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Pathology of Budd–Chiari Syndrome and Hepatic Vein Obstruction

  • Ian R. WanlessEmail author
Chapter

Abstract

The pathology of Budd-Chiari syndrome can be summarized as evidence of hepatic vein thrombosis with secondary congestive injury and parenchymal regeneration. The parenchymal loss reflects the regional distribution of venous obstruction. Thrombosis is characterized by episodic extension, organization, recanalization, and recurrence. Congestive endothelial injury and stasis, usually with hypercoagulable state, causes disease extension. Retrograde portal vein flow may account for the high prevalence of secondary portal vein thrombosis.

The typical histologic appearance is zone 3 hemorrhage into liver cell plates and ischemic necrosis leading to veno-centric cirrhosis. The regenerative response often causes caudate lobe hyperplasia and large regenerative nodules, both of which may be mistaken for neoplasia. Hepatocellular carcinoma and liver cell adenomas also occur.

Although historically a severe disease involving most of the large hepatic veins and often vena cava, imaging allows early discovery and good prognosis.

Imaging is the preferred method of investigation. Biopsy is recommended if the site of obstruction is uncertain or there is suspicion of neoplasia. The differential diagnosis includes congestive heart failure, constrictive pericarditis, shock, injury from toxins, or radiation. Inflammation is minimal unless disease is caused by sarcoidosis or a form of vasculitis. A variety of neoplasms may be found as causative lesions.

Keywords

Biopsy Histology Hepatic vein thrombosis Portal vein thrombosis Congestive injury Congestion Ischemic necrosis Large regenerative nodule Focal nodular hyperplasia Nodular regenerative hyperplasia Hepatocellular carcinoma Veno-centric 

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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Department of PathologyDalhousie UniversityHalifaxCanada

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