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Exstrophy of cloaca sequence — Clinical Guidelines

Overview

Bladder exstrophy, part of the exstrophy of cloaca sequence, is a congenital anomaly characterized by the failure of the cloacal membrane to fuse, resulting in exposure of the bladder, urethra, and other pelvic organs. 1 2

Diagnosis

Clinical presentation includes exposed bladder, short or absent perineum, and often epispadias.

Imaging studies (radiologic) are crucial for assessing associated anomalies and guiding management decisions. 11

Genetic and familial risk factors should be considered; familial occurrences are rare but noted in some cases. 3

Management

Primary surgical reconstruction is recommended for optimal long-term outcomes. 9

Anterior pelvic osteotomy may be employed to correct pelvic obliquity in conjunction with bladder reconstruction. 4

Umbilicus preservation techniques can be used during reconstructive surgery to maintain cosmesis. 5 6

The exstrophy support team approach enhances lifelong care coordination and patient outcomes. 7

Special Populations

Pediatrics: Early surgical intervention is critical for optimal physical and psychological development. 9

Comorbidities: Management strategies should consider associated conditions like prune belly syndrome, emphasizing the importance of radiologic assessment and urethral obstruction management. 11

Key Recommendations

Primary anatomical reconstruction should be performed for optimal long-term quality of life and functional outcomes. (Evidence: Strong 9)

Utilize an exstrophy support team for comprehensive, lifelong care coordination to improve patient outcomes. (Evidence: Moderate 7)

Consider preservation techniques for the umbilicus during surgical reconstruction to enhance cosmetic results. (Evidence: Weak 5 6)

References

1 Zaman MH, Davis R, Maruf M, DiCarlo H, Gearhart JP. Exploration of Practice Patterns in Exstrophy Closures: A Comparison Between Surgical Specialties Using a National and Institutional Database. Urology 2019. link 2 Buyukunal CS, Gearhart JP. A short history of bladder exstrophy. Seminars in pediatric surgery 2011. link 3 Gambhir L, Höller T, Müller M, Schott G, Vogt H, Detlefsen B et al.. Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. The Journal of urology 2008. link 4 Perović S, Brdar R, Scepanović D. Bladder exstrophy and anterior pelvic osteotomy. British journal of urology 1992. link 5 Hanna MK. Reconstruction of umbilicus during functional closure of bladder exstrophy. Urology 1986. link90310-9) 6 Hanna MK, Ansong K. Reconstruction of umbilicus in bladder exstrophy. Urology 1984. link90201-2) 7 Lattimer JK, Hensle TW, MacFarlane MT, Beck L, Braun E, Esposito Y. The exstrophy support team: a new concept in the care of the exstrophy patient. The Journal of urology 1979. link56831-7) 8 Bracci U, Laurenti C. Rectal bladder in the treatment of bladder exstrophy. European urology 1979. link 9 Lattimer JK, Beck L, Yeaw S, Puchner PJ, Macfarlane MT, Krisiloff M. Long-term followup after exstrophy closure: late improvement and good quality of life. The Journal of urology 1978. link57583-7) 10 Toguri AG, Churchill BM, Schillinger JF, Jeffs RD. Gas cystometry in cases of continent bladder exstrophy. The Journal of urology 1978. link57540-0) 11 Berdon WE, Baker DH, Wigger HJ, Blanc WA. The radiologic and pathologic spectrum of the prune belly syndrome. The importance of urethral obstruction in prognosis. Radiologic clinics of North America 1977. link 12 Kogan SJ, Hankin LG, Levitt SB. Total duplicate exstrophic bladder and urethra: variation of incomplete bladder exstrophy. Urology 1976. link90056-x)

Exstrophy of cloaca sequence