Also known as: Intestinal Atresia (Esophageal, Duodenal, Jejunal, Ileal)

Related conditions: Esophageal Atresia, Duodenal Atresia, Jejunal Atresia, Ileal Atresia

1. Description of the problem

Intestinal atresia refers to a complete obstruction of a hollow viscus lumen and can occur at any location in the GI tract. It may be diagnosed prenatally on ultrasound, depending on the degree of obstruction and the gestational age at imaging.


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Clinical symptoms of intestinal atresia depend primarily on location of the atresia. If not a complete obstruction, symptoms may be delayed. Typical symptoms include:

Esophageal Atresia

   Non-bilious vomiting in newborn period

   Drooling

   Inability to feed

Duodenal Atresia

   Gastric distention

   Vomiting – may be bilious

Jejunal / Ileal Atresia

   Abdominal distention – the more distal the lesion, the more distended the abdomen

   Vomiting – may be bilious

2. Emergency Management

Stabilize the newborn.

Evaluate for intestinal atresia and associations with abdominal and other radiographs, consider contrast study.

Surgical management for atresia.

3. Diagnosis

The diagnosis of intestinal atresia includes both physical exam as well as radiographic studies with a focus on diagnosis and identification of associated abnormalities. Often in addition to plain films a contrast study may be necessary.

Esophageal atresia: Nasogastric tube unable to be passed and visualized at esophageal atresia site on abdominal x-ray

Associations: Tracheoesophageal fistula (TEF), VACTERL abnormalities (vertebral, anal, tracheoesophageal, renal, cardiac, limb defects).

Duodenal atresia: “Double bubble” on abdominal x-ray

Associations: Biliary atresia, agenesis of gall bladder, cardiac malformations, renal malformations, vertebral malformations, trisomy 21

Jejunal / Ileal atresia: Dilated loops of the small bowel with air-fluid levels

Associations: Cystic fibrosis, meconium ileus, microcolon.

Epidemiology

Intestinal atresia is often diagnosed prenatally. The incidence is unknown but the most common site of intestinal atresia is the jejunum/ileum, with approximately 1 in 5,000 births; duodenal atresia incidence is approximately 1 in 30,000 births, with a third associated with Down syndrome. The etiology of intestinal atresia is unknown but is believed to be heterogeneous, including a vascular event and/or abnormal epithelial-mesenchymal interactions.

Prognosis

Outcome of intestinal atresia is excellent following surgical repair and typically depends on associated medical conditions or other congenital abnormalities.