Imperforate Anus and Anorectal Malformations

Anorectal malformations vary greatly from child to child. How severe the problem is depends on:

• How far your baby’s digestive tract extends. If the rectum reaches nearly to the normal site of the anus, the problem may be easier to fix.
• How much the nearby muscles are affected.
• Whether a channel connects the rectum to the urinary tract in boys or to the genitals in girls. If it does, surgeons will need to repair the channel.

Some babies with imperforate anus may have problems with their:

• Spine, just above the tailbone
• Heart
• Windpipe (trachea)
• Tube that connects the mouth to the stomach (esophagus)
• Kidneys and bladder
• Arms and legs

When a baby has more than 2 of these problems, doctors say they have VACTERL association. Each letter stands for a possible problem:

• V = vertebral, problems with the bones in the spine
• A = imperforate anus
• C = cardiac, problems with the heart
• TE = tracheoesophageal fistula and esophageal atresia
• R = renal (kidney) problems
• L = limb, problems with the bones in the arms

Girls may have a related problem called cloaca (kloh-AY-kuh). This means the rectum, bladder and vagina do not form right. They share a single opening to the outside of the body instead of each organ having its own opening.

Most of the time, doctors find imperforate anus when they examine a baby right after birth. Often, a physical exam is enough to make the diagnosis. An exam also helps tell how severe it is.

Doctors check:

• The area around your baby’s bottom and genitals for signs of problems
• To see if they can find your baby’s first poop (meconium) and tell where it is located

Your baby’s doctor may ask for some imaging tests: 

• X-ray of the belly and lower spine (sacrum)
• Ultrasound 

These tests can show important details, such as where the rectum ends and whether it connects to other body structures. The tests are important for planning the right surgery and care for your child.

If your baby is diagnosed with imperforate anus, they will need other tests later to check for problems with their spine, heart and kidneys. In girls, doctors will check for cloaca, a related problem where the rectum, bladder and vagina do not form properly.

A baby who has no anus will not be able to eat or pass poop. Babies born with an imperforate anus need treatment soon after birth. Our transport team is very experienced in safely moving babies to Seattle Children’s from their birth hospital. In our Level IV Neonatal Intensive Care Unit (NICU), your baby will receive the highest level of care.

Before surgery, your baby’s doctors will:

• Give your baby fluids and nutrition through an intravenous (IV) line into their vein.
• Insert a tube through your baby’s nose into their stomach (called a nasogastric tube or NG tube). This tube helps keep air out of the stomach so your baby is less likely to vomit.

Your baby will need surgery so poop can leave their body properly. The type and number of surgeries depend on your baby’s needs. 

• If your baby’s rectum ends very near the normal site of the anus, your baby may need only 1 operation within their first few days of life.
• Surgery may be more complex if the rectum ends higher.
• Your baby may also need surgery to repair any channels that connect their rectum to other body structures, such as the urinary or genital tract. In this case, your baby will need other operations before anal repair.

Surgery for imperforate anus has 3 goals:

• Create an anus in the normal place (anoplasty).
• Detach the rectum from other structures and repair these structures, if needed.
• Pull the rectum down to the new anus and connect it.

We will explain what type of surgery your baby needs, how long it will take and the number of procedures your child will need.

• Often the surgeon can operate through your child’s anus and not have to cut into their body.
• Some children have laparoscopic surgery, using very tiny cuts in the belly.
• Some children need open surgery, using larger cuts.

We will give your child medicine to make them sleep without pain during the surgery (general anesthesia). The doctors at Seattle Children’s who give your child anesthesia are board certified, with extra years of training to treat children.

Some babies need time to grow before they can have surgery to fix a complex malformation. Waiting makes surgery easier and safer. While waiting, these babies need a way for poop to leave their bodies.

Surgeons do a procedure called a colostomy (kuh-LOSS-tuh-mee). They:

• Make 2 small openings in your child’s belly wall. Each opening is called a stoma.
• Cut through the bowel.
• Attach the upper end of the bowel to 1 opening. This lets poop pass out of the body into a pouch on the outside.
• Attach the lower end of the bowel to the other opening. This opening lets out normal mucus made in the bowel.

If your child needs a colostomy, we will teach you to care for the stoma and change the pouch. Most of the time, the colostomy is temporary.

When your baby is doing better, the surgeon will connect the 2 segments of the bowel to each other and close the openings in your baby’s belly. Often this happens when a baby is 8 to 12 weeks old.

After surgery for imperforate anus, we will give your child pain medicine for their comfort. Most of the time, children who do not have other health problems stay in the hospital 5 to 7 days. Babies with more complex problems stay longer. Your baby will be ready to go home when they can feed well, gain enough weight and poop through their new anus or a colostomy.

When your baby is ready to go home, our nurses will help set up any supplies you need. You will have plenty of time to practice all your baby’s care before going home.

After surgery, many babies need a tube that passes through their nose to empty their stomach so they don’t vomit (nasogastric tube). How soon your child can feed by mouth after surgery depends on the type of procedure they had. We will explain your child’s eating plan and keep a careful watch to be sure your baby is growing well.

