Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sar...

OBJECTIVES Budd-Chiari syndrome is a rare and serious complication of Behçet's disease, and is the result of occlusion of the major hepatic veins, the adjacent inferior vena cava, or both. The aim of this study was to determine the prevalence, clinical and laboratory findings, and treatment and clinical course of Budd-Chiari syndrome associated with Behçet's disease. METHODS We analyzed retro...

Primary Budd-Chiari syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assess...

Neurologic sequalae of Noonan syndrome have been postulated in the literature. A topic significance is role RASopathy shared pathophysiology Syndrome and Chiari I malformation. In this unique case report, we present a patient with concomitant 4th ventricular outflow obstruction. The highlights importance close clinical suspicion population. We utilize to delve into intricacies known encourage o...

Editor: In the Talal Amer and Omran M. El-Shmam review published in your journal,1 there are two types of classification for Chiari I malformation: type A, which is associated with syringomyelia and type B, which is not associated with syringomyelia. Hans Chiari (1851–1916) first reported Chiari malformation (1891) morphologically as a cerebellar tonsillar herniation with occasional fourth vent...

To the Editor, We read with interest the article by Shukla and Bhatia [1] in a recent issue of the Journal. The authors found that 6 of their patients could not undergo the standard radiological procedures because of technical reasons; reasons for this technical failure were however, not provided. In our experience of adult patients, the TIPSS procedure is usually possible. Of 58 patients who u...

Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. This case report describes the response to eculizumab (a humanized monoclonal antibody that inhibits terminal complement activation) in a 25-y...

budd-chiari syndrome (bcs) refers to thrombosis of hepatic veins as well as intrahepatic or suprahepatic inferior vena cava. we present for the first time a case of possible occurrence of budd- chiari syndrome with the history of depot medroxy progesterone acetate (dmpa) injections in a 33-yearold iranian woman. an underlying disorder can be identified in most of patients with bcs. many of thes...