Article Text
Statistics from Altmetric.com
See article on page 568
The article by Fisher et al(see page 568) on the various techniques that may be used in the management of Budd-Chiari syndrome is timely, and demonstrates some important points. This condition must be considered as part of the differential diagnosis of ascites in all patients, though the mode of presentation may be protean and hence may trap the unwary. The classic syndrome of ascites, hepatomegaly and abdominal pain occurs with obstruction of hepatic venous outflow at any level—from the hepatic venules (e.g. veno-occlusive disease) to the suprahepatic vena cava. Constrictive pericarditis may also mimic the presentation. The syndrome may run a number of clinical courses which also partly determine treatment. The applicability of any treatment is also influenced by the fact that the inferior vena cava may be blocked in 20% of cases, and portal vein thrombosis evident in 10%.
The patient may present with fulminant hepatic failure, with notable coagulopathy, encephalopathy and renal impairment. A more indolent presentation is also well recognised, with ascites with a high protein content, mildly impaired hepatic function, and a variable degree of fibrosis on biopsy. Finally, well compensated patients with hepatic vein obstruction and no ascites have been described; the development of numerous intrahepatic collaterals with time is said to account for this difference. …