Budd-Chiari syndrome contraceptives

Adverse hepatic effects have been linked to the use of oral contraceptives, including hepatic dysfunction, cholestatic jaundice, benign hepatic tumours and peliosis hepatis. In addition, oral contraceptives have a number of less common but important effects on the liver. A correlation between the development of the Budd-Chiari syndrome and COCs has been described, and progestational derivatives have been linked to exacerbations of hepatic porphyria [12]. 

A review of 253 cases of Budd-Chiari syndrome found COC use to be the presumed cause in 9% of patients [29]. There appears to be a correlation between the development of BCS and the oestrogenic component of COCs [12]. Obstruction of the hepatic and portal veins has been documented and has been attributed to the thrombotic effects of the COCs however, the affected women may have had an underlying coagulation defect [16]. Budd-Chiari patients should be advised not to use COCs, as such patients have a demonstrated haematological tendency to form thrombi, and oestrogen-containing contraceptives may increase the risk of recurrent thrombosis [2]. 

Veno-occlusive disease (VOD) describes the occlusion of small hepatic veins and is defined as a radicular form of the Budd-Chiari syndrome. A variety of endotheliotoxic noxae, particularly phytotoxins, are responsible for this clinical picture. In 1951 reports were simultaneously published for the first time both in South Africa (G. Selzer et al.) and Jamaica (K. R. Hill) dealing with this disease of the small venous branches, which results from chronic intoxication with pyrrolizidine alkaloids, (s. pp 548, 571) Similar morphological and clinical effects can also be caused by cytostatic agents (6-mercaptopurine, dacarbazine, thioguanine), azathioprine, contraceptives and exposure to X-rays. Since 1957, the term Stuart-Bras syndrome has also been used to describe the occlusion of the small hepatic veins, (s. p. 832)... 

The hepatic veins may be affected by xenobiotic-induced occlusion resulting from thrombosis or from proliferation starting in the intima and subsequently producing (secondary) thrombosis. An occlusion of the large hepatic veins is known as the Budd-Chiari syndrome. There are two distinct types, the truncular and the radicular form, the latter corresponding to veno-occlusive disease, (s. p. 249) Contraceptives (J.A. Ecker et al., 1966) and cytostatic agents are held responsible. Women develop this type of hepatic disease more than twice as often as men. (s. fig. 29.9)... 

Fig. 29.9 Budd-Chiari syndrome with obliterated radicular vein (see arrow) after administration of contraceptives (van Gieson) (s. tab. 29.10)...

Men and women are affected with the same frequency at almost any age. Familial forms have been described. (63) There is an association with polyarthritis, coeliac disease (58), PSC, PBC (61), sarcoidosis, Budd-Chiari syndrome, and collagenoses or myeloproliferative diseases. A connection with thorotrast (59), immunosuppressives, cytostatics and contraceptives or androgens as... 

Minnema, M.C., Janssen, H.LA., Niermeijer, R, de Man, R.A. Budd-Chiari syndrome combination of genetic defects and the use of oral contraceptives leading to hypercoagulability. X Hepatol. 2000 33 509-512... 

A 39-year-old woman developed idiopathic thrombosis of the posterior tibial vein. Oral contraceptives and resistance to activated protein C were identified as risk factors. After initial treatment with intravenous heparin, she was given phenprocoumon and the oral contraceptive was withdrawn. After 4 months she developed subacute liver failure and phenprocoumon was withdrawn immediately. Autoimmune disease, viral hepatitis, toxic causes, and Budd-Chiari syndrome were excluded. Despite symptomatic treatment, she deteriorated further and orthotopic liver transplantation was performed. Histopathology of the explanted liver further excluded ischemic Uver cell necrosis and Budd-Chiari syndrome. 

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