Duodenal Atresia

Condition: Duodenal Atresia/Stenosis

Overview ("What is it?")

  • Definition
    • The duodenum is the first portion of the intestine that comes right after the stomach. The duodenum is where the bile and pancreatic juice drains.
      • Atresia:  A medical term used to describe a condition where an opening or passage in the body is closed or absent. Duodenal atresia means that there is a blockage between one part of the duodenum to another. There are several types of blockages (Figure 1). There can be an internal layer causing the blockage or two discontinuous segments. Because of the blockage, the portion of duodenum before the blockage is enlarged (dilated) while the part after the blockage is normal or smaller than normal.
      • Stenosis:  A medical term that describes a partial narrowing of an opening or passage. In duodenal stenosis, the narrowing causes slowing of flow of food and fluids through the duodenum.
  • Epidemiology:
    • Duodenal atresia is the most common cause of intrinsic duodenal obstruction in the newborn. It occurs in 1 in 2,500-5,000 live births. In 25-40% of cases, this condition is associated in someone with trisomy 21 (Down syndrome).
    • Probably caused by several factors including genetic and developmental factors.
duodenal atresia
Fig. 1. http://affinity-health.adam.com/graphics/images/en/8940.jpg
http://www.yoursurgery.com/procedures/gia/images/DuodAtresia.jpg

Signs and Symptoms (What symptoms will my child have?")

  • Early signs (Duodenal atresia)
    • Often, duodenal atresia is seen on ultrasound performed on a pregnant mother to check on her baby. Because there is blockage of the duodenum, the fluid that the baby swallows has nowhere to go, resulting in enlargement of the stomach and the duodenum
    • After delivery, the newborn will often start vomiting within a few hours of birth and not tolerate feeds.
      • Most often babies will have bilious (green/yellow) emesis.
  • Later signs/symptoms:  Symptoms of duodenal stenosis happen later after birth.
    • Early on, infants may be able to tolerate some feedings
    • As the baby eats more, the food is hung up at the area of narrowing, leading to vomiting, and problems putting on weight.

Diagnosis ("What tests are done to find out what my child has?")

  • Labs and tests
    • Prenatal ultrasound shows dilated stomach and duodenum and too much fluid in the uterus
    • Abdominal X-ray—air in dilated stomach and duodenum
  • Most of the time, the plain X-ray of the abdomen is enough to make a diagnosis. However, in a patient with a narrowing (instead of complete blockage), other studies may be needed to confirm the diagnosis.
  • Upper Gastrointestinal (GI) Study:  Contrast is placed in stomach and duodenum to check the anatomy of these structures and the flow of food through the system.
  • EGD (Esophagogastroduodenoscopy):  A flexible telescope is placed in the mouth and is gently pushed through the esophagus, stomach and part of the small intestine. The doctor can see the inside of the esophagus, stomach and first part of the small intestine to look for obstruction.
  • If the baby has Trisomy 21, an echocardiogram is needed prior to surgery because there is an association of heart problems in these patients.
  • Conditions that mimic this condition
    • Malrotation (abnormal twist of the bowel)
    • Duodenal duplication
    • Blockage caused by organs compressing the duodenum such as annular pancreas (pancreas is abnormally shaped and encircles the duodenum), preduodenal portal vein (where the main vein entering the liver lies in front of the duodenum).

Treatment ("What will be done to make my child better?")

  • Medicine:  There is no medicine to treat the obstruction, only surgery.
  • Surgery
    • Preoperative preparation
      • Placement of tube (orogastric or nasogastric) in stomach to remove the fluid in the obstructed stomach and duodenum.The tube decreases the risk of vomiting and aspiration (vomit fluid gets in the lungs). 
      • Intravenous antibiotics to decrease infection risk.
    • Procedure
      • Usually performed once the baby has stabilized from delivery and all the information is known to undertake safe surgery.
      • Laparoscopic or open techniques may be used.
        • Laparoscopy is an approach where multiple small cuts are made on the belly to insert a telescope and small instruments.
        • Open technique is an approach were a larger cut is made on the belly to complete the surgery. It is either done up and down (vertical) or side to side (horizontal).
      • Surgeons will make sure that there are no other areas of atresia/stenosis in small and large intestine.
      • The surgeon will identify the area of blockage. The intestine before the blocked segment is sewn on to the intestine beyond the blockage, bypassing the obstruction.
      • If the dilated segment is really big, then it may need to be narrowed (duodenoplasty) so it can work better.
duodenal atresia
  • Postoperative care
    • Continue nasogastric (NG) tube to suction
    • The dilated stomach and duodenum usually take days and even weeks to function normally.
    • The baby usually needs IV (intravenous) nutrition (TPN) while the stomach and duodenum recover.
    • Feeding orally will start when there is only a little fluid that comes from the NG tube. Increasing feeds will be done gradually.
    • Medicines for pain such as acetaminophen (Tylenol) or ibuprofen (Motrin or Advil) or something stronger like a narcotic may be needed to help with pain for a few days after surgery.
  • Risks
    • Leak from area where the duodenum is put back together
    • Narrowing of the bypass
    • Wound infection
    • Slow recovery of the stomach and dilated intestine
  • Benefits:  When the duodenum is put back together, the child is eventually able to eat.

Home Care ("What do I need to do once my child goes home?")

  • Diet:  Normal for age
  • Activity:  Normal for age. Ask surgeon when “tummy time” can resume
  • Wound care:  Can wash incision with soap and water. May not be able to submerge under water for about a week after surgery. By the time the baby is discharged, it should be fine to resume baths.
  • Medicines
    • Nothing specific for surgery
    • By the time the baby is discharged, pain medication is likely not needed.
  • What to call the doctor for
    • Redness, warmth, or drainage from incision
    • Vomiting
    • Fever
  • Follow-up care:  Your child should follow up with his or her surgeon 2-3 weeks after surgery to ensure proper postoperative healing. Later follow up may be needed if vomiting is a problem.

Long-Term Outcomes ("Are there future conditions to worry about?")

  • The dilated duodenum may have abnormal function and may not move well. May require reoperation with revision of intestinal connection and/or tapering of dilated duodenum
  • Reflux:  Usually treated medically
  • Late bowel obstruction from adhesions (scar tissue). Can be treated medically with NG tube but may need surgery to divide scar tissue
  • Survival is over 90%. Deaths are usually related to associated anomalies not duodenal atresia/stenosis.

Updated: 11/2016
Author: Grace Mak, MD
Editors: Patricia Lange, MD; Marjorie J. Arca, MD