Our nurses and lactation specialists will do all that we can to support you in giving your baby breastmilk/chest. To keep your milk flowing and to build a milk supply for your baby, we help you with pumping and storing your milk. When your baby is ready, our nurses and feeding therapists help with the transition to feeding by bottle or breast/chest.

Starting 2 to 3 weeks after surgery, the surgeon will see your child for many follow-up visits to make sure your child is healing and feeding well.

At the first follow-up visit, the surgeon will perform anorectal dilation.

• The surgeon passes a very small, smooth rod into your baby’s anus to gently stretch the opening.
• This keeps your baby’s new anus from getting too narrow as it heals.
• It helps prepare for surgery to close the colostomy, if your child had one.
• Your baby will need dilation each day for many weeks.
• We will teach you and make sure you are comfortable with how to do it at home.

Seattle Children’s provides follow-up care to watch your child’s health, prevent problems and manage symptoms. Our goal is to follow your child until they reach college age to help ensure they are healthy and enjoy a good quality of life.

At regular follow-up visits, the Reconstructive Pelvic Medicine team checks your child’s health. If your child is doing well, these visits may happen only once a year. If your child is having any bowel or other problems, we may see them more often for a while for treatment.

Some children born with anorectal malformations also have problems affecting their urinary tract, genitals, spine, heart or kidneys. Depending on your child’s needs, they may see specialists in the digestive system (gastroenterologists), urinary tract (urologists), female reproductive system (gynecologists) and others.

After surgery and dilation, some children develop good bowel control. But it is common to have some bowel problems off and on throughout their lives. These include constipation or soiling accidents (fecal incontinence). Some children have poor bowel control because of:

• The type of malformation
• Problems with their spine
• Weak pelvic muscles

For children born with imperforate anus, bowel problems are not treated the same as for other children. This is why it’s important to get long-term care from experts. We will partner with you to make sure your child or teen feels safe, understands what is happening and helps make choices about their care in a way that fits their age.

We offer a range of diagnosis and treatment options, such as:

• A full range of motility studies to diagnose and treat children who have problems with how food moves through the digestive tract. Seattle Children's has the only pediatric motility program in the Pacific Northwest.
• Medicines and changes to your child’s diet, activity level and toilet habits to reduce constipation or fecal incontinence.
• Using biofeedback to help your child learn to use their new anus.
• Using devices to stimulate the nerves in the bowels.
• Taking part in our 1-week Bowel Management Treatment Program for children who are old enough for toilet training.
• Having another surgery to improve bowel control, if needed.

Our treatment approach is tailored to the needs of your child and family.

Care for imperforate anus requires knowledge and skill to diagnose the problem, perform complex surgery and provide follow-up care to keep your child healthy for life. Seattle Children’s has this expertise.

• Our Reconstructive Pelvic Medicine (RPM) Program brings together providers from many specialties to treat your whole child. Team members work closely with each other and with you to create the best treatment plan for your child.
• We have all the experts you need. Your child’s team may include a specialist in newborn care (neonatologist), surgeon, urologist, gynecologist, gastroenterologist, dietitian, pathologist or radiologist.
• Our surgery team is very experienced in repairing anorectal malformations.
• Seattle Children’s is part of a worldwide group that does research to improve care for children with disorders like anorectal malformations. Read about the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC).

Many of the children we treat come to us as newborns. We also see children, teens and young adults for diagnosis, surgery and follow-up care.

Often, newborns are referred from their birth center because a malformation is seen right away. Our transport team of physicians, nurses and respiratory therapists are national leaders in safely moving fragile babies. Seattle Children’s has the state’s first Level IV Neonatal Intensive Care Unit (NICU), the highest level of care. We are skilled and equipped to care for the sickest newborns.

We also see many babies and children at any age who:

• Have symptoms but have not been diagnosed yet because their problem is not obvious
• Have been diagnosed and need surgery
• Had surgery somewhere else and want to change doctors
• Want a second opinion about the best treatment

• At Seattle Children’s, you have a full team behind you before, during and after surgery. We work together to meet all your child’s health needs.
• We take time to explain your child’s condition and help you fully understand your treatment options.
• These problems can be uncomfortable and embarrassing for your child and upsetting for you. We partner with you to make sure your child or teen feels safe and understands what is happening. As your child gets older, we include them in decisions about their care.
• Our doctors, nurses, child life specialists and social workers help your family through the challenges of this condition. We connect you to community resources and support groups.
• We work with many children and families from around the Northwest and beyond. We can help with financial counseling, schooling for children in the family, housing, transportation, interpreter services and spiritual care. Learn about our services for patients and families.

After surgery, Seattle Children’s provides follow-up care to watch over your child’s health, prevent problems, manage symptoms and help your child have the best quality of life.

Seattle Children’s has programs focused on problems that some children have after surgery. These include:

• Bowel Management Treatment Program to improve bowel control. Having a plan to prevent soiling accidents helps children be more active, social, happy and independent.
• The only pediatric Motility Program in the Pacific Northwest, for children who have problems with how food moves through their digestive tract.