Duodenal Atresia

What is duodenal atresia?

Duodenal atresia is a rare congenital (present at birth) digestive disorder in which your baby’s duodenum is blocked. The duodenum is the first part of your small intestine. After food passes through your stomach, it enters your duodenum.

In duodenal atresia, the blockage prevents the passage of milk and digestive fluids from your baby’s stomach into their small intestine to continue the digestive process. Babies born with duodenal atresia (pronounced “dew-oh-DEE-nul uh-TREE-zh-uh”) may also have other issues at birth, including heart defects or Down syndrome.

Duodenal atresia is a serious condition, but it’s treatable. It’s important that your baby receives care immediately. Without treatment, babies can’t eat. With treatment, the outcome for babies with the condition is excellent.

Types of duodenal atresia

There are three main types of duodenal atresia:

• Type 1 atresia: In this type, a thin membrane of mucosa blocks the duodenum, but the muscle coat is intact. This type is also called duodenal diaphragm.
• Type 2 atresia: In this type, the duodenum ends are separated but attached by a cord. This type is also called complete duodenal atresia.
• Type 3 atresia: In this type, the duodenum ends are separated, but there’s no tissue between them.

In addition to the three main types of duodenal atresia, some babies have a condition known as duodenal stenosis. Duodenal atresia and duodenal stenosis are both congenital blockages of the small intestine. Babies with duodenal atresia have a complete closure of their duodenum. Babies with duodenal stenosis have an unusually narrow duodenum.

What are the symptoms of duodenal atresia?

In the first few hours after birth, most babies with duodenal atresia experience vomiting. The vomit may be yellow, green or light brown. This typically occurs after a baby’s first feeding. Vomiting worsens if left untreated.

Babies may also experience:

• Low birth weight.
• Swelling in their upper abdomen (belly).
• Constipation.
• Jaundice.

Babies with partial blockages of their duodenum may still vomit during infancy if the blockage is bad enough. Less severe, partial blockages may also occur later in childhood. Duodenal stenosis symptoms that may occur later are more varied and include:

• Indigestion.
• Pain.
• Malabsorption.
• Abdominal mass.

What is the most common cause of duodenal atresia?

Typically, during the fifth and sixth weeks of fetal development, the duodenum is a solid cord. The cells in the center of the solid cord are programmed to die away, forming the hollow tube (lumen) of the duodenum. When that programmed cell death (or “apoptosis”) doesn’t happen, it leads to duodenal atresia.

Partial completion of the process results in duodenal stenosis or other types of blockages. A baby can also have duodenal obstruction from twists in their bowel or pressure from something outside their duodenum that pushes in and collapses it.

Who has a higher risk of getting duodenal atresia or duodenal stenosis?

Duodenal atresia is more common in babies with certain genetic conditions, like Down syndrome. Research doesn’t link any environmental exposures to duodenal atresia and stenosis.

How is duodenal atresia diagnosed?

Healthcare providers may detect duodenal atresia before or after birth.

Before birth (prenatally), providers may detect potential duodenal atresia using the following:

• Fetal ultrasounds: These tests can examine the fetal digestive tract. The presence of two “bubbles” representing the stomach and duodenum or just a large stomach bubble will raise concern for duodenal atresia or stenosis. The ultrasound may also show high levels of amniotic fluid (polyhydramnios). Typically, fetuses swallow and absorb some amniotic fluid in your uterus. But a fetus with duodenal atresia or stenosis may not swallow and absorb amniotic fluid in the usual amounts. This can result in excess amniotic fluid around the fetus.
• Fetal magnetic resonance imaging (MRI): This imaging test may show some similar findings to ultrasound, but it can be helpful in determining the level of the atresia if it’s not clear on ultrasound.

If your healthcare provider suspects or diagnoses duodenal atresia during fetal development, it becomes important to look for chromosomal differences, like those associated with Down syndrome. They can look for these differences with amniocentesis or chorionic villus sampling.

Even if providers suspect duodenal atresia before birth, they can’t verify the diagnosis until your baby is born. After your baby’s birth, healthcare providers may use:

• Abdominal X-rays: This test looks for air or fluid in your baby’s stomach and the first part of their intestine. Typically, for duodenal atresia, the provider will see an enlarged (dilated) stomach and duodenum without air further along in your baby’s intestines. This is called the “double bubble” sign. For duodenal stenosis, partial duodenal obstruction or duodenal atresia with a rare bile duct malformation, the provider may still see air in the intestines beyond the duodenum.
• Barium swallow tests: This test uses a safe amount of contrast (liquid that shows up brightly on X-rays) and fluoroscopy (using real-time X-rays to create a movie). Providers use this test to examine the upper gastrointestinal (GI) tract. Babies usually receive the contrast through a nasogastric tube (runs through their nose to their upper GI tract). This test is most useful to highlight partial duodenal obstructions or stenosis and to look for other causes of symptoms in the absence of a clear diagnosis of duodenal atresia.

