Long-term active problems in patients with cloacal exstrophy: A systematic review

J Pediatr Surg. 2022 Mar;57(3):339-347. doi: 10.1016/j.jpedsurg.2021.08.020. Epub 2021 Sep 4.


Background: Cloacal exstrophy (CE) is the most severe end of the Exstrophy-Epispadias Complex malformations spectrum. Improvements in postnatal management and well-established operative techniques have resulted in survival rates approaching 100%. This systematic review aims to define the prevalence of long-term active medical problems affecting CE patients after the first decade of life.

Methods: PubMed/Medline, Embase, Scopus, and ISI Web of Knowledge databases were used for the literature search. Original articles related to medical, surgical, and psychosocial long-term problems in CE patients >10 years of age were included in the study. Quality assessment of the articles was performed through the Newcastle-Ottawa Scale. Prevalence estimates and 95% CI were assessed for each outcome.

Results: Twelve studies were included. The most common long-term active problems identified were: urinary incontinence with a prevalence ranging from 9.1% to 85%; sexual function issues related to vaginal anomalies with a prevalence ranging from 8.3% to 71.3%, and uterine anomalies, with a prevalence from 14.3% to 71%; gender identity issues in 46, XY patients raised female had a prevalence from 11.1% to 66.7%. There is no documented history of paternity. Impairment of ambulatory capacity was recorded in 13.8% of patients. Only one paper studied psychological well-being, reporting significantly higher levels of depression among gender reassigned patients.

Conclusions: Teenagers and adults born with CE have well defined long-term problems compared to the general population. Recognition and expert management are crucial to improve care and quality of life during and after the transition into adulthood.

Keywords: Cloaca exstrophy; Gastrointestinal outcomes; Gender identity issues; Long-term outcomes; Urinary outcomes.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Animals
  • Bladder Exstrophy* / epidemiology
  • Bladder Exstrophy* / surgery
  • Cloaca
  • Epispadias*
  • Female
  • Gender Identity
  • Humans
  • Male
  • Quality of Life