Budd-Chiari syndrome

The Budd-Chiari syndrome refers to the clinical picture that occurs when there is obstruction of the hepatic veins. The classic acute presentation is with the clinical triad of ascites, hepatomegaly and abdominal pain, although this is non-specific. 

Demographics and clinical presentation

Budd-Chiari syndrome is rare, but the exact frequency is unknown. In Western populations, the commonest cause is thrombosis. Membranous webs have been increasingly described in Asian patients as a cause of obstruction.

The presentation may be acute or chronic:

• acute : results from an acute thrombosis of the main hepatic veins or the IVC. patients may present with rapid onset ascites.
• chronic : the chronic form is related to fibrosis of the intrahepatic veins, presumably related to inflammation

Pathophysiology

The aetiology is mixed and varied. The majority of cases result from thrombosis within the hepatic veins. However, 25% result from external compression that results in obstruction:

• idiopathic : one third of cases
• congenital : web in hepatic vein, interruption of the diaphragm
• venous thrombosis : secondary to dehydration, septicemia, polycythemia rubra vera, antiphospholipid syndrome, pregnancy and the postpartum state, oral contraceptive pill use, sickle cell disease, thrombocytosis, paroxysmal nocturnal hemoglobinuria
• injury and/or inflammation : caused by phlebitis, autoimmune disease, or tumour invasion (renal cell carcinoma, hepatocellular carcinoma, adrenal carcinoma) or  leiomyosarcoma of IVC

Associations

• concurrent portal vein thrombosis

Radiographic features

CT

• inhomogeneous mottled liver with delayed enhancement in the periphery of the liver and around the hepatic veins (nutmeg liver)
• peripheral zones of the liver may appear hypoattenuating because of reversed portal venous blood flow
• caudate lobe enlargement and increased contrast enhancement compared with the remainder of the liver
• inability to identify hepatic veins

Ultrasound

• gallbladder wall thickening, ascites, patchy liver echo pattern, splenomegaly, hypertrophied caudate lobe

Colour-flow doppler

• part of or the entire right hepatic vein with no flow or inappropriately directed flow
• discontinuity between the main hepatic vein and the IVC
• reversed flow in hepatic veins and intra and extrahepatic collaterals
• portal vein changes such as hepatopetal or hepatofugal flow
• low or no flow in the IVC or balanced bidirectional flow
• thrombus or tumour within the IVC

Venography

• complete occlusion of hepatic veins
• stenosis / focal stenosis in intrahepatic IVC may or may not be associated
• spider web appearance and intrahepatic collateral veins

Etymology

Initially described by Budd in 1845 before Chiari lent his first pathological description of "obliterating endophlebitis of the hepatic veins" in 1899.

Treatment and prognosis

Management options include

• long term anticoagulation
• hepatic venoplasty - stenting
• IVC plasty - stenting
• transjugular intrahepatic portosystemic shunts