[Budd-Chiari syndrome: retrospective study of 8 cases assessed with computerized tomography].

PURPOSE

Budd-Chiari syndrome is a rare disease due to the obstruction of hepatic veins and, sometimes, of the inferior vena cava. The disease, whose etiopathogenesis is often unproven, may show acute or chronic clinical course with different features at pathology. We carried out a retrospective study of 8 consecutive patients with the Budd-Chiari syndrome to assess the diagnostic role of CT and to define the main patterns of the disease.

MATERIAL AND METHODS

Our patient population consisted of 8 women with clinical and instrumental suspicion of Budd-Chiari syndrome. Three patients had acute and 5 chronic clinical presentation: the former had had acute symptoms right after delivery, while one of the latter was referred to oral contraceptive abuse, another was diagnosed 18 months after delivery and finally the cause was not known in the other 3 cases. CT was performed with contiguous slices (8 mm) of the upper abdomen, before and after i.v. contrast agent infusion. Liver images were always acquired 15-20 minutes after contrast agent administration.

RESULTS

In the acute patients, CT showed increased liver volume, diffuse parenchymal hypodensity in unenhanced scans, except for some skip areas (segments I and IV, paracaval parenchyma) and strong postcontrast enhancement. Thrombosis of the suprahepatic veins was found in all patients and partial thrombosis of the inferior vena cava in one. Abundant peritoneal effusion was always found. In the chronic patients, CT showed caudate lobe and left lobe hypertrophy, mostly associated with right lobe hypo-atrophy. Moreover the hypertrophic areas showed bright and inhomogeneous contrast enhancement, with a reversed pattern at 15-20 minutes postcontrast. Thrombosis of the suprahepatic veins was found in 3 patients and partial thrombosis of the inferior vena cava in one. Peritoneal effusion was seen in 2 cases.

CONCLUSIONS

The Budd-Chiari syndrome causes anatomical, morphological and volumetric changes in the hepatic parenchyma which are referrable to difficult venous flow and differ in acute vs chronic forms. CT permits to grade liver involvement by the organ's morphodensitometric changes, which are once again different in the acute vs the chronic disease. Moreover, thrombosis of suprahepatic veins and of the inferior vena cava and portal vein is often demonstrated. Therefore CT can be considered a valid diagnostic tool for use in the patients with suspected Budd-Chiari syndrome together with B-mode and color Doppler US.