Cloacal exstrophy is a very rare condition in which a child is born with their internal abdominal organs — the large intestine and bladder — outside of the body and the bladder is separated into two halves. In boys, the penis is usually flat and short and is sometimes split in two. In girls, the clitoris is separated into two halves. Children born with cloacal exstrophy may also have imperforate anus, spinal defects such as spina bifida, and/or other organs exposed including the liver, spleen, colon and rectum (omphalocele).
Cloacal exstrophy occurs during fetal development and is diagnosed during pregnancy through an ultrasound. Once your child is born, it is confirmed through a physical examination.
Cloacal exstrophy is treated with reconstructive surgery. The complexity of the condition requires a multidisciplinary team of colorectal and pelvic health surgeons. The surgical repair is often done in stages based on the complexity of your child’s condition. The descriptions below are general guidelines, but it is essential that each treatment plan is tailored to the needs of the child.
- Often, shortly after birth, the child’s bladder is repaired and placed back into the body. To ensure safe bowel movements, a colostomy is performed. A part of the large intestine is connected to a surgical opening created in the abdomen (stoma) and feces is then passed through the stoma into a sealed pouch outside the body.
- Bladder repair
- This may be done as a single stage procedure where the two bladder halves are closed and placed into the bladder. The bladder repair may also be done with delayed bladder closure, occurring anywhere from 3 months to 2 years old, allowing the bladder plates to grow and the bones of the pelvis to mature to allow for a stronger osteotomy.
- Posterior Sagittal Anorectoplasty (PSARP)/ Pull-Through
- The decision to proceed with anal reconstruction is made in early toddler period. Consideration for pull through depends on the potential for bowel control, length of colon, and ability for child to make solid stool. Other factors taken into consideration are strength and position of the sphincter muscles, severity of spinal anomalies, and ability to participate in a good bowel management program to ensure cleanliness..
As your child ages, vaginal or penile reconstruction surgery may occur to repair physical abnormalities to the genitals.
Prognosis (or Long-term Outcomes)
Children born with cloacal exstrophy may still experience bladder and bowel control problems after surgery. Follow-up with a multidisciplinary team is needed long-term to manage residual complications. If a pull through has been performed, our bowel management team at Texas Children’s Hospital can help optimize your child’s fecal continence.