PSARVUP Surgery for Cloacal Malformations

Our skilled pediatric colorectal surgeons, urologists, and gynecologists have experience in posterior sagittal anorectal vaginal urethroplasty (PSARVUP), surgery to correct structural problems in a baby girl’s pelvic organs. At the Colorectal and Pelvic Center at Children's Health℠, our surgeons, nurses and other providers specialize in caring for children with complex conditions of the intestines, urinary tract and reproductive organs. We offer compassionate care to help your child enjoy their best health and quality of life.

What is PSARVUP surgery?

PSARVUP is reconstructive surgery to treat a cloacal malformation, also called cloaca or persistent cloaca. This congenital (present at birth) condition affects the urinary, digestive, and reproductive organs in a baby girl. The result is a single opening, instead of the typical three openings, in the baby’s genital area.

Our surgeons perform a series of staged surgeries, including PSARVUP, for cloacal malformation treatment. Your baby will typically have PSARVUP when they’re at least several months old to separate the single opening into three openings. Your child will likely need other procedures before or after PSARVUP, depending on the specific structural problems related to the cloaca.

What are the benefits of PSARVUP surgery?

Our surgeons perform PSARVUP surgery to correct a cloaca, a type of anorectal malformation that some baby girls are born with. Females are normally born with three openings in their genital area: a urethra for urine (pee), a vagina for reproduction, and an anus for stool (poop). Babies born with a cloaca have only one opening because the urethra, vagina and anus/rectum are connected as a common channel.

PSARVUP creates a more natural structure in your child’s pelvic organs by separating the urethra, vagina and anus into three openings. The key benefit of PSARVUP surgery is that it allows a child born with a cloaca to pass urine and stool more naturally. A PSARVUP surgery also helps reduce the risk of:

  • Damage to the kidneys
  • Urinary tract infections
  • Problems with reproduction or sexual health, later in life

What are the risks of PSARVUP surgery?

As with any surgery, PSARVUP carries some possible risks, such as:

  • Infection after surgery
  • Bleeding from the surgical site
  • Reaction to anesthesia (medicine to put your child into a sleeplike state for surgery)
  • Excessive pain
  • Breakdown of the wound
  • Strictures (narrowing) in any of the surgically created openings

Children who had cloacal malformations may develop constipation or fecal incontinence (soiling and accidents), even with successful PSARPVUP surgery. These children may benefit from our bowel management program .

In the long term, children who had cloacal malformations may have bladder and kidney problems and require catheterizations (passing a tube to drain urine) or other surgeries to help with bladder control and prevent kidney damage. It’s important for your child to have long-term follow-up care with our team of pediatric urologists to monitor them for any conditions that may develop.

Our years of experience performing PSARVUP helps reduce the risk of rare, potential problems. If a problem occurs, our surgeons and their teams quickly take steps to protect your child’s health. Please contact us immediately if your child gets a high fever or cannot pass urine or stool.

What to expect with PSARVUP surgery

At the Colorectal and Pelvic Center, our surgeons have extensive experience in successfully performing PSARVUP for the most complex cloacal malformations in infants and children. Depending on the specific structures affected by the cloaca, your child may need one or more surgeries over several months to fully correct the condition. Together with our teams, we also provide expert care before and after your child’s procedures to ensure they have the best possible urinary, bowel, and reproductive function.

What to expect before PSARVUP surgery

A cloacal malformation can cause a child to have difficulty passing urine, stool or both. Sometimes urine can build up in the vagina, rather than passing through the common channel. The vagina can then get very large and block the kidneys from draining urine.

If urine builds up, your child will need a procedure to drain the vagina, either catheterization (passing a soft tube up the common channel) or a drain (surgically placing a different soft tube into the vagina). Either of these procedures relieves the blockage to prevent long-term damage to the kidneys.

To allow stool passage, your child will have surgery to create a colostomy, usually within days after birth. In this procedure, the surgeon creates an opening in the belly and connects the large intestine to it. Your child will have a collection bag attached to the opening to allow stool to exit the body.

A colostomy doesn’t affect your child’s eating and digestion, and they will continue to grow before the PSARVUP procedure. This colostomy is temporary until after your child has healed from the PSARVUP procedure.

When your child is about five to six months old, our surgeons plan the PSARVUP procedure. We do imaging and other tests to examine the structures and check for any other malformations that may also need repair. For this procedure, your child will have anesthesia (medicine that puts them in a sleeplike state so that they feel no pain).

What to expect during PSARVUP surgery

We usually recommend PSARVUP surgery when your child is about eight to nine months old and will discuss the timing with you. The goal is to restore not only your child’s anatomy, but also improve their lifelong urinary, bowel and reproductive function. We will discuss with you the type of repair that’s best for your child’s specific anatomy (internal structures) and health needs.

