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Exstrophy and Epispadias Differential Diagnoses

  • Author: Elizabeth B Yerkes, MD; Chief Editor: Marc Cendron, MD  more...
 
Updated: Jul 26, 2016
 
 

Diagnostic Considerations

Other problems to be considered include the following:

  • Patent urachus
  • Persistent cloaca
 
 
Contributor Information and Disclosures
Author

Elizabeth B Yerkes, MD Associate Professor of Urology, Northwestern University Feinberg School of Medicine; Attending Urologist, Division of Urology, Children's Memorial Hospital of Chicago

Elizabeth B Yerkes, MD is a member of the following medical societies: North American Society for Pediatric and Adolescent Gynecology, Phi Beta Kappa, Society for Pediatric Urology, Society for Fetal Urology, Alpha Omega Alpha, American Academy of Pediatrics, American Urological Association

Disclosure: Nothing to disclose.

Coauthor(s)

Richard C Rink, MD 

Richard C Rink, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Urological Association, Indiana State Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Harry P Koo, MD Chairman of Urology Division, Director of Pediatric Urology, Professor of Surgery, Virginia Commonwealth University School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond

Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Urological Association

Disclosure: Nothing to disclose.

Chief Editor

Marc Cendron, MD Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston

Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, New Hampshire Medical Society, Society for Pediatric Urology, Society for Fetal Urology, Johns Hopkins Medical and Surgical Association, European Society for Paediatric Urology

Disclosure: Nothing to disclose.

Additional Contributors

Howard M Snyder, III, MD Professor, Department of Surgery, Division of Pediatric Urology, University of Pennsylvania School of Medicine and Children's Hospital of Philadelphia

Howard M Snyder, III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Urological Association, National Kidney Foundation

Disclosure: Nothing to disclose.

References
  1. Shetty MV, Bhaskaran A, Sen TK. Female epispadias. Afr J Paediatr Surg. 2011 May-Aug. 8(2):215-7. [Medline].

  2. Gambhir L, Höller T, Müller M, Schott G, Vogt H, Detlefsen B. Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol. 2008 Apr. 179(4):1539-43. [Medline].

  3. Reutter H, Qi L, Gearhart JP, Boemers T, Ebert AK, Rosch W. Concordance analyses of twins with bladder exstrophy-epispadias complex suggest genetic etiology. Am J Med Genet A. 2007 Nov 15. 143A(22):2751-6. [Medline].

  4. Wood HM, Babineau D, Gearhart JP. In vitro fertilization and the cloacal/bladder exstrophy-epispadias complex: A continuing association. J Pediatr Urol. 2007 Aug. 3(4):305-10. [Medline].

  5. Jayachandran D, Bythell M, Platt MW, Rankin J. Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol. 2011 Nov. 186(5):2056-60. [Medline].

  6. Ebert AK, Bals-Pratsch M, Seifert B, Reutter H, Rosch WH. Genital and reproductive function in males after functional reconstruction of the exstrophy-epispadias complex--long-term results. Urology. 2008 Sep. 72(3):566-9; discussion 569-70. [Medline].

  7. Kibar Y, Roth C, Frimberger D, Kropp BP. Long-Term Results of Penile Disassembly Technique for Correction of Epispadias. Urology. 2008 Dec 17. [Medline].

  8. Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol. 2006 Aug. 24(3):244-9. [Medline].

  9. Husmann DA, Rathbun SR. Long-term follow up of enteric bladder augmentations: the risk for malignancy. J Pediatr Urol. 2008 Oct. 4(5):381-5; discussion 386. [Medline].

  10. Bujons A, Lopategui DM, Rodríguez N, Centeno C, Caffaratti J, Villavicencio H. Quality of life in female patients with bladder exstrophy-epispadias complex: Long-term follow-up. J Pediatr Urol. 2016 May 28. [Epub ahead of print]. [Medline].

  11. Stein R, Hohenfellner K, Fisch M, et al. Social integration, sexual behavior and fertility in patients with bladder exstrophy--a long-term follow up. Eur J Pediatr. 1996 Aug. 155(8):678-83. [Medline].

  12. Dy GW, Willihnganz-Lawson KH, Shnorhavorian M, et al. Successful pregnancy in patients with exstrophy-epispadias complex: a University of Washington experience. J Pediatr Urol. 2015 Aug. 11(4):213.e1-6. [Medline].

  13. Lee C, Reutter HM, Grasser MF, et al. Gender-associated differences in the psychosocial and developmental outcome in patients affected with the bladder exstrophy-epispadias complex. BJU Int. 2006 Feb. 97(2):349-53. [Medline].

  14. Mukherjee B, McCauley E, Hanford RB, Aalsma M, Anderson AM. Psychopathology, psychosocial, gender and cognitive outcomes in patients with cloacal exstrophy. J Urol. 2007 Aug. 178(2):630-5; discussion 634-5. [Medline].

  15. Ebert A, Scheuering S, Schott G, Roesch WH. Psychosocial and psychosexual development in childhood and adolescence within the exstrophy-epispadias complex. J Urol. 2005 Sep. 174(3):1094-8. [Medline].

  16. Borer JG, Gargollo PC, Kinnamon DD, et al. Bladder growth and development after complete primary repair of bladder exstrophy in the newborn with comparison to staged approach. J Urol. 2005 Oct. 174(4 Pt 2):1553-7; discussion 1557-8. [Medline].

  17. Gargollo PC, Borer JG, Diamond DA, et al. Prospective followup in patients after complete primary repair of bladder exstrophy. J Urol. 2008 Oct. 180(4 Suppl):1665-70; discussion 1670. [Medline].

