Budd-Chiari disease

Budd-Chiari syndrome, since it can also be seen in advanced right-sided cardiac failure, explained by the same hemodynamic effects related to the outflow disturbances within the hepatic veins (Fig. 11.11). In the chronic phase of Budd-Chiari disease, the venous obstruction is well established, giving rise to the appearance of typical comma shaped branching vascular structures, corresponding to an intrahe-patic network of venous collaterals trying to bypass the obstruction. These abnormal vessels tend to be... 

Veno-occlusive disease (VOD) differs from Budd-Chiari syndrome in that it consists of occlusive fibrosis of the small intrahepatic veins. VOD may present as either an acute form with sudden ascites, liver enlargement and rapidly rising bilirubin, or as a chronic form with fibrosis and cirrhosis. One of the main causes of VOD is the use of cyclophosphamide or alkalating agents during conditioning for bone marrow transplantation, where it occurs in up to 20% of cases. Other causes include irradiation, antineoplastic drugs, pyrrolizidine alkaloids and alcohol. 

Budd-Chiari syndrome or veno-occlusive disease... 

Unclear structures can be punctured with a fine needle initially as an exploratory procedure (consistency cyst blood vessel ) before the Menghini needle is used. Reports have been written, for example, on the laparoscopic diagnosis of Budd-Chiari syndrome (190), liver abscesses (232), peliosis hepatis (236,305), FNH (204, 236), Osier s disease (306), and unclarified cholestasis. (280, 299)... 

Further study results are available relating to amyloidosis (29, 111), primary sclerosing cholangitis (73), pe-liosis hepatis (105), Budd-Chiari syndrome (29, 60, 108), schistosomiasis (29, 79), biliary tract diseases (75, 82, 120), echinococcosis (69, 71), and adenoma. (67, 74)... 

An increase in blood both in the sinusoids and in Disse s spaces culminates in hepatomegaly. This can be witnessed particularly in cases of right heart failure, constrictive pericarditis, veno-occlusive disease and the Budd-Chiari syndrome. Inflammation-related hyper-aemia also occurs in acute viral hepatitis. 

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)... 

Budd-Chiari syndrome, heatstroke, ligature of the hepatic artery, shock liver, veno-occlusive disease... 

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)... 

Occlusion of the small hepatic veins is called veno-occlu-sive disease (VOD) (G. Bras et al., 1954 K.L. Stuart et al., 1957). It is identical to the radicular type of the Budd-Chiari syndrome, (s. p. 249) Cytostatics and azathioprine are among the alleged causal agents. (I2l, 130) Diagnosis is based on imaging techniques (ultrasound, CEDS, CT), and sometimes on liver biopsy, (s. fig. 29.10) (see chapter 39)... 

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... 

Liver involvement in Osier-Vaquez disease is rare or not detectable at all. There is, however, evidence of hepato-splenomegaly due to extramedullary haemopoiesis. Of importance here is the association with Budd-Chiari syndrome and veno-occlusive disease. Polycythaemia vera should be considered in cases of aetiologically unclarified portal vein thrombosis. 

With the exception of occasional hepatosplenomegaly, liver involvement is rare. There is again an association with Budd-Chiari syndrome and veno-occlusive disease. 

Anger, B.R., Seifried, E., Scheppach, J., Heimpel, H. Budd-Chiari syndrome and thrombosis of other abdominal vessels in chronic myeloproliferative diseases. Klin. Wschr. 1989 67 818 -825... 

Veno-occlusive disease (VOD) is characterized by thrombosis of the central and small (sublobular) hepatic veins. It is also known as the radicular form of the Budd-Chiari syndrome or as the Stuart-Bras syndrome, (s. tab. 14.5) (75, 92, 93)... 

Bayraktar, Y., Balkanci, F., Bayraktar, M., Calguneri, M. Budd-Chiari syndrome a common comphcation of Behcet s disease. Amer. J. Gastroenterol. 1997 92 858 -862... 

Bhupalan, A., Talbot, K., Forbes, A., Owen, M., Samson, D., Murray-Lyon, I.M. Budd-Chiari syndrome in association with polycystic disease of the hver and kidneys. J. Royal Soc. Med. 1992 85 296- 297... 

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. 

A 49-year-old woman was diagnosed with veno-occlusive disease, a form of Budd-Chiari syndrome. The patient had portal hypertension associated with obliteration of the smaller hepatic venules. A liver biopsy specimen showed centrilobular necrosis and congestion. The woman had been regularly taking two products that contained comfrey, one for 6 months and the other for 4 months. Analyses of products taken indicated an estimated intake of pyrrolizidine alkaloids of 0.49 to 1.45 ng/kg daily. Other supplements being taken by the woman included a number of vitamins, minerals, and sterotrophic adrenal bovine extract (Ridker et al. 1985). 

McDermott, W.V., and P.M. Ridker. 1990. The Budd-Chiari syndrome and hepatic veno-occlusive disease Recognition and treatment. Arch. Surg. 125 (4) 525-527. 

Evidence of toxicity may not become apparent until some time after the alkaloid is ingested. The acute illness has been compared to the most common pro-thrombotic disorders that lead to Budd-Chiari syndrome, portal vein thrombosis, and sinusoidal obstruction syndrome, previously known as hepatic veno-occlusive disease of... 

A reduction in the efferent blood flow via the hepatic veins such as seen in Budd-Chiari syndrome causes several liver flow abnormalities which are quite different in the acute and chronic forms of the disease (Mathieu et al. 1987). in the acute phase, besides obvious liver enlargement, the postsinusoidal obstruction causes a severe reduction in the portal vein flow and a compensatory increase in the arterial flow delivered through the hepatic artery. Since blood flow is not able to perfuse the more peripheral liver areas properly, and since there is a pressure gradient between the arterial vessels and liver veins, functional intrahepatic arterioportal shunts are used that may... 

Cholangiocarcinoma Budd-Chiari syndrome Polycystic liver disease Amyloidosis Hepatic enzyme defects Metastatic neuroendocrine tumour... 

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