Prevalence of Urinary, Prolapse, and Bowel Symptoms in Mayer-Rokitansky-Küster-Hauser Syndrome.

Abstract Background Mullerian agenesis, or Mayer-Rokitansky-Kuster-Hauser syndrome, occurs in 1 in 4500-5000 individuals assigned female sex at birth. Pelvic floor symptoms among individuals with Mayer-Rokitansky-Kuster-Hauser syndrome have not been well-studied and it is unknown how vaginal lengthening treatments affect these symptoms. Objective(s) To assess urinary, prolapse, and bowel symptoms in individuals with MRKH and to determine if symptoms vary by vaginal lengthening treatment. Study Design We conducted a cross-sectional study in 2019 using an online survey distributed by the Beautiful You MRKH Foundation via social media to individuals with Mayer-Rokitansky-Kuster-Hauser syndrome. Demographics, age at and timing of diagnosis, information about VL treatment, urinary symptoms (Michigan Incontinence Symptom Index), prolapse symptoms (Pelvic Organ Prolapse Distress Inventory short-form), and bowel symptoms (Bristol Stool Form Scale) were obtained. Inclusion criteria included: self-reported diagnosis of mullerian agenesis and female sex. Respondents with a history of renal transplant or dialysis, completion of Results Of 808 respondents, 615 met inclusion criteria, representing 40 countries. Eighty-one percent identified as Caucasian. Median age of participants was 29 (IQR 24-36), with a median age at diagnosis of 16 (IQR 15-17). Among respondents, 54% (331/614) had VL treatment, 130 of whom (39%) had undergone surgical vaginal lengthening. Of individuals with Mayer-Rokitansky-Kuster-Hauser syndrome, 70% (428/614) reported having had one or more urinary symptoms and 79% (339/428) reported being bothered by these symptoms. Urinary symptoms included urinary incontinence (34%, 210/614), urinary frequency (40%, 245/614), urinary urgency (40%, 248/614), pain with urination (16%, 97/614), and recurrent urinary tract infections (29%, 177/614). Prolapse symptoms included lower abdominal pressure (41% 248/612), pelvic heaviness or dullness (29%, 177/610), and vaginal bulge (11% 68/609). Constipation and anal incontinence were reported by 25% (153/611) and 25% (153/608), respectively, of the cohort. Aside from recent urinary incontinence and anal incontinence, the prevalence of pelvic floor symptoms did not differ significantly between those with and without vaginal lengthening. Among those with surgical vaginal lengthening, symptomatic vaginal bulge was highest in individuals who underwent a bowel vaginoplasty procedure. Conclusion(s) Urinary, prolapse, and bowel symptoms are common among individuals with Mayer-Rokitansky-Kuster-Hauser syndrome and should be evaluated in this population. Overall, when compared to no vaginal lengthening treatment, having vaginal lengthening treatment is not associated with significant differences in the prevalence of pelvic floor symptoms, with the exception of recent urinary incontinence and anal incontinence. Our data suggest that bowel vaginoplasty may be associated with greater symptoms of vaginal bulge. More robust studies are needed to determine the impact of various vaginal lengthening treatments on pelvic floor symptoms.