Hepatic Vein Thrombosis (Budd-Chiari Syndrome)

Dominique-Charles Valla

doi:10.1055/s-2002-23202

Seminars in Liver Disease, Inhaltsverzeichnis

Semin Liver Dis 2002; 22(1): 005-014
DOI: 10.1055/s-2002-23202

Hepatic Vein Thrombosis (Budd-Chiari Syndrome)

Dominique-Charles Valla

Service d'Hépatologie, Hôpital Beaujon; Université Paris VII; and Unité INSERM U481, Clichy, France

Abstract

ABSTRACT

Hepatic vein thrombosis is caused by one or several thrombogenic conditions, of which myeloproliferative disorders are the most frequent. Thrombosis and its fibrous sequelae can affect the veins diffusely or locally. Severity is determined by the extent and velocity of the thrombotic process. Development of venous collateral vessels is an important compensatory mechanism. Some patients can be totally free of symptoms. Major complications are intractable ascites, liver insufficiency, and gastrointestinal bleeding. Diagnosis can be made via ultrasonography or magnetic resonance imaging in a majority of cases. The main prognostic factors are age, Child-Pugh score, and response of ascites to diuretics. Medical therapy includes control of causal factors, anticoagulation, and nonspecific treatment of complications. Procedures aiming to restore outflow of hepatic blood are indicated in patients with uncontrolled manifestations. Percutaneous angioplasty, followed by portosystemic shunt (including a transjugular intrahepatic portosystemic stent shunt) and eventually liver transplantation can be proposed in a graded manner. The current 10-year survival rate is about 75%.

KEYWORD

Hepatic vein thrombosis - thrombogenic disorders - portosystemic shunt - angioplasty - transplantation

