Advertisement

Long Term Results of Bladder Exstrophy

The Mainz Experience
  • Raimund Stein
  • Margit Fisch
  • Rudolf Hohenfellner
Chapter

Abstract

After primary bladder closure or urinary diversion, other factors apart from the reconstruction gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility.

Between 1968 and 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime.

94% of the patients with rectal reservoirs and 97% of those with a Mainz pouch I are continent day and night, whereas only 3 of 6 patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young Dees procedure are continent. The upper urinary tract remained stable at the latest followup in between 95% (Mainz pouch) and 98% of the renal units (rectal reservoirs).

Of the 104 patients 48 attend school, 4 are in college, 42 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic center, 4 attend kindergarten and 2 are younger than 4 years of age.

In females reconstruction of the external genitalia resulted in normal sexuality and fertility. Reconstruction was performed in 25 and fixation of the uterus in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital recon-struction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered 7 children by Caesarean section.

In male patients cosmetic results were satisfactory, however, at the expense of the fertility. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. 9 males developed epididymitis, necessitating 2 orchiectomies and 3 vasectomies. No patient with reconstruction of the external genitalia ejaculates normally or has fathered children, whereas ejaculation was normal in 3 men who did not undergo genital reconstruction. Of these, 2 men have fathered 4 children.

Conclusion: In individuals with the bladder exstrophy-epispadias complex Education, Occupation and social development is normal. The cosmetic results achieved by genital reconstruction are satisfactory. In female patients, antefixation of the uterus should be performed before or together with an introitusplasty to prevent uterine prolapse. In male patients, however, surgery is performed at the expense of fertility.

Keywords

Cosmetic Result Urinary Diversion External Genitalia Uterine Prolapse Retrograde Ejaculation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    C. R. J. Woodhouse: Exstrophy and epispadias. In: Long-term paediatric urology. Edited by C. R. J. Woodhouse. Oxford, England: Blackwell Scientific Publications, pp. 127–150, 1991.Google Scholar
  2. 2.
    C. R. Blakeley and W. G. Mills: The obestric and gynaecological complications of bladder exstrophy and epispadias. Br J Obestet Gynaecol, 88: 167–173, 1981.CrossRefGoogle Scholar
  3. 3.
    K. A. Burbrige, T. W. Hensle, W. J. Chambers, R. Leb and K. F. Jeter: Pregnancy and sexual function in women with bladder exstrophy. Urology, 28: 12, 1986.CrossRefGoogle Scholar
  4. 4.
    R. J. Rhodes: Cosmetic vulval surgery following repair of ectopia vesicae. Proc. Roy. Soc. Med., 70: 534–537, 1977.PubMedGoogle Scholar
  5. 5.
    J. Dewhurst, P. J. Toplis and J. H. Shepherd: Ivalon sponge hysterosacropexy for genital prolapse in patients with bladder exstrophy. Br. J. Obstet. Gynaecol., 87: 67–69, 1980.PubMedCrossRefGoogle Scholar
  6. 6.
    A. H. Bennett: Exstrophy of bladder treated by ureterosigmoidostomies: long-term evaluation. Urology, 2: 165–168, 1973.PubMedCrossRefGoogle Scholar
  7. 7.
    H. Lepor, E. Sharpio and R. D. Jeffs: Urethral reconstruction in boys with classical bladder exstrophy. J Urol, 131: 512–515, 1984.PubMedGoogle Scholar
  8. 8.
    H. J. Mesrobian, P. P. 4. Kelalis and S. A. Kramer: Long-term followup of cosmetic appearance and genital function in boys with exstrophy: review of 53 patients. J Urol, 136: 256–258, 1986.Google Scholar
  9. 9.
    H. G. Mesrobian, P. P. Kelalis and S. A. Kramer: Long-term followup of 103 patients with bladder exstrophy. Journal of Urology, 139: 719–722, 1988.PubMedGoogle Scholar
  10. 10.
    G. Audry, C. Grapin, S. Loulidi, M. Gruner and J. Brueziere: Avenir genital des garcons atteints d’exstrophie vesicale ou d’epispadias avec incontinence. Ann Urol, 24: 120–124, 1991.Google Scholar
  11. 11.
    M. K. Hanna and D. 1. Williams: Genital function in males with vesical exstrophy and epispadias. Br J Urol, 44: 169–174, 1972.PubMedCrossRefGoogle Scholar
  12. 12.
    A. Zabbo and R. Kay: Ureterosigmoidostomy and bladder exstrophy: A long-term follow-up. Journal of Urology, 136: 396–398, 1986.PubMedGoogle Scholar
  13. 13.
    J. F. Eid, P. Rosenberg, K. Rothaus, D. Mininberg, L. Hoffmann and E. D. Vaughan: Use of tissue expanders in final reconstruction of infra pubic midline scar, mons pubis and vulva after bladder exstrophy repair. Urology, 41: 426–430, 1993.PubMedCrossRefGoogle Scholar
  14. 14.
    F. Marconi, P. Messina, P. Pavanello and R. de Castro: Cosmetic reconstruction of the mons veneris and lower abdominal wall by skin expansion as the last stage of the surgical treatment of bladder exstrophy: a report of three cases. Plast Reconstr Surg, 91: 551–555, 1993.PubMedCrossRefGoogle Scholar
  15. 15.
    M. P. Krisiloff, P. J. Puchner, W. Tretter, M. T. MacFarlane and J. K. Lattimer: Pregnancy in women with bladder exstrophy. J Urol, 119: 478–479, 1978.PubMedGoogle Scholar
  16. 16.
    E. W. Overstreet and J. F. Hinman: Some Gynaecologic aspects of bladder exstrophy. West. J. Surg. Obestet. Gynaecol., 64: 131–137, 1956.Google Scholar
  17. 17.
    C. Clemetson: Ectopia vesicae and split pelvis. Br. J. Obestet. Gynaecol., 65: 973–981, 1958.CrossRefGoogle Scholar
  18. 18.
    E. Sharpio, H. Lepor and R. D. Jeffs: The inheritance of the exstrophy-epispadias complex. J. Urol., 132: 308–310, 1984.Google Scholar
  19. 19.
    W. A. Kennedy, H. T. W., E. A. Reiley, H. E. Fox and T. Haus: Pregnancy after orthotopic continent urinary diversion. Surg. Gynecol. Obstet., 177: 405, 1993.Google Scholar
  20. 20.
    J. P. Gearhart and R. D. Jeffs: Exstrophy of the bladder, epispadias and other bladder anomalies. In: Campbell’s Urology. Edited by R. C. Walsh, A. B. Retik, T. A. Stamey and E. D. Vaughan. New York: Saunders, 2, pp. 1772–1821, 1992.Google Scholar
  21. 21.
    M. D. Bastuba, M. M. Alper and R. D. Oates: Fertility and the use of assisted reproductive techniques in the adult male exstrophy/epispadias patient. Fertil. Steril., 60: 733–740, 1993.PubMedGoogle Scholar
  22. 22.
    G. Palermo, H. Jorisk, M.-P. Derde, M. Camus, P. Devroey and A. van Steirteghem: Sperm characteristics and outcome of human assisted fertilization by subzonal insemination and intracytoplasmatic sperm injection. Fertil. Steril., 59: 826–830, 1993.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1999

Authors and Affiliations

  • Raimund Stein
    • 1
    • 2
  • Margit Fisch
    • 1
  • Rudolf Hohenfellner
    • 1
  1. 1.Department of UrologyJohannes Gutenberg University, Medical SchoolMainzGermany
  2. 2.MainzGermany

Personalised recommendations