A 2-day-old female neonate has bilious vomiting from birth, abdominal distension, and failure to pass meconium. X-ray shows a 'double bubble' sign. At surgery, a duodenal atresia is found at the level of the second part of the duodenum. The embryological basis of this defect is:
- A Volvulus of the midgut at 8 weeks gestation
- B Failure of rotation of the midgut loop
- C Failed recanalization of the duodenal lumen after the solid cord stage (5th–10th week) ✓
- D Annular pancreas compressing the duodenum externally
Explanation
Duodenal atresia results from failure of recanalization of the duodenal lumen that normally undergoes a transient solid epithelial cord phase between 6–10 weeks of gestation, followed by vacuolization and recanalization. Incomplete or failed recanalization produces atresia (most common), stenosis, or a web/membrane. The double bubble sign on X-ray represents air in the stomach and proximal duodenum with no distal gas. Annular pancreas encircles the duodenum externally and is a separate (and occasionally co-existing) lesion; it causes obstruction but is not intrinsic atresia. Surgical repair is diamond-shaped duodeno-duodenostomy (Kimura procedure).
Reference: Bailey & Love's Short Practice of Surgery, 27th ed.
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Written and medically reviewed by the StethoPrep medical team.