Name
BUDD-CHIARI SYNDROME
DESCRIPTION
DETAIL
CAUSES : - THROMBOSIS OF HEPATIC VEINS, OFTEN IN THE SETTING OF POLYCYTHEMIA VERA, MYELOPROLIFERATIVE SYNDROMES, PAROXYSMAL NOCTURNAL HEMOGLOBINURIA - ANTIPHOSPHOLIPID ANTIBODY SYNDROME - ESSENTIAL THROMBOCYTOSIS - ORAL CONTRACEPTIVES OR OTHER HYPERCOAGULABLE STATES. - INVASION OF INFERIOR VENA CAVA BY TUMOR SUCH AS RENAL CELL OR HEPATOCELLULAR CARCINOMA, LEIMYOSARCOMA, ADRENAL CARCINOMA, WILMS TUMOR, RT ATRIAL MYXOMA - IDEOPATHIC MEMBRANOUS OBSTRUCTION OF INF VENA CAVA - HEPATIC IRRADIATION - TT WITH CERTAIN ANTINEOPLASTICS OR INGESTION OF PYRROLIDIZINE ALKALOIDS PRESENT IN CERTAIN HERBAL TEAS ( BUSH TEA DISEASE) CAN CAUSE VENOCLUSIVE DISEASAE AFFECTING SUBLOBULAR BRANCHES OF HEPATIC VEINS & HEPATIC VENULES - PREGNANCY - CHR. INFLAMMATORY DISEASES - CLOTTING DISORDERS - INFECTIONS LIKE * BEHCET DISEASE * INFLAMMATORY BOWEL DISEASE * SARCOIDOSIS * SLE * SJOGREN SYNDROME * MIXED CONNECTIVE TISSUE DISORDERS - INHERITED THROMBOTIC DIATHESIS LIKE * PROTEIN C DEFICIENCY * PROTEIN S DEFICIENCY * ANTITHROMBIN III DEFICIENCY * FACTOR V LEIDEN DEFICIENCY - MISCELLANEOUS LIKE * ALPHA 1-ANTITRYPSIN DEFICIENCY * TRAUMA * DACARBAZINE * URETHANE - IDIOPATHICOTHER TESTS : * ASCITIC FLUID EXAMINATION : PROTEIN CONC>2 GM/DL, WBC < 500 * HEPATIC VENOGRAPHY OR LIVER BIOPSY SHOWING CENTRILOBULAR CONGESTION & SINUSOIDAL DILATATION IN THE ABSENCE OF RT. SIDED HEART FAILURE ESTABLISHES DIAGNOSIS * COLOR FLOW DOPPLER ULTRA SOUND : SENSITIVITY & SPECIFICITY 85 - 90% * MRI - SENSITIVITY & SPECIFICITY 85 - 90%
TYPENOTES
* FOUR DIFFERENT FORMS SEEN - ACUTE, SUBACUTE, CHRONIC & FULMINANT FORM Medical Care: Medical therapy can be instituted for short-term, symptomatic benefit. Medical therapy alone is associated with a high 2-year mortality rate (80-85%). " Management of ascites. " Anticoagulation " Antithrombolytic therapy: This has been used in a few cases. Agents include streptokinase, urokinase, recombinant tissue plasminogen activator, and other modalities. " Angioplasty: This can help relieve obstruction caused by membranous webs. Surgical Care: Offer decompression of the hepatic vasculature if portal hypertension is the cause of the symptoms. Either surgery or a transjugular intrahepatic portosystemic shunt procedure can be performed. Offer liver transplantation if decompensated liver cirrhosis is present. DRUG TREATMENT : 1. ANTICOAGULANTS - WARFARIN 2. FIBRINOLYTIC AGENTS : - STREPTOKINASE - UROKINASE - ALTEPLASE
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
X-RAY CHEST P.A. VIEW( NORMAL ), ULTRA SOUND WHOLE ABDOMEN - MALE, COMPLETE BLOOD COUNT, ECHOCARDIOGRAPHY, ECG, BIOPSY