The Indian Journal of Pediatrics

, Volume 84, Issue 9, pp 715–720 | Cite as

Exstrophy Bladder – Reconstruction or Diversion for the Underprivileged

Review Article


The surgical techniques for management of bladder exstrophy epispadias complex have evolved from staged reconstruction, complete primary repair to radical mobilization. Post-operative complications add to the multiplicity of surgical procedures at each step. The end results are variable with many achieving continence rates of 85–89% only after bladder augmentation and clean intermittent catheterization. The situation is further complicated in resource-poor settings, where illiteracy and poverty are the driving factors for choosing a single operative procedure for creation of low pressure reservoir aiming at upper tract preservation and good primary continence. Thus, primary urinary diversion should be offered as a surgical option to patients with limited access to health care facilities. Yogesh’s cystorectostomy is a modification of Heitz-Boyer-Hovelacque procedure, wherein the bladder plate is directly anastomosed to the recto-sigmoid pouch, without mobilizing the ureters from their original location. The short-term follow-ups are encouraging with all achieving total urinary continence over the ensuing months. The upper tract functions are well preserved, along with huge parental and patient satisfaction and overall improvement in the quality of life.


Bladder exstrophy Developing countries Epispadias Health services accessibility Urinary incontinence 



VS did review of literature, prepared the initial draft and YKS conceptualized the article, described the surgical steps and the initial experience with a novel procedure and sorted the final draft. YKS shall stand as guarantor for the article.

