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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In addition, extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins resulting in formation of ectopic varices, and portal biliopathy.
After hemodynamic resuscitation all such patients should sarjn endotherapy [either sclerotherapy EST or band ligation EVL ]. Another study from Lucknow  documented growth retardation height less than 5th percentile for age in Frequency of gastropathy and gastric varices in children with extrahepatic portal venous obstruction treated with sclerotherapy.
Poddar U Borkar V. 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. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal ehpgo, hypersplenism, and growth retardation.
In another study in 20 children with EHPVO, Dubuisson sarrin al  have shown that almost half of the patients had protein- C, protein-S and anti-thrombin III deficiencies but their levels were normal in all parents, suggesting that these deficiencies are not genetic in origin.
Idiopathic portal hypertension and extrahepatic portal venous obstruction.
Diagnosis is often clinical, supported by simple radiological tools. EST remains the only therapeutic option in children younger than 2 years of age due to insertion difficulty with a comparatively larger banding cylinder. Improved body mass index after mesenterico-portal bypass. Of the two modalities of endotherapy, EST is an established modality to tackle varices in children Table 1. However, since most of the patients are asymptomatic, this approach is recommended only if a therapeutic intervention is contemplated.
In patients with endoscopic failure, a staged procedure portosystemic shunt followed by biliary surgery should be preferred. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.
There is no report of post shunt encephalopathy in EHPVO cases and re-bleed occurs only when the shunt is blocked. There is no controversy about the management of acute variceal bleeding. Most of these bleeding episodes occurred within the first 4 years of variceal eradication. Etiological spectrum of esphageal varices due to portal hypertension in Indian children: Diminished portal blood flow results in decreased insulin delivery to the liver and thereby decreased production of insulin-like growth factor-1 IGF-I and insulin-like growth factor binding protein-3 IGFBP Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO.
Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction; a year follow-up. This is the most physiological shunt as it restores the hepatic blood flow.
Consensus on extra-hepatic portal vein obstruction.
Is it superior to sclerotherapy in children with extrahepatic portal venous obstruction? Surgical guidelines for the management of extra-hepatic portal vein obstruction.
A longer interval 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. Mesoportal bypass for extrahepatic portal vein obstruction in children: Bleeding sarn extrahepatic portal vein obstruction.
Non-cirrhotic portal hypertension – diagnosis and management.
A prospective study of endoscopic esophageal variceal ligation using multiband ligator. However, with the availability of new mesenterico-portal mesenterico-left portal-bypass or Rex shunt, the problems of conventional shunts have been largely overcome. Management of gastric varices and portal hypertensive gastropathy. Two possible mechanisms have been proposed. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: Surgery is primarily indicated when endotherapy fails to control bleeding, in presence of gastric or ectopic varices not amenable to endoscopic management and with delayed sequelae like portal biliopathy and rectal varices.
Management of esophageal varices. Non-cirrhotic portal fibrosis in children. Colonic changes in patients with cirrhosis and in patients with extrahepatic portal vein obstruction.
Variceal bleed and splenomegaly are the commonest presentations.
Bleeding from rectal varices can be managed with sclerotherapy or band ligation. Cholestasis in children with portal vein obstruction. Management of colorectal varices The effect of increased portal pressure in EHPVO is not localized to the esophagus and stomach; it affects saein entire gastrointestinal tract.
Management of gastric varices and portal hypertensive gastropathy Gastric varices: