Cloacal Anomaly

What is a cloacal anomaly?

A cloacal abnormality is when the urinary tract, rectum and vagina merge together to form a common channel that exits the body through one opening. This a rare abnormality that develops in baby girls during the early stages of a pregnancy.

Babies born with a cloacal abnormality have a single opening where the urethra would typically open. The opening is often hooded and elongated. Cloacal abnormalities can have other signs and symptoms depending on where the urinary tract, rectum and vagina merge together.

If these tracts merge together close to the opening, the newborn doesn’t have a visible anus (opening where stool leaves the body). These babies have a short common channel, which allows stool and urine to easily leave the body.

In other cases, the three tracts merge together farther away from the opening. This longer common channel makes it more difficult for stool and urine to leave the body. These cases are more complex, and these babies often have urinary tract infections.

Babies with a cloacal abnormality may also have:

• A malformed anus

• A clitoris that looks more like a penis

• Reproductive organs, bladder, rectum and/or muscles in the pelvis that are not fully developed

• A blockage (obstruction) in the vagina

• More than one vagina and/or cervix

Some babies may also have other problems with their spinal cord, heart or arms and legs.

How are cloacal anomalies diagnosed?

A doctor will examine your baby right after birth to make a diagnosis. Though cloacal anomalies can sometimes be seen on prenatal ultrasounds during pregnancy, it’s difficult to make a definitive diagnosis until the baby is born.

After diagnosis, our team of experts will perform imaging tests so they can better understand the specifics of your child’s condition. These may include:

• X-rays

• Ultrasound

• Endoscopy, which means your baby goes under anesthesia and doctors use a small tube to view inside the common channel

Once a cloacal anomaly is diagnosed, your baby will need other work up to look for other related birth defects:

What causes cloacal anomalies?

At this time, it's not known what causes cloacal abnormalities or how to prevent them. The condition is very rare and occurs in about 1 in 25,000 newborn baby girls.

How are cloacal anomalies treated?

All cloacal anomalies require surgery at some point. Your child's treatment plan will depend on the type and severity of their condition.

Immediate treatment

The immediate goal of treatment is to make sure urine and stool can leave your child's body. Some babies may be able to empty urine and stool through the single opening, but will still need a surgical procedure called a colostomy that diverts the stool away from the urinary tract so that poop comes out through the abdominal wall into a bag instead. For some babies, urine might build up in the vagina and cause blockage of the urinary tract. In these situations, they may need a catheter to drain urine and sometimes may need a surgical procedure called a vaginostomy to drain urine out of the vagina. It is important to ensure urine is draining properly to prevent kidney damage.

During this time, your baby receives around-the-clock care in our nationally ranked Level IV NICU. As your baby grows and becomes stable, doctors will begin to plan for reconstructive surgery.

Once your baby is safely eliminating stool and urine, then the care team will want to ensure that baby is healing from surgery and growing well.

Reconstructive surgery

Between the ages 6 months and 12 months, your baby will have surgery to separate their urethra, vagina and rectum. Cloacal anomalies can have many variations, and it requires extensive planning leading up to surgery. It may take additional endoscopy or Xray procedures to better define the anatomy when your baby is bigger before your team of surgeons would want to perform reconstructive surgery.

Sometimes the surgery can be performed only through the bottom, but more severe anomalies may require surgery through both the bottom and the belly.

It is typical that after reconstructive surgery, your baby would still have a colostomy in order to protect the repair from stool. It will require another surgery later to close the colostomy.

Follow-up care

Our team continues to provide any care your child needs throughout childhood. This can include treatment for:

• Constipation or stool accidents through a personalized bowel management program

• Nutrition and growth

• Urinary leakage, urinary tract infections, and kidney disease

• Reproductive concerns that your child may have as she approaches puberty

• Psychological coping

It is important that your child continues to follow up with a multidisciplinary team of experts for coordinated care throughout her childhood and adolescence. She may have problems with different issues at different stages of life related to being born with a cloacal anomaly.