(1055) Prevalence of Obstructive Sleep Apnea and Other Pharyngeal Symptoms in Pediatric Ehlers Danlos Patients

Introduction: This study seeks to characterize the prevalence of obstructive sleep apnea (OSA) and related surgical interventions, dysphagia, and dysphonia in pediatric Ehlers Danlos syndrome (EDS) patients compared to a control population.

Methods: TriNetX was queried for patients ≤18 years with and without a diagnosis of EDS on January 13, 2024. Patients with a history of acute recurrent tonsillitis were excluded from both the EDS and control cohorts. Patients within the queried groups with a diagnosis of OSA, dysphagia, dysphonia, and common surgical interventions for OSA were recorded and reported in the results as risk ratios.

Results: Out of 10,104, 652 children in the Ehlers Danlos population had a diagnosis of OSA (6.453%, 95% CI: 5.991-6.950), which was a 4.434 times greater risk (95% CI: 4.116-4.776) than the control population (n = 17,208,689). This group also had higher rates of tonsillectomy and adenoidectomy (RR: 2.507), adenoidectomy only (RR:2.152), and tonsillectomy only (RR: 4.486). In addition, 739 Ehlers Danlos patients had dysphagia, a 5.907 times increased risk (95% CI: 5.51-6.332) from the control, and 57 EDS patients had dysphonia, which was a relative risk of 2.901 (95% CI: 2.239-3.759) compared to control.

Conclusions: Pediatric Ehlers Danlos patients had a greater risk of being diagnosed with OSA, dysphagia, and dysphonia, as well as increased rates of undergoing tonsillectomy and adenoidectomy procedures. The increased risk for these diagnoses may be due to poor craniofacial soft tissue integrity in Ehlers Danlos syndrome, which can affect collagen types I and III. The higher rates of tonsillectomy and adenoidectomy surgeries may reflect a general approach to treating pediatric OSA but may not be appropriate for this patient population. Further study into whether pediatric OSA patients with Ehlers Danlos syndrome benefit from tonsillectomy and adenoidectomy as well as alternative appropriate first line treatments.