Ultrasonography in patients with Budd-Chiari syndrome – Diagnostic signs and prognostic implications

Purpose: We analysed sonomorphological signs of Budd-Chiari syndrome (BCS) and their potential for prognosis prediction in a single referral center over a 7-year period.

Patients and methods: Forty-five consecutive patients were included. A uniform therapeutic strategy was applied, consisting of anticoagulation as well as a symptom and response-triggered use of recanalization and/or portosystemic shunt therapy and liver transplantation. Analysis included the frequencies of sonomorphological signs and their predictive value for diagnosis of BCS, mean values of laboratory and colour Doppler data in different therapeutic groups and survival.

Results: Specific ultrasound signs were identified at level of the hepatic veins in 71% of the patients and in 33% at level of the caval vein, i.e. thrombosis, stenosis, fibrotic cord or insufficient recanalization of the vessels. The frequent non-specific signs were splenomegaly (78%), inhomogeneous liver parenchyma (76%), intrahepatic collaterals (73%), caudate lobe hypertrophy (67%), ascites (56%) and extrahepatic collaterals (44%). The combination of specific signs and „caudate lobe hypertrophy“ offered the highest predictive value to identify patients with BCS (p=0.014) with a specifity of 100%. Mean survival was significantly different between the patients with or without portal hypertension (n=25, 41.1±7.6, 95%CI (26.2–55.9) versus n=20, 89.4±4.5, 95%CI (80.5–98.2), p=0.004) and with or without portal vein thrombosis (n=12, 29.8±10.7, 95%CI (8.9–50.7) versus n=33, 79.3±6.1, 95%CI (67.4–91.1), p=0.003).

Conclusions: We present a comprehensive description of sonomorphological signs in BCS. The combination of ultrasound signs „altered hepatic and/or caval veins“ and „caudate lobe hypertrophy“ was the best to diagnose BCS. Patients with portal vein thrombosis or portal hypertension have a poor prognosis.

Keywords: Hepatic outflow obstruction, ultrasound, survival