Imaging Features And Clinical Relevance Of Portal Venous Systems Shown By Extrahepatic Portal Angiography In Children With Extrahepatic Portal Venous Obstruction

Objective:To investigate the findings of portal angiography in children with extrahepatic pottal hypertension and factors associated with morphology of portal venous system.Methods:From February 2008 to October 2017,107 patients(62 boys and 45 girls)with upper gastrointestinal bleeding or splenomegaly and hypersplenism were admitted to our hospital.The cavernous transformation of portal vein was diagnosed by the ultrasound and CT in all children,and a surgery was performed(Rex shunt in 99 cases,Warren shunt in 7 cases,and laparoscopic splenic vessel ligation in one case).Intraoperative superior mesenteric venography was used to determine the morphology of portal venous system.According to the morphology of portal vein showed by portal venogram,all patients were divided into the following groups:groups with and without a visible gastric coronary vein;groups with and without a visible splenic vein;group with the appearance of“cotton mass”and group with the visible collateral veins in portal cavernoma;groups with and without a good visible intrahepatic portal vein.The pre-and post-operative portal pressure,the preoperative incidence of esophageal varices,the time of onset,the incidence of postoperative rebleeding,the pre-and post-operative size of spleen and the age of operation were compared among groups.The proportion of children undergoing gastro-portal bypass was compared between groups with and without a visible gastric coronary vein.The correlation between the appearance of cavernous transformation of the portal vein and the conditions of the coronary vein,splenic vein and intrahepatic portal vein was analyzed.Results:The duration of follow-up was 3-120 months,with an average of 48.1 months.The preoperative incidence of esophageal varices,the time of onset,the postoperative size of spleen and the age of operation were significantly lower in group with the appearance of“cotton mass”than those of group with the visible collateral veins(P<0.05).The incidence of preoperative esophageal varices in group with a visible gastric coronary vein was significantly higher than group without a visible gastric coronary vein,and there was a significant correlation between the visible coronary vein and esophageal varices(P=0.002).The time of onset,the pre-and post-operative length of spleen,the age of operation were significantly lower in group with a good visible intrahepatic portal vein than those of group without a good visible intrahepatic portal vein(P<0.05).There was a significant correlation between the morphology of portal cavernoma and the appearances of gastric coronary vein and intrahepatic portal vein(P<0.05).The visible gastric coronary vein was significantly correlated with the gastro-portal shunt(P=0.000),and group with a visible gastric coronary vein have a higher ratio of children undergoing gastro-portal shunt.Conclusion:There are two types of portal cavernoma:"cotton mass"type and "the visible collateral vein" type.The type of "cotton mass" is an early stage of morphology in cavernous transformation of portal vein,which was usually occurred in children with the smaller age of operation and time of onset,and the lower incidence of esophageal varices.Children with a type of"cotton mass" usually had a better prognosis after Rex shunt.Children with extrahepatic portal hypertension should be treated as soon as finding this disease.The visible gastric coronary vein is an important indicator for performing gastroportal shunt.