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Review Question - QID 106082

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QID 106082 (Type "106082" in App Search)
A 37-year-old-woman delivers a male infant with Down syndrome at 38 weeks. His Apgar scores at one and five minutes are both 8, with scores of 1 for acrocyanosis and irregular breathing. He passes meconium several hours later. Between 24 and 48 hours after birth, the infant has multiple episodes of vomiting despite lack of feedings and does not have additional bowel movements. Vomitus is bilious and nonbloody. On physical exam, the infant is crying, he appears jaundiced, and his abdomen is distended. An abdominal radiograph is performed, which is shown in Figure A. Which is the first step in management of this patient?
  • A

Initiation of phototherapy

0%

0/3

Abdominal ultrasound

0%

0/3

Endotracheal intubation

0%

0/3

Nasogastric tube placement

100%

3/3

Immediate surgical intervention

0%

0/3

  • A

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The clinical presentation and radiograph are consistent with duodenal atresia. Nasogastric or orogastric tube placement is the first step in management prior to surgical intervention.

Duodenal atresia is the congenital failure of the duodenal lumen to recanalize during fetal development. The condition is often seen in infants with Down syndrome and is associated with a number of congenital anomalies including biliary atresia, as well as cardiac and renal malformations. Duodenal atresia presents with vomiting 1-2 days after birth. Abdominal radiograph reveals the "double bubble" sign, caused by gastric and proximal duodenal dilation with narrowing at the pylorus.

Kimura and Loening-Baucke describe the management of infants with duodenal atresia. On diagnosis, intravenous fluids should be initiated and a nasogastric tube should be placed for gastric decompression. Surgery is not immediate and is typically performed between 24 and 48 hours after diagnosis. The standard procedure is a duodenoduodenostomy. With appropriate treatment, the condition has a very good prognosis.

Choundhry et al. describe the congenital abnormalities associated with duodenal atresia. Among the study cohort, 57% of infants with duodenal atresia had additional congenital anomalies. Down's syndrome and cardiac anomalies were most common, occurring in 46% and 31% respectively. Malrotation, bowel atresias, vertebral anomalies, tracheo-esophageal fistula, renal anomalies, VACTERL, and anorectal malformation were also found to occur at lower frequencies.

Figure A shows an abdominal radiograph with the "double bubble" sign, characteristic of duodenal atresia.

Incorrect Answers
Answer 1: Phototherapy is used for decreasing, circulating unconjugated bilirubin levels in management of neonatal jaundice.
Answer 2: An abdominal ultrasound would be performed if the infant's jaundice continues and there is reason to suspect an obstructive cause, such as biliary atresia. There is no role for abdominal ultrasound in the management of duodenal atresia, which is the more pressing issue.
Answer 3: Endotracheal intubation is not indicated, as the infant is not having respiratory distress.
Answer 5: The need for surgery is not immediate. Decompression with a nasogastric or orogastric tube and initiation of IV fluids should be preformed pre-operatively.

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