Preduodenal Portal Vein and Malrotation: What Causes the Obstruction?

Preduodenal portal vein is a rare congenital abnormality, and occurs either as a single malformation, in association with other malformations or as part of "polysplenia" syndrome. Preduodenal portal vein has seldom been reported as a cause of intestinal obstruction, however corrective surgery is nearly always performed. We conducted a 25-year retrospective study in a single centre to investigate the cause of obstruction in patients with preduodenal portal vein. Furthermore, we reviewed the literature on preduodenal portal vein.Over a period of 25 years, preduodenal portal vein was diagnosed in five patients. The diagnosis was made during surgery performed because of symptoms of high intestinal obstruction. All five patients had intestinal malrotation as well and, in all patients, another cause for high intestinal obstruction than preduodenal portal vein was found.Preduodenal portal vein is mainly asymptomatic. It is often associated with other intestinal congenital abnormalities more likely to cause high intestinal obstruction. Therefore, the (paediatric) surgeon should always be alert for another associated cause of intestinal obstruction. Because of the potential for technical problems from preduodenal portal vein during surgery, it nevertheless should be on the surgeon's mind during surgery when the patient has high intestinal obstruction.