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Digestive and Gastrointestinal Conditions

Imperforate Anus and Anorectal Malformations

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What Is Imperforate Anus?

Imperforate anus is a problem with the way a baby's anus or rectum formed. The rectum is the end of the large intestine, where stools (feces) are stored until they leave the body in bowel movements. The anus is the opening that stools pass through as they leave the body.

Imperforate anus is when there is no opening at the end of the digestive tract where the anus normally is. Imperforate anus can take several forms. The digestive tract may end in a closed pouch somewhere inside the body. Or, the rectum may connect to other parts of the body through a channel called a fistula. For example, in boys the rectum may connect through a channel to the urethra, bladder or scrotum. In girls it may connect to the vagina or bladder. Sometimes the channel comes out in front of where the anus would normally be. In other cases, a baby has an anus, but it is too narrow to allow waste to come out.

Children with imperforate anus may have other problems that are associated with the condition. These may include problems with:

  • The bones that make up the spine (vertebrae)
  • The lowest part of the spine, just above the tailbone (sacrum)
  • The windpipe (trachea)
  • The tube that connects the mouth to the stomach (esophagus)
  • Heart
  • Arms and legs
  • Kidneys

When a baby has more than two of these associated problems, doctors say they have VACTRL association (V=vertebral, A=imperforate anus, C=cardiac, T=tracheoesophageal fistula, R=renal, L=limb).

In some babies with imperforate anus, the muscles and nerves that control how the anus works do not form properly.

Girls may have a related condition called cloaca (pronounced cloh-AIK-uh). This means the rectum, bladder and vagina do not form properly. Normally, all three of these organs have separate openings to the outside of the body. With cloaca, they share a single, large opening.

Imperforate Anus in Children

Children with imperforate anus and related anal or rectal problems are born with the conditions. Imperforate anus occurs while the baby is forming inside the mother. Doctors do not know the cause. It affects about 1 in every 5,000 babies.

Imperforate Anus and Anorectal Malformations at Seattle Children’s

We have treated many children with anal or rectal malformations. Each year, we see 15 to 25 new patients with the conditions. In our surgery clinics, we care for and monitor children who have had surgeries to repair the problems. Our surgeons have experience with the surgeries these children need. They have treated children with many different kinds of problems, from the least complex to the most complex cases. Our Reconstructive Pelvic Medicine Clinic brings together many healthcare providers who work as a team to provide long term management. We also have a one-week Bowel Management Treatment Program for children who have trouble with bowel control after surgery.

When you come to Seattle Children's, you have a team of people to care for your child before, during and after surgery. Along with your child's surgeon, you are connected with doctors trained to care for new babies with complex problems (neonatologists), nurses, dietitians, child life specialists, social workers and others. We work together to meet all of your child's health needs and help your family through this experience.

Since 1907, Seattle Children's has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.

Who Treats This at Seattle Children's?

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Spring 2014: Good Growing Newsletter

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