Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review

Abstract

The Ehlers-Danlos syndromes (EDS) and associated hypermobility spectrum disorders (HSD) are a heterogenous group of connective tissue disorders associated with significant morbidity. The urogenital aspects of these disorders are understudied and there is little guidance on the prevalence, types, or outcomes of urogenital complications in EDS/HSD. Our objective was to perform a scoping review to characterize and synthesize the literature reporting urogenital and pelvic complications in EDS/HSD patients. We performed a systematic search of three databases (Medline, CINAHL, Embase) to January 2019. English language, full-text articles reporting on urogenital or pelvic complications in EDS/HSD were included. A total of 105 studies were included (62 case reports/series, 43 observational) involving patients with hypermobile (23%), vascular (20%), classical (12%) EDS, and HSD (24%). Some studies looked at multiple subtypes (11%) or did not report subtype (33%). Reported complications included urinary (41%), gynecological (36%), obstetrical (25%), renal (9%), and men's health problems (7%), with some studies reporting on multiple areas. Urinary and gynecological complications were most prevalent in patients with HSD, while a broad range of complications were reported in EDS. While further research is required, results suggest a higher index of suspicion for urogenital problems is probably warranted in this population.

Pain Symptomatology and Management in Pediatric Ehlers–Danlos Syndrome: A Review

“…Therefore, future research within pediatric EDS which considers the frequency with which more intrusive, severe dysmenorrhea is endorsed would be valuable. Furthermore, given that pain presents as a primary gynecological complaint (e.g., vulvodynia, vestibulodynia, dyspareunia, and generalized pelvic pain [60]) in adult females with EDS, the greater study of such pain, beyond dysmenorrhea, is warranted to facilitate earlier and more effective intervention.…”

High rate of dyspareunia and probable vulvodynia in Ehlers–Danlos syndromes and hypermobility spectrum disorders: An online survey

“…Several EDS studies have categorized dyspareunia and vulvodynia as the same condition (Castori et al, 2012Castori, Morlino, et al, 2013); however, more specifically, dyspareunia is a symptom of vulvodynia. No studies have examined vulvodynia in women with EDS or HSD (Castori et al, 2012;Castori, Morlino, et al, 2013;Chopra et al, 2017;Gilliam, Hoffman, & Yeh, 2020;Hugon-Rodin et al, 2016;Hurst et al, 2014;McIntosh, Mallett, Frahm, Richardson, & Evans, 1995).…”

Obstetrics and gynecology in Ehlers‐Danlos syndrome: A brief review and update

“…Dyspareunia is also reported at an increased frequency in women with EDS. A systematic review of 105 publications was performed, looking at the urogenital and pelvic complications found in EDS and hypermobility spectrum disorders (HSD) (Gilliam, Hoffman, & Yeh, 2019).…”

“…Vulvodynia and pelvic pain also occur with increased frequency in EDS (Castori et al, 2012;Gilliam et al, 2019). The etiology is peripheral sensitization of the vulva or generalized urogenital, pelvic, and/or central nervous sensitivity.…”

“…In the review by Gilliam, Hoffman, and Yeh (2019), 21 of the 23 studies, which looked at pelvic organ prolapse found an association with ESD or HSD. In the study by Nee et al (2019), uterine prolapse was reported by 13.1% of EDS patients and 16.4% reported rectal prolapse.…”