My son was diagnosed with Duodenal Atresia a few months before he was born. For the next few months my wife and I spoke with doctors and researched the condition online. We learned that it is a surprisingly common problem, but there isn’t much information on what to expect. I hope to help explain what Duodenal Atresia is and what to expect as a parent during pregnancy and after birth. It has been a long and exhausting process, but I now have a beautiful, healthy baby boy – hopefully reading what I have learned and experienced will make things easier for you and your child.
What is Duodenal Atresia?
Duodenal Atresia is a condition where the beginning of the intestine, the duodenum, is not properly connected to the rest of the intestine. This can be anything from a complete disconnect to an almost perfect connection with a thin membrane blocking the path. No matter how it presents, the surgery to treat it is the same.
What can I expect soon after diagnosis?
Duodenal Atresia doesn’t have any harmful effects on your baby while it is still in the uterus. When the problem is diagnosed, there won’t be any rush for treatment – your doctor will want to let your pregnancy continue normally. The doctor will probably perform an amniocentesis or schedule one soon. Amniocentesis is the process of drawing out a sample of amniotic fluid for testing. It is performed with a long but narrow needle under an ultrasound. The needle is scary looking, and the process is a bit painful, but it isn’t much worse than most shots (at least not according to my wife).
The scariest part of the entire process is the next two weeks. The lab will perform a chromosomal analysis to check for certain genetic conditions. This is because duodenal atresia is a common symptom of Down’s Syndrome. About 30% of cases of duodenal atresia are caused by Down’s Syndrome. You’re going to worry about this, and there isn’t really anything that comforts it. Just try to remember that 70% of cases are not associated with Down’s. My son’s test came back negative, so unfortunately I can’t help prepare you if you get different results. The hospital should have a genetecist and counselors to help explain what to expect in that case.
Your doctor will also set up a surgical consult for you. Duodenal atresia does require surgery shortly after birth (more about that later – it isn’t as bad as you might think), and you will have a chance to discuss things with the surgeon. Duodenal atresia is a common enough problem that your doctor should be able to refer you to a surgeon that handles it on a regular basis. My surgeon claimed to see about a dozen cases a year.
What can I expect during pregnancy?
LOTS of ultrasounds. My wife was in for ultrasounds every two weeks, and towards the end of her pregnancy was in once or twice a week. This helps the doctor keep an eye on things to make sure your child is developing as expected and to see if there are any changes in the presentation. It is possible that there was a misdiagnosis, and further observations will help ensure that the problem is definitely duodenal atresia.
You can also expect a condition called polyhydramnios, which basically translates as “lots of fluid.” Your child can’t swallow properly, so the fluid isn’t being processed like it would be in a normal pregnancy. The amount of extra fluid varies case-by-case. My wife was one of the most extreme cases the doctors had seen – her uterine measurements were being compared to octomom! Polyhydramnios can cause extra discomfort and will wear you out sooner. If you are working, you may need to take leave sooner than expected.
The most serious side-effect of polyhydramnios is the chance of premature labor. Your body doesn’t know how long you have been pregnant, just how big your uterus is and how much pressure is in it. Because the uterus is larger, it is possible for labor to start sooner than normal. Part of your regular checkups and ultrasounds will be measuring your uterus and cervix. If your doctor thinks that you are at risk of coming early, he/she may prescribe drugs to reduce fluid production or even physically drain some fluid in a process similar to amniocentesis. Your doctor will be committed to keeping your child in as long as possible.
What can I expect after birth?
You should have a few minutes to see your child immediately after birth, but the nurses will want to get him/her to the NICU as quickly as possible. As soon as you have recovered enough to move to a wheelchair they will be happy to bring you to be with your child. It only took about an hour or two before my wife was ready, and the time moves surprisingly fast.
Once you get to the NICU, your child will already have some tubes and wires attached. Expect an IV because your child will have to rely on it for nutrition and hydration until after the surgery. Three electrodes to measure heart rate and breathing are standard. There will probably be an oxygen monitor strapped to your child’s foot. The most difficult of all is the drainage tube – it will be inserted into your child’s stomach through his/her mouth to drain the excess fluid and stomach acid that builds up before the blockage. You will be allowed to hold your child – the nurses will help make sure that the tubes and wires are moved safely. After a while you will probably get used to moving them out of the way yourself.
It is a great idea to visit the NICU during pregnancy. You can meet some of the staff and become familiar with the area and policies. This will help make the transition less stressful.
When is the Surgery?
The surgeon will want to wait a few days before the surgery. This will let your child gain some strength and get used to breathing on his own. Surgery on brand new babies can sometimes cause them to switch back to fetal breathing because of the respirator – waiting these few days eliminates the risk.
The surgery will take about two hours. With family or friends there to support you, this time goes by quickly. At this point you will have met with your surgeon enough to trust him/her to do a good job. The surgeon will make a slit on each end of the blockage, and connect the slits together. He/she will also run a feeding tube through the connection. Odds are your child’s appendix will also be removed during the surgery – it can be done through the same incision, and according to our surgeon the risks of removing it then are so low that your child is more likely to develop complications due to appendicitis later in life than to develop complications from the removal. The surgery will leave a tiny scar. The bandage still hasn’t fallen off of my son, so I can’t tell you much about how it looks.
What can I expect after the surgery?
When you get back to the NICU, your child will be on a respirator. This isn’t because he/she needs it to breathe, but because the transition is easier once your child wakes up. This may be different in your case – our hospital has a tunnel with poor ventilation between the OR and NICU, so the respirator was needed during that run as a precaution.
Your child will have one more tube – a narrow feeding tube. In our case, it was inserted through our son’s nose.
Within a day or two your child’s IV will be replaced with a more nutritious fluid, and lipids (fat) will be added. Small feedings will be performed via the feeding tube. Pumped breast milk can be used. Our NICU had a pump that mothers could borrow – each mom was provided with their own set of of cups to keep, so it isn’t like using a used pump. Once the fluid stops building up in your child’s stomach, the drainage tube will be removed. As your child starts taking more food through the tube, the IV will be turned down.
Eventually you child’s surgery will heal to the point where you can begin small oral feedings. Unfortunately, because your child could not suckle for the first several days, you may have trouble breastfeeding. There will be specialists available to help teach you and your child proper technique. It can be frustrating, but eventually things get better. Once your child is taking all of his/her feedings orally, you should be able to take him/her home!
What can I expect long-term?
You will have a few more appointments scheduled to make sure things are healing right, but then you’re done for good! Your child will lead a perfectly normal life with no special dietary restrictions.
All of this information is from doctors and surgeons we worked with at Bronson Methodist Hospital in Kalamazoo, MI, and from my own personal experience.