Prevalence of Scoliosis in Hypermobile Ehlers-Danlos Syndrome

Abstract

Objective: The main purpose of this study was to evaluate the prevalence, form, and severity of scoliosis in a population of adults meeting the 2017 criteria for hypermobile Ehlers-Danlos syndrome (hEDS). The second objective was to compare the prevalence of scoliosis versus other criteria at initial hEDS diagnosis. Methods: A retrospective study looking at the frequency and severity of scoliosis in adults (N=28) meeting the 2017 diagnostic criteria for hEDS through analysis of a full spine EOS® X-ray (EOS imaging, Paris, France) performed at the initial diagnosis. Severity was defined by the Cobb angle. Results: At the initial diagnosis, the mean age was 30.1 years (standard deviation [SD]: ±10.18 years). Twenty-nine percent (n=8/28) of patients fulfilling hEDS criteria presented with scoliosis. Thirty-two percent (n=9/28) of patients had scoliotic inflection and 39% (n=11/28) had no scoliosis. Scoliosis was mild-to-moderate with a mean Cobb angle of 13.6° (SD: ±3.5°). None of the patients had severe scoliosis requiring surgery. Compared to the 2017 diagnostic criteria, it is noteworthy that scoliosis prevalence in this present study population ranks at the level of the most frequent ones. Conclusion: This study provides interesting information regarding frequency of scoliosis and scoliotic inflection in a group of patients with hEDS. Although the patients did not present with severe forms of scoliosis at initial diagnosis, the results highlight the importance of systematically looking for scoliosis in adult and young patients, in order to follow progression and ensure appropriate management.