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Condition

Pediatric Anorectal Malformations

What You Need to Know

Anorectal malformations are birth defects in which a baby's anus, rectum and associate muscles and nerves do not develop properly.

Types of Anorectal Malformations

There are several types of anorectal malformation, including:

  • Cloacal malformation. The vagina, rectum and urinary tract are combined into a single channel.
  • Imperforate anus. The anal opening is missing or blocked.
  • Rectal atresia. The rectum does not connect to the anus.
  • Rectal stenosis. The anus or rectum is too small to allow stool to pass.

Treatment

Surgical treatments for anorectal malformation include:

  • Colostomy. Creates an opening for the colon, or large intestine to excrete waste in a small bag located outside of the body.
  • Posterior Sagittal Anorectoplasty (PSARP). Connects the rectum to the anal opening as well as close any abnormal openings.
  • Colostomy Closure. The closing of the colostomy.

Recovery

Recovery from anorectal reconstructive surgery will typically require two to four days in the hospital. For several months after surgery, anal dilations may need to be performed to help stretch the anus to the appropriate size for your child’s anatomy and age.

The Children’s National Colorectal & Pelvic Reconstruction team has exceptional experience treating even the most complex anorectal malformation cases, such as imperforate anus, and specializes in the repair of the colorectal, urologic and gynecologic organs.

What are anorectal malformations?

Anorectal malformations occur when a baby’s anal opening, rectum and the associated sphincter muscles and nerves that help the body determine that it is time to go to the bathroom do not develop properly. As a result, the patient is unable to have normal bowel movements, which can lead to a host of other health problems.

Anorectal malformations are congenital defects that occur in developing babies early on in pregnancy. They can range from mild to complex and can develop differently in males and females. Treatment and prognosis will vary depending on the type and severity of the malformation.

What are the different types of anorectal malformations in children?

There are several types of anorectal malformation, each of which has its unique characteristics. Doctors typically classify malformations based on where the rectum ends in the abdomen and if there is a fistula – an abnormal connection that develops between organs which can cause stool to pass into other parts of the abdomen. Types of anorectal malformations include:

  • Cloacal malformation: the vagina, rectum and urinary tract are combined into a single channel
  • Imperforate anus: the anal opening is missing or blocked
  • Rectal atresia: the rectum does not connect to the anus
  • Rectal stenosis: the anus or rectum is too small to allow stool to pass

How are anorectal malformations treated?

Most babies with an anorectal malformation will need one or more specialized surgeries to correct the problem. Our highly experienced colorectal surgeons are trained in the latest surgical approaches for all types of anorectal malformations including implementing leading-edge, minimally invasive techniques.

Surgical repair for anorectal malformation includes:

Colostomy

A colostomy is a surgical procedure that creates an opening for the colon, or large intestine to excrete waste in a small bag located outside of the body. Most babies born with an anorectal malformation will need a temporary colostomy within the first few days of life. The colostomy will ensure the baby can properly pass stool, helps to normalize digestion and greatly reduces the risk of infection.

Posterior Sagittal Anorectoplasty (PSARP)

Anorectal reconstructive surgery, also known as posterior sagittal anorectoplasty (PSARP), is performed to connect the rectum to the anal opening as well as close any abnormal openings that may interfere with the ability to have a normal bowel movement.  In cases where there is an absence of an anal opening, your surgeon will create a new one.

The majority of PSARP procedures are performed when a baby is between one and six months old. The type of PSARP performed will depend on the position of the rectum and anus (whether it is low or high), how well the sphincter muscles work, and if there are any abnormal openings (called fistulas) that need to be repaired. For appropriate cases, pediatric surgeons at Children’s National Hospital use minimally invasive, laparoscopic techniques to help reduce the damage to surrounding structures and minimize pain post-surgery. 

Colostomy Closure

Your child’s colostomy will remain in place for approximately eight weeks following the reconstructive surgery to allow the rectum and anal opening to heal before coming in contact with any waste. When your child is ready and completely healed, the colostomy will be surgically closed. Within several days, they will begin passing stools on their own through the anus. At first, stools will be loose and occur frequently. To protect your child’s skin from diaper rash and irritation, safe topical treatments should be applied. As your child continues to heal, their stools will normalize, becoming firmer and less frequent.

