Budd–Chiari syndrome

Budd–Chiari syndrome

The Budd–Chiari syndrome a ff ects 1/1 /uni00A0 000 /uni00A0 000 adults and is a collective term for conditions that impede hepatic venous outflow at any level from the small hepatic veins to the junction of the IVC with the right atrium. Cardiac and pericardial diseases and sinusoidal obstruction syndrome are excluded from this definition. It principally a ff ects young women, who present the classic triad of abdominal pain, ascites and hepato megaly . A hypercoagulable condition such as antithrombin 3, protein C or protein S deficiency is identified in 75% of patients, extrinsic compression in 25% and rarely congenital or acquired IVC webs. The liv er becomes acutely congested, with impaired liver function and portal hypertension; ascites and oesophageal var ices develop. Fulminant hepatic failure may result fr om acute thrombosis but in the majority of cases abdominal discomfort and ascites are the main presenting features. If chronic, the liver progresses to established cirrhosis. Colour and pulsed Doppler ultrasonography and CT scanning together with detailed hae matological studies will usually identify the cause. The diagno sis should be suspected in patients with ascites where a CT scan demonstrates a large, congested liver or cirrhosis with gross enlargement of the caudate lobe resulting from preservation and hypertr ophy of the segment due to direct venous drainage to the IVC. Further IVC compression or occlusion and portal George Budd , 1808–1882, Professor of Medicine, King’s College Hospital, London, UK. Hans Chiari , 1851–1916, Austrian pathologist, later Professor at the University of Strasbourg, France. Christian Johann Doppler , 1803–1853, Professor of Experimental Physics, Vienna, Austria, enunciated the ‘Doppler principle’ in 1842. Jacques Caroli , 1902–1979, Professor of Medicine, Hôpital St Antoine, Paris, France, described the disease in 1958. Jean-Martin Charcot , 1825–1893, French neurologist and Professor of Pathology , Hôpital Pitié-Salpêtrière, Paris, France. hypertrophy . Treatment of Budd–Chiari syndrome depends on the stage of disease at presenta tion and the specific findings in each patient. Fulminant liver failure , established cirrhosis and complications of portal hypertension may require liver trans - plantation. If the liver parenchyma is relatively normal TIPSS or a side-to-side portocaval shunt should be considered and IVC compression relieved by the insertion of a retrohepatic expandable metallic stent. W ith e ff ective Budd–Chiari syn - drome treatment prognosis depends on whether it is possible to treat the underlying pathology . Patients usually require lifelong anticoagulation.