Extrahepatic portal hypertension in children

Developmental abnormalities and liver disease in childhood

“…[140][141][142] venous lake with extension into the gastroduodenal ligament. [81][82][83] It is a major cause of portal hypertension and may account for as many as 30% of all children with bleeding oesophageal varices. 84 A thrombotic diathesis due to inherited abnormalities of anticoagulant proteins is very rarely a factor in the pathogenesis.…”

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

“…Many authors have reported that the cause of extrahepatic portal hypertension is extrahepatic portal vein obstruction, and the term extrahepatic portal hypertension is almost synonymous with that of extrahepatic portal vein obstruction [4][5][6][10][11][12][13][14][15]. It has also been described that prehepatic blockade of the portal vein results in the formation of multiple hepatopetal collaterals, which is called cavernous transformation, and this is a common characteristic of extrahepatic portal obstruction [5,16,17].…”

“…It has also been described that prehepatic blockade of the portal vein results in the formation of multiple hepatopetal collaterals, which is called cavernous transformation, and this is a common characteristic of extrahepatic portal obstruction [5,16,17].…”

“…It has been reported that many cases of extrahepatic portal hypertension are caused by portal vein obstruction [5,6]. However, based on the findings of ultrasound, portal venography, computed tomography (CT) scanning, and magnetic resonance imaging, we have previously proposed that extrahepatic portal hypertension had embryological cause, in which the common bile duct went around behind the dorsal anastomosis of the vitelline vein when the duodenum rotated, and the portal vein was lifted by the common bile duct and coiled itself around the duct [7].…”

Extrahepatic portal vein obstruction in a neonate

“…Extrahepatic portal venous obstruction is rare, but it is a significant cause of portal hypertension in children. Although its etiology is unclear, the probable underlying causes of extrahepatic portal obstruction are congenital malformation, trauma, exchange transfusion, omphalitis, septicemia, umbilical catheterization, protein C deficiency, splenectomy, pulmonary hypertension, thrombotic hematological or embryological 1–8 . Odieve et al reported 30 cases of extrahepatic portal hypertension of unknown origin 9 .…”