https://doi.org/10.4081/pmc.2023.323
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
Accepted: 2 August 2023
Abstract Views: 753
PDF: 316
HTML: 0
HTML: 0
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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: https://doi.org/10.1186/1750-1172-4-23
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: https://doi.org/10.1155/2014/587064
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: https://doi.org/10.1016/j.jpurol.2020.09.003
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: https://doi.org/10.1016/j.jpurol.2017.11.008
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: https://doi.org/10.4103/1596-3519.84704
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: https://doi.org/10.1016/S0022-5347(05)65805-3
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: https://doi.org/10.2106/00004623-199502000-00003
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: https://doi.org/10.1016/j.ijscr.2018.04.041
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: https://doi.org/10.1007/s00345-009-0436-7
Abdur-Rahman LO. The perils of bladder exstrophy repairs in Africa. Ann Afr Med 2014;13:232-233
DOI: https://doi.org/10.4103/1596-3519.142301
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: https://doi.org/10.4314/cajm.v46i12.8577
Hollowell JG, Ransley PG. Surgical Management of Incontinence in Bladder Exstrophy. Br J Urol 1991;68:543-548.
DOI: https://doi.org/10.1111/j.1464-410X.1991.tb15402.x
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: https://doi.org/10.1111/1471-0528.13934
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: https://doi.org/10.1111/j.1464-410X.1979.tb03580.x
Fisch M, Wammack R, Müller SC, Hohenfellner R. The Mainz pouch II (sigma rectum pouch). J Urol 1993;149:258-263.
DOI: https://doi.org/10.1016/S0022-5347(17)36050-0
Mingin GC, Stock JA, Hanna MK. The Mainz II pouch: experience in 5 patients with bladder exstrophy. J Urol 1999;162:846-848
DOI: https://doi.org/10.1097/00005392-199909010-00075
Inouye BM, Di Carlo HN, Young EE, et al. Secondary reclosure in classic bladder exstrophy: challenges and outcomes. Urology 2015;85:1179-1182
DOI: https://doi.org/10.1016/j.urology.2015.01.029
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: https://doi.org/10.3389/fped.2019.00110
Cuckow PM, Cao KX. Meeting the challenges of reconstructive urology – Where are we now? J Pediatr Surg 2019;54:223-228
DOI: https://doi.org/10.1016/j.jpedsurg.2018.10.070
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: https://doi.org/10.1007/s00345-006-0053-7
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: https://doi.org/10.1097/JU.0000000000000505
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: https://doi.org/10.1016/B978-1-4160-3204-5.00030-X
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: https://doi.org/10.4103/1596-3519.122688
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: https://doi.org/10.4103/0189-6725.150985
Wiersma R. Overview of bladder exstrophy: a third world perspective. J Pediatr Surg 2008;43:1520-1523.
DOI: https://doi.org/10.1016/j.jpedsurg.2007.11.005
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: https://doi.org/10.11604/pamj.2014.17.172.3031
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: https://doi.org/10.1016/j.afju.2013.09.003
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: https://doi.org/10.1016/j.jpedsurg.2015.10.065
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: https://doi.org/10.1016/j.ucl.2018.06.009
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: https://doi.org/10.1001/jamasurg.2018.0067
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: https://doi.org/10.1016/S0022-5347(05)00035-2
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: https://doi.org/10.1007/978-1-4757-3056-2_16
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: https://doi.org/10.1016/j.juro.2015.08.076
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: https://doi.org/10.1016/j.urology.2016.11.026
Reifsnyder JE, Hanna MK. Advances in bladder substitution and creation of neobladders in children. F1000Res. 2019 Nov 26;8:F1000 Faculty Rev-1992.
DOI: https://doi.org/10.12688/f1000research.16607.1
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: https://doi.org/10.1111/j.1464-410X.2004.04963.x
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
How to Cite
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). https://doi.org/10.4081/pmc.2023.323
Copyright (c) 2023 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Similar Articles
- A. Gentili, L. Pasini, V. Bachiocco, V. Landuzzi, L. Giuntoli, M. Lima, S. Baroncini, Cardiocirculatory intraoperative assessment during single-shot caudal anaesthesia in children: comparison between levobupivacaine and ropivacaine , La Pediatria Medica e Chirurgica: Vol. 34 No. 3 (2012)
- S. Rugolotto, R. Beghini, I. Cogo, G. Sidoti, E.M. Padovani, A. Pietrobelli, Epoprostenol for very low birth weight (VLBW) infants: a novel dilution protocol , La Pediatria Medica e Chirurgica: Vol. 35 No. 5 (2013)
- Salvatore Fabio Chiarenza, Elena Carretto, Valeria Bucci, Samuele Ave, Giuseppe Pulin, Cosimo Bleve, Uretero-pelvic junction obstruction in children: Is vascular hitch an effective and safe solutions in very long term outcome? Report of 25 years follow-up , La Pediatria Medica e Chirurgica: Vol. 45 No. 1 (2023)
- Andrea Zangari, Carmine Noviello, Camilla Todesco, Mercedes Romano, Letizia Trotta, Carmine Botta, Ilaria Cascone, Salvatore Scommegna, Gabriele Vasta, Vito Briganti, Alfonso Papparella, Satisfaction and results of the subareolar incision as treatment for gynecomastia in adolescents: experience of two centers , La Pediatria Medica e Chirurgica: Vol. 46 No. 2 (2024)
- Chiara Costantini, Federica Fati, Elisa Pani, Fabio Beretta, Silvia Bisoffi, Giosuè Mazzero, Elisa Negri, Clara Revetria, Hamid R. Sadri, Enrico Ciardini, Two-balloon epistaxis catheter to ensure vaginal patency in a complex case of vaginoplasty for vaginal agenesis: a case report , La Pediatria Medica e Chirurgica: Vol. 45 No. 2 (2023)
- Giovanni Murialdo, Attilia Piazzi, Giuseppe Badolati, Enrico Calcagno, Agostino Berio, Oculo-auriculo-vertebral spectrum with myopathy and velopharyngeal insufficiency. A case report with a non-branchiomeric muscle biopsy , La Pediatria Medica e Chirurgica: Vol. 38 No. 2 (2016)
- Maria Enrica Miscia, Maria Castellano, Stella Chiarini, Giuseppe Lauriti, Marco Casaccia, Pierluigi Lelli Chiesa, Gabriele Lisi, Pediatric primary spontaneous pneumothorax: a comparison of treatment at pediatric surgery vs. thoracic surgery departments , La Pediatria Medica e Chirurgica: Vol. 45 No. 1 (2023)
- C. Pietrasanta, B. Ghirardi, M.F. Manca, S. Uccella, C. Gualdi, E. Tota, L. Pugni, F. Mosca, Herpesviruses and breast milk , La Pediatria Medica e Chirurgica: Vol. 36 No. 3 (2014)
- Sara Uccella, Agnese De Carli, Ida Sirgiovanni, Paola Schiavolin, Giuseppe Damiano, Beatrice Ghirardi, Francesca Maglioli Carpano, Laura Bassi, Silvana Gangi, Odoardo Picciolini, Monica Fumagalli, Fabio Mosca, Survival rate and neurodevelopmental outcome of extremely premature babies: an 8-year experience of an Italian single neonatal tertiary care center , La Pediatria Medica e Chirurgica: Vol. 37 No. 3 (2015)
- Fabio Mosca, Maria Lorella Giannì, Human milk: composition and health benefits , La Pediatria Medica e Chirurgica: Vol. 39 No. 2 (2017)
You may also start an advanced similarity search for this article.