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

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.