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Budd Chiari Syndrome

Budd Chiari Syndrome is characterized by congestive hepatopathy and thrombotic or non-thrombotic obstruction of hepatic venous outflow. This condition is uncommon and occurs in about 0.001% of the population. Budd Chiari Syndrome can have a multifactorial etiology, with primary myeloproliferative diseases being the leading cause and hypercoagulable state occurring in about 75% of patients. The primary form of this condition arises from endoluminal venous lesion-like thrombosis. The secondary form of Budd Chiari Syndrome develops from neighboring structures. While Budd Chiari Syndrome is potentially lethal, it can be cured.

Budd Chiari Syndrome Condition Overview

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Budd Chiari Syndrome Diagnosis

The initial diagnostic procedure for Budd Chiari Syndrome is doppler ultrasonography to estimate blood flow. At least two hepatic veins must be blocked or have reversed or turbulent flow to indicate the presence of disease. Patients with Budd Chiari Syndrome may present with massive ascites or abrupt onset of ascites, unexplained chronic or thrombogenic liver disease, or fulminant hepatic failures. Computed tomographic (CT) scans or magnetic resonance imaging (MRI) should be used as a secondary diagnostic tool. MRIs give the advantage of showing the hepatic vein thrombosis and allow for easier evaluation of the IVC. Liver biopsy can be used for differential diagnosis such as right-sided heart failure, infectious hepatitis, metastatic liver disease, cystic fibrosis, and other conditions.

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Dr. Aditya Bagrodia interviews Dr. Vitaly Margulis, professor of urology at UT Southwestern Medical Center, about locally advanced kidney cancer. They discuss various topics including classification of locally advanced kidney cancers, various imaging modalities for staging cancer, special considerations for tumor-thrombus formation, targeted therapy vs. checkpoint inhibitors, and robotic vs. open nephrectomies.

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Budd Chiari Syndrome Treatment

Treatment strategies for Budd Chiari Syndrome become more invasive as the condition escalates. The main goals are to alleviate obstruction, prevent clot progression, limit progressive liver injury, and manage other complications. Initial treatments are targets towards the complications of portal hypertension and other underlying diseases. Then, treatments focus on correcting the hepatic venous outflow obstruction. Therapeutic interventions rely on anticoagulants, but thrombolysis and stenting are other options that have proved successful. Acute forms of Budd Chiari Syndrome may be treated with a transjugular intrahepatic portosystemic shunt or surgical decompression. In extreme cases, where all other therapies fail, patients can receive a liver transplant.

Budd Chiari Syndrome Articles

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Renal cell carcinoma with tumor thrombus in the interior vena cava

Tumor thrombus formation is common in locally advanced kidney cancer, and can lead to bland thrombus, pulmonary emboli, and even Budd-Chiari syndrome. The risk of pulmonary emboli creates unique challenges during kidney cancer surgery.

References

[1] Aydinli, M., & Bayraktar, Y. (2007, May 21). Budd-Chiari Syndrome: Etiology, pathogenesis and diagnosis. World journal of gastroenterology. Retrieved October 18, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4147117/
[2] Hernandez-Gea, V., De Gottardi, A., Leebeek, F. W. G., Ratou, P.-E., Salem, R., & Garcia-Pagan, J. C. (2019, July 1). Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. Journal of Hepatology. Retrieved October 18, 2021, from https://www.journal-of-hepatology.eu/article/S0168-8278(19)30130-8/fulltext

Disclaimer: The Materials available on https://www.BackTable.com are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.

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