Gut 1986 Sep;27(9):1101-5

Budd-Chiari syndrome presenting as fulminant hepatic failure

Powell-Jackson PR, Ede RJ, Williams R

Abstract
Two cases of the Budd-Chiari syndrome are described in whom the diagnosis was
finally confirmed at necropsy. The presentation was with encephalopathy,
occurring within eight weeks of first symptoms and coming therefore within the
definition of fulminant hepatic failure. In one, thought to have non-A, non-B
hepatitis, encephalopathy progressed to grade 4 coma with death 12 days after
presentation. In the other, mistakenly thought to have intra-abdominal
malignancy, an exploratory laparotomy exacerbated the encephalopathy with death
three weeks later. In neither case were non-invasive investigations, such as
ultrasound and isotope scanning, carried out which might have facilitated an
earlier diagnosis and consideration for orthotopic liver transplantation,
probably the most appropriate form of therapy for these very severe case