Nejm 2004;350:578; J Clin Gastro 2000;30:155; Brit J Surg 1995;82:1023

Cause: Budd-Chiari syndrome is caused by obstruction of the normal hepatic venous outflow. This can occur at the level of the hepatic veins or in the vena cava. Many diseases can result in venous obstruction. Mechanical causes include congenital membranous obstruction of the IVC (common in Asia) or posttraumatic, postsurgical, or invading malignancy such as HCC. Hypercoagulable causes include paroxysmal nocturnal hemoglobinuria; polycythemia vera; other myeloproliferative disorders; deficiencies of protein C, S, or antithrombin III; estrogens; or other hypercoagulable states. About 30% of cases are idiopathic.

Epidem: In a large U.S. series, 83% of pts were women, and the mean age was 37 yr (range 14-68) (Ann Surg 1990;212:144).

Pathophys: There are 3 hepatic veins that drain the liver. The course of the Budd-Chiari syndrome is determined by the degree of obstruction of these veins and the acuity of onset. When obstruction occurs, there can be increase in the direct flow from the caudate lobe to the IVC. This results in caudate hypertrophy that may be seen on imaging studies. Venous obstruction causes hypoxia and increased sinusoidal pressure, with resultant hepatomegaly and varying degrees of liver failure. When onset is sudden and massive, FHF may occur (p 30). When the obstruction is gradual or limited, portal HTN (ascites and varices) is a greater problem than hepatocellular failure.

Sx: The sx can vary widely, as indicated. There may be evidence of RUQ pain from Glisson’s capsule distention, distention from ascites, and a complaint of generalized weakness (Lancet 1993;342:718).

Si: Ascites is almost universal. Tender hepatomegaly is common with acute presentations. Prominent veins may be seen over the abdominal wall or back. Those with acute presentations may also have evidence of hepatic encephalopathy or hepatorenal syndrome. Those with chronic presentations predominantly have findings of portal HTN and cirrhosis. Leg edema is common with IVC obstruction.

Crs: Mortality is almost universal if the disease is nottreated. The course varies widely depending on the extent and acuity of obstruction and the underlying predisposing condition. If hepatic venous drainage can be restored, the majority of pts survive long term (Gut 1999;44:568).

Cmplc: Liver failure, variceal hemorrhage, encephalopathy.

Diff Dx: The disease needs to be considered in any pt presenting with ascites and a tender liver. Some pts present with evidence of portal HTN or cirrhosis. The usual pitfall in dx is to fail to consider this unusual disease. Once this disease is considered, the dx can usually be made by imaging studies. The broad diff dx is that of jaundice or cirrhosis (p 30).