Imperforate Anus, Female, Total

Imperforate anus is a birth defect. The little girl was born without the opening where bowel movements, or stool, leave the body (anus). The opening may be missing. It could be in the wrong place. Or, it may be blocked. Stool normally passes through the large intestine (colon) and the last section of the colon (rectum) and then out through the anus. When that cannot happen, stool may back up in her body. Or, sometimes it leaves through another opening. Most of the time, an imperforate anus can be fixed with surgery.

CAUSES

The condition occurs because the fetus (unborn child) did not develop correctly in the womb. However, no one knows just why this happens. Often, babies born with this condition have other birth defects, too.

SYMPTOMS

  • No anus is visible.

  • The baby's belly (abdomen) is swollen.

  • The infant does not produce any stool in her first days of life.

  • Stool comes out through the opening of the vagina (a canal from the uterus, or the womb, to the outside).

DIAGNOSIS

To decide if a baby girl has an imperforate anus, her healthcare provider will need to:

  • Do a physical exam of the child.

  • Order abdominal X-rays. Ultrasound (using sound waves) may also be used.

  • If she does have an imperforate anus, additional X-rays and/or ultrasound studies may be ordered to look for other birth defects.

TREATMENT

  • Surgery will be needed. The goal is to create a normal rectum and anus for the child.

  • A new opening may be made. An old opening may be closed. Often, more than one operation is done.

  • The procedure will vary from child to child. Just what is done will depend on your child's condition.

  • If reconstructive surgery cannot be done right away, a temporary opening for stool to leave the body may be needed. This is called a colostomy. With a colostomy:

  • An opening from the colon to the outside is made through the abdomen. This allows stool to collect in a bag on the outside of the child's body.

  • The bag will stay in place for awhile even after she has reconstructive surgery for a new anus. The colostomy will be closed off once everything is working as it should.

PROGNOSIS

Surgery to correct imperforate anus is usually successful. However, some children may always have trouble controlling bowel movements.

HOME CARE INSTRUCTIONS

Surgery for imperforate anus can sometimes be done before a newborn leaves the hospital. If this cannot happen, a colostomy may be done in the meantime. Then, the child may be cared for at home while waiting for surgery.

  • Before the child leaves the hospital, you will be shown how to handle the colostomy. Ask for tips to help you deal with the bag and a baby who wriggles around a lot. Your caregiver will tell you:

  • How often the bag should be changed

  • How often it should be cleaned. What to use to clean it.

  • If the area on the child's body can get wet.

  • How the area should be cleaned.

  • Make sure the infant takes any medications her caregivers have prescribed. Follow the directions carefully.

  • Keep all follow-up appointments with the child's surgeon and other caregivers.

SEEK MEDICAL CARE IF:

  • The treated area does not heal. It might look red or bloody.

  • You have any questions about medications.

  • You have questions about caring for the colostomy.

  • The child develops a fever of 101° F (38.3° C) or higher.

SEEK IMMEDIATE MEDICAL CARE IF:

  • The child cannot produce stool.

  • She develops a fever of more than 102.0° F (38.9° C).