Because of associated abnormalities affecting connective tissue in various organs including airways, hypermobility syndrome has been associated with high risk for the development sleep apnea. Ehlers-Danlos syndrome (EDS) and Marfan syndrome (MFS) represent the most common hypermobility syndromes; therefore, the purpose of this review was to examine the prevalence of obstructive sleep apnea (OSA) in these populations.
All publications and poster presentations written in English found through August 2018 that describe the prevalence of sleep apnea among people with EDS or MFS were included.
A total of 13 studies were identified, 7 for EDS and 6 for MFS. A combined random prevalence rate of OSA across both populations was 48.9% (95% confidence interval 38.3-59.6), with a slightly higher rate of 59.7% (39.7-77.0) for MFS versus 39.4% (28.8-51.1) for EDS. However, a high degree of heterogeneity across studies was found in both groups (EDS group: Q = 28.6 and I2 = 79.0; MFS group: Q = 37.1 and I2 = 86.5). When directly compared to the general population, patients with EDS/MFS were on average six times more likely (odds ratio 6.28 [95% confidence interval 3.31-11.93], P < 0.001, Z = 5.61) to have a diagnosis of OSA.
OSA is a previously underestimated EDS/MFS-related complication. The high prevalence of OSA might be the result of bony and soft-tissue abnormalities associated with these hypermobility syndromes. Untreated OSA is thought to worsen cardiovascular complications especially among those with MFS. Further research is needed to better delineate whether the prevalence of OSA is moderated by factors such as sex, body mass index, bony structure, and disorder subtype.