Membranous Budd-Chiari syndrome in Caucasians

Abstract

Objective: Budd-Chiari syndrome (BCS) is characterized by an obstruction of hepatic venous outflow. Membranous obstruction of the inferior vena cava (IVC) is a curable cause of primary BCS but is very rare in Western Europe. To date, there is only very limited information on membranous BCS in the Western world. We here report the diagnosis and management of five Caucasian patients with membranous BCS.

Material and methods: Out of 23 patients with BCS diagnosed between 2004 and 2007 we identified five with a membranous web of the IVC. Diagnostic evaluation of BCS included laboratory tests, ultrasound Doppler imaging, CT and MRI.

Results: The clinical presentation of membranous BCS was heterogeneous. The time frame from first clinical symptoms to diagnosis ranged from 3 weeks to 60 years. Liver cirrhosis was misdiagnosed in 4/5 patients. CT did not establish the correct diagnosis of membranous BCS in any of our patients. In contrast, abdominal Doppler ultrasonography showed collaterals and a web in the IVC which was confirmed by Doppler-MRI and hepatovenography. Four patients underwent interventional treatment with balloon dilatation of short-segment venous stenoses or complete occlusions. Therapy was successful: in all cases it resulted in a normalized extrahepatic blood flow and reduction of spleen size.

Conclusions: Membranous BCS may be underdiagnosed in Caucasians. Doppler ultrasound should be used as the initial diagnostic procedure for membranous BCS. Although CT is considered the "gold standard" in addition to angiography, it could not detect membranous obliteration in our cases. Patients can be effectively treated by interventional endovascular therapy.