Obliterative portal venopathy in cystic fibrosis-related liver disease without cirrhosis

Obliterative portal venopathy without cirrhosis in explants from 2 patients (ages 10 and 16.8 years) with portal hypertension and nodular cystic fibrosis-related liver disease on imaging. For some patients with this phenotype, the portal hypertension can be managed with transjugular intrahepatic portosystemic shunt (TIPS) rather than transplantation.
(A) Nodular regenerative hyperplasia (centrolobular sinusoidal dilation). The hyperplastic hepatocytes in the center (*) compress the periphery hepatocytes into thin cords (thick arrows).
(B) Diminished portal vein diameters in portal tracts with thick coat of smooth muscle surrounding the portal vein (arrow). There is no evidence of cholestasis. (Arrow head: bile duct; dashed arrow: hepatic artery.)