Duodenal Atresia

What is duodenal atresia?

Duodenal atresia is a condition that some babies are born with (congenital disorder). Babies with duodenal atresia have a closure in the first part of their small intestines (duodenum). The closure causes a mechanical blockage that prevents the passage of milk and digestive fluids.

What is the duodenum?

The duodenum is the first part of the small intestine. After food passes through the stomach, it enters the duodenum.

What is the difference between duodenal atresia and duodenal stenosis?

Duodenal atresia and duodenal stenosis are both congenital blockages of the small intestine.

Babies with duodenal atresia have a complete closure in the first part of their duodenum. Babies with duodenal stenosis have an unusually narrow duodenum.

How common is duodenal atresia?

Duodenal atresia and stenosis are both rare. Worldwide, only 1 in 7,500 to 1 in 40,000 babies are born with either condition. The conditions affect boys and girls equally.

Who might get duodenal atresia or duodenal stenosis?

Duodenal atresia and stenosis have not been found to be related to any environmental exposures. Duodenal atresia is more common in babies with certain genetic conditions.

What other conditions are associated with duodenal atresia?

Almost 1 in 3 duodenal atresia diagnoses occur in babies who also have Down syndrome. Around 1 in 6 babies with duodenal atresia or stenosis who do not have Down syndrome also have heart disease, but that number increases to 1 in 2 or 3 babies when they are found to have Down syndrome. Additional anomalies, including problems with the bones that make up the spine, the esophagus and airway, the kidneys, the arms or legs, other parts of the intestines, and the anus may occur in 15% or less of patients with duodenal atresia or stenosis.

What causes duodenal atresia?

Normally, during the fifth and six weeks of fetal development, the duodenum is a solid cord. Programmed death of cells in the center of the solid cord leads to the formation of the normal cavity (lumen) of the duodenum. Failure of that programmed cell death (or “apoptosis”) 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 the bowel or pressure from something outside the duodenum that pushes in and collapses the 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. A baby may vomit after the first feeding. Vomiting worsens if left untreated. In these cases, it is important to distinguish duodenal atresia from malrotation.

Babies may also experience:

• Low birth weight.
• Premature birth.
• Swelling in the upper abdomen (belly).

Patients with partial blockages of the duodenum may still present with vomiting in infancy if the blockage is bad enough. Less severe, partial blockages may also present later in childhood. Late presenting symptoms are more varied and include:

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

How is duodenal atresia diagnosed?

Healthcare providers may detect duodenal atresia before or after 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 the uterus. But a fetus with duodenal atresia or stenosis may not swallow and absorb amniotic fluid in normal 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 is not clear on ultrasound.

If duodenal atresia is suspected or diagnosed prenatally, it becomes important to look for chromosomal defects, such as those associated with Down syndrome. This can be done with amniocentesis or chorionic villus sampling.

• Amniocentesis: This is a test to check for genetic or chromosomal conditions, such as Down syndrome. The procedure involves analyzing a small sample of your amniotic fluid.

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

• Abdominal X-rays: This test looks for air or fluid in the stomach and first part of the intestine. Classically, for duodenal atresia, one will see an enlarged (dilated) stomach and duodenum without air further along in the intestines. This is called the “double-bubble” sign. For duodenal stenosis, partial duodenal obstruction or duodenal atresia with a rare bile duct malformation, air may still be seen in the intestines beyond the duodenum.
• Barium swallow tests: This test uses a safe amount of barium (white liquid that shows up 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 barium through a nasogastric tube (tube that runs through the nose to the upper GI tract). This test is most useful to define 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?

Duodenal atresia is repaired with surgery, but it is not an emergency. Evaluation for associated conditions 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.

Before surgery, babies with the condition cannot breastfeed or bottle-feed. Instead, they need parenteral nutrition (getting food through an IV or directly to the stomach). These babies will also have a tube placed into their stomach through either their nose or mouth to remove air and fluid that build up from the obstruction.

What are the types of duodenal atresia surgery?

Duodenal atresia is treated with a surgery called a duodenoduodenostomy. This is done by connecting the portions of the duodenum before and after the obstruction, effectively bypassing the obstruction.

Some types of duodenal blockages may need to be removed surgically. In rare cases, partial duodenal obstructions may be managed endoscopically with dilation. When the obstruction is caused by compression from something outside of the duodenum, the surgical repair will depend on the problem identified by your surgeon.

What is the recovery process after duodenal atresia surgery?

After surgery, babies recover in the neonatal intensive care unit (NICU). It may take a week or more until their intestines wake up and are ready to feed. During that time, a tube will be kept in place to decompress the 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 are able to take all their food by mouth and are gaining weight. Usually, babies stay in the hospital for two to three weeks. Length of hospital stay varies depending on whether or not other conditions have been diagnosed in association with the duodenal atresia.

Can you treat duodenal atresia before birth?

Healthcare providers cannot treat duodenal atresia until after a baby is born. However, 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 excess 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 decrease age at diagnosis and surgery, reduce the time until the baby is able to tolerate full feeds by mouth, reduce hospital length of stay and avoid common problems with fluid and electrolyte management.

How can I prevent duodenal atresia?

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

What is the outlook for babies with duodenal atresia?

For babies who do not receive treatment for duodenal atresia, it can be deadly. Babies who have surgery to correct the condition typically have excellent outcomes.

Some babies have other conditions associated with duodenal atresia, such as 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 else should I ask my doctor?

You may also want to ask your healthcare provider:

• What is my baby’s treatment plan?
• 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 will have another child with duodenal atresia?

A note from Cleveland Clinic

In duodenal atresia, babies have a blockage in the first part of their small intestine. That blockage prevents milk and intestinal fluids from traveling beyond the first part of the small intestine. Babies born with duodenal atresia usually vomit after their first feeding. The treatment for duodenal atresia is surgery to bypass or remove the obstruction. Babies who receive timely surgical treatment typically experience excellent outcomes.