Congenital absence of portal vein with nodular regenerative hyperplasia of the liver

Congenital absence of the portal vein is a very rare anomaly. The intestinal and splenic venous drainage bypasses the liver and drain into the inferior vena cava (IVC). Two cases of such anomaly are described. Both cases were investigated by US coupled with echo-colour Doppler examination, CT and MR imaging, followed by digital subtraction angiography (DSA) and liver biopsy. In the first case the splenic and superior mesenteric vein formed a venous trunk which emptied directly into the IVC; in the second case, the splanchnic blood flowed into a dilated hepatofugal inferior mesenteric vein which connected to the left internal iliac vein. In both cases nodular regenerative hyperplasia of the liver was present, presumably due to an abnormal hepatic cell response to the absent portal flow. The particular contribution of MR imaging to the diagnosis of both vascular abnormalities and liver parenchyma derangement and its advantages over the other diagnostic techniques is emphasized. The clinical and radiological features of 17 previously reported cases are reviewed. (orig.)