Sarin SK(1), Agarwal SR. Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World. Endoscopic Management. S. K. Sarin, Cyriac Abby Philips, Rajeev Khanna tal vein obstruction (EHPVO), noncirrhotic portal fibrosis. (NCPF; or idiopathic PHT. Extrahepatic Portal Vein Obstruction (EHPVO). Nonā€Cirrhotic Shiv Kumar Sarin MD, DM. Director Treatment of chronic EHPVO in children.

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Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation.

Introduction Extrahepatic portal venous obstruction EHPVO is the commonest cause of portal hypertension in children [12] and one of the common causes in adults in India. Correction of extrahepatic portal vein thrombosis by the meserteric to left portal vein bypass. Magnetic resonance MR imaging with intravenous gadolinium injection delineate the cavernoma and biliary changes simultaneously and may be extremely useful in children.

Extrahepatic portal vein obstruction in children: Long term outcome after injection sclerotherapy for esophageal varices in children with extra hepatic portal hypertension. Another study from Lucknow [55] documented growth retardation height less than 5th percentile for age in Portal-vein obstruction in children leads to growth retardation. Assessment of injection sclerotherapy in the management of children with oesophageal varices.

Consensus on extra-hepatic portal vein obstruction.

Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Nevertheless, EST cannot be totally ruled out as a therapeutic modality, especially sarih children.


Surgery is primarily indicated when endotherapy fails to control bleeding, in presence of gastric sairn ectopic varices not amenable to endoscopic ehpvk and with delayed sequelae like portal biliopathy and rectal varices.

Endoscopic sclerotherapy for esophageal varices in children with extrahepatic portal vein obstruction; a follow -up study. Longterm studies after endotherapy have shown almost no mortality.

Endoscopic retrograde cholangiopancreatography in the management of pancreaticobiliary disorders in children.

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Rectosigmoid varices and other mucosal changes in patients with portal hypertension. Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. Poddar U Borkar V.

In another study Chaudhary et al [50] documented relief of jaundice in five of seven patients within weeks of shunt surgery and the remaining two patients required second stage hepaticojejunostomy.

In adults, beta-blockers are recommended for patients who have bled from portal hypertensive gastropathy; and shunts TIPS or surgical are recommended ehpvoo those patients in whom beta-blockers are contraindicated or have failed.

The definitive method of diagnosis of portal biliopathy is ERCP.

Portal biliopathy is universal in adults and common in children but symptomatic cases are mainly in adults; thereby suggesting a progressive nature of the condition.

Shunt surgery should be considered for asymptomatic portal biliopathy in presence of another indication like growth failure, symptomatic hypersplenism or ectopic varices. The exact mechanism of the growth failure is not known. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Endoscopic band ligation followed by sclerotherapy. Variceal bleed and splenomegaly are the commonest presentations. On the other hand, EVL has the advantages of rapid eradication of varices requiring fewer sessions and portending fewer complications.


Management of esophageal varices. Though the role of anticoagulation therapy remains controversial in adults,12 there is no role of anticoagulation therapy in children with EHPVO. Band ligation plus sclerotherapy versus sclerotherapy alone in children with extrahepatic portal vein obstruction.

Extrahepatic portal vein obstruction.

Endoscopic sclerotherapy in children: Treatment of bleeding esophagogastric varices due to extrahepatic portal hypertension: Cholangiopathy associated with portal hypertension: Management of gastric varices and portal hypertensive gastropathy. The natural history of portal hypertensive gastropathy: The mesoportal bypass MPB is a new shunt that restores mesenteric blood flow to the liver through the Rex venous recessus portion of left portal vein joining the umbilical vein.

Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction. Chawla Y, Dilawari JB. This indicates while in some patients compression plays an important role, in others ischemia alone saron in combination with compression causes sarij changes.

Biliary changes in extrahepatic portal venous obstruction: With the availability of the most physiological shunt mesenteric-left-portal bypass or Rex the management paradigm of EHPVO swrin changed from endotherapy to primary shunt surgery. Irreversible changes in the portal vein may decrease the compliance of the portal vein for adaptation if the shunt is done late.