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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Management of esophageal varices. Endoscopic retrograde cholangiopancreatography in the management of pancreaticobiliary disorders in children.

Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. As of now, we do not have sufficient data to say what proportions of EHPVO cases are suitable for this new shunt. Management of gastric varices and portal hypertensive gastropathy.

Extrahepatic portal vein obstruction.

However, with the availability of new mesenterico-portal mesenterico-left portal-bypass or Rex shunt, the problems of conventional shunts have been largely overcome.

Emergency shunt surgeries have become a rarity in the era of endoscopic management. Extrahepatic portal vein obstruction in children: Earlier all attention was concentrated on esophageal varices in EHPVO because the main manifestation was variceal bleeding. Non-cirrhotic portal fibrosis in children. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: This is the most physiological shunt as it restores the hepatic blood flow. H-type shunt with an autologous venous graft for treatment of portal hypertension in children.

Cholangiopathy associated with portal hypertension: Magnetic resonance MR imaging with intravenous gadolinium injection delineate the cavernoma and biliary changes simultaneously and may be extremely useful in children. Endosonographic evaluation of the rectum in children with extrahepatic portal venous obstruction.


Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Symptomatic biliary obstruction can be managed endoscopically but shunt surgery followed by biliary bypass if necessary seems to be the best management option. However, if there is bleeding from PHG shunt surgery should be considered as the benefit of long-term beta-blockers in EHPVO in children has not been proven in clinical trials.

Bleeding from rectal varices can be managed with sclerotherapy or band ligation. Evolving consensus in portal hypertension. Studies in children have shown that hereditary or acquired coagulation disorders do not play a role in the pathogenesis of EHPVO in children. Two possible mechanisms have been proposed. In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis.

Cholestasis in children with portal vein obstruction.

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Some people believe that shunt surgery should be done first in all cases with symptomatic biliopathy and if it fails to resolve biliary obstruction then second stage biliary surgery is recommended. Pant Hospital, New Delhi, India. Liver function tests took 2 to 2. Although the liver may appear normal, functional compromise develops in the long term. There are two main approaches to prevent shpvo bleed: This suggests that children with EHPVO have growth failure and decreased growth velocity despite adequate nutrition.

Factor V Leiden and prothrombin gene GA mutations are uncommon in portal vein thrombosis in India. Aetiology and management of extrahepatic portal vein obstruction in children: Primary biliary tract surgery has significant morbidity and mortality due to extensive collaterals around the bile ducts.

Non-cirrhotic portal hypertension – diagnosis and management.

The natural history of portal hypertensive gastropathy: Long term outcome after injection sclerotherapy for esophageal varices in children with extra hepatic portal hypertension.


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. Most of these bleeding episodes occurred within the first 4 years of variceal eradication. In adults, beta-blockers are recommended for patients who have bled from portal hypertensive gastropathy; and shunts TIPS or surgical are recommended in those patients in whom beta-blockers are contraindicated or have failed.

Half of the re-bleed cases were managed with endotherapy for esophageal varices while the remaining half had to be undertaken for shunt surgery for bleeding gastric varices. A prospective study of endoscopic esophageal variceal ligation using multiband ligator. EHPVO is the commonest cause of portal hypertension and variceal bleeding in children. Nevertheless, EST cannot be totally ruled out as a therapeutic modality, especially in children.

Significantly increased levels of growth hormone and decreased levels of insulin-like growth factor-1 IGF-1 and insulin-like growth factor binding protein-3 IGFBP-3 have been noted in EHPVO patients, suggesting growth hormone resistance. 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.

Thapa BR, Mehta S.

Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen.