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Cloacal Exstrophy

Cloacal Exstrophy

What Is Cloacal Exstrophy? 

Cloacal exstrophy is a birth defect in which part of the abdomen is open and some of the abdominal organs (the bladder and intestines) are on the outside of the body. It often causes a separation of both male and female genitalia, and the anus may be closed. It occurs in 1 out of every 200,000 to 400,000 births. 

The colon in these patients is often very short. In the past, all of these patients would have had a permanent stoma (opening on the outside of the abdomen for stool collection into a bag). However, this decision is now made based on the individual patient, since some may be able to have their stoma closed. 

Patients with cloacal exstrophy benefit most from a multidisciplinary care team working together to address all their medical needs. This approach helps achieve the best long-term outcome. 

Initial Management

  • The first priority after birth is to ensure that your child is stable. Tests are done to rule out any related anomalies.
  • Due to the separation of genitalia it is important to complete chromosomal testing to determine your child’s gender.
  • Surgery to close the opening in the abdomen (and bladder if possible) and to create a stoma is completed.

Long-Term Management

  • Your child should maintain close follow-up with their team of doctors, especially the surgeon and urologist.
  • Proper nutrition is very important for these children. Because of their short colon, you child could lose too much salt through their stool. This can also affect how much sugar is absorbed in the body which may hinder their growth. 
    • Your child will need to have their urine sodium checked soon after the stoma is created. This test is repeated until the results are normal. 
    • Some children need to take sodium pills orally in order to get their sodium to a normal level.
  • When your child is ready for potty training, the surgeon may order a study to test the length of their colon and examine the stool in the stoma bag. If your child’s colon has grown and their stool is of thicker consistency, the surgeon may offer bowel management through the stoma. This involves giving an enema through the stoma to clean out the stool. Your child may also need a special diet and/or medications to thicken and slow down the stool. 
  • If your child’s stoma bag is able to remain empty for 24 hours they may be able to have a closure of the stoma and surgery to reconstruct the anus and genitalia.
  • If your child is unable to have their stoma closed, the surgeon will discuss with you the timing of their reconstructive surgeries to be performed without the stoma closure.

Last Updated 01/2020