Congenital duodenal obstruction (CDO) occurs due to intrinsic and extrinsic mechanisms but is most often caused by intrinsic duodenal atresia and stenosis. This review will summarize the history, epidemiology, and etiologies associated with the most common causes of CDO. The clinical presentation, complex diagnostic considerations, and current surgical repair options for duodenal atresia and stenosis will also be discussed. Finally, both historical and recent controversies which continue to affect the surgical decision-making in the management of these patients will be highlighted.
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