Budd-Chiari syndrome associated with oral contraceptive steroids

Abstract

Oral contraceptive steroids (OCS) have been implicated as the cause of a number of instances of hepatic vein thrombosis (Budd-Chiari syndrome). Survival appears to be related to early diagnosis and treatment, but there does not appear to be a consensus as to the most appropriate management of these patients. Portosystemic shunting has frequently been advocated, although the results have been quite variable. Some patients appear to do well with conservative measures only. In the effort to obtain a clearer understanding of the effects of different medical and surgical therapies in this disorder, we analyzed the treatment of 47 cases associated with OCS, 29 of which were found in the literature and 18 additional cases identified through a questionnaire survey mailed to members of the AASLD. Surgery had been performed in 27 of these 47 patients (57%); 17 patients had been treated medically (36%); two individuals had received unspecified treatment, and one patient died before any treatment could be initiated. In the surgical group 13 patients underwent portosystemic shunt surgery with six surviving up to 5 years. Two patients survived more than 15 months following orthotopic liver transplant and one patient is well after partial hepatectomy. Of 10 patients who underwent exploratory laparotomy (three with the intent to perform a shunt), seven died postoperatively. Mean survival for the surgically treated group was 19.4 months (range 10 days to 7 years). Of those patients treated medically with combinations of diuretics, anticoagulants, antiplatelet agents, fibrinolytic agents, and peritoneovenous shunts to control ascites, 11 (65%) have survived from 3 months to 6 years (mean survival 29.0 months). We conclude that a satisfactory response may accompany either medical or surgical management of patients with Budd-Chiari syndrome associated with OCS. Patients with severe occlusive disease may benefit most from surgical decompression of the hepatic veins. However, for those with mild to moderate disease, the proper role for operative intervention remains to be defined.

A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome

“…Anticoagulant therapy has been used widely in BCS to prevent propagation of the thrombi. 9,10,13,[17][18][19][20][21][22][23] Again, most reports of the effectiveness of this form of treatment have been anecdotal and lacking in long-term follow-up. There is no evidence that the use of either heparin or warfarin produces dissolution of established thrombosis.…”

Aged Budd-Chiari syndrome attributed to chronic deep venous thrombosis with alcoholic liver cirrhosis

“…Previously reported conditions that predispose to thrombosis are myeloproliferative disorders such as polycythemia vera and essential thrombocytosis, including preclinical stage, 10 and abnormal clotting tendencies due to deficiencies of antithrombin III, 11 protein C, 12,13 or protein S, 14 blood constituents that protect against thrombosis. Oral contraceptive use, 15 the presence of lupus anticoagulant 16 and anticardiolipin antibodies, 17 paroxysmal nocturnal hemoglobinuria (PNH), 18 and factor V Leiden mutation 19 also predispose to thrombosis. Less well documented associations were reported, such as ulcerative colitis, 20 Behcet's disease, 21 fat overload syndrome, 22 idiopathic granulomatous venulitis, 23 and pregnancy.…”

Successful Liver Transplantation in a Patient With Budd-Chiari Syndrome Caused by Homozygous Factor V Leiden

“…At that time, her disease was presumed to be secondary to oral contraceptive use, and a case report was published by investigators at another institution. 6 On review of her chart, she had normal values for partial thromboplastin time, antithrombin III, plasminogen, protein C (free and total), and protein S. Her prothrombin time was mildly elevated at 13.7 seconds (international normalized ratio, 1.4). In addition, she had abnormal platelet aggregation but normal factor VIII activity.…”