UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Surgical management and postoperative outcome of children with bladder exstrophy

Author
Joseph G Borer, MD, FAAP, FACS
Section Editor
Laurence S Baskin, MD, FAAP
Deputy Editor
Melanie S Kim, MD

INTRODUCTION

Bladder exstrophy is a complex congenital anomaly that is treated surgically (picture 1 and figure 1). Surgery is challenging, as bladder exstrophy involves the urinary, reproductive, and musculoskeletal systems, and, in some patents, the intestinal tract. Major goals of reconstruction are preservation of normal kidney function, development of adequate bladder storage and function (ie, urinary continence), and provision of acceptable genital cosmetic appearance and function.

The surgical management and postoperative outcomes of children with bladder exstrophy are discussed here. The clinical manifestations and initial management of infants with bladder exstrophy are discussed separately. (See "Clinical manifestations and initial management of infants with bladder exstrophy".)

SURGICAL APPROACHES

The following approaches, or variations of such, are used to manage bladder exstrophy. The first two interventions result in correction of the underlying defect.

Modern staged repair of bladder exstrophy (MSRBE) involves three staged operations and was initially developed in the 1970s [1,2]. Subsequent modifications have been made over the past several years.

Complete primary repair of bladder exstrophy (CPRBE) was introduced in 1989 with the hope that a single operation would achieve the goals of continence and preservation of renal function [3]. However, follow-up studies demonstrate that many children still require multiple procedures following CPRBE [4]. (See 'Choice of surgical approach' below.)

                          

