Advertisement

Bladder and Cloacal Exstrophy

  • Jason J. Howard
  • James S. Huntley
  • Jonathan G. Schoenecker
  • Sattar Alshryda
  • Joao Pippi Salle
Chapter

Abstract

Bladder exstrophy is a rare condition, resulting from a failure of fusion of midline tissues at the lower abdominal region (including the anterior urogenital elements), wherein the inner surface of the urethra and posterior bladder wall are exposed anteriorly, associated with bilateral hemipelvic posterolateral rotation, a substantial pubic diastasis, and a deficient lower abdominal wall. The goals of treatment include a successful primary closure and the eventual achievement of urinary continence. Successful and sustained urogenital reconstruction is dependent on a tension-free closure of the anterior soft tissues, facilitated by pelvic osteotomies, to avoid wound dehiscence and need for revision surgery. This chapter will offer a comprehensive review of the associated musculoskeletal and urogenital pathoanatomy, clinical aspects and natural history, for both classic bladder exstrophy and cloacal exstrophy, as well as a description of pelvic osteotomy techniques. A brief overview of the principles of urogenital reconstruction in bladder exstrophy will also be covered.

Keywords

Bladder exstrophy Cloacal exstrophy Pelvic osteotomies Innominate osteotomy Urogenital reconstruction 

