Histopathology of Budd–Chiari Syndrome

Abstract

The histopathological changes in Budd–Chiari syndrome (BCS) overlap with those of sinusoidal obstruction syndrome (SOS) and of cardiac or pericardiac disorders resulting in right cardiac failure. These conditions, however, are different on both clinical and pathological grounds and need to be differentiated from BCS. This review is centred on the three main aspects of BCS in diagnostic liver histopathology: (1) general histopathology of BCS; (2) implications for liver biopsy interpretation; and (3) BCS in the liver allograft. The histological features of BCS form a complex spectrum which is shaped differently in each individual case according to the topographical distribution and chronological evolution of the obliterative insult, its upstream effect of the hepatic vascularisation and the consequent parenchymal injury, scarring and remodelling. Sampling variation limits the use of liver biopsy for prognostication in patients with BCS.

Keywords

• Budd–Chiari syndrome
• sinusoidal dilatation
• congestion
• perisinusoidal fibrosis
• nodular regenerative hyperplasia
• parenchymal extinction