Cloacal Malformation

What is a Cloacal Malformation?

When a female baby is born with a cloacal malformation, the body’s systems for digestion, urination, and reproduction come together into one shared tract. Instead of having separate openings for the rectum, urethra, and vagina, there is just one opening.

Cloacal malformation is rare and can vary in severity. It can cause problems with going to the bathroom and may affect reproductive health later on.

Surgery is usually needed to help restore each system’s natural function and give your child the best chance at a healthy future.

Signs and Symptoms of Cloacal Malformation

Signs of cloacal malformation often appear after birth. You or your child’s doctor may notice that your baby doesn’t pass stool or urine as expected. Sometimes there’s swelling in the belly, or a single opening where there should be more than one.

Over time, children may also have trouble with bladder or bowel control. Every child is different, but early symptoms are often a sign that testing is needed to understand the severity of the condition. If you think your baby may have cloacal malformation, schedule an appointment with your pediatrician.

CHRISTUS Children's General Surgery Clinic

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Pediatric General Surgery Clinic

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Pediatric General Surgery Clinic

CHRISTUS Children's General Surgery Clinic in San Antonio provides exceptional surgical care for infants, children, and teens. 

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Care That Supports Every Part of Your Child's Journey

When your child needs care for a cloacal malformation, you deserve a team that sees the whole picture — and the whole child. That means caring for more than one system at a time. It means staying with you, from the first questions to the last follow-up.

At CHRISTUS Children’s, specialists in pediatric surgery, urology, gynecology, and gastroenterology come together to create one care plan — shaped just for your child. Nutrition, bowel care, and healing are all connected here.

And so are you. Every step includes guidance, answers, and support from a team that knows this path and walks it with families every day.

Diagnosis of Cloacal Malformation

Most cases of cloacal malformation are diagnosed soon after birth, especially if your baby isn’t passing urine or stool. Your doctor may use imaging tests to understand how the urinary, digestive, and reproductive systems are connected. Sometimes, more detailed studies are needed as your child grows. These tests help the care team plan the safest, most effective treatment for your child.

Treatment for Cloacal Malformation

Every child’s needs and anatomy are different, so the first step is often imaging and gentle exams to learn how the organs are connected.

Many babies will need a temporary colostomy soon after birth to help them pass stool safely while the body heals and grows. A colostomy is a surgery that helps your baby pass stool by creating a small opening in the belly, where waste leaves the body into a bag. This is temporary, giving the body time to heal before reconstructive surgeries.

What is the long-term outcome for patients with a cloacal malformation?

The long-term outcomes for children with cloacal malformations have improved significantly with advances in surgical techniques and aftercare:

  • Bowel and urinary function: Many patients can have good control over their bowel and urinary functions. However, some may need ongoing bowel management or intermittent catheterization to help with urination.
  • Reproductive health: With appropriate surgical repair, many patients can have normal sexual function. Fertility may be affected depending on the severity of the malformation and the surgical interventions required.
  • Quality of life: Most children with cloacal malformations can live normal, active lives. However, they will need long-term care from a health care team. This team includes urologists, gynecologists, and colorectal specialists.
  • Psychological support: Your child and family may need help to deal with the condition. This is especially true during adolescence when body image and sexual health become more important.

It is important for you, as parents and caregivers, to work closely with your child's health care team. This will help ensure the best outcomes for your child.

PSARVUP Surgery for Cloacal Malformation

The primary surgery to fix a cloacal malformation is known as a Posterior Sagittal Anorectal Vaginalplasty, or PSARVUP. This surgery aims to separate the three channels—rectum, urethra and vagina—and create individual openings for each.

Steps of the surgery:

  • Pre-surgical preparation: All babies born with a cloacal malformation receive a colostomy in their first week of life. This helps divert stool and allows the intestines to rest before the final surgery.
  • Surgical repair: At about six months of age, your child may be ready for the full repair, and the definitive surgery will be performed. The surgeon will carefully dissect and separate the rectum, urethra, and vagina, bringing them to the surface to create three distinct openings.
  • Reconstruction: Depending on your child’s specific anatomy, further reconstruction may be necessary to ensure proper function and appearance.

The procedure may require one or multiple stages, depending on the complexity of the malformation.

Understanding PSARVUP Surgery

Posterior Sagittal Anorectal Vaginal Urethral Plasty, or PSARVUP, is the primary surgery used to repair cloacal malformations. It is a detailed procedure designed to give each organ its own healthy, working pathway.

Here’s a step-by-step of what happens during the PSARVUP surgery:

Anesthesia and Positioning

  • Your child will be fully asleep and won’t feel pain during the procedure. The care team will gently position her for the surgery.

    Creating the Surgical Opening

  • A straight incision is made down the center of the buttocks for access. If needed, small tools may also be used through the belly, called a laparoscopy, to safely move the organs into place.

    Separating the Organs

  • The rectum, vagina, and urethra are carefully separated.
  • The rectum is brought down to create a new anal opening.
  • The vagina and urethra are guided into the right positions to allow for future menstruation and normal urination.

    Rebuilding the Openings

  • Each organ is brought to the surface with its own opening:
  • A new anus is created in the correct place.
  • Separate openings are reconstructed for urination and the vaginal area so they can function normally.

    Closing the Incision

  • The muscles around the new anus are positioned to help with bowel control, and the skin is stitched closed with care. After healing, your child will have three distinct openings for bowel movements, urination, and reproductive health.