Image 6 Shows a normal portal triad. Image 7 Shows increased perisinusoidal reticulin. Diagnosis Hepatoportal Sclerosis Discussion Hepatoportal sclerosis is essentially portal hypertension due to increased presinusoidal pressure in the absence of cirrhosis. Hepatoportal Sclerosis without an apparent cause in the USA is rare, but when it does occur, it is often misdiagnosed. There is a childhood variant with onset as young as 20 months.
Patients typically have splenomegally or, less commonly, ascites or no symptoms at all.
Portal vien thrombosis is present in a number of patients. Liver function studies are normal or there is mild elevation in Alkaline Phosphatase. Pancytopenia is also seen. Hepatic vein pressure gradient may be normal or increased. Portal venous pressure is higher than wedged hepatic vein pressure.