Urinary tract issues frequent in EDS patients: Review
Surgery more difficult for EDS patients, researchers say
People with Ehlers-Danlos syndrome (EDS) frequently experience urinary problems, most commonly urinary incontinence and pelvic organ prolapse, according to a review study.
Urinary incontinence is the involuntary release of urine. Pelvic organ prolapse occurs when pelvic organs, such as the bladder, parts of the large intestine, or reproductive organs, drop from their positions. Urinary incontinence has been reported in 50%-60% of EDS patients and pelvic organ prolapse in 29%-75% of cases.
Similarly high rates of these urinary problems were reported among people with joint hypermobility syndrome (JHS), a condition marked by joint hypermobility, or abnormally mobile joints, also a hallmark of most EDS types.
The findings show that EDS and JHS patients “suffer from more frequent and more severe lower urinary tract disorders than the general population, and it seems more difficult to opt for invasive treatments given the risk of complication or surgical failure,” the researchers wrote.
The study, “Lower urinary tract involvement in Ehlers Danlos and Joint Hypermobility Syndromes: Review of the literature,” was published in The French Journal of Urology.
Lower urinary tract in EDS
EDS encompasses a group of genetic conditions that affect the connective tissues — those that provide structure to joints, skin, blood vessels, and other tissues and organs. Most types of EDS are associated with soft and fragile skin that is easily damaged, as well as joint hypermobility.
Hypermobile EDS, the most common EDS type, shares some symptoms with JHS, including joint hypermobility and muscle and/or bone damage. The two conditions have been compared and studied together.
While a large portion of the urinary system is made of connective tissue, little is known about lower urinary tract involvement in EDS and JHS. The lower urinary tract comprises the bladder and urethra, the tube that carries urine from the bladder to out of the body.
To estimate the prevalence and management of lower urinary tract issues in people with EDS or JHS, researchers in France systematically reviewed published studies up to December 2022 reporting such rates and management strategies.
A total of 73 studies were included in the analysis: 48 regarding EDS and 25 focusing on JHS.
Both EDS and JHS patients were more affected by lower urinary tract disorders than the general population. Those disorders included urinary incontinence, pelvic organ prolapse, uncontrollable need to urinate, and recurrent urinary infections.
Regarding EDS, urinary incontinence, assessed by patient surveys, clinical evaluation, and severity (regarding leakage frequency and need for surgical intervention), was reported in 50%-60% of cases.
Rates of pelvic organ prolapse in EDS patients ranged between 29% and 75%. This condition was estimated to affect 37% of women older than 50, who were diagnosed at a mean age of 41, and the condition significantly affected quality of life, according to three studies.
But one study reported pelvic organ prolapse only affected in 1.33% of women with EDS.
Bladder diverticula, a condition marked by the formation of pouches out of the bladder wall, was reported in 16% to 25% of EDS patients, and was described in several case reports. It was also associated with multiple urinary tract infections in six clinical cases.
There were some reports of spontaneous bladder rupture, while 18% of EDA patients reported the need to regularly urinate at night.
Rates of urinary problems were similarly high among people with JHS. Particularly, urinary incontinence was reported in 41%-73% of cases, and recurrent urinary tract infections in 24%. People with JHS were also two times more likely to develop pelvic organ prolapse.
Treatment of urinary symptoms included physical therapy or drug treatments such as desmopressin and oxybutynin. Surgical treatments were used to manage urinary incontinence and prolapse, but among patients with hereditary EDS or JHS, this approach was associated with a risk of post-surgery complications and recurrence.
“This is probably linked to the quality of the tissue, and raises the question of surgical methods, which should perhaps be different from those used in the general population,” the researchers wrote. “In the absence of a wealth of scientific literature on the subject, and with the emergence of specialized multidisciplinary medical units, studies focusing on functional and morphological aspects will need to be carried out.”
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