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Wed Feb 24 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Gearhart JP, Jeffs RD. State-of-the-art reconstructive surgery for bladder exstrophy at the Johns Hopkins Hospital. Am J Dis Child 1989; 143:1475.
  2. Jeffs RD, Charrios R, Mnay M, et al. Primary closure of the exstrophied bladder. In: Current Controversies in Urologic Management, WB Saunders, Philadelphia 1972. p.235.
  3. Grady RW, Mitchell ME. Newborn exstrophy closure and epispadias repair. World J Urol 1998; 16:200.
  4. Gargollo PC, Borer JG, Diamond DA, et al. Prospective followup in patients after complete primary repair of bladder exstrophy. J Urol 2008; 180:1665.
  5. Ansell JS. Surgical treatment of exstrophy of the bladder with emphasis on neonatal primary closure: personal experience with 28 consecutive cases treated at the University of Washington hospitals from 1962 to 1977: techniques and results. J Urol 1979; 121:650.
  6. Dodson JL, Surer I, Baker LA, et al. The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome. J Urol 2001; 165:1656.
  7. Baradaran N, Stec AA, Schaeffer AJ, et al. Delayed primary closure of bladder exstrophy: immediate postoperative management leading to successful outcomes. Urology 2012; 79:415.
  8. Mitchell ME, Bägli DJ. Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol 1996; 155:300.
  9. Grady RW, Carr MC, Mitchell ME. Complete primary closure of bladder exstrophy. Epispadias and bladder exstrophy repair. Urol Clin North Am 1999; 26:95.
  10. Grady RW, Mitchell ME. Complete primary repair of exstrophy. Surgical technique. Urol Clin North Am 2000; 27:569.
  11. Shnorhavorian M, Grady RW, Andersen A, et al. Long-term followup of complete primary repair of exstrophy: the Seattle experience. J Urol 2008; 180:1615.
  12. Braga LH, Lorenzo AJ, Jrearz R, et al. Bilateral ureteral reimplantation at primary bladder exstrophy closure. J Urol 2010; 183:2337.
  13. Borer JG, Vasquez E, Canning DA, et al. An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care. J Urol 2015; 193:1802.
  14. Schaeffer AJ, Johnson EK, Logvinenko T, et al. Practice patterns and resource utilization for infants with bladder exstrophy: a national perspective. J Urol 2014; 191:1381.
  15. Baradaran N, Cervellione RM, Stec AA, Gearhart JP. Delayed primary repair of bladder exstrophy: ultimate effect on growth. J Urol 2012; 188:2336.
  16. Borer JG, Strakosha R, Bauer SB, et al. Combined cystometrography and electromyography of the external urethral sphincter following complete primary repair of bladder exstrophy. J Urol 2014; 191:1547.
  17. Gargollo P, Hendren WH, Diamond DA, et al. Bladder neck reconstruction is often necessary after complete primary repair of exstrophy. J Urol 2011; 185:2563.
  18. Gearhart JP, Baird A, Nelson CP. Results of bladder neck reconstruction after newborn complete primary repair of exstrophy. J Urol 2007; 178:1619.
  19. Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 2007; 3:311.
  20. Shnorhavorian M, Song K, Zamilpa I, et al. 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.
  21. Wallis MC, Oottamasathien S, Wicher C, et al. Padded self-adhesive strap immobilization following newborn bladder exstrophy closure: the Utah straps. J Urol 2013; 190:2216.
  22. Mushtaq I, Garriboli M, Smeulders N, et al. Primary bladder exstrophy closure in neonates: challenging the traditions. J Urol 2014; 191:193.
  23. Borer JG. Are osteotomies necessary for bladder exstrophy closure? J Urol 2014; 191:13.
  24. Diseth TH, Bjordal R, Schultz A, et al. Somatic function, mental health and psychosocial functioning in 22 adolescents with bladder exstrophy and epispadias. J Urol 1998; 159:1684.
  25. Baker LA, Gearhart JP. The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol 1998; 16:205.
  26. Hammouda HM, Kotb H. Complete primary repair of bladder exstrophy: initial experience with 33 cases. J Urol 2004; 172:1441.
  27. Mesrobian HG, Kelalis PP, Kramer SA. Long-term followup of 103 patients with bladder exstrophy. J Urol 1988; 139:719.
  28. Shaw MB, Rink RC, Kaefer M, et al. Continence and classic bladder exstrophy treated with staged repair. J Urol 2004; 172:1450.
  29. Mouriquand PD, Bubanj T, Feyaerts A, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003; 92:997.
  30. Lepor H, Shapiro E, Jeffs RD. Urethral reconstruction in boys with classical bladder exstrophy. J Urol 1984; 131:512.
  31. Borer JG, Gargollo PC, Hendren WH, et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 2005; 174:1674.
  32. Baird AD, Mathews RI, Gearhart JP. The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences. J Urol 2005; 174:1421.
  33. Surer I, Baker LA, Jeffs RD, Gearhart JP. Modified Young-Dees-Leadbetter bladder neck reconstruction in patients with successful primary bladder closure elsewhere: a single institution experience. J Urol 2001; 165:2438.
  34. Hafez AT, El-Sherbiny MT, Shorrab AA, El-Mowafi H. Complete primary repair of bladder exstrophy in children presenting late and those with failed initial closure: single center experience. J Urol 2005; 174:1549.
  35. Cervellione RM, Husmann DA, Bivalacqua TJ, et al. Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol 2010; 6:450.
