The exstrophy experience: A national survey assessing urinary continence, bladder management, and oncologic outcomes in adults.

INTRODUCTION AND OBJECTIVE: The bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of congenital genitourinary malformations with an incidence of 1:10,000 to 1:50,000. Advances in reconstructive surgical techniques have improved clinical outcomes, but there is a paucity in data about disease sequela in adulthood. This is the largest survey to date in the United States exploring the urinary continence, bladder management, and oncologic outcomes in adults with BEEC. METHODS: Respondents were over the age of 18 with a diagnosis of bladder exstrophy, cloacal exstrophy, or epispadias. They were treated at the authors' institution, included in the Association for the Bladder Exstrophy Community (A-BE-C) mailing list, and/or engaged in A-BE-C social media. A survey was created using uniquely designed questions and questionnaires. Survey responses between May 2020 and July 2020 were processed using Research Electronic Data Capture (REDCap). Quantitative and qualitative statistics were used to analyze the data with significance at p < 0.05. RESULTS: A total of 165 patients completed the survey. The median age was 31.5 years (IQR 25.9-45.9). Many patients considered themselves continent of urine, with a median satisfaction score of 74 (IQR 50-97) on a scale from 0 (consider themselves to be completely incontinent) to 100 (consider themselves to be completely continent). There was less leakage among those with a continent urinary diversion compared to those who void or catheterize per urethra (p = 0.003). Patients with intestinal-urinary tract reconstruction, such as augmentation cystoplasty or neobladder creation, were more likely to perform bladder irrigations (p = 0.03). Patients with continent channels were more likely to report UTI than all other forms of bladder management (89.0% vs. 66.2%, p = 0.003). Three (1.9%) patients were diagnosed with bladder cancer. A small portion of patients (27.2%) were given bladder cancer surveillance recommendations by a physician. DISCUSSION: Most patients achieved a satisfactory level of urinary continence, with the highest continence rates in those with a continent urinary diversion. Those with intestinal-urinary tract reconstruction were more likely to perform bladder irrigations, perhaps to avoid complications from intestinal mucous production. The rates of self-reported UTI and were higher in patients with continent channels, but recurrent UTIs were not affected by the type of genitourinary reconstruction. Bladder cancer exists in this population, highlighting the need for long-term follow-up. CONCLUSION: Most BEEC patients achieve a satisfactory level of urinary continence, with the best outcomes in those with a continent urinary diversion. This population requires long-term follow-up with a transitional urologist to ensure adequate oncologic care.

Patient-reported outcomes in adult females with bladder exstrophy: A study of long-term sexual, reproductive and urinary outcomes using social media.

INTRODUCTION: Limited data exist on patient-reported outcomes in adults with bladder exstrophy (BE). We partnered with the Association for the Bladder Exstrophy Community (A-BE-C) using social media to survey adult females with BE. The aim of the study was to assess long-term patient-reported sexual, reproductive and continence outcomes. MATERIALS AND METHODS: Between December 3, 2018 and January 18, 2019, A-BE-C promoted an anonymous survey of adult females with BE on social media. The survey included the Female Sexual Function Index (FSFI) and International Consultation on Incontinence Questionnaire - Short Form (ICIQ-SF) in addition to questions on demographics, reproductive and gynecological outcomes. RESULTS: A total of 130 women with a median age of 30 years (IQR 26, 41) completed the survey. The majority of women were born in the United States (N = 86, 66.2%). Women reported a median of 10 (IQR 5, 17) surgeries performed for their condition. The mean ICIQ-SF score was 6.2 ± 6.2 (moderate). Only 19.2% (N = 25) reported volitionally voiding and the majority reported intermittent catheterization through a catheterizeable channel or the urethra (Summary Table). The mean FSFI score was (20.1 ± 9.0), indicating risk for sexual dysfunction (FSFI < 26.55). Of the respondents, 28.5% (N = 37) were treated for uterine prolapse, 56.9% (N = 74) required surgery in order to use tampons or have penetrative intercourse, and over half (55.4%) reported some degree of dissatisfaction with the appearance of their external genitalia. Forty-seven women (36.2%) reported pregnancies, and of these 32 (68.1%) reported complications with pregnancy. Outcomes of 100 pregnancies included miscarriage/abortion (41.0%), preterm vaginal (3.0%), preterm cesarean section (19.0%), term vaginal (2.0%), and term cesarean section (35.0%). Seven (15.2%) women identified having children with a medical diagnosis, none of which included BE. Infertility was reported in 17.3% (22/127) women. Women identified sexual function, fertility, and body image as areas that need future research. CONCLUSION: This is the largest study of patient-reported outcomes in females with BE achieved through partnership with an advocacy group and social media. We found that continence and sexual function were both impaired in adult women with BE. Respondents reported poor obstetric and gynecological outcomes including infertility, uterine prolapse, vaginal stenosis, and need for multiple surgeries. BE surgeons, through multi-institutional collaboration, should continue to reassess surgical techniques at initial repair to better address these functional issues. These results should be a part of counseling parents of newborns with BE.