Volltext

Referenzen

REFERENCES

1 Stuart K, Bras G. Veno-occlusive disease of the liver. Q J Med . 1957; 26 291-314
2 Parker R GF. Occlusion of the hepatic veins in man. Medicine (Baltimore) . 1959; 38 369-402
3 Akiyoshi H, Terada T. Centrilobular and perisinusoidal fibrosis in experimental congestive liver in the rat. J Hepatol . 1999; 30 433-439
4 Witte C L, Witte M H, Dumont A E. Lymph imbalance in the genesis and perpetuation of the ascites syndrome in hepatic cirrhosis. Gastroenterology . 1980; 78 1059-1068
5 Beattie C, Sitzmann J V, Cameron J L. Mesoatrial shunt hemodynamics. Surgery . 1988; 104 1-9
6 Menu Y, Sebag G, Vilgrain V. Budd-Chiari syndrome: MR evaluation. Diagn Int Radiol . 1990; 2 23-28
7 Miller W J, Federle M P, Straub W H. Budd-Chiari syndrome: imaging with pathologic correlation. Abdom Imaging . 1993; 18 329-335
8 Soyer P, Rabenandrasana A, Barge J. MRI of Budd-Chiari syndrome. Abdom Imaging . 1994; 19 325-329
9 Gonzalez-Flecha B, Reides C, Cutrin J C. Oxidative stress produced by suprahepatic occlusion and reperfusion. Hepatology . 1993; 18 881-889
10 Henrion J. Ischemia/reperfusion injury of the liver: pathophysiologic hypotheses and potential relevance to human hypoxic hepatitis. Acta Gastroenterol Belg . 2000; 63 336-347
11 Tanaka M, Wanless I R. Pathology of the liver in Budd-Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. Hepatology . 1998; 27 488-496
12 Vilgrain V, Lewin M, Vons C. Hepatic nodules in Budd-Chiari syndrome: imaging features. Radiology . 1999; 210 443-450
13 Mahmoud A E, Helmy A S, Billingham L. Poor prognosis and limited therapeutic options in patients with Budd-Chiari syndrome and portal venous system thrombosis. Eur J Gastroenterol Hepatol . 1997; 9 485-489
14 Gupta S, Barter S, Phillips G W. Comparison of ultrasonography, computed tomography and 99mTc liver scan in diagnosis of Budd-Chiari syndrome. Gut . 1987; 28 242-247
15 Tavill A S, Wood E J, Kreel L. The Budd-Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology . 1975; 68 509-518
16 Cho K J, Geisinger K R, Shields J J, Forrest M E. Collateral channels and histopathology in hepatic vein occlusion. AJR . 1982; 139 703-709
17 Hadengue A, Poliquin M, Vilgrain V. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology . 1994; 106 1042-1047
18 Hirshberg B, Shouval D, Fibach E. Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd-Chiari syndrome. J Hepatol . 2000; 32 574-578
19 Janssen H L, Meinardi J R, Vleggaar F P. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. Blood . 2000; 96 2364-2368
20 Denninger M H, Chait Y, Casadevall N. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology . 2000; 31 587-591
21 Mahmoud A EA, Mendoza A EA, Meshokhes A N. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. Q J Med . 1996; 89 37-43
22 Rosenberg R D, Aird W C. Vascular-bed-specific hemostasis and hypercoagulable states. N Engl J Med . 1999; 340 1555-1564
23 Rosendaal F R. Venous thrombosis: a multicausal disease. Lancet . 1999; 353 1167-1173
24 Schafer A I. Hypercoagulable states: molecular genetics to clinical practice. Lancet . 1994; 344 1739-1742
25 Deltenre P, Denninger M H, Hillaire S. Factor V Leiden-related Budd-Chiari syndrome. Gut . 2001; 48 264-268
26 Anger B R, Seifried E, Scheppach J. Budd-Chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases. Klin Wochenschr . 1989; 67 818-825
27 Wanless I R, Peterson P, Das A. Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. Hepatology . 1990; 12 1166-1174
28 Valla D, Casadevall N, Lacombe C. Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. Ann Intern Med . 1985; 103 329-334
29 Reid C D, Chanarin I, Lewis J. Formes frustes in myeloproliferative disorders. Identification by the growth of an endogenous erythroid clone in vitro in patients with arterial vascular disease. Lancet . 1982; 1 14-15
30 Hillmen P, Lewis S M, Bessler M. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med . 1995; 333 1253-1258
31 Socie G, Mary J Y, de Gramont A. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology. Lancet . 1996; 348 573-577
32 Valla D, Dhumeaux D, Babany G. Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology . 1987; 93 569-575
33 Pelletier S, Landi B, Piette J C. Antiphospholipid syndrome as the second cause of non-tumorous Budd-Chiari syndrome. J Hepatol . 1994; 21 76-80
34 Mahmoud A E, Elias E, Beauchamp N. Prevalence of the factor V Leiden mutation in hepatic and portal vein thrombosis. Gut . 1997; 40 798-800
35 Price D T, Ridker P M. Factor V Leiden mutation and the risks for thromboembolic disease: a clinical perspective. Ann Intern Med . 1997; 127 895-903
36 Valla D, Le M G, Poynard T. Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. Gastroenterology . 1986; 90 807-811
37 Valla D. Drug-induced vascular and sinusoidal lesions of the liver. Baillieres Clin Gastroenterol . 1988; 2 481-500
38 Dilawari J B, Bambery P, Chawla Y. Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine (Baltimore) . 1994; 73 21-36
39 Valla D, Benhamou J P. Obstruction of the hepatic veins or suprahepatic inferior vena cava. Dig Dis . 1996; 14 99-118
40 Bismuth E, Hadengue A, Hammel P. Hepatic vein thrombosis in Behcet's disease. Hepatology . 1990; 11 969-974
41 Brinson R R, Curtis W D, Schuman B M. Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis. Dig Dis Sci . 1988; 33 1615-1620
42 Marteau P, Cadranel J F, Messing B. Association of hepatic vein obstruction and coeliac disease in North African subjects. J Hepatol . 1994; 20 650-653
43 Aikat B K, Bhusnurmath S R, Chhuttani P N. Hepatic vein obstruction-a retrospective analysis of 72 autopsies and biopsies. Ind J Med Res . 1978; 67 128-144
44 Gupta S, Blumgart L H, Hodgson H J. Budd-Chiari syndrome: long-term survival and factors affecting mortality. Q J Med . 1986; 60 781-791
