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Bladder Exstrophy

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Pediatric Surgery

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Abstract

Bladder exstrophy falls on a spectrum spanning a gamut from the relatively benign isolated epispadias to the severe cloacal exstrophy. In regards to bladder exstrophy, the disorder is characterized by a lower midline defect, with the bladder lumen being exposed. In addition to the bladder, there are defects to the genitalia, pelvic bony anatomy, rectum, anus, and abdominal wall. The condition is thought to be an abnormal development of the midline, specifically the cloacal membrane. Following birth, these patients require surgical intervention for functional closure of the bladder. After the closure of the bladder, there are several surgical milestones to recapitulate the normal lower genitourinary tract anatomy. That includes repair of epispadias in males and a continence surgery for both males and females.

Throughout the last several decades, treatment success has improved in regard to functional closure. The management of these patients has turned to improving quality of life, especially in terms of patients achieving urinary continence. Advances in modern medicine have rendered this major congenital anomaly compatible with a long life into adulthood. Because most experience is isolated to large centers with experienced exstrophy surgeons, data may not be generalizable. With more collaborative efforts between institutions and centralization in care at high volume centers, questions still unclear in the management of these patients may hopefully be elucidated in the near future.

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References

  • Arlen AM, Smith EA (2014) Disorders of the bladder and cloacal anomaly. Clin Perinatol 41(3):695–707

    PubMed  Google Scholar 

  • Benz KS, Dunn E, Maruf M, Facciola J, Jayman J, Kasprenski M et al (2018) Novel anatomical observations of the prostate, prostatic vasculature and penile vasculature in classic bladder exstrophy using magnetic resonance imaging. J Urol 200(6):1354–1361

    PubMed  Google Scholar 

  • Buyukunal CSN, Gearhart JP (2011) A short history of bladder exstrophy. Semin Pediatr Surg 20(2):62–65

    PubMed  Google Scholar 

  • Cervellione RM, Husmann DA, Bivalacqua TJ, Sponseller PD, Gearhart JP (2010) Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol 6(5):450–456

    PubMed  Google Scholar 

  • Cervellione RM, Mantovani A, Gearhart J, Bogaert G, Gobet R, Caione P et al (2015) Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe. J Pediatr Urol 11(6):337

    Google Scholar 

  • Connolly JA, Peppas DS, Jeffs RD, Gearhart JP (1995) Prevalence and repair of inguinal hernias in children with bladder exstrophy. J Urol 154(5):1900–1901

    CAS  PubMed  Google Scholar 

  • Davis R, Maruf M, Dunn E, DiCarlo H, Gearhart JP (2019) The role of anatomic pelvic dissection in the successful closure of bladder exstrophy: an aid to success. J Pediatr Urol 15(5):559.e1–559.e7

    CAS  PubMed  Google Scholar 

  • Dunn EA, Kasprenski M, Facciola J, Benz K, Maruf M, Zaman MH et al (2019) Anatomy of classic bladder exstrophy: MRI findings and surgical correlation. Curr Urol Rep 20(9):48

    PubMed  Google Scholar 

  • Ebert A-K, Zwink N, Reutter HM, Jenetzky E, Stein R, Hölscher AC et al (2020) Treatment strategies and outcome of the exstrophy-epispadias complex in Germany: data from the German CURE-Net. Front Pediatr 8:174

    PubMed  PubMed Central  Google Scholar 

  • Gearhart J, Benchaim J, Jeffs R, Sanders R (1995) Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 85(6):961–964

    CAS  PubMed  Google Scholar 

  • Goldman S, Szejnfeld PO, Rondon A, Francisco VV, Bacelar H, Leslie B et al (2013) Prenatal diagnosis of bladder exstrophy by fetal MRI. J Pediatr Urol 9(1):3–6

    PubMed  Google Scholar 

  • Goyal A, Fishwick J, Hurrell R, Cervellione RM, Dickson AP (2012) Antenatal diagnosis of bladder/cloacal exstrophy: challenges and possible solutions. J Pediatr Urol 8(2):140–144

    PubMed  Google Scholar 

  • Grady RW, Mitchell ME (1999) Complete primary repair of exstrophy. J Urol 162(4):1415–1420

    CAS  PubMed  Google Scholar 

  • Hamper UM, Gearhart JP, Dahnert WF, Sheth S, Jeffs RD (1991) Bladder exstrophy-epispadias complex: prostatic evaluation by transrectal ultrasonography. Prostate 19(2):133–140