References

  1. 1.
    O'Phelan EH. Iliac osteotomy in exstrophy of the bladder. J Bone Joint Surg Am. 1963;45:1409–22.CrossRefGoogle Scholar
  2. 2.
    Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success. Urology. 2003;62:1109e13.CrossRefGoogle Scholar
  3. 3.
    Shultz WG. Plastic repair of exstrophy of bladder combined with bilateral osteotomy of ilia. J Urol. 1958;79:453–8.CrossRefGoogle Scholar
  4. 4.
    Suson KD, Sponseller PD, Gearhart JP. Bony abnormalities in classic bladder exstrophy: the urologist's perspective. J Pediatr Urol. 2013;9:112–22.CrossRefGoogle Scholar
  5. 5.
    O’Donnell B. The lessons of 40 bladder extrophies in 20 years. J Pediatr Surg. 1984;19:547–9.CrossRefGoogle Scholar
  6. 6.
    Kasat LS, Borwanker SS. Factors responsible for successful primary closure in bladder exstrophy. Pediatr Surg Int. 2000;16:194–8.CrossRefGoogle Scholar
  7. 7.
    Purves JT, Gearhart JP. Pelvic Osteotomy in the Modern Treatment of the Exstrophy-Epispadias Complex. EAU-EBU Update Series. 2007;5:188–96.CrossRefGoogle Scholar
  8. 8.
    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 Am. 1995;77:177–89.CrossRefGoogle Scholar
  9. 9.
    Stec AA, Tekes A, Ertan G, Phillips TM, Novak TE, Solaiyappan M, Huisman TA, Sponseller PD, Gearhart JP. Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol. 2012;188:1535–42.CrossRefGoogle Scholar
  10. 10.
    Greene WB, Dias LS, Lindseth RE, Torch MA. Musculoskeletal problems in association with cloacal exstrophy. J Bone Joint Surg Am. 1991;73:551–60.CrossRefGoogle Scholar
  11. 11.
    Ebert AK, Falkert A, Brandl R, Hirschfelder H, Koller M, Rösch WH. Pelvic-floor imaging using three-dimensional ultrasonography and magnetic resonance imaging in the long term follow-up of the bladder-exstrophy-epispadias complex. BJU Int. 2010;105:248–53.CrossRefGoogle Scholar
  12. 12.
    Lottmann HB, Melin Y, Cendron M, Lombrail P, Beze-Beyrie P, Cendron J. Bladder exstrophy: evaluation of factors leading to continence with spontaneous voiding after staged reconstruction. J Urol. 1997;158:1041–4.CrossRefGoogle Scholar
  13. 13.
    Stec AA, Pannu HK, Tadros YE, et al. Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 2001;166:1444.CrossRefGoogle Scholar
  14. 14.
    Kaar SG, Cooperman DR, Blakemore LC, Thompson GH, Petersilge CA, Elder JS, Heiple KG. Association of bladder exstrophy with congenital pathology of the hip and lumbosacral spine: a long-term follow-up study of 13 patients. J Pediatr Orthop. 2002;22:62–6.PubMedGoogle Scholar
  15. 15.
    Nordin S, Clementson C, Herrlin K, Hägglund G. Hip configuration and function in bladder exstrophy treated without pelvic osteotomy. J Pediatr Orthop. 1996;5:119–22.Google Scholar
  16. 16.
    Sutherland D, Pike L, Kaufman K, Mowery C, Kaplan G, Romanus B. Hip function and gait in patients treated for bladder exstrophy. J Pediatr Orthop. 1994;14:709–14.CrossRefGoogle Scholar
  17. 17.
    Lascombes P, Dautel G, Grosdidier G. Anatomical basis of pelvic growth in bladder exstrophy. Surg Radiol Anat. 1989;11:85–9.CrossRefGoogle Scholar
  18. 18.
    Yazici M, Kandemir U, Atilla B, Eryilmaz M. Rotational profile of lower extremities in bladder exstrophy patients with unapproximated pelvis: a clinical and radiologic study in children older than 7 years. J Pediatr Orthop. 1999;19(4):531–5.CrossRefGoogle Scholar
  19. 19.
    Mundy A, Kushare I, Jayanthi V, Samora W, Klingele KE. Incidence of Hip Dysplasia With Bladder Exstrophy. J Pediatr Orthop. 2016;36:860–4.Google Scholar
  20. 20.
    Jani MM, Sponseller PD, Gearhart JP, Barrance PJ, Genda E, Chao EY. The hip in adults with classic bladder exstrophy: a biomechanical analysis. J Pediatr Orthop. 2000;20:296–301.PubMedGoogle Scholar
  21. 21.
    Castagnetti M, Gigante C, Perrone G, Rigamonti W. Comparison of musculoskeletal and urological functional outcomes in patients with bladder exstrophy undergoing repair with and without osteotomy. Pediatr Surg Int. 2008;24:689–93.CrossRefGoogle Scholar
  22. 22.
    Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of bladder exstrophy in the United States. J Urol. 2005;173:1728–31.CrossRefGoogle Scholar
  23. 23.
    Cervellione RM, Mantovani A, Gearhart J, Bogaert G, Gobet R, Caione P, Dickson AP. Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe. J Pediatr Urol. 2015;11:337.e1–6.CrossRefGoogle Scholar
  24. 24.
    Dutta HK. Cloacal exstrophy: a single center experience. J Pediatr Urol. 2014;10:329–35.CrossRefGoogle Scholar
  25. 25.
    Suson KD, Novak TE, Gupta AD, Benson J, Sponseller P, Gearhart JP. Neuro-orthopedic manifestations of the omphalocele exstrophy imperforate anus spinal defects complex. J Urol. 2010;184:1651–5.CrossRefGoogle Scholar
  26. 26.