In some cases, the surgeon repairs the cloaca with a single incision between the buttocks (posterior approach). For more complex cloacal malformations, your child may need an abdominal (belly) incision in addition to the posterior incision.

What to expect after PSARVUP surgery

Your child will recover in the hospital for several days after PSARVUP. Your child will go home from the hospital with a urinary catheter (soft tube) to help them pass urine. Their first follow-up visit will be a few weeks after surgery, and we’ll examine them to ensure they’re healing well without any issues.

You may need to perform anal dilations to help prevent scar tissue from closing the newly created anus. Your child’s care team will teach you how to perform dilations, which you will do at home until the anal area heals.

After two to three months, we will operate again to close the colostomy and check the urethra, vagina and rectum. After this procedure, your child will pass stool through the large intestine and out of the body through the rectum and anus.

A child who has had a cloaca may have difficulty with toilet training. At our Colorectal and Pelvic Center, we offer a comprehensive bowel management program to help your child achieve bowel control. We work closely with you and your child to customize a program specifically for their unique needs.

How do I prepare my child for surgery?

Your child’s care team will explain how to prepare your child for surgery. Here are some general steps:

  • Depending on the specific nature of the cloaca, your child may need to go to the hospital the day before surgery for a bowel prep.
  • If your child does not need to go to the hospital the day before surgery, the nurse will call you 48 hours (2 days) before the surgery date. They will explain:
    • Guidelines for your child’s food and drink
    • What time you need to arrive at the hospital
  • If your child takes medicine, ask the care team whether they should stop or keep taking it before surgery. Don’t give your child any medicine on the morning of surgery unless your child’s care team tells you to.
  • On the day of surgery, the care team will make sure your child is ready for surgery.

What questions should I ask my provider about PSARVUP surgery?

As you decide on treatment options for your child, some questions you may want to ask include:

  • Are there other treatment options for a cloaca?
  • How soon will I be able to see my child after PSARVUP?
  • How long will my child need to stay in the hospital after PSARVUP?
  • Will my child go home with any special equipment after PSARVUP?

PSARVUP Surgery for Cloacal Malformations Doctors and Providers

At Children’s Health, we’re committed to providing the best possible care for your child, from infancy through young adulthood. Our expert team of colorectal surgeons, urologists, gynecologists, gastroenterologists, psychologists and Child Life specialists is ready to support you and your child throughout their journey.

Frequently Asked Questions

  • Are there other treatment options available for cloacal malformations?

    All types of cloacal malformations need surgery to create three separate structures for the urethra, vagina and rectum/anus. PSARVUP surgery enables a baby girl to pass urine and stool safely and also helps them have normal female sexual and reproductive functions later in life.

  • Will my child have normal urinary, bowel and sexual function after a cloaca?

    Most children recover well after PSARVUP surgery for cloacal malformation repair. Typically, children with more complex malformations are more likely to experience difficulties with urinary, bowel and sexual function later in life.

    After a cloaca repair, most children have difficulty with bowel function and may need a bowel management program to help with toilet training. Some children may need to use a catheter to help with urinary continence (bladder control).

    Most women who had a cloaca as a baby have typical sex lives as adults. They can become pregnant and bear children, although they may need to have cesarean sections instead of vaginal births.

  • Will my child need lifelong care after a cloaca?

    Children born with cloacal malformations need ongoing follow-up care because the condition can increase the risk of certain urological, gynecological and colorectal conditions. Some children may experience constipation, urinary tract infections, and bowel and urinary incontinence, which, if not treated promptly, can lead to more serious conditions.

    Some children may need additional surgeries later in life. It’s important to continue to bring your child to the Colorectal and Pelvic Center for follow-up care, where we can adjust their care plan as needed.

    During the teen years, many young women develop new health problems that can affect their quality of life. We’ll evaluate your daughter when she begins puberty to ensure that she will be able to menstruate. As needed, our pediatric and adolescent gynecologist offers treatment in a sensitive, confidential manner for problems affecting young women’s reproductive health.

    Young women may develop issues with body self-image and sexual function and may benefit from psychological support. Our center offers access to pediatric psychologists, Child Life specialists and social workers for therapy and other services. Being proactive can empower young adults to take a larger role in managing their own condition, which can result in lifelong health benefits.

    As your child becomes an adult, their health needs change. We can help your child transition to an adult care provider who is experienced in treating women with cloacal malformations and other anorectal malformations (ARMs). Some adults who received care for an ARM as children may experience infertility, obstetric challenges and kidney disease or failure, among other problems. For these reasons, it’s critical that they continue with ongoing care throughout their lives.

Resources