Extrahepatic portal vein obstruction how is extrahepatic portal vein obstruction abbreviated. It is a distinct disorder that excludes portal vein thrombosis pvt occurring with liver cirrhosis or. Pdf extrahepatic portalvein obstruction in pregnancy. Extra hepatic portal venous obstruction in children narendra k. Imaging and radiological interventions in extrahepatic. Original article transjugular intrahepatic portosystemic. Extrahepatic portal vein obstruction how is extrahepatic. Coagulation profile and platelet function in patients with. To define the natural course of extrahepatic nonmalignant partial portal vein thrombosis pvt, including the progression from partial to complete pvt, in patients with cirrhosis who had undergone multidetector computed tomography ct. The medical records of 108 children 67 male, median age 4.
The effect of ehhpvo portal pressure in ehpvo is not localized saein the esophagus and stomach. Preoperative portal vein embolization for extension of hepatectomy indications. Extrahepatic portal vein obstruction in egyptian children. Extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal hypertension, especially in third world countries. The portal vein carries blood from bowel to the liver. Extrahepatic portal vein obstruction and portal vein. Extrahepatic portal vein obstruction ehpvo refers to the obstruction of the extrahepatic portal vein with or without involvement of the intrahepatic portal vein branches, splenic andor superior mesenteric vein.
Review article management of bleeding in extrahepatic portal. Moreover, operations like a splenectomy and proximal lienorenal shunt eliminate a large painful spleen and hypersplenism and restore a normal growth pattern in children. Extrahepatic portal vein obstruction is a condition in which there is mechanical obstruction to portal venous flow. Hepatic congestion due to right heart failure andor triscuspid regurgitation is a prominant feature and a reflection of elevated right atrial pressure. The major lobar fissure, also referred to as the principal plane or cantlie line, is represented by the dashed line and divides the anatomical right and left liver.
If you have access to a journal via a society or association membership, please browse to your society journal, select an article to view, and follow the instructions in this box. Patients present with splenomegaly or variceal bleeding. Backgroundmalignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis epvt. Dec 11, 2017 in the english literature, portal vein obstruction was first reported in 1868 by balfour and stewart, who described a patient presenting with an enlarged spleen, ascites, and variceal dilatation. Extra hepatic portal vein obstruction linkedin slideshare. Reappraisal of shunt surgery for extrahepatic portal vein. The diagnosis is easily confirmed by doppler ultrasonography.
Ultrasonographic identification of dilated intrahepatic bile. Portal hypertension and bleeding esophageal varices their. Fast fusion 3d sweep of the liver at the porta hepatis with power doppler confirms a flow void in the right portal vein. The thrombosis may extend into intrahepatic portal venous branches, giving rise to combined extra hepatic and intrahepatic portal venous obstruction 5. Extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal hypertension, especially in third.
Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein thrombosis pvt is a frequent complication of liver cirrhosis, but it can also occur as a primary vascular disorder amid absent liver disease. Ehbdo is an uncommon but often lifethreatening problem that may require timely surgical intervention. Imaging findings in incidental intrahepatic portal venous shunts. Management of extra hepatic portal venous obstruction ehpvo. Heart failure leads to both morphological and functional changes in the liver. Multimodality imaging of primary extrahepatic portal vein. Portal vein obstruction due to chronic liver disease, neoplasm, or postsurgery is a separate. It may be a partial or complete obstruction of the main portal vein with or without obstruction to its tributaries, in the absence of liver cirrhosis or malignancy. Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic. In other words, ipo and iho in japan have hepatitis as a common etiologic factor, and the difference between them lies only in severity and location of fibrosis causing flow resistance. These results suggest that hepatitis is the cause of hepatic fibrosis found in most cases of ipo as in iho.
Extrahepatic portal vein aneurysm journal of vascular surgery. Imaging of spleno portal axis is the mainstay for the diagnosis of ehpvo. The obstruction of the portal vein is diagnosed through the presence of intraluminal thrombus in the portal vein andor portal vein cavernoma fig. Aetiology and management of extrahepatic portal vein. The portal cavernomis composed of two venous systems including the paracholedochal veins, which run parallel to the ductal wall, and the epicholedochal venous plexus, located on the surface of the bile duct 3, 4 fig. As many as half of all patients with ehpvo children and adults have no predisposing cause 15. In western countries, extrahepatic portal vein obstruction ehpvo occurring in the absence of liver cirrhosis and portal invasion or constriction by a malignant tumor accounts for a small but not negligible proportion of cases of portal hypertension in adults. The main risk in pregnant women with this condition is variceal bleeding. Patient may need blood thinners or other treatments. This leads to increased portal vein and hepatic sinusoid pressures as the blood flow stagnates. Results of a stepwise approach to extrahepatic portal vein obstruction in children.
