Diagnosis and Management of Budd Chiari Syndrome: An Update

Abstract

Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.

Budd-Chiari syndrome and liver transplantation

“…Doppler ultrasonography, contrast-enhanced triphasic computed tomography, and magnetic resonance imaging are sufficient to reveal these diagnostic features of BCS (31,34). Nowadays, there is no call for direct or retrograde venography solely to diagnose BCS, but patients with BCS are usually referred to an interventional radiologist to confirm the diagnosis and for therapeutic interventions (35).…”

“…In contrast, findings associated with portal hypertension such as splenomegaly, esophageal varices, encephalopathy, and gastrointestinal bleeding are more commonly seen in the chronic form. In some patients, the hepatic sinusoids may be completely decompressed via large portosystemic and intrahepatic collaterals so that they are completely asymptomatic (31).…”

Successful Angioplasty of a Hepatic Vena Caval Branch in a Patient with Budd-Chiari Syndrome Due to Occlusion of the Three Major Hepatic Veins

“…The main risk after stent deployment is migration. Therefore, stenting should be performed for lesions that respond inadequately to balloon dilatation, immediate recoil, or recurrent stenosis or occlusion [2].…”

“…The classical symptom triad seen in BCS includes abdominal pain, ascites, and hepatomegaly, but patients can present with other symptoms, such as liver dysfunction, jaundice, portal hypertensive gastroenteropathy, gastroesophageal varices, splenomegaly, thrombocytopenia, venous thrombosis, and leg edema/ulcers [2,3]. These conditions are usually managed using diuretics, anticoagulants, sodium restriction, and other palliative measures, although such treatments often fail or are only transiently effective [2].…”

“…Therefore, PTV is preferred if any of the HV are accessible, while portacaval shunting should be performed otherwise [5]. Liver transplantation is performed to treat patients with fulminant hepatic failure or those who are refractory to other invasive treatments [2]. PTV of a single HV is the treatment of choice for BCS when a patient has isolated HV occlusion [3], and PTV is most successful when performed on the least obstructed of the three major HV [5].…”