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Current Urology Reports

, 20:48 | Cite as

Anatomy of Classic Bladder Exstrophy: MRI Findings and Surgical Correlation

  • Emily A. DunnEmail author
  • Matthew Kasprenski
  • James Facciola
  • Karl Benz
  • Mahir Maruf
  • Mohammad H. Zaman
  • John Gearhart
  • Heather Di Carlo
  • Aylin Tekes
New Imaging Techniques (S Rais-Bahrami and K Porter, Section Editors)
  • 53 Downloads
Part of the following topical collections:
  1. Topical Collection on New Imaging Techniques

Abstract

Purpose of Review

The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation.

Recent Findings

Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors’ experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy.

Summary

Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve.

Keywords

Classic bladder exstrophy Exstrophy-epispadias complex Magnetic resonance imaging Anatomy Surgical repair Imaging guidance 

Notes

Compliance with Ethical Standards

Conflict of Interest

Emily A. Dunn, Matthew Kasprenski, James Facciola, Karl Benz, Mahir Maruf, Mohammad H. Zaman, John Gearhart, Heather Di Carlo, and Aylin Tekes each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Jayachandran D, Bythell MW, Platt MW, Rankin J. Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol. 2011;186(5):2056–60.CrossRefGoogle Scholar
  2. 2.
    Vermeij-Keers C, Hartwig NG, van der Werff JF. Embryonic development of the ventral body wall and its congenital malformations. Semin Pediatr Surg. 1996;5(2):82–9.PubMedGoogle Scholar
  3. 3.
    Manner J, Kluth D. The morphogenesis of the exstrophy-epispadias complex: a new concept based on observations made in early embryonic cases of cloacal exstrophy. Anat Embryol (Berl). 2005;210(1):51–7.  https://doi.org/10.1007/s00429-005-0008-6.CrossRefGoogle Scholar
  4. 4.
    Muecke EC. The role of the cloacal membrane in exstrophy: the first successful experimental study. J Urol. 1964;92:659–67.CrossRefGoogle Scholar
  5. 5.
    Blaschko SD, Cunha GR, Baskin LS. Molecular mechanisms of external genitalia development. Differentiation. 2012;84(3):261–8.  https://doi.org/10.1016/j.diff.2012.06.003.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Sinclair AH, Berta P, Palmer MS, Hawkins JR, Griffiths BL, Smith MJ, et al. A gene from the human sex-determining region encodes a protein with homology to a conserved DNA-binding motif. Nature. 1990;346(6281):240–4.  https://doi.org/10.1038/346240a0.CrossRefPubMedGoogle Scholar
  7. 7.
    Yamada G, Satoh Y, Baskin LS, Cunha GR. Cellular and molecular mechanisms of development of the external genitalia. Differentiation. 2003;71(8):445–60.  https://doi.org/10.1046/j.1432-0436.2003.7108001.x.CrossRefPubMedGoogle Scholar
  8. 8.
    Hamilton W, Boyd J, Mossman H. Human embryology. 4th ed. Cambridge, England: Heffer; 1972.CrossRefGoogle Scholar
  9. 9.
    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(2):177–89.CrossRefGoogle Scholar
  10. 10.
    Stec AA, Pannu HK, Tadros YE, Sponseller PD, Wakim A, Fishman EK, et al. Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights. Urology. 2001;58(6):1030–5.CrossRefGoogle Scholar
  11. 11.
    Stec AA, Pannu HK, Tadros YE, Sponseller PD, Fishman EK, Gearhart JP. Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 2001;166(4):1444–9.CrossRefGoogle Scholar
  12. 12.
    Stec AA, Baradaran N, Tran C, Gearhart JP. Colorectal anomalies in patients with classic bladder exstrophy. J Pediatr Surg. 2011;46(9):1790–3.  https://doi.org/10.1016/j.jpedsurg.2011.03.019.CrossRefPubMedGoogle Scholar
  13. 13.
    Williams AM, Solaiyappan M, Pannu HK, Bluemke D, Shechter G, Gearhart JP. 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol. 2004;172(4 Pt 2):1702–5.CrossRefGoogle Scholar
  14. 14.
    Tekes A, Ertan G, Solaiyappan M, Stec AA, Sponseller PD, Huisman TA, et al. 2D and 3D MRI features of classic bladder exstrophy. Clin Radiol. 2014;69(5):e223–9.  https://doi.org/10.1016/j.crad.2013.12.019.CrossRefPubMedGoogle Scholar
  15. 15.
    Stec AA, Tekes A, Ertan G, Phillips TM, Novak TE, Solaiyappan M, et al. Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol. 2012;188(4 Suppl):1535–42.  https://doi.org/10.1016/j.juro.2012.02.039.CrossRefPubMedGoogle Scholar
  16. 16.
    Gargollo PC, Borer JG, Retik AB, Peters CA, Diamond DA, Atala A, et al. Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patients before and after complete primary repair of bladder exstrophy. J Urol. 2005;174(4 Pt 2):1559–66; discussion 66.CrossRefGoogle Scholar
  17. 17.
    Halachmi S, Farhat W, Konen O, Khan A, Hodapp J, Bagli DJ, et al. Pelvic floor magnetic resonance imaging after neonatal single stage reconstruction in male patients with classic bladder exstrophy. J Urol. 2003;170(4 Pt 2):1505–9.  https://doi.org/10.1097/01.ju.0000087463.92231.b1. CrossRefPubMedGoogle Scholar
  18. 18.
