Treatment of patients with Budd-Chiari syndrome. Literature review
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https://doi.org/10.52784/27112330.115Keywords:
Budd-Chiari syndrome, transjugular intrahepatic portosystemic shunt, anticoagulation, angioplasty, liver transplantation.Abstract
Budd-Chiari syndrome (SBC), described in 1845, is defined as the obstruction of hepatic venous flow in the absence of heart or pericardial disease. In Colombia there are no clear epidemiological data of this pathology, that around the world is considered rare. It is diagnosed by demonstrating the obstruction of the flow of the hepatic veins. It has various clinical manifestations such as fever, ascites, abdominal pain and collateral circulation, among others. In certain cases, it is asymptomatic but in the great majority it is accompanied by prothrombotic pathologies. Initial management depends on the patient’s condition; however, staggered management has been proposed, beginning with anticoagulation, continuing with angioplasty, then with transjugular intrahepatic portosystemic shunt (TIPS), and finally, with liver transplantation. The prognosis depends on an early diagnosis and proper treatment. In the best circumstances, a five-year survival is achieved in 90% of cases, while in the absence of treatment, the one-year mortality rate reaches the same percentage.
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Khan F, Armstrong MJ, Mehrzad H, Chen F, Neil D, Brown R, et al. Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Aliment Pharmacol Ther 2019;49:840-63. https://doi.org/10.1111/apt.15149.
European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol 2016;64:179-202. https://doi.org/10.1016/j.jhep.2015.07.040.
Li T, Zhai S, Pang Z, Ma X, Cao H, Bai W, et al. Feasibility and midterm outcomes of percutaneous transhepatic balloon angioplasty for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction. J Vasc Surg 2009;50:1079-1084. https://doi.org/10.1016/j.jvs.2009.06.049.
Mancuso A. An update on the management of Budd-Chiari syndrome: The issues of timing and choice of treatment. Eur J Gastroen Hepat 2015;27:200-203. https://doi.org/10.1097/MEG.0000000000000282.
Li Y, De Stefano V, Li H, Zheng K, Bai Z, Guo X, et al. Epidemiology of Budd-Chiari syndrome: A systematic review and meta-analysis. Clin Res Hepatol Gastroenterol 2018;43:468-474. https://doi.org/10.1016/j.clinre.2018.10.014.
Valla DC. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol 2004;19:S204-211. https://doi.org/10.1111/j.1440-1746.2004.03642.x.
Hernández-Gea V, De Gottardi A, Leebeek FWG, Rautou PE, Salem R, García-Pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol 2019;71:175-199. https://doi.org/10.1016/j.jhep.2019.02.015.
Darwish-Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009;151:167-175. https://doi.org/10.7326/0003-4819-151-3-200908040-00004.
Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HLA, Leebeek FWG. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood 2012;120:4921-4928. https://doi.org/10.1182/blood-2011-09-376517.
Seligsohn U, Lubetsky A. Genetic susceptibility to venous thrombosis. N Engl J Med 2001;344:1222-1231. https://doi.org/10.1056/NEJM200104193441607.
Ziakas PD, Poulou lS, Rokas GI, Bartzoudis D, Voulgarelis M. Thrombosis in paroxysmal nocturnal hemoglobinuria: sites, risks, outcome. An overview. J Thromb Haemost 2007;5:642-645. https://doi.org/10.1111/j.1538-7836.2007.02379.x.
Valla DC. Budd-Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int 2018;12:168-180. https://doi.org/10.1007/s12072-017-9810-5.
Qi X, Yang Z, De Stefano V, Fan D. Methylenetetrahydrofolate reductase C677T gene mutation and hyperhomocysteinemia in Budd-Chiari syndrome and portal vein thrombosis: A systematic review and meta-analysis of observational studies. Hepatol Res 2014;44:E480-498. https://doi.org/10.1111/hepr.12348.
Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd-Chiari syndrome: a common complication of Behçet's disease. Am J Gastroenterol 1997;92:858-862.
Bismuth E, Hadengue A, Hammel P, Benhamou JP. Hepatic vein thrombosis in Behçet's disease. Hepatology 1990;11:969-974. https://doi.org/10.1002/hep.1840110610.
