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The bladder exstrophy-epispadias complex (BEEC) belongs to one of the most devastating urological malformations affecting the complete urinary tract including the genitalia. A semi-standardized questionnaire was used to evaluate the functional and psychosocial developmental outcome in 122 affected patients recruited from German-speaking self-referral support groups. The questionnaire covered mode of reconstruction, subjective assessment of continence, milestones of child development, education, school performance, level of life satisfaction, anxieties, and in patients older than 16 years partnership experience. The results showed a mean of one surgery in the age group 0-4, of four surgeries in both age groups 5-13 and 14-20, and seven surgeries in patients aged 20 and over. The continence rates for the latter three age groups were 36, 64, and 80%, respectively. We found that the neurocognitive development was unremarkable. Most patients showed above average performance in school and in their profession. On the other hand, they exhibited psychological anxieties and worries in their experience with sexuality and partnerships. Future studies will be necessary to clarify the association of urological status, functional impairments, and psychosocial adaptation as a basis for improved approaches to comprehensive care and support.
The bladder exstrophy-epispadias complex (BEEC) belongs to one of the most devastating urological malformations affecting the complete urinary tract including the genitalia. A semi-standardized questionnaire was used to evaluate the functional and psychosocial developmental outcome in 122 affected patients recruited from German-speaking self-referral support groups. The questionnaire covered mode of reconstruction, subjective assessment of continence, milestones of child development, education, school performance, level of life satisfaction, anxieties, and in patients older than 16 years partnership experience. The results showed a mean of one surgery in the age group 0-4, of four surgeries in both age groups 5-13 and 14-20, and seven surgeries in patients aged 20 and over. The continence rates for the latter three age groups were 36, 64, and 80%, respectively. We found that the neurocognitive development was unremarkable. Most patients showed above average performance in school and in their profession. On the other hand, they exhibited psychological anxieties and worries in their experience with sexuality and partnerships. Future studies will be necessary to clarify the association of urological status, functional impairments, and psychosocial adaptation as a basis for improved approaches to comprehensive care and support.
Background The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction. Aim The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population. Methods We conducted a descriptive, cross-sectional study of men diagnosed with classic presentations of bladder exstrophy or epispadias. Outcomes Patients were asked to complete 4 validated questionnaires: the International Index of Erectile Function (IIEF)-5, the Erection Hardness Score (EHS), the Self-Esteem and Relationship, and the International Consultation Incontinence modular Questionnaire-Short Form. Fertility potential was assessed with semen analysis and a non-validated questionnaire. Results 38 Patients 18–64 years old (M [mean] = 32.2) completed the questionnaires. The average IIEF-5 score was 18.1/25 (ranging from 3–25; SE = 7.62), with results indicating that 55% of the sample had normal erectile function. Results also showed higher scores for patients with normal spermatozoa concentration (M = 22.75, SE = 1.89, P = .08) than for those with oligospermia (M = 17.30, SE = 8.53, P = .08). Results on the IIEF-5 also indicated higher scores for patients who conceived children without assisted reproductive technologies (ART) (M = 22.83, SE = 2.317, P = .02) than for patients without children (M = 15.76, SE = 8.342, P = .02). The average EHS was 3.43/4 (ranging from 1–4, SE = 0.9). EHS was higher for patients who had reconstruction than for patients who had cystectomy (M = 3.88, SE = 1.07 and 2.78, SE = 1.09, P = .02). The average total Self-Esteem and Relationship score was 67.04/100 (ranging from 10.71–96.43, SE = 22.11). The average total International Consultation Incontinence modular Questionnaire-Short Form score was 4.97/21 (ranging from 0–18, SE = 5.44), higher score indicating more urinary incontinence. Among the patients surveyed, 31.6% were parents at the time of study and 50% of them benefited from ART. With regards to the 14 semen analyses performed, only 7.1% produced normal results and 44.7% indicated that ejaculation was weak and dribbling. Clinical Translation Erectile function appears to be decreased and psychological aspects of sexuality indicate low self-esteem about sexual relationship. Although ethical problems could not allow prospective spermograms, our cohort is large enough to provide significant data. Conclusions Early sperm storage for future ART, sexual medicine management, and complementary genital reconstruction in adulthood constitute potential treatment options for this population.
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