Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report

Submitted: 11 May 2023
Accepted: 2 August 2023
Published: 29 August 2023
Abstract Views: 93
PDF: 84
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Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure’s potential long-term risks, which will necessitate a limited but regular follow-up.

Ebert AK, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis 2009;4:23-39 DOI:

Gearhart JP, Jeffs RD. Exstrophy-epispadias complex and bladder anomalies. In: Walsh, PC, Retnik AB, Vaughan ED, Wein AJ. (eds.): Campbell's Urology. (7th ed.), 1998

Inouye B M, Tourchi A, Di Carlo HN, et al. Modern Management of the Exstrophy-Epispadias Complex. Surg Res Practice 2014:587064. DOI:

Wu WJ, Maruf M, Mnyevitch R, et al. Delaying primary closure of classic bladder exstrophy: When is it too late? J Pediatr Urol 2020;16:834-838. DOI:

Ahn JJ, Shnorhavorian M, Katz C, et al. Early versus delayed closure of bladder exstrophy: A National Surgical Quality Improvement Program Pediatric analysis. J Pediatr Urol 2018;14:27.e1-27.e5. DOI:

Sanni RB, Denoulet D, Coulibaly B, et al. The treatment of bladder exstrophy at the University Hospital Center in Treichville (Abidjan). Ann Urol (Paris) 1997;31:371-374.

Hosseini SM, Sabet B, Zarenezhad M. Abdominal wall closure in bladder exstrophy complex repair by rectus flap. Ann Afr Med 2011;10:243-245. DOI:

Stec AA, Pannu HK, Tadros YE, et al. Pelvic floor anatomy in classical bladder exstrophy by using 3-dimensional computerized tomography: Initial insights. J Urol 2001;166:1444-1449. DOI:

Sponseller PD, Bisson LJ, Gearhart JP, Jeffs RD, Magid D, Fishman E. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg 1995;77:177-189. DOI:

Eldirdiri S, Elmushly RM, Elazhary SG. Late presentation of ectopia vesica with malignant transformation. A case report and review. Int J Surg Case Rep 2018;48:43-46 DOI:

Mansour AM, Sarhan OM, Helmy TE, et al. Management of bladder exstrophy epispadias complex in adults: Is abdominal closure possible without osteotomy? World J Urol 2010;28:199-204. DOI:

Abdur-Rahman LO. The perils of bladder exstrophy repairs in Africa. Ann Afr Med 2014;13:232-233

Andrawes SS, Kanyi SS, Poenaru D. Mainz Pouch II for Bladder Extropy, 18 years’ experience, Poster, 20th Congress International Society of Urology, October 10-11, 2020.

Mteta KA, Mbwambo JS, Eshleman JL, Aboud MM, Oyieko W. Urinary diversion in children with mainly exstrophy and epispadias: Alternative to primary bladder closure. Central Afr J Surg 2000; 46:318-320 DOI:

Hollowell JG, Ransley PG. Surgical Management of Incontinence in Bladder Exstrophy. Br J Urol 1991;68:543-548. DOI:

Wilkinson J, Pope R, Kammann TJ, et al. The ethical and technical aspects of urinary diversions in low-resource settings: a commentary. BJOG 2016;123:1273-1277. DOI:

Spence HM, Hoffman WW, Fosmire GP. Tumour of the colon as a late complication of ureterosigmoidostomy for exstrophy of the bladder. Br J Urol 1979;51:466-470. DOI:

Fisch M, Wammack R, Müller SC, Hohenfellner R. The Mainz pouch II (sigma rectum pouch). J Urol 1993;149:258-263. DOI:

Mingin GC, Stock JA, Hanna MK. The Mainz II pouch: experience in 5 patients with bladder exstrophy. J Urol 1999;162:846-848 DOI:

Inouye BM, Di Carlo HN, Young EE, et al. Secondary reclosure in classic bladder exstrophy: challenges and outcomes. Urology 2015;85:1179-1182 DOI:

Promm M, Roesch WH. Recent Trends in the Management of Bladder Exstrophy: The Gordian Knot Has Not Yet Been Cut. Front Pediatr 2019;7:110 DOI:

Cuckow PM, Cao KX. Meeting the challenges of reconstructive urology – Where are we now? J Pediatr Surg 2019;54:223-228 DOI:

Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol 2006;24:244-249. DOI:

Maruf M, Manyevitch R, Michaud J, et al. Urinary Continence Outcomes in Classic Bladder Exstrophy: A Long-Term Perspective. J Urol 2020;203:200-205 DOI:

Purves JT, Gearhart JP. The Bladder Exstrophy–Epispadias– Cloacal Exstrophy complex in Pediatric urology / [edited by] John P. Gearhart, Richard C. Rink, Pierre D.E. Mouriqand. — 2nd ed. 2010 Saunders DOI:

