Duodenal atresia is a condition in which the first part of the small bowel (the duodenum) has not developed properly. It is not open and cannot allow the passage of stomach contents.
Causes, incidence, and risk factors
The cause of duodenal atresia is unknown, but it is thought to result from problems during an embryo's development in which the duodenum does not normally change from a solid to a tube-like structure.
Duodenal atresia is seen in more than 1 in 10,000 live births. Approximately 20-30% of infants with duodenal atresia have Down syndrome. Duodenal atresia is often associated with other birth defects.
Symptoms
Upper abdominal swelling may or may not be present
Early vomiting of large amounts, which may be greenish (containing bile)
Continued vomiting even when infant has not been fed for several hours
Absent urination after first few voidings
Absent bowel movements after first few meconium stools
Signs and tests
A fetal ultrasound may show excessive amounts of amniotic fluid in the womb, a condition calledpolyhydramnios. It may also show swelling of the baby's stomach and part of the duodenum.
An abdominal x-ray may show air in the stomach and first part of duodenum, with no air beyond that. This is known as the double-bubble sign.
Treatment
A tube is placed to decompress the stomach. Dehydration and electrolyte abnormalities are corrected by providing fluids through an intravenous tube. An evaluation for other congenital anomalies should be performed.
Surgery to correct the duodenal blockage is necessary, but is not an emergency. The surgical approach will depend on the nature of the abnormality. Associated problems (such as those related to Down syndrome) must be treated as appropriate.
Expectations (prognosis)
Recovery from the duodenal atresia is expected after treatment. Untreated, the condition is deadly.
Complications
Other birth defects
After surgery, there may be late complications such as:
Swelling of the first part of the small bowel (megaduodenum)
Problems with movement through the intestines
Gastroesophageal reflux
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