Etiological spectrum of esophageal varices due to portal hypertension in Indian children: Is it different from the West?

Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion. ( J CLIN EXP HEPATOL 2015;5:22-40)

show abstract

“…67,68 Bleeding usually occurs in the first or second decade of life. The bleed is well tolerated due to underlying normal functioning liver.…”

Section: Chronic Portal Vein Thrombosis (Extra Hepatic Portal Venous mentioning

confidence: 99%

Portal Vein Thrombosis

Chawla

Bodh

2015

Journal of Clinical and Experimental Hepatology

151

147

View full text Add to dashboard Cite

show abstract

“…Thrombosis of the extrahepatic portal vein in the absence of cirrhosis is the major cause of portal hypertension and variceal bleeding in children in developing countries 17,18 and the second most common cause of portal hypertension in the world. 19 In these cases, the presence of variceal bleeding triggers investigations for presence of cirrhosis and its underlying cause, which may include a liver biopsy.…”

Section: Discussionmentioning

confidence: 99%

Hepatic arterial buffer response: pathologic evidence in non-cirrhotic human liver with extrahepatic portal vein thrombosis

et al. 2016

View full text Add to dashboard Cite

Increase in hepatic arterial flow in response to reduced portal flow (hepatic arterial buffer response) has been demonstrated experimentally and surgically. We provide pathologic evidence for hepatic arterial buffer response in non-cirrhotic patients with extrahepatic portal vein thrombosis and elucidate the histopathologic spectrum of non-cirrhotic portal vein thrombosis. Liver biopsies and resections from non-cirrhotic patients with extra-hepatic portal vein thrombosis were retrieved. Morphologic features, extent of CD34 staining, outer diameters, luminal diameters and wall thickness of hepatic arteries cut in cross-section and outer diameters of cross-sectioned paired bile ducts were compared with age-and gender-matched controls. There were 12 male and 9 female patients. Measurements of 280 and 193 arteries from patients and controls, respectively, demonstrated statistically significant (Po 0.05) arterial dilatation (increase in percentage of arterial lumen to outer diameter) and arterial wall thinning in resection specimens of non-cirrhotic patients with extra-hepatic portal vein thrombosis. Subtle and/or focal dilatation of central veins, portal veins and sinusoids; focal trabecular thinning/ thickening and mild ductular reaction were common findings in both the patient and control groups. Diffuse and obvious changes, and portal vein absence or attenuation were seen only in the patient group. Capillarization of sinusoids was not seen on CD34 stain. Two patients showed significant ductular reaction, one of who developed biliary strictures on follow-up. Hepatic arterial dilatation and wall thinning in non-cirrhotic patients with portal vein thrombosis provide pathologic evidence of hepatic arterial buffer response in the human liver. Obvious and diffuse sinusoidal dilatation and absence or attenuation of portal veins are highly suggestive of extrahepatic portal vein thrombosis in non-cirrhotic patients with portal hypertension. Periportal shunt vessels, hypervascular portal tracts, muscularized portal veins, large thick-walled or dilated arteries aid diagnosis but are rare findings. Normal or near-normal biopsies do not rule out portal vein thrombosis.

show abstract

“…The majority of children (85% to 90%) with EHPVO present with variceal bleeding (Arora, 1998;Poddar, 2008;Valla, 2002). The usual presentation for children with EHPVO is sudden, unexpected and, often, massive hemetemesis.…”

Section: Variceal Bleedingmentioning

confidence: 99%

“…Extrahepatic portal venous obstruction (EHPVO) is the commonest cause of portal hypertension in children and also one of the common causes in adults in India and other tropical countries (Arora, 1998;Poddar, 2008;Poddar 2000). In India, EHPVO is responsible for portal hypertension in about one third cases of adults and more than half of the cases in children (Sarin 2002;.…”

Section: Introductionmentioning

confidence: 99%