Cloacal exstrophy is a very rare congenital (from birth) defect that can cause a variety of conditions:
- Omphalocele. An omphalocele is a type of hernia where the child’s intestine or other abdominal organs stick out of the belly button. They are covered by a thin layer of tissue but are exposed and vulnerable.
- Exstrophy of the cloaca. A cloaca is a common passageway for feces, urine, and reproduction. In exstrophy of the cloaca, part of the intestine that should be joined is separated by part of the bladder. Boys with cloacal exstrophy also may have undescended testicles and epispadias.
- Imperforate anus. An imperforate anus prevents stool from passing out of the rectum. If the passageway is completely blocked, emergency surgery is necessary.
- Spinal defects. Many children with cloacal exstrophy also have spina bifida.
Bladder exstrophy is a milder form of cloacal exstrophy. In cloacal exstrophy, defects of the urethra, bladder, and bowel are present. In bladder exstrophy, defects of the urethra and bladder are present.
For more information on bladder/cloacal extrophy, visit the International Birth Defects Information Systems Web site.
What is the cause?
The cause for this condition is unknown, but there is a slightly larger incidence of cloacal exstrophy within families, which suggests some genetic component to the condition.
How is it treated?
Before treating cloacal or bladder exstrophy, the medical team will gain an understanding of exactly how this condition affects your child. Often, the diagnosis is made via fetal ultrasound while the baby is still in utero.
After birth, your child likely will undergo a variety of tests, which may include a CT scan, MRI, ultrasound, and/or endoscopy, which will help determine exactly how your child is affected by this condition.
Both bladder exstrophy and cloacal exstrophy require a series of surgeries that begin just after birth and continue for several years:
- Initial closure surgery. Surgeons close the bladder, reposition pelvic bones, and repair the genitalia. After surgery, patients may be left with epispadias, which must be repaired later after the child has had a chance to recover from the initial closure surgery.
- Epispadias repair. This surgery typically is performed when the child is under one year old.
- Ureter/bladder sphincter reconstruction surgery. When your child’s bladder has grown to a sufficient size, another surgery must be performed to reposition the ureters (tube from the kidneys to the bladder) and create a bladder sphincter. In the majority of cases, this surgery allows your child to achieve control of urine and become toilet-trained.
Children with cloacal exstrophy have all these procedures performed and there may be other procedures performed as well, depending on your child’s needs:
- Colostomy surgery. Soon after a child with cloacal exstrophy is born, a surgery will be performed to allow stool to pass outside the body. This is done by creating a colostomy (a pouch on the outside of the body that collects waste).
- Colostomy pouch removal. If your child is born with enough intestine, and is capable of forming solid stool, another surgical procedure at a later date will eliminate the need for a colostomy pouch.
About surgery for bladder/cloacal exstrophy at Children’s
The pediatric urology surgery team at Children’s provides next-generation care to neonatal infants, newborns, children, and adolescents from throughout the Upper Midwest. The team consistently performs some of the most cutting-edge surgical procedures available, including newborn surgery, minimally invasive surgery, and robotic surgery, when appropriate. Urologic surgery is performed at Children’s – Minneapolis, Children’s – St. Paul and Children’s – Minnetonka.
- If you are a family member looking for a Children’s specialist in urology surgery, please call the Center for Pediatric Urology at 1-800-992-6983.
- If you are a health professional looking for a consultation or referral information, please call Children’s Physician Access at 1-866-755-2121 (toll-free).