children with EDS referred to sleep clinics. Methods: This is a retrospective review of medical records and polysomnography tests of children with EDS younger than 18 years who were referred to the sleep clinic. Demographic information and medical history were collected, and polysomnography tests were reviewed. Questionnaires completed during previous clinic visits, including the Pediatrics Sleep Questionnaire (PSQ), Epworth Sleepiness Scale (ESS), and Pediatric Quality of Life Inventory (PedsQL), were also evaluated. Results: Sixty-five patients with EDS-hypermobility type were included. The mean age was 13.15 ± 3.9 years. There were 68% of patients who were female, and 91% of patients were Caucasian. The mean follow-up period was 1.14 ± 1.55 years. Common sleep diagnoses included insomnia (n = 14, 22%), obstructive sleep apnea (OSA) (n = 17, 26%), periodic limb movement disorder (PLMD) (n = 11, 17%), and hypersomnia (n = 10, 15%). In addition, 65% required pharmacologic treatment and 29% were referred to behavioral sleep medicine. For OSA, two patients required continuous positive airway pressure. A significant improvement was observed in the PSQ, ESS, and PedsQL scores during follow-up visits after treatment (n = 34; P =.0004, 0.03, and 0.01, respectively). Conclusions: There is a high prevalence of sleep disorders, including OSA, insomnia, PLMD, and hypersomnia in children with EDS referred to sleep clinics. Specific management can improve quality of life and questionnaire scores of this patient population. Our study emphasizes the importance of screening for sleep disorders in children with EDS.

Original languageEnglish
Pages (from-to)623-629
Number of pages7
JournalJournal of Clinical Sleep Medicine
Volume14
Issue number4
DOIs
StatePublished - 15 Apr 2018

Keywords

  • Ehlers-Danlos
  • Obstructive
  • Pediatrics
  • Sleep apnea

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