  18. Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol. 1999 Oct. 162(4):1415-20. [Medline].

  19. Mitchell ME. Bladder exstrophy repair: complete primary repair of exstrophy. Urology. 2005 Jan. 65(1):5-8. [Medline].

  20. Shnorhavorian M, Grady RW, Andersen A, Joyner BD, Mitchell ME. Long-term followup of complete primary repair of exstrophy: the Seattle experience. J Urol. 2008 Oct. 180(4 Suppl):1615-9; discussion 1619-20. [Medline].

  21. Bhatnagar V. Bladder exstrophy: An overview of the surgical management. J Indian Assoc Pediatr Surg. 2011 Jul. 16(3):81-7. [Medline]. [Full Text].

  22. Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: A contemporary series. J Pediatr Urol. 2007 Aug. 3(4):311-315. [Medline].

  23. Leclair MD, Villemagne T, Faraj S, Suply E. The radical soft-tissue mobilization (Kelly repair) for bladder exstrophy. J Pediatr Urol. 2015 Dec. 11(6):364-5. [Medline].

  24. Ben-Chaim J, Hidas G, Wikenheiser J, et al. Kelly procedure for exstrophy or epispadias patients: anatomical description of the pudendal neurovasculature. J Pediatr Urol. 2016 Jun. 12(3):173.e1-6. [Medline].

  25. Anwar AZ, Mohamed MA, Hussein A, Shaaban AM. Modified penile disassembly technique for boys with epispadias and those undergoing complete primary repair of exstrophy: long-term outcomes. Int J Urol. 2014 Sep. 21(9):936-40. [Medline].

  26. Levitt MA, Mak GZ, Falcone RA Jr, Pena A. Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients. J Pediatr Surg. 2008 Jan. 43(1):164-8; discussion 168-70. [Medline].

  27. Mathews R, Gearhart JP, Bhatnagar R, Sponseller P. Staged pelvic closure of extreme pubic diastasis in the exstrophy-epispadias complex. J Urol. 2006 Nov. 176(5):2196-8. [Medline].

  28. Inouye BM, Tourchi A, Di Carlo HN, Young EE, Mhlanga J, Ko JS, et al. Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy. J Pediatr Urol. 2014 Aug 12. [Medline].

  29. Houk CP, Lee PA. Consensus statement on terminology and management: disorders of sex development. Sex Dev. 2008. 2(4-5):172-80. [Medline].

  30. Husmann DA, Gearhart JP. Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol. 2004 Oct. 172(4 Pt 2):1696-700; discussion 1700-1. [Medline].

  31. Schaeffer AJ, Purves JT, King JA, Sponseller PD, Jeffs RD, Gearhart JP. Complications of primary closure of classic bladder exstrophy. J Urol. 2008 Oct. 180(4 Suppl):1671-4; discussion 1674. [Medline].

  32. Krishnamsetty RM, Rao MK, Hines CR, et al. Adenocarcinoma in exstrophy and defunctional ureterosigmoidostomy. J Ky Med Assoc. 1988 Aug. 86(8):409-14. [Medline].

  33. Reiner WG, Gearhart JP, Kropp B. Suicide and suicidal ideation in classic exstrophy. J Urol. 2008 Oct. 180(4 Suppl):1661-3; discussion 1663-4. [Medline].

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Open bladder plate and urethra with bifid clitoris in female patient with classic bladder exstrophy. Note low-set umbilicus and anteriorly displaced anus. Courtesy of Richard Rink, MD.
Male with classic bladder exstrophy. Note low-set umbilicus and short, broad, upturned phallus. Distance between phallus and scrotum is abnormal. Courtesy of Richard Rink, MD.
External view in female patient with epispadias. Labia are separated anteriorly. Courtesy of Richard Rink, MD.
Female patient with epispadias with labia retracted. The clitoris is bifid, and the urethra is open dorsally. Courtesy of Richard Rink, MD.
Penopubic epispadias in male patient. Typical spadelike configuration of glans penis with incomplete foreskin, dorsal urethral plate, and open bladder neck. Courtesy of Richard Rink, MD.
46,XY newborn with cloacal exstrophy. A very large omphalocele is present, and the hemibladders flank the exstrophic cecal plate. The phallus is small and bifid, with the hemiglans and hemiscrotum located just distal to the bladder. Courtesy of Martin Kaefer, MD.
Lipomeningocele in the same newborn shown in Image 6, a 46,XY newborn with cloacal exstrophy. Courtesy of Martin Kaefer, MD.
Superior vesical fissure variant of exstrophy. Genitalia are normal. Patent urachus is considered in the differential diagnosis, although the opening is well below the umbilicus. Rectus abdominis and pubic abnormalities are found in the exstrophic variant but not with patent urachus. Courtesy of Richard Rink, MD.
Female with covered exstrophy. The umbilicus is very low, the pubic bones are widely separated, and the rectus muscles are divergent. The bladder is intact, but the patient has undergone bladder neck reconstruction to achieve urinary continence. The external genitalia are normal. Courtesy of Elizabeth Yerkes, MD.
Typical appearance of pelvis in patient with exstrophy-epispadias complex with wide separation of pubic symphysis.
Bilateral vesicoureteral reflux and small-capacity bladder after initial exstrophy closure. Contrast escapes readily through the incompetent bladder neck.
Use of plastic wrap to protect the delicate bladder mucosa in a newborn with bladder exstrophy. Courtesy of Richard Rink, MD.
Subtotal prolapse of bladder wall through patulous bladder neck after bladder closure in female with cloacal exstrophy. The bladder mucosa appears healthy, and urine is draining freely. Courtesy of Richard Rink, MD.
 
 
 
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