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

Area of Science:

• Pediatric Surgery
• Urology
• Reproductive Medicine

Background:

• Cloacal exstrophy (CE) is a severe congenital malformation.
• Improved survival rates necessitate understanding long-term health issues.
• Focus on active medical problems beyond the first decade of life.

Purpose of the Study:

• To systematically review long-term medical problems in CE patients.
• To define the prevalence of these issues after age 10.
• To inform clinical management and improve patient outcomes.

Main Methods:

• Comprehensive literature search of major databases (PubMed, Embase, Scopus, Web of Knowledge).
• Inclusion of original articles on medical, surgical, and psychosocial issues in CE patients >10 years old.
• Quality assessment using the Newcastle-Ottawa Scale and prevalence estimation with 95% confidence intervals.

Main Results:

• Urinary incontinence (9.1%-85%) and sexual function issues (8.3%-71.3%) are highly prevalent.
• Uterine anomalies (14.3%-71%) and gender identity issues in 46,XY patients (11.1%-66.7%) are common.
• Impaired ambulatory capacity (13.8%) and psychological distress, including depression, are noted.

Conclusions:

• Adolescents and adults with CE experience distinct long-term health challenges.
• Expert management is essential for addressing these issues.
• Improved care and quality of life are achievable through recognition and intervention.