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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Frequency of gastropathy and gastric varices in children with extrahepatic portal venous obstruction treated with sclerotherapy. Besides variceal bleeding, which is the commonest presentation, patients sarij have symptomatic portal biliopathy, hypersplenism, and growth retardation.

The natural history of portal hypertensive gastropathy: Despite the clear cut benefit ehpov EVL when used alone, there is a higher risk of recurrence of varices as it is difficult to ligate smaller varices, and because perforators and paraesophageal collaterals remain patent after EVL.

The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. Abstract Extrahepatic portal venous obstruction EHPVO is the commonest cause of portal hypertension and variceal bleeding in children.

J Pediatr Gastroenterol Nutr. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Hence, more attention is now being paid to the other effects of portal hypertension on the gastrointestinal tract, namely gastric varices and portal hypertensive gastropathy PHG.

By using EVL as a primary treatment, one can achieve rapid eradication or down gradation of varices with fewer complications and by using low-dose EST following EVL, one can block perforators and paraesophageal collaterals, thereby reducing the risk of recurrence. A longer ehpbo up to 1 year and documentation of a patent shunt with decompressed collaterals on color Doppler or MR angiography may help in determining the optimum time for surgery.


Extrahepatic portal vein obstruction.

Portal-vein obstruction in children leads to growth retardation. Hence the management of EHPVO is no longer restricted to the management of variceal bleeding alone; it encompasses the management of growth failure, portal biliopathy, colorectal varices, massive splenomegaly with its associated complications like infarction, hypersplenism, physical disability due to pain, early satiety etc. It may present with occult bleed or as an unusual cause of overt gastrointestinal bleeding.

Indeed, EVL has become the preferred mode of treatment shpvo variceal bleeding in adults. Management of portal biliopathy Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO.

Surgical guidelines for the management of extra-hepatic portal vein obstruction. In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis.

The risk of bleeding is more with secondary gastric varices varices appearing after eradication of esophageal varicesisolated gastric varices IGV1 and gastroesophageal varices along the greater curvature of stomach GOV2. Report of the Baveno IV consensus workshop on methodology of diagnosis asrin therapy in portal hypertension. Primary biliary tract surgery has significant morbidity and mortality due to extensive collaterals around the bile ducts.

Surgical shunts revert the complications secondary to PHT. This observation again supports the compression theory in most cases of portal biliopathy.

Extrahepatic portal vein obstruction.

Portal hypertension in north Indian children. Further the prevalence of rectal varices is more in EHPVO than in cirrhosis, probably due to the duration of portal hypertension or selective redistribution of portal pressure along the inferior mesenteric vein consequent to thrombosis at the junction of splenic and superior mesenteric veins.


Of the two modalities of endotherapy, EST is an established modality to tackle varices in children Table 1. Endoscopic band ligation followed by sclerotherapy. Results of surgical portosystemic shunts. Magnetic resonance MR imaging with intravenous gadolinium injection delineate the cavernoma and biliary changes simultaneously and may be extremely useful in children. These findings suggest diminished anabolic action of growth hormone on muscle growth affecting the lean muscle mass, and its lipolytic effect resulting in decreased adiposity.

Prevalence, classification and natural history of gastric varices: Regarding endotherapy for variceal bleeding, there is no doubt that band ligation is superior to sclerotherapy. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: Conventional shunts proximal or distal splenorenal are not possible in almost one-third of cases due to blockage of splenic vein SV or small SV.

The exact mechanism of the growth failure is not known. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction.

Consensus on extra-hepatic portal vein obstruction.

Extrahepatic portal venous obstruction. Portal hypertensive gastropathy in non-cirrhotic patients. 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. Is it superior to sclerotherapy in children with extrahepatic portal venous obstruction?