    Reimers J. The stability of the hip in children. A radiological study of the results of muscle surgery in cerebral palsy. Acta Orthop Scand. 1980;184:1–100.CrossRefGoogle Scholar
  27. 27.
    Yazici M, Sözübir S, Kilicoglu G, Bernay F, Incesu L, Ariturk E. Three-dimensional anatomy of the pelvis in bladder exstrophy: description of bone pathology by using three-dimensional computed tomography and its clinical relevance. J Pediatr Orthop. 1998;18:132–5.PubMedGoogle Scholar
  28. 28.
    Gargollo PC, Borer JG, Retik AB, Peters CA, Diamond DA, Atala A, Barnewolt CE. Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patientsbefore and after complete primary repair of bladder exstrophy. J Urol. 2005;174:1559–66.CrossRefGoogle Scholar
  29. 29.
    Inouye BM, Lue K, Abdelwahab M, Di Carlo HN, Young EE, Tourchi A, Grewal M, Hesh C, Sponseller PD, Gearhart JP. Newborn exstrophy closure without osteotomy: Is there a role? Pediatr Urol. 2016;12:51.e1–4.CrossRefGoogle Scholar
  30. 30.
    Arlen AM, Cooper CS, Morcuende J, Austin JC. Safety and efficacy of spica casts for immobilization following initial bladder closure in classic bladder exstrophy. J Pediatr Urol. 2011;7:456–9.CrossRefGoogle Scholar
  31. 31.
    Shnorhavorian M, Song K, Zamilpa I, Wiater B, Mitchell MM, Grady RW. Spica casting compared to Bryant's traction after complete primary repair of exstrophy: safe and effective in a longitudinal cohort study. J Urol. 2010;184:669–73.CrossRefGoogle Scholar
  32. 32.
    Shoukry AI, Ziada AM, Morsi HA, Habib EI, Aref A, Badawy HA, et al. Outcome of complete primary bladder exstrophy repair: single center experience. J Pediatr Urol. 2009;5:496e9.CrossRefGoogle Scholar
  33. 33.
    Braga LH, Lorenzo AJ, Jrearz R, Bagli DJ, Pippi Salle JL. Bilateral ureteral reimplantation at primary bladder exstrophy closure. J Urol. 2010;183:2337–41.CrossRefGoogle Scholar
  34. 34.
    McLorie GA. Osteotomy: who can get by without one? Dialogues Pediatr Urol. 2007;29:8.Google Scholar
  35. 35.
    Elsayed ER, Alam MN, Sarhan OM, Elsayed D, Eliwa AM, Khalil S. Closure of bladder exstrophy with a bilateral anterior pubic osteotomy: Revival of an old technique. Arab J Urol. 2011;9:203–6.CrossRefGoogle Scholar
  36. 36.
    Frey P, Cohen SJ. Anterior pelvic osteotomy: a new operative technique facilitating primary bladder exstrophy closure. Br J Urol. 1989;64:641–3.CrossRefGoogle Scholar
  37. 37.
    Gibbon AJ, Maffulli N, Fixsen JA. Horizontal pelvic osteotomies for bladder exstrophies: a preliminary report. J Bone Joint Surg Br. 1991;73-B:896–8.CrossRefGoogle Scholar
  38. 38.
    Sponseller PD, Gearhart JP, Jeffs RD. Anterior innominate osteotomies for failure or late closure of bladder exstrophy. J Urol. 1991;146:137–40.CrossRefGoogle Scholar
  39. 39.
    Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Am. 2001;83:184–93.CrossRefGoogle Scholar
  40. 40.
    Segev E, Ezra E, Binyamini Y, Wientroub S, Ben-Chaim J. A combined vertical and horizontal pelvic osteotomy approach for repair of bladder exstrophy: the Dana experience. Isr Med Assoc J. 2004;6:749–52.PubMedGoogle Scholar
  41. 41.
    Satsuma S, Kobayashi D, Yoshiya S, Kurosaka M. Comparison of posterior and anterior pelvic osteotomy for bladderexstrophy complex. J Pediatr Orthop B. 2006;15:141–6.CrossRefGoogle Scholar
  42. 42.
    Jones D, Parkinson S, Hosalkar HS. Oblique pelvic osteotomy in the exstrophy/epispadias complex. J Bone Joint Surg Br. 2006;88:799–806.CrossRefGoogle Scholar
  43. 43.
    McKenna PH, Khoury AE, McLorie GA, et al. Iliac osteotomy: a model to compare the options in bladder and cloacal exstrophy reconstruction. J Urol. 1994;151:182–6.CrossRefGoogle Scholar
  44. 44.
    Cervellione RM. The use of pelvic osteotomy in cloacal exstrophy. Semin Pediatr Surg. 2011;20:119–22.CrossRefGoogle Scholar
  45. 45.
    Inouye BM, Tourchi A, Di Carlo HN, Young EE, Mhlanga J, Ko JS, Sponseller PD, Gearhart JP. Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy. J Pediatr Urol. 2014;10:1244–8.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Jason J. Howard
    • 1
  • James S. Huntley
    • 2
  • Jonathan G. Schoenecker
    • 3
  • Sattar Alshryda
    • 4
  • Joao Pippi Salle
    • 5
  1. 1.Weill Cornell MedicineChief of Orthopaedic Surgery, Sidra MedicineDohaQatar
  2. 2.Senior Attending Physician in Pediatric, Orthopedic SurgerySidra MedicineDohaQatar
  3. 3.Vanderbilt University Medical Center, Jeffrey Mast Chair of Orthopaedic Hip and Trauma SurgeryNashvilleUSA
  4. 4.Clinical Director of Paediatric Trauma and Orthopaedic SurgeryRoyal Manchester Children Hospital, Manchester University NHS Foundation TrustManchesterUK
  5. 5.Division of UrologySidra MedicineDohaQatar

Personalised recommendations