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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The first ninety patients. IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions.

In a study of five cases, Dhiman et al [49 ] showed that sarih ERCP done after weeks of shunt surgery demonstrated total disappearance of cholangiographic changes in one, partial disappearance in two and no change in remaining two cases.

There are two main approaches to prevent further bleed: 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. Mesoportal bypass for extrahepatic portal vein obstruction in children: Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors.

J Pediatri Gastroenterol Nutr. Surgical guidelines for the management of extra-hepatic portal vein obstruction. The prevalence and spectrum of colonic lesions in srain with cirrhosis and noncirrhotic portal hypertension. Frequency of gastropathy and gastric varices in children with extrahepatic portal venous obstruction treated with sclerotherapy. Other indications of shunt surgery include symptomatic hypersplenism, growth retardation, portal biliopathy, massive splenomegaly affecting the quality of life, rare blood group, and remote area of residence.


PHG classically described in cirrhosis.

There is a paradigm shift in EHPVO management towards shunt surgery after the introduction of mesenteric-left-portal vein bypass or Rex shunt as it provides complete cure of the condition. The effect of lienorenal shunts.

Non-cirrhotic portal hypertension – diagnosis and management.

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.

Most of these bleeding episodes occurred within the first 4 years of variceal eradication. The only pediatric study by Gauthier-Villars et al [53] 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. After hemodynamic resuscitation all such patients should undergo endotherapy [either sclerotherapy EST or band ligation EVL ].

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Surgical shunts revert the complications secondary to PHT. Poddar U Borkar V. Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass. Nevertheless, EST cannot be totally ruled out as a therapeutic modality, especially in children.

Consequently recurrence of varices has been shown to be higher with EVL. The low prevalence in pediatric studies clearly suggests that the duration of portal hypertension plays an important role in the development of rectal varices. Liver function tests took 2 to 2. In a follow up study from our institute we have shown that factor V Leiden and prothrombin gene mutations were uncommon in children with EHPVO. Portal hypertension, children, biliopathy, growth, sclerotherapy.


Management of gastric varices and portal hypertensive gastropathy. Portal-vein obstruction in children leads to growth retardation.

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 This indicates while in some patients compression plays an important role, in others ischemia alone or in combination with compression causes biliary changes.

There are many postulated etiologies of EHPVO namely umbilical sepsis, umbilical vein catheterization, abdominal trauma, surgery, intra-abdominal sepsis, dehydration, congenital agenesis or atresia of portal vein.

Non-cirrhotic portal hypertension – diagnosis and management.

Treatment of bleeding esophagogastric varices due to extrahepatic portal hypertension: In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis. The natural history of portal hypertensive gastropathy: Biliary changes in extrahepatic portal venous obstruction: Pseudosclerosing cholangitis in extrahepatic portal venous obstruction.

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.

Further research is needed to understand the pathogenesis and natural history of these disorders.