duodenal atresia
Pronunciation: doo-oh-DEE-nal ah-TREE-zhah
Congenital obstruction of the duodenum due to failure of recanalization during fetal development.
Full Definition
Duodenal atresia is a congenital anomaly characterized by complete obstruction of the duodenal lumen, most commonly occurring distal to the ampulla of Vater. This condition results from failure of recanalization during the 8th to 10th week of gestation when the duodenal lumen normally reopens after temporary obliteration. Duodenal atresia is strongly associated with Down syndrome (occurring in 20-30% of cases) and polyhydramnios during pregnancy due to inability to swallow amniotic fluid. The classic radiological finding is the 'double bubble' sign on abdominal X-rays. Pathologically, the duodenum shows a complete membrane (type I) or gap with fibrous cord connection (type II) or complete separation (type III).
Usage
Usage note: Distinguish from duodenal stenosis (partial obstruction). Strong association with Down syndrome should be noted.
In Context
- "Surgical exploration confirmed type II duodenal atresia with a fibrous cord connecting the proximal and distal segments." — Operative report
- "The association of duodenal atresia with Down syndrome prompted chromosomal analysis." — Clinical assessment