  36. Stein R, Stöckle M, Fisch M, et al. The fate of the adult exstrophy patient. J Urol 1994; 152:1413.
  37. Avolio L, Koo HP, Bescript AC, et al. The long-term outcome in men with exstrophy/epispadias: sexual function and social integration. J Urol 1996; 156:822.
  38. Ben-Chaim J, Jeffs RD, Reiner WG, Gearhart JP. The outcome of patients with classic bladder exstrophy in adult life. J Urol 1996; 155:1251.
  39. Mathews RI, Gan M, Gearhart JP. Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int 2003; 91:845.
  40. Smeulders N, Woodhouse CR. Neoplasia in adult exstrophy patients. BJU Int 2001; 87:623.
  41. Eraklis AJ, Folkman MJ. Adenocarcinoma at the site of ureterosigmoidostomies for exstrophy of the bladder. J Pediatr Surg 1978; 13:730.
  42. Hollowell JG, Ransley PG. Surgical management of incontinence in bladder exstrophy. Br J Urol 1991; 68:543.
  43. Lottmann HB, Melin Y, Cendron M, et al. Bladder exstrophy: evaluation of factors leading to continence with spontaneous voiding after staged reconstruction. J Urol 1997; 158:1041.
  44. Surer I, Baker LA, Jeffs RD, Gearhart JP. Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex. J Urol 2001; 165:2425.
  45. Lloyd JC, Spano SM, Ross SS, et al. How dry is dry? A review of definitions of continence in the contemporary exstrophy/epispadias literature. J Urol 2012; 188:1900.
  46. Capolicchio G, McLorie GA, Farhat W, et al. A population based analysis of continence outcomes and bladder exstrophy. J Urol 2001; 165:2418.
  47. McMahon DR, Cain MP, Husmann DA, Kramer SA. Vesical neck reconstruction in patients with the exstrophy-epispadias complex. J Urol 1996; 155:1411.
  48. Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the bladder exstrophy population: predictors of success? Urology 2001; 57:774.
  49. Gearhart JP, Ben-Chaim J, Sciortino C, et al. The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder? Urology 1996; 47:240.
  50. Mathews R, Sponseller PD, Jeffs RD, Gearhart JP. Bladder neck reconstruction in classic bladder exstrophy: the role of osteotomy in the development of continence. BJU Int 2000; 85:498.
  51. Casale P, Grady RW, Waldhausen JH, et al. Cloacal exstrophy variants. Can blighted conjoined twinning play a role? J Urol 2004; 172:1103.
  52. Reiner WG, Gearhart JP, Jeffs R. Psychosexual dysfunction in males with genital anomalies: late adolescence, Tanner stages IV to VI. J Am Acad Child Adolesc Psychiatry 1999; 38:865.
  53. Ebert A, Scheuering S, Schott G, Roesch WH. Psychosocial and psychosexual development in childhood and adolescence within the exstrophy-epispadias complex. J Urol 2005; 174:1094.
  54. Koo HP, Avolio L, Duckett JW Jr. Long-term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol 1996; 156:2037.
  55. Mathews R, Hubbard JS, Gearhart JP. Ureteral reimplantation before bladder neck plasty in the reconstruction of bladder exstrophy: indications and outcomes. Urology 2003; 61:820.
  56. Diseth TH, Emblem R, Schultz A. Mental health, psychosocial functioning, and quality of life in patients with bladder exstrophy and epispadias - an overview. World J Urol 1999; 17:239.
  57. Reiner WG, Gearhart JP. Anxiety disorders in children with epispadias-exstrophy. Urology 2006; 68:172.
  58. Reiner WG, Gearhart JP, Kropp B. Suicide and suicidal ideation in classic exstrophy. J Urol 2008; 180:1661.
  59. Reddy SS, Inouye BM, Anele UA, et al. Sexual Health Outcomes in Adults with Complete Male Epispadias. J Urol 2015; 194:1091.
  60. Mesrobian HG, Kelalis PP, Kramer SA. Long-term followup of cosmetic appearance and genital function in boys with exstrophy: review of 53 patients. J Urol 1986; 136:256.
  61. Woodhouse CR, Hinsch R. The anatomy and reconstruction of the adult female genitalia in classical exstrophy. Br J Urol 1997; 79:618.
  62. Deans R, Liao LM, Wood D, et al. Sexual function and health-related quality of life in women with classic bladder exstrophy. BJU Int 2015; 115:633.
  63. Lattimer JK, MacFarlane MT, Puchner PJ. Male exstrophy patients: a preliminary report on the reproductive capability. Trans Am Assoc Genitourin Surg 1978; 70:42.
  64. Hanna MK, Williams DI. Genital function in males with vesical exstrophy and epispadias. Br J Urol 1972; 44:169.
  65. Deans R, Banks F, Liao LM, et al. Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study. Am J Obstet Gynecol 2012; 206:496.e1.
  66. 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.
  67. Suominen JS, Helenius I, Taskinen S. Long-term orthopedic outcomes in patients with epispadias and bladder exstrophy. J Pediatr Surg 2012; 47:1821.
  68. Gobet R, Weber D, Horst M, et al. Long-term followup (37 to 69 years) in patients with bladder exstrophy treated with ureterosigmoidostomy: psychosocial and psychosexual outcomes. J Urol 2009; 182:1819.
  69. Mednick L, Gargollo P, Oliva M, et al. Stress and coping of parents of young children diagnosed with bladder exstrophy. J Urol 2009; 181:1312.
  70. Schaeffer AJ, Yenokyan G, Alcorn K, et al. Health related quality of life in adolescents with bladder exstrophy-epispadias as measured by the Child Health Questionnaire-Child Form 87. J Urol 2012; 188:1924.
  71. Pennison MC, Mednick L, Rosoklija I, et al. Health related quality of life in patients with bladder exstrophy: a call for targeted interventions. J Urol 2014; 191:1553.