Fact-sheet: Duodenal atresia
Updated on 09/17/2021 at 12:16 PM
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Definition
Definition: embryopathy, complete (atresia) or incomplete (stenosis) absence of the duodenal lumen.
-Sporadic form++, autosomal dominant transmission suggested in some families
-Isolated in 30 to 52% of cases
-Associated malformations: Trisomy 21+++ (20 to 30%), cardiac
anomaly (25%), musculoskeletal, urinary
-Prenatal diagnosis in 80-90% of cases
Clinical features
Clinical presentation: bilious vomiting, neonatal obstruction with scaphoid abdomen from the first hours of life
Diagnostic delay if obstruction is less severe
Isolated failure to thrive possible
Radiography
Typical upper GI series finding:
"double bubble" sign, air trapped in the first portion of the duodenum and the stomach due to dilation of the stomach and first portion of the duodenum (upstream of the stenosis)
Ultrasound
Ultrasound:
-dilation of the stomach and first portion of the duodenum upstream of the stenosis, flat downstream small bowel loops
-normal appearance of the pylorus
Management
Volvulus risk!!!
Good prognosis with early surgical treatment
Classification
3 types:
-Type 1: duodenal web (65%)
-Type 2: complete duodenal atresia, fibrous cord (8%)
-Type 3: complete duodenal atresia, complete separation (8%)