Shrestha SM, Okuda K, Uchida T, Maharjan KG, Shrestha S, Joshi BL, et al. Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal. J Gastroenterol Hepatol 1996;11:170-179. https://doi.org/10.1111/j.1440-1746.1996.tb00056.x.
Valla DC. Primary Budd-Chiari syndrome. J Hepatol 2009;50:195-203. https://doi.org/10.1016/j.jhep.2008.10.007.
Miller WJ, Federle MP, Straub WH, Davis PL. Budd-Chiari syndrome: imaging with pathologic correlation. Abdom Imaging 1993;18:329-235. https://doi.org/10.1007/BF00201775.
Li CM, Wang ZG, Gu YQ, Yu HX, Chen B, Li Z, et al. Management of complex patients with Budd-Chiari syndrome. Ann Vasc Surg 2010;24:301-307. https://doi.org/10.1016/j.avsg.2009.07.013.
MacNicholas R, Olliff S, Elias E, Tripathi D. An update on the diagnosis and management of Budd-Chiari syndrome. Expert Rev Gastroenterol Hepatol 2012;6:731-744. https://doi.org/10.1586/egh.12.56.
Ferral H, Behrens G, Lopera J. Budd-Chiari Syndrome. Am J Roentgenol 2012;199:737-745. https://doi.org/10.2214/AJR.12.9098.
Cura M, Haskal Z, Lopera J. Diagnostic and interventional radiology for Budd-Chiari syndrome. RadioGraphics 2009;29:669-681. https://doi.org/10.1148/rg.293085056.
Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y. Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins. Hepatobiliary Pancreat Dis Int 2013;12:278-285. https://doi.org/10.1016/S1499-3872(13)60045-4.
Senzolo M, Cholongitas EC, Patch D, Burroughs AK. Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome. Nat Clin Pract Gastroenterol Hepatol 2005;2:182-190. https://doi.org/10.1038/ncpgasthep0143.
Hadengue A, Poliquin M, Vilgrain V, Belghiti J, Degott C, Erlinger S, et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 1994;106:1042-1047. https://doi.org/10.1016/0016-5085(94)90765-X.
Okuda K, Kage M, Shrestha SM. 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. https://doi.org/10.1002/hep.510280505.
Mukund A. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011;3:169-177. https://doi.org/10.4329/wjr.v3.i7.169.
Goel RM, Johnston EL, Patel KV, Wong T. Budd-Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J 2015;91:692-697.
Gao Y, Chen S, Yu C. Applicability of different endovascular methods for treatment of refractory Budd-Chiari syndrome. Cell Biochem Biophys 2011;61:453-460. https://doi.org/10.1007/s12013-011-9211-7.
Murad SD, Valla DC, de Groen PC, Zeitoun G, Hopmans JAM, Haagsma EB, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508. https://doi.org/10.1002/hep.20064.
Seijo S, Plessier A, Hoekstra J, Dell'era A, Mandair D, Rifai K, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology 2013;57(5):1962-1968. https://doi.org/10.1002/hep.26306.
Sakr M, Abdelhakam SM, Elsayed SA, Allam EH, Farid AM, Abdelmoaty W, et al. Validation of prognostic indices in Egyptian Budd-Chiari syndrome patients: A single-center study. World J Gastroenterol 2017;23:629-637. https://doi.org/10.3748/wjg.v23.i4.629.
Wang YD, Xue HZ, Zhang X, Xu ZQ, Jiang QF, Shen Q, et al. Clinical and pathological features and surgical treatment of Budd-Chiari syndrome-associated hepatocellular carcinoma. Chin Med J 2013;126:3632-3638.
Fu Y, Sun YL, Ma XX, Xu PQ, Feng LS, Tang Z, et al. Necessity and indications of invasive treatment for Budd-Chiari syndrome. Hepatobiliary Pancreat Dis Int 2011;10:254-260. https://doi.org/10.1016/S1499-3872(11)60042-8.
Langlet P, Escolano S, Valla D, Coste-Zeitoun D, Denie C, Mallet A, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501. https://doi.org/10.1016/S0168-8278(03)00323-4.
Dang XW, Xu PQ, Ma XX, Xu DQ, Zhu YJ, Zhang YS. Surgical treatment of Budd-Chiari syndrome: Analysis of 221 cases. Hepatobiliary Pancreat Dis Int 2011;10:435-438. https://doi.org/10.1016/S1499-3872(11)60074-X.
