Lower urinary tract involvement in Ehlers-Danlos and Joint Hypermobility syndromes: Review of the literature

Abstract

Introduction: Ehlers-Danlos syndrome (EDS) and Joint Hypermobility syndrome (JHS) are still poorly understood, with a prevalence of 1/5000 for EDS and 1/500 for JHS. They are characterized by multisystem involvement. Urological involvement has been little studied. The aim was to carry out a review of the literature on the urological involvement of EDS and JHS.

Method: A review of the literature was carried out using the following databases: Pubmed, Canadian Hospitals and EMBASE. Search terms were "Ehlers-Danlos" or "Joint Hypermobility" associated with "Urology", "Bladder", "Pelvic Organ Prolapse", "Urinary Retention", "Leak", "Leakage", "Urinary Incontinence", "Urinary Tract Infection" and "Urdodynamic", no filters were added.

Results: Seventy-three articles were included for a total of 259 found. The prevalence of urinary incontinence in EDS is estimated at 50-60%, and that of pelvic organ prolapse (POP) at 29-75%. Bladder diverticula are also frequently reported. For JHS, the prevalence of urinary incontinence is estimated between 40 and 73%, that of POP increased with 73% of stage greater than 2, g-JHS patients are almost 3 times more affected by prolapses (OR=2.37) which seem more severe. Patients with vesicoureteral reflux, most often severe, are more affected by joint hypermobility (OR=2.79). Few studies have been carried out on urological assessment and treatment modalities.

Conclusion: EDS patients often have urinary incontinence, pelvic organ prolapse or bladder diverticula. JHS patients frequently have urinary incontinence, pelvic organ prolapse and vesicoureteral reflux.

Keywords: Bladder diverticula; Ehlers-Danlos; Joint hypermobility; Urinary incontinence; Urology.