Duodenal Atresia: Diagnosis and Treatment

How Is Duodenal Atresia Diagnosed in Babies?

Duodenal atresia can usually be diagnosed during pregnancy at the second trimester survey ultrasound. An obstetrician (doctor who specializes in pregnancy, childbirth and women’s reproductive health) can also diagnosis duodenal atresia if the mother develops polyhydramnios. This can be seen on an ultrasound or if the mother’s belly shows measurements that are larger than expected. If the measurements are larger than expected, there also is also a higher chance of premature delivery.

The clearest sign of duodenal atresia during prenatal ultrasound is the double bubble sign. This is when doctors can see 2 bubbles in your baby’s abdomen (belly area). These bubbles are the enlarged, fluid-filled stomach and duodenum that show that there is an obstruction in the duodenum.

While the double bubble may be enough to diagnose a complete blockage, your baby might need more testing to check for narrowing of the duodenum. Obstetricians may recommend the following additional tests:

• Amniocentesis. This is when doctors remove a small sample of fluid from the amniotic sac and check it for genetic conditions, such as trisomy 21.
• Echocardiogram. This is an ultrasound of your baby’s heart. The ultrasound checks for other anomalies (unusual medical concerns). This is especially important if your baby has Down syndrome because they are more likely to have other birth defects in the heart.

Doctors can also evaluate for duodenal atresia after birth through one or both of the following tests:

• Upper gastrointestinal study. This is a test in which doctors check how well food and fluid flows your baby’s stomach into the intestines. Doctors check this by giving contrast dye to drink and watching it move through your baby’s stomach and checking how it flows into the intestines.
• Esophagogastroduodenoscopy (EDG). This is when a doctor checks for an obstruction by placing a small, flexible telescope in the mouth that goes down to into the stomach and into the small intestine.

How Is Duodenal Atresia Treated in Babies?

There are no treatments for duodenal atresia at the prenatal stage (before your baby is born). Duodenal atresia can only be treated with surgery to repair the connection between the stomach and the intestines. Surgery is usually done 1-3 days after birth.

It is important to create a plan with doctors to make sure you and your baby get the care you need before and after birth. This plan is created by your care team within the Fetal Care Program at Mass General for Children (MGfC).

Who Is on My Baby’s Care Team?

Your team is specialized in providing individual attention to you and your baby’s needs. This team works together and with your family to design and carry out your and your baby’s medical care. 

The first members you meet are the program coordinators, who will help you and your family before, during and after birth, and at your baby’s appointments.

Other members of your team include:

• Your obstetrician or maternal fetal medicine provider, who checks for signs of polyhydramnios or the need for an amnioreduction to remove excess fluid and ease the swelling in the mother’s body, if needed
• Pediatric surgeon, who will monitor your baby for other health concerns and fix the blockage after your baby is born.
• Pediatric cardiologist (heart doctor)
• Neonatologist (doctor who cares for newborns in the Neonatal Intensive Care Unit (NICU) and NICU nurses. The team will work together and be responsible for designing and carrying out your and your baby’s complete plan.

How Does Duodenal Atresia Affect My Baby’s Birth?

In most cases, your baby can be born naturally at their due date without any additional problems. If the mother develops polyhydramnios, baby may be delivered early. If you develop polyhydramnios, the doctor will talk with you about an early deliver plan, if needed.

What Happens After Birth?

After birth, your baby will be admitted to the neonatal intensive care unit. There, they will be examined by the neonatologist and pediatric surgeon. The care team will order more imaging tests and plan the next steps in your baby’s care. 

Babies with duodenal atresia can usually breathe on their own, but they cannot nurse or take a bottle before the surgery because of the blockage. At first, eating will be a challenge. As your baby heals, the surgeons will allow your baby to increase the volume of milk slowly until they reach the full amount they need to grow. During this time, the care recommends that mothers store their breastmilk until your baby is ready to eat by mouth.

What Can I Expect Before, During and After Surgery?

Before Surgery

• Once your baby is diagnosed, there is no medicine to treat duodenal atresia but the surgeons will do surgery to correct the blockage so your baby can eat.
• Doctors will place a small drainage tube in your baby’s stomach to remove fluids. This helps prevent your baby from vomiting their stomach contents.

During Surgery

• Surgeons will fix the blocked intestine so that the duodenum can begin to work properly. The surgery may be done with laparoscopic techniques (with a tiny camera and instruments). This way, the incisions on your baby’s belly are small and the recovery may be faster.
• If the portion of intestine affected by duodenal atresia is large, the surgeon may also want to do a duodenoplasty (a procedure to make the tube the right size). This helps make sure the duodenum will work properly.

After Surgery

• Because the surgery is performed under general anesthesia, your baby will return to the NICU after. Sometimes, babies need a ventilator to help with breathing at first. They are usually able to be taken off the ventilator quickly. 
• It usually takes about 2-3 days to start feedings, but it may take as long as 7-14 days for the duodenum to work properly after surgery. In the meantime, your baby will receive all necessary calories and nutrients through an IV. Your baby will come off IV nutrition gradually. 
• Even when your baby's intestines work properly, it may take several weeks before your baby can digest all the good they need to grow on their own. 
• Your baby can be discharged once they gain weight and are feeding without difficulty.  

What Are the Risks of Duodenal Atresia Surgery?

Risks of duodenal atresia surgery can include:

• Infection inside the abdomen
• Internal bleeding
• Reflux (acid reflux or heartburn)
• Leaking from the repaired bowel
• Occasionally, if the duodenum does not work properly after surgery, your baby may need some medication to help with digestion

Many babies have slow recovery of digestion after, but they normally recover in a few weeks. Occasionally babies will have slow digestion for longer than a few weeks and may need medication to help.

How Should I Care for My Baby After Surgery?

Your baby’s care team will give you more detailed instructions on how to care for your baby after surgery. You can also follow these tips once you go home:

• Wash the incisions with soap and water
• Do not soak or submerge your baby’s wounds under water for one week after surgery.
• By the time your baby goes home, it is unlikely that they will have pain from the incision. If so, Tylenol® should work well for pain relief.

When Should I Call the Doctor?

Call the doctor if your baby is recovering at home and has a fever, is vomiting, or has drainage from incision.

When Should I Schedule My Baby’s Follow-Up Appointment?

Schedule your baby’s follow-up appointment a few weeks after surgery to make sure they are healing and recovering well. Schedule the appointments by calling the Fetal Care Program at 857-238-2273.