Extra- and Intrahepatic Portal Hypertension without Cirrhosis (Hepatoportal Sclerosis)

Mikkelsen, William P.; Edmondson, Hugh A.; Peters, Robert L.; Redeker, A G; Reynolds, Telfer B.

doi:10.1097/00000658-196510000-00006

Cited by 286 publications

(132 citation statements)

References 17 publications

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“…In addition, all these features may be present in the entity called 'hepatoportal sclerosis' or 'obliterative portal venopathy', which is characterized by obliteration of the intrahepatic branches of the portal vein in the presence of a patent extrahepatic portal vein. 24,25 The finding of 'periportal shunt vessels' in two specimens from the control group may reflect tangential cuts of branching portal veins and the associated difficulty of accurately defining a periportal shunt vessel.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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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.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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“…Thus, clearly, IPH is decreasing in Japan where the ratio of cirrhosis to IPH was similar to that of India in the past. When Mikkelsen et al 6 documented hepatoportal sclerosis in Los Angeles, there were 36 cases seen in the preceding 18 years, namely two cases per year, at this large liver center. Bioulac-Sage et al at Bordeaux University, France, saw 10 cases of hepatoportal sclerosis in 8 years.…”

Section: See Article On Pagementioning

confidence: 95%

“…4,5 Hepatoportal sclerosis (HPS) is its counterpart in the USA as documented by Mikkelsen et al 6 Banti 7 described a new disorder characterized by splenomegaly, anemia and subsequent development of gastrointestinal bleeding and ascites, terminating in death due to hemorrhage or hepatic failure. He thought that this was primarily a disease of the spleen and emphasized fibroadenia of the spleen as the pathognomonic feature.…”

Section: See Article On Pagementioning

confidence: 99%

Non‐cirrhotic portal hypertension: Why is it so common in India?

Okuda

2002

J of Gastro and Hepatol

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“…Nevertheless, hepatic dysfunction and encephalopathy have been reported by Mikkelsen et al 130 with or without portal diversion in patients with extrahepatic portal block; in two such patients Warren et al 125 achieved reversal of encephalopathy after disconnecting the portalsystemic shunts and restoring the hepatopetal flow.…”

Section: The Species Factor In Encephalopathymentioning

confidence: 96%

The ECK fistula in animals and humans

Starzl¹

1983

Current Problems in Surgery

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Extra- and Intrahepatic Portal Hypertension without Cirrhosis (Hepatoportal Sclerosis)

Cited by 286 publications

References 17 publications

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

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

Non‐cirrhotic portal hypertension: Why is it so common in India?

The ECK fistula in animals and humans

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