Preduodenal portal vein is rare, with 63 cases reported in the literature. In general, this anomaly occurs in children with associated small bowel obstruction. We report a newborn infant who presented with duodenal stenosis, mongolism, and preduodenal portal vein. Treatment consisted of a duodenoduodenal anastomosis without mobilizing the portal vein. The correlation between imaging techniques and the operative findings is discussed. Because identification of preduodenal portal vein at surgery is important, preoperative sonography may be useful in selected cases to define the position of the vein.
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