A rex shunt mesenterictoleft portal vein bypass is considered a more physiologically rational treatment for ehpvo in children compared to other portosystemic systemic shunts, as it eliminates the untoward effects of depriving the liver of splanchnic portal flow and significant morbidities. No thrombus was found in the portal vein, which was anastomosed end to end. Extrahepatic portal vein obstruction is an important cause of portal hypertension among children. Recognition of the characteristic changes and knowledge of the portal venous anatomy makes it possible to diagnose extrahepatic biliary obstruction with a high degree of confidence. Clinically, it is very important to differentiate bet ween acute. Extrahepatic portal veins obstruction ehpvo is a vascular disorder of the liver which is defined as obstruction in the extra hepatic portal vein with or without involvement of the intrahepatic portal veins or splenic or superior mesenteric veins. Background malignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis epvt. Extrahepatic portal vein obstruction ehpvo is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Extrahepatic portal vein obstruction radiology reference. The extrahepatic factors are portal or splenic vein thrombosis, cavernous transformation of the portal or splenic veins, hepatic vein thrombosis and the cruveilhierbaumgarten syndrome. The arrow indicates the formation of a portal cavernoma.
Chiari syndrome bcs hepatic venous outflow tract obstruction hvoto extrahepatic portal vein obstruction ehpvo portal hypertension v. Extrahepatic portal vein obstruction ehpvo is the usual cause for portal hypertension in children. Portal vein thrombosis may arise as a complication of cirrhosis. Causes of extrahepatic portal vein obstruction include thrombosis and invasion or constriction by a malignant tumor.
Ehpvo associated with pancreatitis and abdominal lymph node tuberculosis compressing the portal vein was excluded. Hepatic cirrhosis, hepatic encephalopathy, transjugular intrahepatic portosystemic shunt, portal vein thrombosis, covered stent introduction portal vein thrombosis pvt is an obstructive deep vascular disease and one of the major complications of liver cirrhosis 1, 2. Extrahepatic portal venous obstruction ehpvo is a heterogenous disease with regards to etiology, pathogenesis, age, and geographical location. Biliary abnormalities associated with extrahepatic portal. Controversy exists concerning the proper therapy for bleeding gastroesophageal varices secondary to noncirrhotic portal vein obstruction 23,24.
We evaluated ehpvo children for prevalence, nature, and relation of colonic changes with disease duration, extent of splenoportal axis spa thrombosis, portal hypertensive gastropathy phg, and esophageal varices evs. In infants, omphalitis and other forms of infection are the established causes, and in adults pylephlebitis, biliary tract infection, and injury to the portal vein during surgery are. Mortality of patients with epvt may be associated with these concurrent medical conditions or with manifestations of portal hypertension, such as variceal haemorrhage. Physical examination was negative except mild right upper quadrant tenderness. Risk factors for thrombophilia in extrahepatic portal vein. Extrahepatic portal vein obstruction ehpvo, although rare in children. It may or may not extend into the intrahepatic portal vein. In the english literature, portal vein obstruction was first reported in 1868 by balfour and stewart, who described a patient presenting with an enlarged spleen, ascites, and variceal dilatation. Extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Extrahepatic portal vein obstruction ehpvo is an important cause of portal hypertension in children.
Guidelines for the diagnosis and treatment of extrahepatic. Hepatocellular functions have been reported to be normal. Portal vein thrombosis pvt is a relatively common complication in patients with liver cirrhosis, but might also occur in absence of an overt liver. There was no significant medical or surgical history. To study a singlecentre experience of the management of extrahepatic portal vein obstruction ehpvo in children during the last 3 decades. Extrahepatic portal vein obstruction without portal hypertension signs was characterized by thick extensive hepatopetal collaterals or patency of some intrahepatic portal veins.
Extrahepatic portalvein obstruction ehpvo is defined as obstruction of the extrahepatic portal vein with or without the involvement of the intrahepatic portal veins or splenic or superior mesenteric veins. Acute portal vein thrombosis unrelated to cirrhosis. Aug 25, 2014 extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Surgical exploration revealed extrahepatic portal vein obstruction with giant inferior mesenteric vein and colonic varices. Treatment of portal vein obstruction by percutaneous. Extrahepatic portal vein obstruction ehpvo is characterized by features of recent thrombosis or portal hypertension with portal cavernoma as a sequel of portal vein obstruction. In developing countries, it is a common cause of portal hypertension, accounting for up to 30% of all variceal bleeders. Branches of the hepatic artery and biliary ducts follow those of the portal vein. Extrahepatic obstruction of the portal vein with bleeding.
