Management of Ehlers-Danlos Related OSA with Home Sleep Testing and Auto-CPAP

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Introduction: Ehlers-Danlos syndrome (EDS) represents a group of connective tissue disorders that includes increased tissue laxity and cartilage defects. Upper airway and nasal-maxillary cartilage defects increase the risk of obstructive sleep apnea (OSA) and 32% of patients with this syndrome are reported to have OSA. Report of Case: A 43 years-old man diagnosed with EDS presented with night time awakenings associated with loud snoring and daytime sleepiness. Neck size was 42.5 cm, body-mass index of 27.94, tonsils +1 and Friedman tongue position 1. Past medical history included aortic aneurysm and sinus pauses. He was scheduled later that week for pacemaker placement for sinus pauses associated with syncopal episodes. To expedite the patient's treatment of suspected OSA before pacemaker placement, we opted for home sleep testing. The test showed respiratory index of 21 events/hour, oxygen saturation nadir of 84% and 25 minutes of oxygen desaturation to 88% or lower. Continuous positive airway pressure (CPAP) in auto mode with pressure range of 4?20 cmH2O was ordered and patient reported improvement of symptoms after starting treatment. On follow up patient described better sleep and more energy through the day. However, he later reported that his wife witnessed him snoring with CPAP therapy although the machine reported control of respiratory events with apnea/hypopnea index of 3.1 per hour. He also complained of residual sleepiness throughout the day with daily naps lasting 1?2 hours. Consequently, a titration study with CPAP has been scheduled. Conclusion: Home sleep test is an appropriate method for diagnosis of OSA in patients with EDS and suspected OSA which can improve quality of life and decrease morbidity. However, given complexity of sleep disordered breathing in patients with connective tissue disorders auto-CPAP may not effectively treat OSA in this population and an in-lab titration study should be considered.

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