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Chronic Budd–Chiari Syndrome
and Abdominal Varices

N engl j med 357;25：e29

44岁男性，20年慢性肝病史，反复上消化道出血，意识状态进行性恶化。

44-year-old man with a 20-year history of chronic liver disease presented with recurrent
acute upper gastrointestinal bleeding and worsening mental status. He was emaciated, with marked abdominal
distention due to ascites, and had prominent superficial veins over the anterior abdominal wall, with flow
directed upward. Liver-function tests were elevated, with an aspartate aminotransferase level of 240 U per liter (normal
range, 0 to 35), an alanine aminotransferase level of 367 U per liter (normal range, 0 to 35), a total bilirubin
level of 4.6 mg per deciliter (78.7 μmol per liter) (normal range, 0.3 to 1.0 [5.1 to 17.1]), and an albumin level of 1.8 g
per deciliter (normal range, 3.5 to 5.5). Upper gastrointestinal endoscopy showed large esophagogastric varices and
portal gastropathy. Computed tomography revealed a suprahepatic inferior vena caval web. Severe portal hypertension
with variceal bleeding and encephalopathy due to cirrhosis of the liver may be caused by chronic obstruction of the
hepatic venous outflow tract, such as that which may occur as a result of an inferior vena caval web. An inferior vena
caval web is a not-uncommon cause of chronic (or acute) Budd–Chiari syndrome in India. In this case, percutaneous
transfemoral recanalization of the inferior vena caval web was attempted but failed. The patient died at home from
variceal bleeding a few days later.

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