Martens P, Nevens F. Budd-Chiari syndrome. United Eur Gastroent 2015;3:489-500. https://doi.org/10.1177/2050640615582293.
Angeli P, Bernardi M, Villanueva C, Francoz C, Mookerjee RP, Trebicka J, et al. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol 2018;69:406-460. https://doi.org/10.1016/j.jhep.2018.08.009.
Zhang Y, Zhao H, Yan D, Xue H, Lau WY. Superior mesenteric vein-caval-right atrium y shunt for treatment of Budd-Chiari syndrome with obstruction to the inferior vena cava and the hepatic veins-a study of 62 patients. J Surg Res 2011;169:e93-99. https://doi.org/10.1016/j.jss.2011.02.030.
Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006;44:1308-1316. https://doi.org/10.1002/hep.21354.
Fitsiori K, Tsitskari M, Kelekis A, Filippiadis D, Triantafyllou K, Brountzos E. Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome patients: Results from a single center. Cardiovasc Intervent Radiol 2014;37:691-697. https://doi.org/10.1007/s00270-013-0697-9.
Zhang Q, Xu H, Zu M, Gu Y, Wei N, Wang W, et al. Catheter-directed thrombolytic therapy combined with angioplasty for hepatic vein obstruction in Budd-Chiari syndrome complicated by thrombosis. Exp Ther Med 2013;6:1015-1021. https://doi.org/10.3892/etm.2013.1239.
Zhang QQ, Xu H, Zu MH, Gu YM, Shen B, Wei N, et al. Strategy and long-term outcomes of endovascular treatment for Budd-Chiari syndrome complicated by inferior vena caval thrombosis. Eur J Vasc Endovasc Surg 2014;47:550-557. https://doi.org/10.1016/j.ejvs.2014.01.014.
Cui YF, Fu YF, Wei N, Zhu HC, Xu H. Retrograde puncture assisted hepatic vein recanalization in treating Budd-Chiari syndrome with segmental obstruction of hepatic vein. Radiol Medica 2015;120:1184-1189. https://doi.org/10.1007/s11547-015-0557-6.
Peynircioglu B, Shorbagi AI, Balli O, Cil B, Balkanci F, Bayraktar Y. Is there an alternative to TIPS? Ultrasound-guided direct intrahepatic portosystemic shunt placement in Budd-Chiari syndrome. Saudi J Gastroentero 2010;16:315-318. https://doi.org/10.4103/1319-3767.70633.
Tripathi D, Sunderraj L, Vemala V, Mehrzad H, Zia Z, Mangat K, et al. Long-term outcomes following percutaneous hepatic vein recanalization for Budd-Chiari syndrome. Liver Int 2017;37:111-120. https://doi.org/10.1111/liv.13180.
Qi X, Yang M, Fan D, Han G. Transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome: A critical review of literatures. Scand J Gastroenterol 2013;48:771-784. https://doi.org/10.3109/00365521.2013.777775.
Inafuku H, Morishima Y, Nagano T, Arakaki K, Yamashiro S, Kuniyoshi Y. A three-decade experience of radical open endvenectomy with pericardial patch graft for correction of Budd-Chiari syndrome. J Vasc Surg 2009;50:590-593. https://doi.org/10.1016/j.jvs.2009.03.040.
Mancuso A. Budd-Chiari syndrome management: Lights and shadows. World J Hepatol 2011;3:262-264. https://doi.org/10.4254/wjh.v3.i10.262.
Zhang B, Jiang ZB, Huang MS, Zhu KS, Qian JS, Shan H. Effects of percutaneous transhepatic interventional treatment for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction. J Vasc Surg Venous Lymphat Disord 2013;1:392-399. https://doi.org/10.1016/j.jvsv.2013.05.008.
Hidaka M, Eguchi S. Budd-Chiari syndrome: Focus on surgical treatment. Hepatol Res 2017; 47:142-148. https://doi.org/10.1111/hepr.12752.
Mackiewicz A, Kotulski M, Zieniewicz K, Krawczyk M. Results of liver transplantation in the treatment of Budd-Chiari syndrome. Ann Transplant 2012;17:5-10. https://doi.org/10.12659/AOT.882630.
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