How is duodenal atresia treated?

Healthcare providers repair duodenal atresia with surgery. Before surgery, your baby’s provider will evaluate them for associated conditions, which is important to optimize the timing of repair. Most babies born with duodenal atresia will receive surgery within two to three days of birth. Some babies may need other problems addressed before their duodenal atresia treatment.

Before surgery, babies with the condition can’t breastfeed (chestfeed) or bottle-feed. Instead, they get food through an IV (parenteral nutrition). A provider will also place a tube into their stomachs through either their noses or mouths to remove air and fluid that build up from the obstruction.

Duodenal atresia surgery

Providers treat duodenal atresia with a surgery called a duodeno-duodenostomy (pronounced “dew-ODD-den-oh-dew-oh-den-OST-oh-me”) or duodeno-jejunostomy. Your baby’s provider will connect the portions of their duodenum before and after the obstruction, effectively bypassing the obstruction.

In rare cases, they’ll manage partial duodenal obstructions endoscopically with dilation. When compression from something outside of their duodenum causes the obstruction, the surgical repair will depend on the problem identified by your surgeon.

In almost all cases, a provider can do this with minimally invasive surgery. This method uses micro-incisions (3 mm) and generally avoids open abdominal surgery (laparotomy).

What is the recovery process after duodenal atresia surgery?

After surgery, babies recover in a neonatal intensive care unit (NICU) or similar unit. It may take a week or more until their intestines wake up and are ready to feed. During that time, a tube will stay in place to decompress your baby’s stomach of any air and fluid. Some babies also need mechanical ventilation for a few days to help them breathe.

Babies can leave the hospital when they’re able to take all their food and are gaining weight. Usually, babies stay in the hospital for two to three weeks. Length of hospital stay varies depending on whether your baby’s provider diagnosed other conditions in association with the duodenal atresia and how well your baby responds to treatment.

Can you treat duodenal atresia before birth?

Healthcare providers can’t treat duodenal atresia until after a baby is born. But if they detect duodenal atresia before birth, they can take extra steps to lower the risk of birth complications.

For example, too much amniotic fluid buildup can increase the risk of preterm labor. If necessary, your provider may use amnioreduction. This procedure removes some amniotic fluid.

Your healthcare provider will also be able to offer prenatal counseling and suggest delivery at a facility that can care for your baby. This may:

• Lead to earlier diagnosis and treatment.
• Reduce the time until your baby can tolerate full feeds by mouth.
• Shorten hospital length of stay.
• Avoid common issues with fluid and electrolyte management.

How can I prevent duodenal atresia?

There’s no known way to prevent duodenal atresia. If your healthcare providers detect duodenal atresia during pregnancy, they’ll carefully monitor you to reduce the risks of complications.

What is the outlook for babies with duodenal atresia?

For babies who don’t receive treatment for duodenal atresia, it can be fatal. Babies who have surgery to correct the condition typically have excellent outcomes. Earlier diagnosis and treatment increase the odds of positive outcomes.

Some babies have other conditions associated with duodenal atresia, like Down syndrome. These babies may have other challenges, even after duodenal atresia surgery. Your baby’s care team can help you prepare and know what to expect.

What questions should I ask my baby’s healthcare provider?

You may want to ask your baby’s provider the following questions:

• What’s my baby’s treatment plan?
• Which type of surgery: minimally invasive or open surgery?
• Are there any risks associated with my baby’s treatment plan?
• What kind of follow-up care will my baby need?
• What signs of complications do I need to watch for after surgery?
• What are the chances I’ll have another child with duodenal atresia?

A note from Cleveland Clinic

While duodenal atresia sounds serious, it’s important to remember that it’s a treatable disorder. Many hospitals are well-equipped to diagnose and address it before or shortly after birth. Surgery to correct the blockage has a high success rate, and with proper care, most babies with duodenal atresia go on to live healthy lives. If you have concerns about duodenal atresia, talking to your healthcare provider can help ease your anxieties and guide you through the next steps.