Abstract
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.
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References
Mahajan JK, Rao KL. Exstrophy-epispadias complex- issues beyond the initial repair. Indian J Urol. 2012;28:382–7.
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.
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.
Epidemiology of bladder exstrophy and epispadias: a communication from the International Clearing House for Birth Defects Monitoring Systems. Teratology. 1987;36:221–7.
Bennet AH. Exstrophy of bladder treated by ureterosigmoidostomies, long-term evaluation. Urology. 1973;2:165–8.
Ives E, Coffey R, Carter CO. A family study of bladder exstrophy. J Med Genet. 1980;17:139–41.
Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of bladder exstrophy in the United States. J Urol. 2005;173:1728–31.
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.
Coffey RC. Production of aseptic ureteroenterostomy: by a suture transfixing the ureteral wall and the intestinal mucosa. JAMA. 1930;94:1748–50.
Lepor H, Jeffs RD. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol. 1983;130:1142–5.
Baker LA Gearhart JP. The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol. 1998;16:205–11.
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.
Shaw MB, Rink RC, Kaefer M, et al. Continence and classic bladder exstrophy treated with staged repair. J Urol. 2004;172:1450–3.
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.
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.
Mollard P, Mouriquand PD, Buttin X. Urinary continence after reconstruction of classical bladder exstrophy (73 cases). Br J Urol. 1994;73:298–302.
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.
Bhatnagar V. Bladder exstrophy: an overview of the surgical management. J Indian Assoc Pediatr Surg. 2011;16:81–7.
Shoukry AI, Shoukry I. Management of bladder exstrophy in adulthood: report of 5 cases. J Pediatr Urol. 2013;9:575–8.
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.
Purves JT. Modern approaches in primary exstrophy closure. Semin Pediatr Surg. 2011;20:79–84.
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.
Hendren WH. Exstrophy of the bladder: an alternative method of management. J Urol. 1976;115:195–202.
Mitchell ME, Piser JA. Intestinocystoplasty and total bladder replacement in children and young adults: followup in 129 cases. J Urol. 1987;138:579–84.
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.
Koo HP, Avolio L, Duckett JW Jr. Long-term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol. 1996;156:2037–40.
Pahernik S, Beetz R, Schede J, Stein R, Thüroff JW. Recto sigmoid pouch (Mainz pouch II) in children. J Urol. 2006;175:284–7.
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.
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.
Smeulders N, Woodhouse CRJ. Neoplasia in adult exstrophy patients. BJU Int. 2001;87:623–8.
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.
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.
Leadbetter GW Jr, Zickerman P, Pierce E. Ureterosigmoidostomy and carcinoma of the colon. J Urol. 1979;121:732–5.
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.
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.
Stöckle M, Becht E, Voges G, Riedmiller H, Hohenfellner R. Ureterosigmoidostomy: an outdated approach to bladder exstrophy? J Urol. 1990;143:770–4.
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.
Reiner WG, Gearhart JP, Kropp B. Suicide and suicidal ideation in classic exstrophy. J Urol. 2008;180:S1661–4.
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.
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.
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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.
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Sarin, Y.K., Sekhon, V. Exstrophy Bladder – Reconstruction or Diversion for the Underprivileged. Indian J Pediatr 84, 715–720 (2017). https://doi.org/10.1007/s12098-017-2419-9
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DOI: https://doi.org/10.1007/s12098-017-2419-9