Chinda JY, Abubakar AM, Dabkana TM, et al. Exstrophy anomalies: Preliminary review of 18 cases in Maiduguri, North-eastern Nigeria. Ann Afr Med 2013;12:212-216 DOI:

Jumbi T, Mwika P, Shahbal S, et al. Challenges in the management of bladder exstrophy. An African case series and review of literature. Surg Case Rep Rev 2020;4:1-5

Kouame BD, Kouame GS, Sounkere M, et al. Aesthetic, urological, orthopaedic and functional outcomes in complex bladder exstrophy-epispadias’ management. Afr J Paediatr Surg 2015;12:56-60 DOI:

Wiersma R. Overview of bladder exstrophy: a third world perspective. J Pediatr Surg 2008;43:1520-1523. DOI:

Mouafo Tambo FF, et al. Exstrophie vésicale fermée à propos d'un cas rare à l'Hôpital Gynéco-Obstétrique et Pédiatrique de Yaoundé. Revue Africaine de Chirurgie et Spécialités 2015;9:247-55.

Traore MT, Niang L, Jalloh M, et al. Prise en charge de l'exstrophie vésicale à propos de 7 cas. Uro Andro 2016;5:247-249.

Tshimbayi M, Ndua D, Kazadi C, et al. Exstrophie vésicale: à propos d'un cas diagnostiqué tardivement. Pan Afr Med J 2014;17:172 DOI:

Ziouziou I, Karmouni T, El Khader K, et al. L'exstrophie vésicale chez l'adulte: A propos de 5 cas. Afr J Urol 2014;20:53-58. DOI:

Poenaru D. The burden of pediatric surgical disease in low-resource settings: Discovering it, measuring it, and addressing it. J Pediatr Surg 2016;51:216-220 DOI:

Van Batavia JP, Shukla AR, Joshi RS, Reddy PP. Pediatric Urology and Global Health: Why now and how to build a successful global outreach program Urol Clin North Am 2018;45:623–631 DOI:

Joshi RS, Shrivastava D, Grady R, et al. A Model for Sustained Collaboration to Address the Unmet Global Burden of Bladder Exstrophy-Epispadis Complex and Penopubic Epispadis: The International Bladder Exstrophy Consortium. JAMA Surg 2018;153:618-624 DOI:

Rubenwolf PC, Hampel C, Roos F, et al. Continent Anal Urinary Diversion in Classic Bladder Exstrophy: 45-Year Experience. Urology 2017;100:249-254

Pahernik S, Beetz R, Schede J, Stein R, Thüroff JW. Rectosigmoid pouch (Mainz Pouch II) in children. J Urol 2006;175:284-287 DOI:

Stein R, Fisch M, Hohenfellner R Applications of Ureterosigmoidostomy in Bladder Exstrophy. In: Gearhart JP, Mathews R. (eds) The Exstrophy—Epispadias Complex. 1999. Springer, Boston, MA DOI:

Rubenwolf P, Thomas C, Thüroff JW, Stein R. Sexual Function, Social Integration and Paternity of Males with Classic Bladder Exstrophy following Urinary Diversion. J Urol 2016;195:465-470 DOI:

Rubenwolf PC, Hampel C, Roos F, et al. Continent Anal Urinary Diversion in Classic Bladder Exstrophy: 45-Year Experience. Urology 2017;100:249–54. DOI:

Reifsnyder JE, Hanna MK. Advances in bladder substitution and creation of neobladders in children. F1000Res. 2019 Nov 26;8:F1000 Faculty Rev-1992. DOI:

Bastian PJ, Albers P, Haferkamp A, Schumacher S, Müller SC. Modified ureterosigmoidostomy (Mainz Pouch II) in different age groups and with different techniques of ureteric implantation. BJU In 2004;94:345-349 DOI:

Alemu MH Mainz II pouch: continent urinary diversion, for bladder exstrophy epispadia complex and irreparable VVF: a 5-year comprehensive retrospective analysis. Ethiop Med J 2010;48:57-62

LeBlanc D, Muma K, Ayele F, etv al. Management of bladder exstrophy using the Mainz II procedure in a resource-limited setting: a multisite study. J Can Chir 2015;58:166

Ayele F, Chung K, Smith A, Poenaru D. Urinary diversion using Mainz Pouch II in bladder exstrophy: an Ethiopian experience. Ann Ped Surg 2015;11:65–112

Calisti, A., Belay, K., Mombo, A., Nugud, F. A., Salman, D. Y., & Lelli Chiesa, P. (2023). Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report. La Pediatria Medica E Chirurgica, 45(2).


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