How is Cloacal Exstrophy Treated?
Newborns with cloacal exstrophy will need a multidisciplinary team to plan treatment that is specific to the type and extent of the condition. This care will start at birth and will be ongoing as the child develops.
Initial Management
The first priority after birth is to ensure your child is stable. This includes tests to rule out any heart problems. Surgery is usually done within one to two days after birth. The goal of this first surgery is to separate the bowel from the bladder. At the same time, the intestine will be placed back into the abdomen, and the abdominal wall will be closed. At this time, a procedure called a Hindgut rescue is done. During this procedure, a surgeon creates a stoma (a small opening in the abdomen) at the end of the colon. This will allow the stool (poop) to pass through the intestine to the outside of the body into a collection bag. A urologist will also join the bladder together. They may place the bladder into the abdomen.
A coordinated care team will monitor your child to make sure they are recovering well from the surgery. The care team will also do chromosomal testing to determine gender. Gender reconstruction procedures typically wait until the child can be a part of the final decision.
From there, the next few weeks and months are focused on getting your child proper nutrition. This care is done in collaboration with the intestinal rehabilitation team, including dietitians.
After initial treatment and recovery, your care team will use ultrasounds and MRI scans to check for any spinal abnormalities. Some of these findings may need surgery to correct. Your child may have a second surgery within the first year of life to close the bladder and reconstruct the pelvis. Your care team will coordinate procedures to limit the number of times your child has to go under anesthesia.
Long-Term Management
After your child’s initial procedures, close follow-up with your care team is needed. Proper nutrition will continue to be important for your child.
Some patients with cloacal exstrophy may need a permanent stoma (opening on the outside of the abdomen for stool collection into a bag), but this decision is individualized, and stoma closure may be possible for some patients. When a stoma is present, careful monitoring of a child’s growth is important, because excess salt (sodium) loss through the stoma can slow growth. To support proper nutrition, healthcare providers regularly check urine sodium levels during follow-up visits. Sodium monitoring begins when the stoma is created and continues periodically as long as the stoma remains, especially if there are growth concerns. Some children need oral sodium supplements to maintain normal sodium levels and support healthy growth.
Traveling with a child with cloacal exstrophy may require extra care. Families need to have all medical supplies with them when traveling. Parents should also know where the nearest hospital and emergency room are located and when to seek care.
As your child grows, your surgeon will check how well your child’s bowels and bladder are working. They will also check how well your child can hold poop and urine.
The care team may:
- Measure how long the colon is
- Check what the poop is like (soft or thick)
This helps the team decide if it may be safe to close the stoma (the opening on the belly where poop comes out). Between ages 1 and 5, some children may be able to have the stoma closed. This would require surgery to create an anus (anoplasty) and may also include surgery on the genitals. Most children will need bowel care through the stoma before it can be closed. Bowel care may include giving an enema through the stoma to clean out poop, and/or special foods to help make poop thicker. If bowel care works well, closing the stoma may be an option.
Some patients and families choose to keep the stoma. This choice depends on how well the bowel care works and what feels right for the patient and family. The decision is made together with the family and care team. Some patients may need surgery on the genitals. The timing is different for every child and often this decision is delayed until the child is old enough to help decide.
