What is duodenal atresia?
Duodenal atresia is a problem with the development of a baby's duodenum, the part of the small intestine that connects the stomach to the rest of the intestine. Instead of an open tube into the intestine, a baby with duodenal atresia has a blockage or gap between the duodenum and intestine. This prevents food and fluid from going to the small intestine and forces it back upwards instead.
Causes of duodenal atresia
The cause of duodenal atresia is unknown. During pregnancy, your baby’s duodenum normally changes from a solid form to a tube (recanalization). Duodenal atresia occurs when recanalization does not completely happen.
Duodenal atresia is present in more than one in 5,000 live births. About one-third of infants with duodenal atresia may have Trisomy 21 (Down syndrome) as well as other congenital differences. Your doctor may recommend additional testing, such as amniocentesis or genetic testing, to identify any other areas of concern.
Types of duodenal atresia
Duodenal atresia can be partial or complete. In a partial atresia, the area is narrowed so much that nothing can pass through. In a complete atresia, there is a section missing from the duodenum, which creates a gap between the stomach and intestines where they should be joined. In either case, the blocked part of the duodenum is called the atretic area.
Signs and symptoms of duodenal atresia
During pregnancy, duodenal atresia causes extra amniotic fluid to build up. This is known as polyhydramnios. This is because your baby cannot get the fluid from the stomach to the intestine. An ultrasound during pregnancy as well as an X-ray after birth shows a "double bubble." This is caused by fluid and air in your baby’s stomach and duodenum, where it gets trapped rather than moving on to the intestine.
A baby born with duodenal atresia may have a stomach area that appears to be normal, or they may have symptoms such as:
Stomach area swelling
Early vomiting that is often green
Continued vomiting even when the infant has not been fed
Little or no stooling (pooping)
Treating Duodenal Atresia
After birth, your care team will place a tube through your baby’s nose or mouth into the stomach and use suction to empty the contents. Then, they’ll do an abdominal X-ray to look for the "double bubble" and any other areas of concern. During this time, your baby will be fed by fluid through a vein (IV fluid). The medical team will do a complete exam and sometimes other tests like an ultrasound.
Surgery to correct the duodenal blockage is necessary, usually one or two days after birth. An operation known as a duodenostomy removes the blockage. The surgeon makes a cut in the right upper side of the stomach area. Sometimes this operation is done through very small incisions (minimally invasive technique). Then the surgeon finds the location of the blockage and opens the blockage. The surgeon will sew the two pieces of intestine together.
Recovery and prognosis after surgery
For babies with no other complications or related conditions, the prognosis after surgical repair of the atresia is excellent—around 95% survival rate.
Recovery from surgery usually takes about 5 to 10 days. The intestine needs that time to heal and be able to work again. During this time, your baby will be fed by vein and will receive pain medications as needed. Feedings are started when the intestine shows it is working. Signs of this include passing gas, having stools (pooping) and less output from the tube in your baby’s nose. If you are planning on breastfeeding, you can pump and freeze breast milk until your baby is able to start feedings.
The length of your baby’s stay in the NICU depends on how soon they can tolerate full feedings and gain weight. After discharge, a pediatrician will monitor your baby. Your baby also will come back to Children’s Mercy to see the pediatric surgeon or any other necessary specialists.
Choosing the best home for your child’s care
At Children’s Mercy, our skilled professionals can care for you and your baby before, during and after delivery. Our Fetal Health Center works with families like yours every day to help correct and prevent problems that happen during prenatal development.
You can choose to deliver your baby right in the Fetal Health Center’s Special Care Delivery unit, so that your baby can immediately receive care from our neonatal specialists while still remaining close by to you. Or you might deliver elsewhere and come to Children’s Mercy for a scheduled surgery after your baby is born.
Our team of pediatric surgeons was one of the first to apply minimally invasive surgical techniques (surgery through very small incisions) to the treatment of duodenal atresia. Our expertise in minimally invasive surgery means we are able to treat most babies with duodenal atresia using these specialized techniques to minimize pain and scars for your child. Our team of highly experienced specialists will follow your child as they grow to ensure that they are meeting their developmental milestones and nutritional requirements.
If your child has more complex medical needs, such as Down syndrome or a heart condition, we have all the specialists you may need in one place. In many cases, we can coordinate your appointments to make your visit as productive as possible.
Want a second opinion?
If you’ve recently received a diagnosis or would like a second opinion, experience matters. We're happy to share our expertise with you. Call (816) 855-1800 to make an appointment with our team.
Highest level of surgical care
Children’s Mercy is one of only 30 locations in the nation to be verified by the American College of Surgeons as a Level 1 Children’s Surgery Center.
Neonatal Follow-Up Clinics: Care for your growing child
We recognize that many of our NICU graduates have special health care needs related to growth and development. The Neonatal Follow-Up Clinics provide follow-up services for your babies after they're home from the NICU.