Polysplenia with agenesis of the dorsal pancreas and preduodenal portal vein, about a case

Abstract

Purpose of the study: Polysplenia is a complex polymalformative syndrome that is frequently accompanied by an inconsistent spectrum of visceral and vascular anatomical abnormalities and is extremely undiscovered in adulthood. The objective of this article is to limit the intraoperative risks generated by the lack of knowledge of these anatomical variations by insisting on the inconstancy of all these variations and the perfect knowledge of the reference anatomy and the exploration of the anatomical variations in imaging before the surgery.

Patient and methods: The patient was 50-year-old who was hospitalized in our department for gastric adenocarcinoma. During her extension assessment, a polysplenia syndrome was accidentally discovered on the scan images, which showed an exceptional association between preduodenal portal vein, agenesis of the dorsal pancreas, and polysplenia.

Conclusion: There are neither specific clinical symptoms of the polysplenia syndrome, nor any biological sign; hence, the interest of recognizing this pathology is to avoid diagnostic errors, but also to guide the surgeon during the surgical act performed.

Keywords: Agenisis of the dorsal pancreas; Polysplenia; Preduodenal portal vein; Total gastrectomy.