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The ultimate challenge of cloacal exstrophy
Justine M Schober1, Polly A Carmichael, Melissa Hines
1Department of Psychology, City University, London, United Kingdom.
The Journal of Urology
|December 18, 2001
Summary
Cloacal exstrophy presents complex system abnormalities, including spinal and genitourinary issues. Gender assignment and long-term quality of life remain significant challenges requiring further research.
Area of Science:
- Pediatric Surgery
- Urology
- Developmental Biology
Background:
- Cloacal exstrophy (CE) is a rare congenital anomaly with significant multi-system involvement.
- Management of CE requires a multidisciplinary approach, addressing complex anatomical and physiological challenges.
Purpose of the Study:
- To review system abnormalities in cloacal exstrophy and address the complexities of gender assignment.
- To explore gender identity, role behavior, and sexual orientation in patients with CE.
Main Methods:
- Comprehensive literature review of cloacal exstrophy management, outcomes, and concurrent anomalies.
- Original research evaluating gender identity in female-raised patients with CE.
Main Results:
- High incidence of spinal dysraphism (approaching 100%) and cord tethering.
- Improved survival and reduced morbidity for short bowel syndrome with advances in nutrition and antibiotics.
- Neurological impact on bladder and lower extremity function; improved continence with Mitrofanoff reconstruction.
- Genital abnormalities noted in males (30% with absent/diminutive penis); uterine anomalies common in females, with generally normal ovaries/tubes and no documented fertility.
Conclusions:
- Surgical strategies and timing for gender assignment in CE remain controversial.
- A critical need exists for research into gender development and quality of life in CE patients.
- A cautious, watchful approach is recommended during maturation into adulthood.