What is duodenal atresia?

Atresia is the term used when a part of a body passage (like the bowel) is missing or does not form properly. The most common place for this to happen is in the first part of a baby’s bowel (the duodenum). This is called duodenal atresia. This means that the baby’s stomach is not connected to the rest of the baby’s bowel.

What causes duodenal atresia?

Duodenal atresia is a rare condition. However, unfortunately we do not know what causes it. It is not caused by something you have or have not done. Around 70% of babies with duodenal atresia may have structural problems affecting another part of their body such as their heart or another part of their gut.

In each cell of our body, the genetic information (DNA) is packaged into 46 chromosomes. Having an incorrect number of chromosomes (e.g. in Down’s Syndrome) or a change in the DNA (a gene) on one of these chromosomes can affect the way cells work and how organs in the body form. Around one third of babies with duodenal atresia may have an underlying genetic problem such as Down’s syndrome.

How do we find duodenal atresia?

Around 60% of babies with duodenal atresia will be identified during pregnancy. This is most commonly at the 20-week ultrasound scan (between 18-20+6 weeks) or if you have further scans later in pregnancy. It may be suspected if there is an increased amount of fluid around your baby.

If duodenal atresia is suspected, then you will be referred to a team of experts to help plan your care for this pregnancy (fetal medicine unit).

What tests and follow up will I be offered?

When you attend the fetal medicine unit, a specialist will ask you some questions about your pregnancy, any previous pregnancies, family history and your health. A second scan will be performed to confirm your baby has duodenal atresia. The specialist will also have a detailed look at the baby to look for any other structural problems. There will also be a specialist ultrasound assessment of your baby’s heart (fetal echocardiogram). The specialist will discuss with you the options for testing the genetics of baby by amniocentesis.

During your pregnancy you will have regular ultrasound scans to monitor the duodenal atresia and baby’s growth. It is common for babies to have an increased amount of amniotic fluid. This can increase the risk of going into labour early. The specialist may suggest an internal (vaginal) scan to assess the length of the neck of the womb (cervix). This can help assess the risk of early labour. Depending on how many weeks pregnant you are and the amount of fluid around baby, the specialist may discuss using a needle test to remove some fluid around baby to reduce the risk of early labour. 

We will generally recommend inducing your labour slightly before your due date, from 38 weeks if there are no other complications. It is safe for most women to aim for a vaginal delivery.

What is the treatment for duodenal atresia?

A specialist baby doctor (neonatologist) will be present at the time of the delivery of your baby. The baby will be cared for in the neonatal unit (NICU) after delivery. Your baby will have a line placed in their arm or through their umbilical cord to allow blood tests to be performed and to keep your baby hydrated. A tube will be placed through your baby’s nose down to their stomach (nasogastric or NG tube). Some babies may also require some support with their breathing.

Your baby will have an X-ray of their tummy performed a few hours after being born. If this confirms the diagnosis of a bowel atresia, then your baby will require an operation in the first few days of life. A specialist surgeon will assess your baby and discuss with you the options for treatment.  

Operations are performed with the baby asleep (under general anaesthetic). The operation involves a cut in the tummy and the bowel is joined around the missing or blocked area. Any treatments and operations will be explained to you in detail, and you will have the opportunity to ask questions.

After the operation, your baby will closely monitored in NICU or on the surgical ward. Feeding will gradually commence with breast or bottle milk through the nasogastric (NG) tube. It can take several weeks for the bowel to start working normally and babies will need feeding support through the line into their vein during this time. As they recover, lines will be removed, and they can start feeding normally.

Follow-up

Most children with duodenal atresia and no other problems will grow up to live normal lives. Some children will need ongoing support with their feeding.

Choices and support

Having a child diagnosed with a congenital anomaly can be a scary and difficult experience. Please don’t hesitate to chat to your medical team or GP who can let you know about the support available for you and your family.

Antenatal Results and Choices        https://www.arc-uk.org