Prevalence of obstructive sleep apnea and related oropharyngeal symptoms in pediatric Ehlers Danlos patients.

OBJECTIVE

To evaluate the prevalence of obstructive sleep apnea (OSA), oropharyngeal symptoms, and surgical OSA treatment in children with Ehlers Danlos syndrome (EDS) compared to control.

STUDY

DESIGN

Retrospective cohort study.

SETTING

TriNetX database.

METHODS

TriNetX was used to query for patients ≤18 years old with and without a diagnosis of EDS. Patients with any recorded occurrence of OSA, oropharyngeal symptoms, and adenotonsillar surgeries were recorded and reported.

RESULTS

Among 10104 patients ≤18 years old with EDS, 652 (6.45 %, 95 % CI: 5.99-6.95) had a recorded diagnosis of OSA with a 4.43 (95 % CI: 4.12-4.78) times higher risk of diagnosis compared to a control cohort (n = 17208689). Pediatric EDS patients also had a higher risk of dysphagia and dysphonia at 5.91 (95 % CI: 5.51-6.33) and 2.90 (95 % CI: 2.24-3.76), respectively. Additionally, children with EDS were more likely to undergo an adenotonsillectomy compared to control (2.51, 95 % CI: 2.23-2.82).

CONCLUSION

Children with EDS are at a higher risk of developing OSA and other oropharyngeal symptoms like dysphagia and dysphonia. These patients are also more likely to undergo adenotonsillar surgeries as a first-line approach to correcting obstructive sleep apnea. Otolaryngologists and speech language pathologists can play a major beneficial role in working with pediatric EDS patients in the management and treatment of oropharyngeal symptoms.