45 Uddin W, Ramage J K, Portmann B. Hepatic venous outflow obstruction in patients with polycystic liver disease: pathogenesis and treatment. Gut . 1995; 36 142-145
46 Ahn S S, Yellin A, Sheng F C. Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg . 1987; 5 28-37
47 Valla D, Hadengue A, el Younsi M. Hepatic venous outflow block caused by short-length hepatic vein stenoses. Hepatology . 1997; 25 814-819
48 Okuda K, Kage M, Shrestha S M. Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology . 1998; 28 1191-1198
49 Bismuth H, Sherlock D J. Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg . 1991; 214 581-589
50 Zeitoun G, Escolano S, Hadengue A. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology . 1999; 30 84-89
51 Kreel L, Freston J W, Clain D. Vascular radiology in the Budd-Chiari syndrome. Br J Radiol . 1967; 40 755-759
52 Rector W G, Redeker A G. Direct transhepatic assessment of hepatic vein pressure and direction of flow using a thin needle in patients with cirrhosis and Budd-Chiari syndrome. An effective alternative to hepatic vein catheterization. Gastroenterology . 1984; 86 1395-1399
53 Bellin M F, Challier E, Valla D. Budd-Chiari syndrome: value of duplex sonography and colour Doppler imaging for diagnosis and follow-up. Eur Radiol . 1995; 5 379-386
54 Ohta M, Hashizume M, Tomikawa M. Analysis of hepatic vein waveform by Doppler ultrasonography in 100 patients with portal hypertension. Am J Gastroenterol . 1994; 89 170-175
55 Millener P, Grant E G, Rose S. Color Doppler imaging findings in patients with Budd-Chiari syndrome: correlation with venographic findings. AJR . 1993; 161 307-312
56 Mathieu D, Kracht M, Zafrani E. Budd-Chiari syndrome. In: Ferrucci J, Mathieu D, eds. Advances in Hepatobiliairy Radiology St. Louis: CV Mosby 1990: 3-8
57 Ludwig J, Hashimoto E, McGill D. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc . 1990; 65 51-55
58 Wanless I, Liu J, Butany J. Role of thrombosis in the pathogenesis of congestive hepatic fibrosis (cardiac cirrhosis). Hepatology . 1995; 21 1232-2137
59 Wanless I, Wong F, Blendis L. Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension. Hepatology . 1995; 21 1238-1247
60 Harbin W, Robert N, Ferrucci J. Diagnosis of cirrhosis based on regional changes in hepatic morphology. A radiological and pathological analysis. Radiology . 1980; 135 273-283
61 Mathieu D, Vasile N, Grenier P. Portal thrombosis: dynamic CT features and course. Radiology . 1985; 154 737-741
62 Min A D, Atillasoy E O, Schwartz M E. Reassessing the role of medical therapy in the management of hepatic vein thrombosis. Liver Transplant Surg . 1997; 3 423-429
63 Campbell D A, Rolles K, Jamieson N. Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome. Surg Gynecol Obstet . 1988; 166 511-518
64 Schmets L, Hagege H, Merlet C. [Porto-hepatic thrombosis, revealing paroxysmal nocturnal hemoglobinuria, followed by regression induced by heparin therapy]. Gastroenterol Clin Biol . 1993; 17 955-958
65 Brearly S, Hawker P C, Dykes P W. A lethal complication of peripheral vein vasopressin infusion. Hepatogastroenterology . 1985; 32 224-225
66 Fisher N C, McCafferty I, Dolapci M. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut . 1999; 44 568-574
67 McMullin M F, Hillmen P, Jackson J. Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria. J Intern Med . 1994; 235 85-89
68 Bilbao J I, Pueyo J C, Longo J M. Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc Intervent Radiol . 1997; 20 112-119
69 Lopez R R, Benner K G, Hall L. Expandable venous stents for treatment of the Budd-Chiari syndrome. Gastroenterology . 1991; 100 1435-1441
70 Pisani-Ceretti A, Intra M, Prestipino F. Surgical and radiologic treatment of primary Budd-Chiari syndrome. World J Surg . 1998; 22 48-54
71 Vogt P R, Andersson L C, Jenni R. Dorsocranial liver resection and direct hepatoatrial anastomosis for hepatic venous outflow obstruction: long-term outcome and functional results. Am J Gastroenterol . 1996; 91 539-544
72 Henderson J M, Warren W D, Millikan W J. Surgical options, hematologic evaluation, and pathologic changes in Budd-Chiari syndrome. Am J Surg . 1990; 159 41-50
73 Kohli V, Pande G K, Dev V. Management of hepatic venous outflow obstruction. Lancet . 1993; 342 718-722
74 Orloff M J, Johansen K H. Treatment of Budd-Chiari syndrome by side-to-side portacaval shunt: experimental and clinical results. Ann Surg . 1978; 188 494-512
75 Shaked A, Goldstein R M, Klintmalm G B. Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. Surg Gynecol Obstet . 1992; 174 453-459
76 Vons C, Smadja C, Bourstyn E. Results of portal systemic shunts in Budd-Chiari syndrome. Ann Surg . 1986; 203 366-370
77 Panis Y, Belghiti J, Valla D. Portosystemic shunt in Budd-Chiari syndrome: long-term survival and factors affecting shunt patency in 25 patients in Western countries. Surgery . 1994; 115 276-281
78 Cobo F, Cervantes F, Garcia-Pagan J C. [Budd-Chiari syndrome associated with chronic myeloproliferative syndromes: analysis of 6 cases]. Med Clin (Barcelona) . 1996; 107 660-663
79 Blum U, Rossle M, Haag K. Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology . 1995; 197 805-811
80 Ganger D R, Klapman J B, McDonald V. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol . 1999; 94 603-608
81 Watanabe H, Shinzawa H, Saito T. Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction. Hepatogastroenterology . 2000; 47 839-841
82 Ringe B, Lang H, Oldhafer K J. Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation?. <~>A single-center experience with 50 patients. Hepatology . 1995; 21 1337-1344
83 Halff G, Todo S, Tzakis A G. Liver transplantation for the Budd-Chiari syndrome. Ann Surg . 1990; 211 43-49
84 Hemming A W, Langer B, Greig P. Treatment of Budd-Chiari syndrome with portosystemic shunt or liver transplantation. Am J Surg . 1996; 171 176-181