Patterns of portal vein occlusion and their aetiological significance

Abstract

Clinical and angiographic findings in 53 patients with portal vein occlusion were reviewed to determine the relationship between the pattern of venous occlusion and its aetiology. Five major patterns were identified. Group 1 comprised three children with idiopathic intrahepatic portal vein occlusion alone (hepatoportal sclerosis). Those in group 2 (25 patients), with occlusion of the main portal vein, accounted for almost half the total number and for most of those with a probable congenital aetiology. The ten patients in group 3 had angiographic occlusion and collateralization of the main portal and superior mesenteric veins. Intra-abdominal sepsis, other than that from pancreatic disease, was associated with this pattern of venous occlusion in which the splenic vein is spared. No patients with isolated portal and splenic vein occlusion (group 4) were recorded. Widespread thrombosis throughout the portal venous system (group 5; 15 patients) had a multifactorial aetiology but was typically observed in hypercoagulable states that were also associated with hepatic vein occlusion or other deep vein thromboses.

Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension

“…At autopsy, examination of the hilum of the liver confirmed that cavernous transformation was caused by a large number of small vessels instead of a large vessel and the lesser omentum was a fibrous fleshy plate about 1 cm thick [19]. However, the vessels described in some published reports are not numerous fine vessels, but rather tortuous large vessels, and are similar to the g-shaped extrahepatic portal vein that we found using 3D-CTP in this study [9,[20][21][22].…”

Portal Vein Thrombosis

“…It is hypothesized that EHPVO in children has initial component of phlebosclerosis, with thrombosis as a secondary event, 57 due to infection or a primary thrombotic disorder. Omphalitis, neonatal umbilical sepsis, umbilical vein cannulation, repeated abdominal infections, sepsis, abdominal surgery and trauma can also result in EHPVO.…”

Portal hypertension due to portal venous thrombosis: Etiology, clinical outcomes

“…The design of the studies and the limits evaluating the cause of PVT has changed parallel to the evolution in medical and genetic technology. In previous studies between 1979-1997, PVT were attributed mostly to trauma (5%-17%), intraabdominal sepsis (5%-36%), umbilical sepsis (5%-12%) [6] (important cause of PVT in children), pancreatitis (4%-5%) and prothrombotic disorders (2%-28%), but in nearly 50% of the patients etiology remained unidentified [7][8][9][10][11] . But with the advent of better medical care, potent antibiotic treatment, advanced medical technology, discovery of various causes of genetic thrombophilia and with better understanding of the coagulation system, this profile has also changed.…”