    CAS  PubMed  Google Scholar 

  • Johal NS, Arthurs C, Cuckow P, Cao K, Wood DN, Ahmed A et al (2019) Functional, histological and molecular characteristics of human exstrophy detrusor. J Pediatr Urol 15(2):154.e1–154.e9

    CAS  PubMed  Google Scholar 

  • Kasprenski M, Benz K, Maruf M, Jayman J, Di Carlo H, Gearhart J (2020a) Modern Management of the Failed Bladder Exstrophy Closure: a 50-yr experience. Eur Urol Focus 6(2):383–389

    PubMed  Google Scholar 

  • Kasprenski M, Maruf M, Davis R, Jayman J, Benz K, Michaud J et al (2020b) Penile disassembly in complete primary repair of bladder exstrophy: time for re-evaluation? Urology 137:146–151

    PubMed  Google Scholar 

  • Kelly JH (1995) Vesical exstrophy: repair using radical mobilisation of soft tissues. Pediatr Surg Int 10(5–6):298–304. https://doi.org/10.1007/BF00182207

    Article  Google Scholar 

  • Ludwig M, Ching B, Reutter H, Boyadjiev SA (2009) Bladder exstrophy-epispadias complex. Birt Defects Res A Clin Mol Teratol 85(6):509–522

    CAS  Google Scholar 

  • Mahfuz I, Darling T, Wilkins S, White S, Cheng W (2013) New insights into the pathogenesis of bladder exstrophy-epispadias complex. J Pediatr Urol 9(6 Pt B):996–1005

    PubMed  Google Scholar 

  • Maruf M, Manyevitch R, Michaud J, Jayman J, Kasprenski M, Zaman MH et al (2020) Urinary continence outcomes in classic bladder exstrophy: a long-term perspective. J Urol 203(1):200–205

    PubMed  Google Scholar 

  • Mathews R, Gosling JA, Gearhart JP (2004) Ultrastructure of the bladder in classic exstrophy: correlation with development of continence. J Urol 172(4 Part 1):1446–1449

    PubMed  Google Scholar 

  • Meldrum KK, Mathews R, Gearhart JP (2001) Hugh Hampton young: a pioneer in pediatric urology. J Urol 166(4):1415–1417

    CAS  PubMed  Google Scholar 

  • Meldrum KK, Mathews RI, Nelson CP, Gearhart JP (2005) Subspecialty training and surgical outcomes in children with failed bladder exstrophy closure. J Pediatr Urol 1(2):95–99

    PubMed  Google Scholar 

  • Muecke EC (1964) The role of the cloacal membrane in exstrophy: the first successful experimental study. J Urol 92:659–667

    CAS  PubMed  Google Scholar 

  • Nelson CP, Dunn RL, Wei JT (2005) Contemporary epidemiology of bladder exstrophy in the United States. J Urol 173(5):1728–1731

    PubMed  Google Scholar 

  • Pathak P, Ring JD, Delfino KR, Dynda DI, Mathews RI (2020) Complete primary repair of bladder exstrophy: a systematic review. J Pediatr Urol 16(2):149–153

    PubMed  Google Scholar 

  • Poli-Merol ML, Watson JA, Gearhart JP (2002) New basic science concepts in the treatment of classic bladder exstrophy. Urology 60(5):749–755

    CAS  PubMed  Google Scholar 

  • Reutter H, Boyadjiev SA, Gambhir L, Ebert A-K, Rösch WH, Stein R et al (2011) Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe. J Pediatr 159(5):825–831.e1

    PubMed  PubMed Central  Google Scholar 

  • Sack BS, Kryger JV, Mitchell ME, Durkee CT, Lyon R, Groth TW (2015) Clinical pathway for early discharge after complete primary repair of exstrophy and epispadias by using a spica cast. J Pediatr Urol 11(4):212.e1–212.e4

    PubMed  Google Scholar 

  • Sathish Kumar KV, Mammen A, Varma KK (2015) Pathogenesis of bladder exstrophy: a new hypothesis. J Pediatr Urol 11(6):314–318

    Google Scholar 

  • Siffel C, Correa A, Amar E, Bakker MK, Bermejo-Sánchez E, Bianca S et al (2011) Bladder exstrophy: an epidemiologic study from the international clearinghouse for birth defects surveillance and research, and an overview of the literature. Am J Med Genet C Semin Med Genet 157C(4):321–332

    PubMed  Google Scholar 

  • Silver RI, Yang A, Ben-Chaim J, Jeffs RD, Gearhart JP (1997) Penile length in adulthood after exstrophy reconstruction. J Urol 157(3):999–1003