Compliance with Ethical Standards

Conflict of Interest


Source of Funding



  1. 1.
    Mahajan JK, Rao KL. Exstrophy-epispadias complex- issues beyond the initial repair. Indian J Urol. 2012;28:382–7.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Gearhart JP. The bladder exstrophy-epispadias-cloacal exstrophy complex. In: Gearhart JP, Rink RC, Mouriquand PDE, editors. Pediatric Urology, Chapter 32, vol. 2. Philadelphia: WB Saunders Co.; 2001. p. 511–46.Google Scholar
  3. 3.
    Boyadjiev SA, Dodson JL, Radford CL, et al. Clinical and molecular characterization of the bladder exstrophy-epispadias complex: analysis of 232 families. BJU Int. 2004;94:1337–43.CrossRefPubMedGoogle Scholar
  4. 4.
    Epidemiology of bladder exstrophy and epispadias: a communication from the International Clearing House for Birth Defects Monitoring Systems. Teratology. 1987;36:221–7.Google Scholar
  5. 5.
    Bennet AH. Exstrophy of bladder treated by ureterosigmoidostomies, long-term evaluation. Urology. 1973;2:165–8.CrossRefGoogle Scholar
  6. 6.
    Ives E, Coffey R, Carter CO. A family study of bladder exstrophy. J Med Genet. 1980;17:139–41.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of bladder exstrophy in the United States. J Urol. 2005;173:1728–31.CrossRefPubMedGoogle Scholar
  8. 8.
    Simon J. Ectopia vesicae (absence of the anterior walls of the bladder and pubis abdominal parietis); operation for directing the orifices of the ureters into the rectum; temporary success; subsequent death; autopsy. Lancet. 1852;60:568–70.CrossRefGoogle Scholar
  9. 9.
    Coffey RC. Production of aseptic ureteroenterostomy: by a suture transfixing the ureteral wall and the intestinal mucosa. JAMA. 1930;94:1748–50.Google Scholar
  10. 10.
    Lepor H, Jeffs RD. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol. 1983;130:1142–5.CrossRefPubMedGoogle Scholar
  11. 11.
    Baker LA Gearhart JP. The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol. 1998;16:205–11.CrossRefPubMedGoogle Scholar
  12. 12.
    Ray AK, Mukherjee NN, Mukherjee S, Mukherjee P. Total correction of bladder exstrophy – our experience in 37 patients. Indian J Urol. 2002;18:117–9.Google Scholar
  13. 13.
    Shaw MB, Rink RC, Kaefer M, et al. Continence and classic bladder exstrophy treated with staged repair. J Urol. 2004;172:1450–3.CrossRefPubMedGoogle Scholar
  14. 14.
    Mouriquand PD. Bubanj T, Feyaerts, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int. 2003;92:997–1001.Google Scholar
  15. 15.
    Gargollo PC, Borer JG, Diamond DA, et al. Prospective follow up in patients after complete primary repair of bladder exstrophy. J Urol. 2008;180:1665–70.CrossRefPubMedGoogle Scholar
  16. 16.
    Mollard P, Mouriquand PD, Buttin X. Urinary continence after reconstruction of classical bladder exstrophy (73 cases). Br J Urol. 1994;73:298–302.Google Scholar
  17. 17.
    Venkatramani V, Chandrasingh J, Devasia A, Kekre NS. Exstrophy-epispadias complex presenting in adulthood: a single center review of presentation, management and outcomes. Urol. 2014;84:1243–7.CrossRefPubMedGoogle Scholar
  18. 18.
    Bhatnagar V. Bladder exstrophy: an overview of the surgical management. J Indian Assoc Pediatr Surg. 2011;16:81–7.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Shoukry AI, Shoukry I. Management of bladder exstrophy in adulthood: report of 5 cases. J Pediatr Urol. 2013;9:575–8.CrossRefPubMedGoogle Scholar
  20. 20.
    Dodson JL, Surer I, Baker LA, Jeffs RD, Gearhart JP. The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome. J Urol. 2001;165:1656–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Purves JT. Modern approaches in primary exstrophy closure. Semin Pediatr Surg. 2011;20:79–84.CrossRefPubMedGoogle Scholar
  22. 22.
    Jeffs RD, Schwartz GR. Ileal conduit urinary diversion in children: computer analysis of followup from 2 to 16 years. J Urol. 1975;114:285–8.CrossRefPubMedGoogle Scholar
  23. 23.
    Hendren WH. Exstrophy of the bladder: an alternative method of management. J Urol. 1976;115:195–202.CrossRefPubMedGoogle Scholar
  24. 24.
    Mitchell ME, Piser JA. Intestinocystoplasty and total bladder replacement in children and young adults: followup in 129 cases. J Urol. 1987;138:579–84.CrossRefPubMedGoogle Scholar
  25. 25.
    Stein R, Fisch M, Black P, Hohenfellner R. Strategies for reconstruction after unsuccessful or unsatisfactory primary treatment of patients with bladder exstrophy or incontinent epispadias. J Urol. 1999;161:1934–41.CrossRefPubMedGoogle Scholar
  26. 26.
    Koo HP, Avolio L, Duckett JW Jr. Long-term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol. 1996;156:2037–40.CrossRefPubMedGoogle Scholar
  27. 27.
    Pahernik S, Beetz R, Schede J, Stein R, Thüroff JW. Recto sigmoid pouch (Mainz pouch II) in children. J Urol. 2006;175:284–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Miles-Thomas J, Gearhart JP, Gearhart SL. An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy. J Gastrointest Surg. 2006;10:473–7.CrossRefPubMedGoogle Scholar
  29. 29.
    Gobet R, Weber D, Renzulli P, Kellenberger C. Long-term follow up (37-69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol. 2009;5:190–6.CrossRefPubMedGoogle Scholar
  30. 30.
    Smeulders N, Woodhouse CRJ. Neoplasia in adult exstrophy patients. BJU Int. 2001;87:623–8.CrossRefPubMedGoogle Scholar
  31. 31.
    Harzmann R, Kopper B, Carl P. Cancer induction by urinary drainage or diversion through intestinal segments? [Article in German]. Urologe A. 1986;25:198–203.PubMedGoogle Scholar
  32. 32.
    Urdaneta LF, Duffell D, Creevy CD, Aust JB. Late development of primary carcinoma of the colon following ureterosigmoidostomy: report of three cases and literature review. Ann Surg. 1966;164:503–13.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Leadbetter GW Jr, Zickerman P, Pierce E. Ureterosigmoidostomy and carcinoma of the colon. J Urol. 1979;121:732–5.CrossRefPubMedGoogle Scholar
  34. 34.
    Woodhouse CR; British Society for Gastroenterology; Association of Coloproctology for Great Britain and Ireland. Guidelines for monitoring of patients with ureterosigmoidostomy. Gut. 2002;51:V15–6.Google Scholar
  35. 35.
    Pettersson L, Tranberg J, Abrahamsson K, Pettersson S, Sillen U, Jonsson O. Half century of followup after ureterosigmoidostomy performed in early childhood. J Urol. 2013;189:1870–5.CrossRefPubMedGoogle Scholar
  36. 36.
    Stöckle M, Becht E, Voges G, Riedmiller H, Hohenfellner R. Ureterosigmoidostomy: an outdated approach to bladder exstrophy? J Urol. 1990;143:770–4.CrossRefPubMedGoogle Scholar
  37. 37.
    Reiner WG, Gearhart JP, Jeffs R. Psychosexual dysfunction in males with genital anomalies: late adolescence, Tanner stages IV to VI. J Am Acad Child Adolesc Psychiatry. 1999;38:865–72.CrossRefPubMedGoogle Scholar
  38. 38.
    Reiner WG, Gearhart JP, Kropp B. Suicide and suicidal ideation in classic exstrophy. J Urol. 2008;180:S1661–4.CrossRefGoogle Scholar
  39. 39.
    Lee C, Reutter HM, Grässer MF, Fisch M, Noeker M. Gender-associated differences in the psychosocial and developmental outcome in patients affected with the bladder exstrophy-epispadias complex. BJU Int. 2006;97:349–53.CrossRefPubMedGoogle Scholar
  40. 40.
    Dodson JL, Furth SL, Yenokyan G, et al. Parent perspectives of health related quality of life for adolescents with bladder exstrophy-epispadias as measured by the child health questionnaire-parent form 50. J Urol. 2010;184:1656–61.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryMaulana Azad Medical CollegeNew DelhiIndia
  2. 2.Division of Urology, Renal Transplant and Robotics, Medanta – The MedicityGurgaonIndia

Personalised recommendations