Caring for Your Child After Reconstructive Surgery for an Anal Malformation

Learn how to care for your child after reconstructive surgery. Before performing any of steps from this video, consult your child's pediatrician.

Olivia Cleckley, MSN, RN, CPN, offers advice on colorectal care.

How will my child recover from surgery for anorectal malformation?

Recovery from anorectal reconstructive surgery will typically require two to four days in the hospital. For several months after surgery, anal dilations may need to be performed to help stretch the anus to the appropriate size for your child’s anatomy and age. Although surgery restores some function, the nerves and sphincter muscles your child relies on to let them know they need to go to the bathroom may be weak.

After surgery, some children continue to experience difficulty with constipation, soiling themselves and have problems potty training, but these can all be successfully managed using a bowel management program. If necessary, this program will help restore your child to maximum function and strengthen their muscle after surgery. To combat constipation, a combination of a healthy diet, plenty of fiber and laxatives (if needed) should be maintained. You should speak with your surgeon about what recovery will mean for your child.

Appointments

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Types of Malformations

    Rectovestibular fistula

    Rectovestibular Fistula
    In this malformation the urethra and vagina are normal, and the distal rectum ends in the vestibule, just behind the vagina.  This malformation is ideally suited for a perineal preserving PSARP, which moves the rectum to within the center of the sphincter and does not cut the perineal body.

    Variations in anorectal malformations in females

    Variations of the distal rectum
    1. Vestibular fistula
    2. Perineal fistula with the fistula exiting anterior to the sphincteric ellipse in the perineal body
    3. Perineal fistula with the fistula exiting within the sphincteric ellipse, at its most anterior extent
    4. Anal stenosis – a narrow opening at the center of the sphincteric ellipse
    5. A normal anus in size and located within the sphincteric ellipse, but anteriorly located with a smaller perineal body
     

    Anal stenosis in a female

    Rectoperineal fistula - Position 4
    The distal rectum exits as a fistula into the center of the sphincter mechanism.

    Rectoperineal fistula in a female 1

    Rectoperineal fistula - Position 2

    The distal rectum exits as a fistula into the perineal body.

    Rectoperineal fistula in a female 2

    Rectoperineal fistula - Position 3
    The distal rectum exits as a fistula into the perineal skin, but within the sphincter complex at its anteriormost extent.

    Rectal altresia-stenosis

    Rectal Altresia-Stenosis - Kay Update
    Anal stenosis is a rare malformation in which the anal opening is very small, but is located within the sphincter mechanism.  The anomaly may at first be missed on physical exam, and the patient only recognized when they present with constipation, due to the ineffective emptying via the small anal opening. This anorectal malformation is associated with a presacral mass, usually a teratoma.
    Chief of the Division of Colorectal & Pelvic Reconstruction Dr. Marc Levitt interacts with patient (baby).

    Colorectal Treatment at Children's National Hospital

    The Division of Pediatric Colorectal & Pelvic Reconstruction offers the latest advancements in diagnosis and treatment for all types of pediatric colorectal disorders. Discover more about the treatments we offer.

    Chief of the Division of Colorectal & Pelvic Reconstruction Dr. Marc Levitt interacts with patient (baby).
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    PATIENT STORY

    Stella's Story

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    Providers Who Treat Anorectal Malformations

    Departments that Treat Anorectal Malformations

    Dr. Marc Levitt treats baby colorectal patient accompanied by parents.

    Pediatric Colorectal & Pelvic Reconstruction

    The Division of Pediatric Colorectal & Pelvic Reconstruction offers the latest advancements in diagnosis and treatment for all types of pediatric colorectal disorders.

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    Bowel Management Program

    Learn more about our Bowel Management Program.

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    The Zickler Family Prenatal Pediatrics Institute

    The Zickler Family Prenatal Pediatrics Institute at Children's National Hospital in Washington, D.C., provides specialized care for babies during pregnancy, delivery and after birth.