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). Non‐Cirrhotic Shiv Kumar Sarin MD, DM. Director Treatment of chronic EHPVO in children.
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Diagnosis is often clinical, supported by simple radiological tools. Portal hypertensive gastropathy in children. Endoscopic retrograde cholangiopancreatography in the management of pancreaticobiliary disorders in children. Zarin of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction.
With the availability of effective endotherapy, the mortality due to variceal bleeding has become a rarity but the morbidity due to late sequelae like growth failure, portal bilipathy, gastric varices, rectal varices etc.
Biliary abnormalities ehpvk with extrahepatic portal venous obstruction.
Non-cirrhotic portal hypertension – diagnosis and management.
On the other hand, EVL has the advantages of rapid eradication of varices requiring fewer sessions and portending fewer complications. The etiology and clinical presentation are different in children and adults.
Though the role of anticoagulation therapy remains controversial in adults,12 there is no role of anticoagulation therapy in children with EHPVO. Portal hypertensive gastropathy and gastric varices before esophageal variceal sclerotherapy and after obliteration. The prevalence and spectrum of colonic lesions in patients with cirrhosis and noncirrhotic portal hypertension.
Acute gastric variceal bleeding can be controlled with tissue glue N-acetyl-2butyl- yanoacrylate injection.
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. Extrahepatic portal venous obstruction and obstructive jaundice: Portal hypertension in north Indian eupvo.
Consensus on extra-hepatic portal vein obstruction.
The low ephvo in pediatric studies clearly suggests that the duration of portal hypertension plays an important role in the development of rectal varices. Studies in children have shown that hereditary or acquired coagulation disorders do not play a role in the pathogenesis of EHPVO in children. Earlier all attention was concentrated on esophageal varices in EHPVO because the main manifestation was variceal bleeding.
Portal-vein obstruction in children leads to growth retardation. There is no controversy about the management of acute variceal bleeding. Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction; a year sarkn.
Portal hypertension, children, biliopathy, growth, sclerotherapy.
Extrahepatic portal vein obstruction.
Bleeding from rectal varices can be managed with sclerotherapy or band ligation. However, with the availability of new mesenterico-portal mesenterico-left portal-bypass or Rex shunt, the problems of conventional shunts have been largely overcome.
Primary biliary tract surgery has significant morbidity and mortality due to extensive collaterals around the bile ducts.
On the other hand, the alternate hypothesis suggests that shunting of blood away from the liver results in impaired synthesis of factors needed for normal growth. Although the liver may appear normal, functional compromise develops in the long term. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: A significant proportion of cases in adults are due to procoagulant state but the same has not been documented in children.
This observation again supports the compression theory in most cases of portal biliopathy. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction. Shunt surgery should be considered for asymptomatic portal biliopathy in presence of another indication like growth failure, symptomatic hypersplenism or ectopic varices. Endoscopic sclerotherapy in children. The only pediatric study by Gauthier-Villars et al  in 8 children with symptomatic biliopathy in EHPVO showed regression of cholestasis in all cases after shunt surgery mesocaval shunt in 6 and Rex in 2.
Prevalence, classification and natural history of gastric varices: Aetiology and management of extrahepatic portal vein obstruction in children: Colonic changes in patients with cirrhosis and in patients with extrahepatic portal vein obstruction.
There are two main approaches to prevent further bleed: Management of esophageal varices. Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO.
In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis. The definitive method of diagnosis of portal biliopathy is ERCP. Endoscopic band ligation followed by sclerotherapy: This has been substantiated in other studies in children. Evolving consensus in portal hypertension.