Overview
Cloacal exstrophy is a rare but severe congenital condition where the abdominal organs, bladder, and intestines are exposed outside the body. It occurs during fetal development when the lower abdominal wall and pelvic structures do not form properly. Babies with this condition require immediate medical attention and multiple surgeries to reconstruct the urinary, gastrointestinal, and reproductive systems. In Korea, advanced pediatric surgical teams provide comprehensive, multidisciplinary care for this rare anomaly.
What is Cloacal Exstrophy?
Cloacal exstrophy (also called OEIS complex: Omphalocele, Exstrophy of the bladder, Imperforate anus, Spinal defects) is a birth defect involving:
- Split or exposed bladder
- Exposed intestines or omphalocele (sac-like covering of abdominal organs)
- Imperforate anus (no anal opening)
- Genital abnormalities
- Sometimes associated with spinal malformations
This condition is extremely rare, occurring in about 1 in 200,000–400,000 live births worldwide.
Symptoms
- Abdominal organs (intestines, bladder) visible outside the body at birth
- Imperforate anus (no anal opening)
- Split or malformed genitalia
- Spinal defects (such as spina bifida)
- Urinary incontinence and poor bladder control
- Feeding difficulties in newborns
- Possible kidney abnormalities
Causes
The exact cause is unknown, but it is believed to result from errors in embryonic development of the abdominal wall and cloaca during the first trimester.
- Genetic predisposition (rare)
- Environmental factors (maternal health, exposure to toxins)
- Sporadic developmental abnormality (most common)
Risk Factors
- Family history of abdominal wall or bladder defects
- Certain maternal health conditions (e.g., diabetes, obesity)
- Possible genetic syndromes
- Twin pregnancies (slightly higher risk)
Complications
- Life-threatening infection risk immediately after birth due to exposed organs
- Urinary incontinence and kidney problems
- Bowel dysfunction (constipation, incontinence)
- Infertility or reproductive challenges in adulthood
- Repeated need for surgeries throughout childhood
- Psychosocial issues due to body image and chronic health problems
Prevention
Since the cause is not fully understood, prevention is difficult. However, some measures may reduce risk:
- Adequate folic acid intake before and during pregnancy
- Proper maternal health management (control of diabetes, obesity)
- Avoidance of alcohol, smoking, and harmful medications during pregnancy
- Prenatal screening and early ultrasound detection
Treatment Options in Korea
Korea provides advanced, specialized care for complex congenital anomalies, including cloacal exstrophy. Treatment requires multiple stages and a multidisciplinary approach.
- Immediate Care after Birth
- Protecting exposed organs with sterile coverings
- Stabilizing the infant’s condition (fluids, infection control, respiratory support)
- Surgical Management
- Initial Surgery (Neonatal Stage): Closing abdominal wall, separating bladder halves, creating a temporary colostomy (to allow stool passage).
- Bladder Reconstruction: Later surgeries to create urinary continence and protect kidney function.
- Bowel and Anus Surgery: Creating a functional anal opening and restoring digestive continuity.
- Genital Reconstruction: Surgery to reconstruct genitalia and improve function/appearance.
- Orthopedic and Spine Surgery: For associated spinal defects if present.
- Long-Term Support
- Urology follow-up for bladder and kidney function
- Colorectal and gastrointestinal management
- Orthopedic and neurological monitoring
- Psychosocial and family support
- Fertility and reproductive counseling in adulthood