    Ebert AK, Reutter H, Ludwig M, Rosch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis. 2009;4:23.  https://doi.org/10.1186/1750-1172-4-23.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Inouye BM, Tourchi A, Di Carlo HN, Young EE, Gearhart JP. Modern management of the exstrophy-epispadias complex. Surg Res Pract. 2014;2014:587064.  https://doi.org/10.1155/2014/587064.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Stec AA, Baradaran N, Gearhart JP. Congenital renal anomalies in patients with classic bladder exstrophy. Urology. 2012;79(1):207–9.  https://doi.org/10.1016/j.urology.2011.09.022.CrossRefPubMedGoogle Scholar
  21. 21.
    Goldman S, Szejnfeld PO, Rondon A, Francisco VV, Bacelar H, Leslie B, et al. Prenatal diagnosis of bladder exstrophy by fetal MRI. J Pediatr Urol. 2013;9(1):3–6.  https://doi.org/10.1016/j.jpurol.2012.06.018.CrossRefPubMedGoogle Scholar
  22. 22.
    Silver RI, Yang A, Ben-Chaim J, Jeffs RD, Gearhart JP. Penile length in adulthood after exstrophy reconstruction. J Urol. 1997;157(3):999–1003.CrossRefGoogle Scholar
  23. 23.
    Schlegel PN, Gearhart JP. Neuroanatomy of the pelvis in an infant with cloacal exstrophy: a detailed microdissection with histology. J Urol. 1989;141(3):583–5.CrossRefGoogle Scholar
  24. 24.
    Woodhouse CR, Kellett MJ. Anatomy of the penis and its deformities in exstrophy and epispadias. J Urol. 1984;132(6):1122–4.CrossRefGoogle Scholar
  25. 25.
    Gearhart JP, Yang A, Leonard MP, Jeffs RD, Zerhouni EA. Prostate size and configuration in adults with bladder exstrophy. J Urol. 1993;149(2):308–10.CrossRefGoogle Scholar
  26. 26.
    • Benz KS, Dunn E, Maruf M, Facciola J, Jayman J, Kasprenski M, et al. Novel anatomical observations of the prostate, prostatic vasculature and penile vasculature in classic bladder exstrophy using magnetic resonance imaging. J Urol. 2018;200(6):1354–61.  https://doi.org/10.1016/j.juro.2018.06.020 In this article, the authors discuss MRI depiction of prostate anatomy and course of neurovascular bundles in CBE males prior to repair. Identification of anatomical landmarks prior to surgery may help to avoid neurovascular damage during genital reconstruction. CrossRefPubMedGoogle Scholar
  27. 27.
    Woodhouse CR, Hinsch R. The anatomy and reconstruction of the adult female genitalia in classical exstrophy. Br J Urol. 1997;79(4):618–22.CrossRefGoogle Scholar
  28. 28.
    • Benz KS, Dunn E, Solaiyappan M, Maruf M, Kasprenski M, Jayman J, et al. Novel observations of female genital anatomy in classic bladder exstrophy using 3-dimensional magnetic resonance imaging reconstruction. J Urol. 2018;200(4):882–9.  https://doi.org/10.1016/j.juro.2018.04.071 In this article, the authors discuss MRI depiction of CBE female genitalia, which were previously less well-described compared with their male counterparts. Identification of the clitoral bodies may help to avoid their injury during surgery. CrossRefPubMedGoogle Scholar
  29. 29.
    •• Di Carlo HN, Maruf M, Massanyi EZ, Shah B, Tekes A, Gearhart JP. 3D MRI-guided pelvic floor dissection in bladder exstrophy: a single arm trial. J Urol. 2019.  https://doi.org/10.1097/JU.0000000000000210 In this article, the authors describe preoperative MRI used in conjunction with surgical navigation software, which aids the dissection of small and indistinct soft tissue structures during surgical repair of children with CE and CBE. CrossRefGoogle Scholar
  30. 30.
    Baka-Jakubiak M. Combined bladder neck, urethral and penile reconstruction in boys with the exstrophy-epispadias complex. BJU Int. 2000;86(4):513–8.CrossRefGoogle Scholar
  31. 31.
    Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol. 1999;162(4):1415–20.CrossRefGoogle Scholar
  32. 32.
    Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success. Urology. 2003;62(6):1109–13.CrossRefGoogle Scholar
  33. 33.
    Gearhart JP, Peppas DS, Jeffs RD. The failed exstrophy closure: strategy for management. Br J Urol. 1993;71(2):217–20.CrossRefGoogle Scholar
  34. 34.
    Gearhart JP, Forschner DC, Jeffs RD, Ben-Chaim J, Sponseller PD. A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy. J Urol. 1996;155(2):689–93.CrossRefGoogle Scholar
  35. 35.
    Cervellione RM, Husmann DA, Bivalacqua TJ, Sponseller PD, Gearhart JP. Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol. 2010;6(5):450–6.  https://doi.org/10.1016/j.jpurol.2010.04.007.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Emily A. Dunn
    • 1
    Email author
  • Matthew Kasprenski
    • 2
  • James Facciola
    • 3
  • Karl Benz
    • 2
  • Mahir Maruf
    • 2
  • Mohammad H. Zaman
    • 2
  • John Gearhart
    • 2
  • Heather Di Carlo
    • 2
  • Aylin Tekes
    • 1
  1. 1.Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological ScienceJohns Hopkins University School of MedicineBaltimoreUSA
  2. 2.Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Charlotte Bloomberg Children’s HospitalJohns Hopkins HospitalBaltimoreUSA
  3. 3.Russell H. Morgan Department of Radiology and Radiological ScienceJohns Hopkins University School of MedicineBaltimoreUSA

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