    CAS  PubMed  Google Scholar 

  • Sirisreetreerux P, Lue KM, Ingviya T, Friedlander DA, Di Carlo HN, Sponseller PD et al (2017) Failed primary bladder exstrophy closure with osteotomy: multivariable analysis of a 25-year experience. J Urol 197(4):1138–1143

    PubMed  Google Scholar 

  • Stec AA, Pannu HK, Tadros YE, Sponseller PD, Fishman EK, Gearhart JP (2001a) Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol 166(4):1444–1449

    CAS  PubMed  Google Scholar 

  • Stec AA, Pannu HK, Tadros YE, Sponseller PD, Wakim A, Fishman EK et al (2001b) Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights. Urology 58(6):1030–1035

    CAS  PubMed  Google Scholar 

  • Stec AA, Baradaran N, Gearhart JP (2012a) Congenital renal anomalies in patients with classic bladder exstrophy. Urology 79(1):207–209

    PubMed  Google Scholar 

  • Stec AA, Baradaran N, Schaeffer A, Gearhart JP, Matthews RI (2012b) The modern staged repair of classic bladder exstrophy: a detailed postoperative management strategy for primary bladder closure. J Pediatr Urol 8(5):549–555

    PubMed  Google Scholar 

  • Stringer MD, Duffy PG, Ransley PG (1994) Inguinal hernias associated with bladder exstrophy. Br J Urol 73(3):308–309

    CAS  PubMed  Google Scholar 

  • Surer I, Baker LA, Jeffs RD, Gearhart JP (2001) Modified young-Dees-Leadbetter bladder neck reconstruction in patients with successful primary bladder closure elsewhere: a single institution experience. J Urol 165(6 Pt 2):2438–2440

    CAS  PubMed  Google Scholar 

  • Suson KD, Stec AA, Shimoda LA, Gearhart JP (2012) Initial characterization of exstrophy bladder smooth muscle cells in culture. J Urol 188(4S):1521–1527

    PubMed  Google Scholar 

  • Suson KD, Inouye B, Carl A, Gearhart JP (2016) Congenital renal anomalies in cloacal exstrophy: is there a difference? J Pediatr Urol 12(4):207.e1–207.e5

    CAS  PubMed  Google Scholar 

  • Tekes A, Ertan G, Solaiyappan M, Stec AA, Sponseller PD, Huisman TA et al (2014) 2D and 3D MRI features of classic bladder exstrophy. Clin Radiol 69(5):e223–e229

    CAS  PubMed  Google Scholar 

  • Williams AM, Solaiyappan M, Pannu HK, Bluemke D, Shechter G, Gearhart JP (2004) 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol 172(4 Pt 2):1702–1705

    PubMed  Google Scholar 

  • Wood HM, Trock BJ, Gearhart JP (2003) In vitro fertilization and the cloacal-bladder exstrophy-epispadias complex: is there an association? J Urol 169(4):1512–1515

    PubMed  Google Scholar 

  • Wood HM, Babineau D, Gearhart JP (2007) In vitro fertilization and the cloacal/bladder exstrophy-epispadias complex: a continuing association. J Pediatr Urol 3(4):305–310

    PubMed  Google Scholar 

  • Zaman M, Kasprenski M, Maruf M, Benz K, Jayman J, Friedlander D et al (2019) Impact of pelvic immobilization techniques on the outcomes of primary and secondary closures of classic bladder exstrophy. J Pediatr Urol 15(4):382.e1–382.e8

    CAS  PubMed  Google Scholar 

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Acknowledgements

We would like to thank Dr. Amanda C. North for her contribution to the first edition of this chapter.

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Authors and Affiliations

  1. Division of Pediatric Urology, Cohen Children’s Medical Center of New York, Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, NY, USA

    Wayland J. Wu

  2. James Buchannan Brady Urological Institute, Roberts Jeffs Division of Pediatric Urology, Charlotte Bloomberg Children’s Hospital, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Wayland J. Wu & John P. Gearhart

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  1. Wayland J. Wu
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  2. John P. Gearhart
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Correspondence to John P. Gearhart .

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Editors and Affiliations

  1. Beacon Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

  2. University Clinic of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria

    Michael E. Höllwarth

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Wu, W.J., Gearhart, J.P. (2023). Bladder Exstrophy. In: Puri, P., Höllwarth, M.E. (eds) Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-81488-5_96

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  • DOI: https://doi.org/10.1007/978-3-030-81488-5_96

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-81487-8

  • Online ISBN: 978-3-030-81488-5

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