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Bladder exstrophy from childhood into adult life
J Ben-Chaim1, S G Docimo, R D Jeffs
1Department of Urology, Johns Hopkins Hospital and University School of Medicine, Baltimore, MD 21287-2101, USA.
Journal of the Royal Society of Medicine
|January 1, 1996
Summary
Bladder exstrophy, a rare congenital condition, results from abnormal development of the cloacal membrane. This developmental anomaly leads to the exstrophic urinary tract or cloacal exstrophy, depending on urorectal septum timing.
Area of Science:
- Developmental biology
- Pediatric surgery
- Urology
Background:
- Bladder exstrophy is a rare congenital anomaly with an incidence of 1 in 30,000 to 50,000 live births.
- The male to female ratio for bladder exstrophy ranges from 1.5-5:1.
Purpose of the Study:
- To elucidate the developmental mechanisms underlying bladder exstrophy and cloacal exstrophy.
Main Methods:
- Review of embryological development.
- Analysis of the role of the cloacal membrane and urorectal septum.
Main Results:
- Persistence or overgrowth of the cloacal membrane impedes normal mesenchymal ingrowth.
- This disruption causes lower abdominal muscle divergence and abnormal genital ridge fusion.
- The timing of urorectal septum ingrowth during rupture dictates the type of exstrophy (classic bladder exstrophy or cloacal exstrophy).
Conclusions:
- Bladder exstrophy arises from specific disruptions in early embryonic development.
- Understanding these mechanisms is crucial for diagnosing and managing exstrophy conditions.