Duodenal Atresia

INTRODUCTION:
  • It is thought to represent a failure of recanalisation of the bowel lumen that is a solid tube early in fetal life (~11-12 weeks).
  • Approximately 20–40 percent of all infants with duodenal atresia have Down syndrome.
  • During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios.
  • After birth, duodenal atresia may cause abdominal distension, especially of the upper abdomen.
  • Bilious vomiting commonly occurs within the first day of life.
  • Duodenal atresia (DA) is thought to occur as a result of failure of vacuolization of the duodenum from its solid cord stage. 
  • The range of anatomic variants includes duodenal stenosis causing constipation , mucosal web with intact muscular wall (so-called windsock deformity); two ends separated by a fibrous cord, or complete separation with a gap within the duodenum.
  • In most cases, the duodenal obstruction is distal to the ampulla of Vater, and infants present with bilious emesis in the neonatal period. In patients with a mucosal web, the symptoms of postprandial emesis may occur later in life.
ASSOCIATED ANOMALIES:
  • Prematurity
  • Down syndrome
  • Maternal polyhydramnios
  • Malrotation
  • Annular pancreas
  • Biliary atresia.
  • Other anomalies, such as cardiac, renal, esophageal, and anorectal anomalies, are also common. 
DIAGNOSIS:
  • The diagnosis of duodenal atresia is usually confirmed by radiography.
  1. An X-ray of the abdomen shows two large air filled spaces, the so-called "double bubble" sign. 
  2. The air is trapped in the stomach and proximal duodenum, which are separated by the pyloric sphincter, creating the appearance of two bubbles visible on x-ray.
TREATMENT:
  • Early treatment includes removing fluids from the stomach via a nasogastric tube, and providing fluids intravenously. 
  • The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy), which may be performed openly or laparoscopically
Exam Question
  • Treatment of duodenal atresia is Duodenoduodenostomy
  • Double bubble sign is seen in Duodenal atresia
  • Anomaly associated with duodenal atresia is Down syndrome
  • A newborn presenting with intestinal obstruction and constipation showed, on abdominal X ray, multiple air fluid levels is suggestive of Duodenal atresia
  • Most common cause of Acute Intestinal Obstruction in neonates is Duodenal atresia
  • Commonest cause of intestinal obstruction in down's syndrome is Duodenal atresia
  • Antenatal double bubble appearance on ultrasound is due to Duodenal atresia

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