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Bladder exstrophy: We need to improve. A lot

Published:November 15, 2021DOI:https://doi.org/10.1016/j.jpurol.2021.11.007

      Summary

      Introduction

      Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients' point of view are needed to get a real-life perspective on the problem.

      Study design

      A self-developed questionnaire concerning sexual, psychosexual and psychosocial outcomes was sent to the adult members of the Brazilian Exstrophy Group.

      Results

      Fifty out of 67 adults from the group (74.5%) responded to the questionnaire. Failure of initial bladder closure attained 62%. Almost ¾ of the patients had augmentation cystoplasty. Bladder lithiasis was common. Esthetic procedures were frequently done. Repetitive UTI (n = 32, 64%) and kidney scars/disease (n = 20, 40%) were frequent. Most (88%) patients either depend on CIC or remain incontinent. Sexual problems predominated in males. Surgery for continence often failed, requiring re-operations, but the prognosis without these procedures was comparatively worse. Continent patients underwent more surgeries (mean 18, 13 and 9 procedures in continent, imperfectly continent and incontinent patients, respectively). Augmented patients more frequently achieved dryness (p = 0.0035). Two-thirds of the women underwent vaginoplasties, but dyspareunia/feeling of “tight” vagina still affected a quarter of them. Four women (15.4%) delivered healthy children. 91.7% of the males reported “normal” erections, but sexual inhibition was common due to feeling of having a small penis (n = 18, 75%). Persistent dorsal curvature and abnormal ejaculation were common (58.3% and 77.1%, respectively). Patients' comments related mainly to mental health issues/need for specialized care, limitations of medicine to cure/treat their disease, unavailability of experts, especially adult specialists, embarrassment over deformities and insufficient information about disease/treatment/prognosis.

      Discussion

      Most BE patients are well-integrated into society, but feelings of sadness and low self-esteem are common. Most welcome procedures to become dry, despite self-catheterization. The results of bladder neck reconstruction are far from perfect, despite multiple attempts and bladder augmentation was often necessary. Volitional voiding is uncommon. Sexual problems are worse for males, and sexual avoidance is common. Sexual function and self-image are inter-related. It seems reasonable to offer selective esthetic procedures to improve social/sexual interaction. Obstetric complications are common, especially UTI, need for ureteral and/or conduit stenting, abnormal fetal positioning, uterine prolapse, technical problems during surgical deliveries and prematurity.

      Conclusion

      Summary Table
      Total Female Male Perfectly continent Incontinent
      N 50 26 24 21 11
      Age (mean) 32.36 31.46 33.33 30.57 38.64
      >1 closure 31 (62%) 13 (50%) 18 (75%) 16 (76.2%) 5 (45.5%)
      Augmentation 36 (72%) 21 (80.8%) 15 (62.5%) 18 (85.7%) 3 (27.3%)
      Comparison between continent and incontinent, significant.
      BNR 21 (42%) 13 (50%) 8 (33.3%) 11 (52.4%) 2 (18.2%)
      BNC 23 (46%) 12 (46.2%) 11 (45.8%) 14 (66.7%) 3 (27.3%)
      Comparison between continent and incontinent, significant.
      Secondary BNC 12/23 (51.14%) 8/13 (63%) 4/11 (36.4%)
      comparison between males and females, significant.
      7/14 (50%) 1/3 (9.1%)
      Comparison between continent and incontinent, significant.
      Esthetic surgery 18 (36%) 14 (53.9%) 4 (16.7%)
      comparison between males and females, significant.
      8 (31%) 0
      Comparison between continent and incontinent, significant.
      Cystolithotomy 23 (46%) 12 (46.2%) 11 (45,8%) 13 (61.9%) 2 (18.2%)
      Comparison between continent and incontinent, significant.
      BE hampered schooling 31 (62%) 14 (53.9%) 17 (70,8%) 12 (57.1%) 6 (54.6%)
      On CIL 32 (64%) 19 (73.1%) 11 (45.8%) 17 (76.2%)
      2 cases treated with ureterosigmoidostomy and one patient that did not respond to continence-related questions excluded.
      1
      Comparison between continent and incontinent, significant.
      Incontinent 12 (24%) 5 (13.2%) 7 (29.2%) 0 (0) 11 (100%)
      BE limits friendships 12 (24% 6 (20.1%) 9 (25%) 6 (28.6%) 1
      BElimits romantic relationships 29 (58%) 10 (38.5%) 19 (79.2%)
      comparison between males and females, significant.
      13 (61.9%) 4 (36.4%)
      BElimits sexual relationships 31 (62%) 12 (46.2%) 19 (79.2%)
      comparison between males and females, significant.
      6 (54.5%) 6 (54.6%)
      Deformities/scars limit social relationships 36 (72%) 16 (61.5%) 20 (83.3%)
      comparison between males and females, significant.
      16 (76.2%) 7 (63.6%)
      UTI 32 (64%) 22 (84.6%) 10 (41.7%)
      comparison between males and females, significant.
      11 (52.4%) 7 (63.6%)
      Kidney disease/scar 20 (40%) 11 (42.3%) 9 (37.5%) 5 (23.8%) 4 (36.4%)
      a comparison between males and females, significant.
      b Comparison between continent and incontinent, significant.
      c 2 cases treated with ureterosigmoidostomy and one patient that did not respond to continence-related questions excluded.

      Keywords

      Abbreviations:

      BE (bladder exstrophy), QOL (quality of life), LMIC (low medium income country), BNR (bladder neck reconstruction), BNI (bladder neck injection), BNC (bladder neck closure), MACE (Malone-type antegrade colonic enema conduit), UTI (urinary tract infection), CIC (clean intermittent catheterization), HRQOL (health-related quality of life)
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