Esophageal variceal bleeding caused by hypoplasia of the portal vein in a patient with the Klippel-Trenaunay syndrome.

The case of a patient affected by Klippel-Trenaunay syndrome presenting with esophageal variceal bleeding caused by hypoplasia of the vena porta is reported. Hemostasis was achieved by performing a proximal spleno-renal shunt. We discuss the likely association of this mesodermal development abnormality and vascular disorders of the portal vein.