Budd-Chiari syndrome

The most common change is a short-term rise in serum transaminases (208), which often abates if treatment is continued. An increase in serum alkaline phosphatase is usual, while serum transaminases can be normal to markedly increased (209). Long-term use leads to changes in hepatic ultrastructure, with involvement of the mitochondria, which develop crystalline inclusions. Furthermore, hypertrophy of the smooth endoplasmic reticulum and changes in the biliary canaliculi have been shown (210,211). These changes are not usually accompanied by any clinical symptoms. The Budd-Chiari syndrome can occur in connection with thromboembolism. 

Hepatic venous thrombosis, also known as Budd-Chiari syndrome, is caused by hypercoagulable disorders precipitated by pregnancy, infection, and birth control medication. An acute painful abdomen, sudden enlargement of the liver, and the presence of ascites make up a triad of clinical symptoms that are important in the diagnosis of this syndrome. Myeloproliferative disorders such as polycythemia vera and paroxysmal nocturnal dyspnea were previously thought to be responsible. Factor V Leiden and prothrombin 20210 mutations are also known to be responsible, Other intraabdominal thromboses include portal vein thrombosis, mesenteric vein thrombosis and renal vein thrombosis. 

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. 

The hepatic vessels may be visualised by conventional angiography or venography. These are invasive techniques requiring the injection of contrast media into the artery or vein via catheters during radiographic screening. Stenoses or occlusions are identified, e.g. occlusion of the hepatic veins in Budd-Chiari syndrome. 

Oestrogen receptors are found in liver tissue, and consequently the high levels of oestrogens contained in COCs that travel to the liver on first pass from the portal circulation may account for the number of hepatic complications associated with these preparations, e.g. cholestatic jaundice, hepatocellular carcinoma, Budd-Chiari syndrome [2]. 

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]. 

The Budd-Chiari syndrome (BCS) is thought to be related to abnormal coagulation and is diagnosed by the identification of obstruction of large hepatic veins in the absence of tumour invasion or compression... 

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]. 

Mitchell MC, Boitnott JK, Kaufman S (1982) Budd-Chiari syndrome Etiology, diagnosis and management. Medicine 61 199-218. 

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

Bolondi, L., Gaiani, S., Li Bassi, S., Zironi, G., Bonino, F., Brunette, M., Barabara, L. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology 1991 100 1324—1331... 

Sakugawa, H., TUgashionna, A., Oyakawa, T., Kadena, K., Kinjo, F., Saito, A. Ultrasound study in the diagnosis of primary Budd-Chiari syndrome (obstruction of the inferior cava) (Japan ). Gastroenterol. Japon. 1992 27 69-77... 

Volk, B.A., Schdlmerich, J., Blllmann, R, Gerok, W. Vaskulare Erkran-kungen der Leber kavemose Pfortadertransformation, M. Osier und Budd-Chiari-Syndrom. Ultraschall 1984 5 117-121... 

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)... 

Bhargava, D.K., Arora, A., Dasarathy, S. Laparoscopic features of the Budd-Chiari-Syndrome. Endoscopy 1991 23 259—261... 

Budd-Chiari syndrome may be imaged in CT as hypodense zones the findings are not, however, reliable. (27) A definitive diagnosis can be obtained by angiography or MRI. 

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)... 

Arita, T., Matsunaga, N., Kobayashi, H. Budd-Chiari syndrome peripheral abnormal intensity of the liver on magnetic resonance imaging. Clin. Radiol. 2000 55 640-642... 

Noohe, T.C., Semelka, R.C., Woosley, J.T., Pisano, E.D. Ultrasound and MR findings in acute Budd-Chiari syndrome with histopathologic correlation. J. Comput. Assist. Tomogr. 1996 20 819 —822... 

In the Budd-Chiari syndrome, the central area of the liver shows a normal or even increased concentration of radioactivity, whereas the peripheral regions of both lobes of liver exhibit reduced or even no uptake ( hot spots and multiple focal storage defects). Only the caudate lobe shows increased activity due to its separate venous flow, it is not functionally affected by hepatic vein thrombosis. (26)... 

Powell-Jackson, PB., Karanl, J., Ede, R., Mire, H., Williams, R. Ultrasonic scanning and 99m Tc sulphur colloid scintigraphy in diagnosis of Budd-Chiari syndrome. Gut 1986 27 1502-1506... 

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. 

Liver cirrhosis (37), liver tumours, liver echinococcosis, portal vein thrombosis, thrombosis of the splenic vein, right heart failure, Budd-Chiari syndrome, peliosis hepatis (39), etc. 

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)... 

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