It can also occlude the portal vein lumen completely or par tially with the thrombus in the central area. Extrahepatic obstruction of the portal vein is a well known cause of hypertension in childhood and 55 out of the 97 patients 57 per cent seen with this condition presented before they were 15. To establish guidelines for the diagnosis and treatment of ehpvo in children, a group of. Extrahepatic portal vein obstruction and portal vein thrombosis in. The postolt course was routine, and the patient is well 7 months later. The asian pacific association for the study of the liver apasl had set up a working party on portal hypertension in 2002 with a mandate to develop consensus on various aspects of portal hypertension. A stepwise approach to diagnosis and treatment kulkarni rd 1, thomas em2, zendejas i3, fair jh and andreoni ka2 1department of surgery, university of texas medical branch, usa 2department of surgery, university of florida, usa 3imc, canyon surgical associates, usa. In a recent article by yale and crummy entitled splenic vein thrombosis and bleeding esophageal varices, the value of splenoportography and transarterial portography was emphasized in the preoperative evaluation of patients with bleeding gastroesophageal varices to allow identification of extrahepatic or segmental portal hypertension. Extrahepatic portal vein obstruction ehpvo is a common cause of portal hypertension in developing countries. Extrahepatic portalvein obstruction in pregnancy sciencedirect. Natural course of extrahepatic nonmalignant partial portal.
Use of mesorex shunt with transposition of the coronary vein. Extrahepatic portal vein obstruction listed as ehpo. Diagnosis of extrahepatic portal vein obstruction jama. The vast majority of cases are due to primary thrombosis of the portal vein. The chart showing pdf series, html series, scan qr codes html series. Noncirrhotic portal hypertension extrahepatic portal vein obstruction pratap sagar tiwari total slides. Rex bypass mrb and portosystemic surgical shunt pss are both used to treat extrahepatic portal vein obstruction ehpvo in children. The etiology and clinical presentation are different in. Extrahepatic portal hypertension due to congenital. Extrahepatic portal vein obstruction ehpvo refers to the obstruction of the extrahepatic pv with or without involvement of the intrahepatic pv branches, splenic vein or superior mesenteric vein.
Extrahepatic portal vein obstruction ehpvo is defined as obstruction of the extrahepatic portal vein with or without the involvement of the intrahepatic portal veins or splenic or superior mesenteric veins. Pdf consensus on extrahepatic portal vein obstruction. A dilated portal vein diameter of greater than or 15 mm is a sign of portal hypertension, with a sensitivity estimated at 12. Portal vein obstruction does not affect the liver function unless the patient has an underlying liver disease such as cirrhosis. International journal of medical science and innovative. Extrahepatic portal vein obstruction ehpvo, leading to portal hypertension, is the most common cause of upper gastrointestinal bleeding in children in india. Portaltoright portal vein bypass for extrahepatic portal. The pathology may be intraluminal, mural, or extramural table 161. Mortality of patients with epvt may be associated with. Extrahepatic portal vein obstruction patients without portal hypertension remained free of its signs for more than 3 years of follow up and, in fact, ehpo without. For successful application of the mesorex shunt, eligible patients must fulfill two preconditions. Introduction portal hypertension is a clinical manifestation which is defined as the presence of a hepatic venous pressure gradient 5 mmhg 1.
Introduction portal hypertension is the comm onest cause of upper gastrointestinal bleeding in children and up to 30% of cases with upper gastrointestinal hemorrhage can be fatal. Patient characteristics and natural history also vary between patient populations. Extrahepatic portal venous obstruction, although rare in the western world, is a common cause of major and life threatening upper gastrointestinal bleeding among the poor in developing countries. Portal vein thrombosis was first seen by stewart and balfour in the late 1860s in a patient with splenomegaly, ascites, and variceal dilatation. Portal hypertension and bleeding esophageal varices their occurrence in the absence of both intrahepatic and extrahepatic obstruction of the portal vein william a. A flow void is noted in the right portal vein which exhibits an echogenic lumen. Though mortality related to variceal bleeding is uncommon, morbidity due to massive splenomegaly with hypersplenism, growth failure, ectopic varices like rectal varices and portal biliopathy is significant. Management of bleeding in extrahepatic portal venous obstruction. The medical literature on colonic changes in children with extrahepatic portal venous obstruction ehpvo is limited. Prevalence, nature, and predictors of colonic changes in. Results of a stepwise approach to extrahepatic portal vein. Extrahepatic portal vein obstruction ehpo is a frequent cause of portal hypertension in children, but it also occurs in adults as a sequela to portal thrombosis.
Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction ehpvo in children article pdf available january 20 with 6,226 reads how we measure reads. Anatomia bouchet miembro superior pdf title, anatomia. Successful recanalization of portal vein thrombosis before. Etiology and longterm outcome of extrahepatic portal vein. India, thrombosis of portal vein is the main cause of ehpvo.
Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non. Extrahepatic portal vein obstruction in the pediatric age clinmed. Portal vein occlusion doctors answer your questions. Extrahepatic portal vein obstruction ehpvo refers to the obstruction of the extrahepatic portal vein with or without involvement of the intrahepatic portal vein branches, splenic andor. Proceedings of the fifth baveno international consensus workshop, fifth edition. Various aetiologies, definitions and presentations have been described from the west that are different from the east. Any obstruction of the venous vasculature of the liver is referred to as buddchiari syndrome, from the venules to the right atrium. It is a distinct disorder that excludes pvt occurring in concurrence with liver cirrhosis or hepatocellular carcinoma. Extrahepatic portal vein obstruction is the most common cause of noncirrhotic portal hypertension in children and young adults in developing countries. Extrahepatic portalvein obstruction ehpvo is a common cause of portal hypertension in developing countries. Good quality randomized a established scientific evidence. The etiology of ehpvo is diverse and risk factors are usually detected in less than half of patients, and include perinatal events such as umbilical catheterization and sepsis, and. Rex bypass for extrahepatic portal vein obstruction.
The purpose of our study was to describe the imaging findings in incidentally discovered intrahepatic portal venous shunts. Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction in children. The aim of this study was to describe the clinical presentation, possible risk factors, upper. The risk of variceal bleeding in children with portal vein obstruction was expected to decrease in adolescence because of the development of. It is characterized mainly by portal vein thrombosis pvt, and is detected in 40% of children with upper gastrointestinal bleeding ugib caused by esophageal varices. Primary extrahepatic portal vein obstruction in adults. The portal vascular anomaly, which presumably was responsible for the portal hypertension, was probably due to failure of communication between the em bryonic vitelline veins or to atresia of the portal vein secondary to pressure from the abnormal hepatic lobulation in utero.
This study analyzed our experience with ehpvo in adults aged 20 years. Portal vein obstruction an overview sciencedirect topics. Extrahepatic portal vein obstruction ehpvo, although rare in children, is an important cause of portal hypertension and upper gastrointestinal ugi bleeding in the pediatric age group1,2. The intrahepatic causes of portal hypertension include, among others, such conditions as portal cirrhosis, biliary cirrhosis, schistosomiasis and hemochromatosis. Extrahepatic portal venous obstruction ehpvo is the commonest cause of portal hypertension and variceal bleeding in children. Access to society journal content varies across our titles. It refers to thrombosis originating in the main portal vein. Parasagittal scans of the main right portal vein were the most sensitive for detection of intrahepatic ductal dilatation. Extrahepatic portal vein obstruction and idiopathic portal hypertension are entities that encompass the spectrum of vascular diseases of the liver. Intrahepatic portal venous shunts are uncommon hepa. On doppler ultrasonography, a slow velocity of portal vein are diagnostic of portal hypertension. Biliary abnormalities associated with portal biliopathy. Extrahepatic bile duct obstruction ehbdo is defined as the lack of bile transit into the duodenum due to blockage along the common bile duct cbd or at its junction with the duodenum.
Extrahepatic portal venous obstruction ehpvo is a common cause of portal hypertension in the developing countries, and constitutes up to 40% of all patients with portal hypertension. Extrahepatic portal vein obstruction in adults detected by. Portal biliopathy is defined as biliary changes in patients with. A 49yearold presented with intermittent epigastric and right upper quadrant abdominal pain with no associated or alleviating factors. Extrahepatic portal vein obstruction ehpvo is an important cause of portal hypertension ph among children.
The etiology is heterogeneous and there are few evidences related to the optimal treatment. Isolated splenic vein or superior mesenteric veinocclusion does not constitute ehpvo. Portal vein thrombosis can occur with inflammatory conditions or cancers. The aim of this study was to analyse the outcome of mrb and pss to select patients who could benefit from a prophylactic mrb. Endoscopy sclerotherapy is the best option for the control of acute variceal bleeding.
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