Abstract
Introduction: Budd-Chari syndrome (BCS) is a rare condition defined by the obstruction of hepatic venous outflow. BCS is a relatively infrequent cause of acute liver failure (ALF), accounting for less than 1% of cases. Treatment for acute BCS consists of a stepwise approach, requiring anticoagulation, angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation.
Case Report: We present the case of a 31-year-old female patient with BCS, which led to ALF and subsequent multiple organ failure, which was successfully treated with TIPS and endovascular coil placement. Initial diagnostic workup revealed the complete obstruction of the hepatic venous outflow, spleno-mesenteric confluent thrombosis, and biochemical criteria of ALF. Her condition rapidly deteriorated towards multiple organ failure. At one point, the MELD score was 42, while the SOFA score predicted a mortality rate of >95%. Following continuous venovenous hemodiafiltration with cytokine adsorbent filters, TIPS was inserted, resulting in a portal pressure gradient (PPG) of 14 mmHg. Following TIPS, the patient had persistent ascites and later presented an episode of gastric variceal bleeding with endoscopic and surgical treatment failure. TIPS revision with further dilation led to a final PPG of 6 mmHg. During the procedure, selective embolization by coil placement of the spleno-gastric collateral circulation ultimately resolved the variceal bleeding. In the aftermath, the patient had complete organ failure remission and was successfully discharged with no ascites, encephalopathy, or significant impairment regarding daily life activities.
Conclusion: In the rare setting of BCS complicated with ALF and portal hypertension-related complications, TIPS and endovascular embolization provide a unique, effective, and against-all-odd solution.
[1]
Garcia-Pagán JC, Buscarini E, Janssen HLA, et al. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol 2016; 64(1): 179-202.
[http://dx.doi.org/10.1016/j.jhep.2015.07.040] [PMID: 26516032]
[http://dx.doi.org/10.1016/j.jhep.2015.07.040] [PMID: 26516032]
[2]
de Franchis R, Bosch J, Garcia-Tsao G, et al. Baveno VII – Renewing consensus in portal hypertension. J Hepatol 2021; 1-16.
[http://dx.doi.org/10.1016/j.jhep.2021.12.022] [PMID: 35120736]
[http://dx.doi.org/10.1016/j.jhep.2021.12.022] [PMID: 35120736]
[3]
Qi X, Han G, Guo X, et al. Review article: The aetiology of primary Budd-Chiari syndrome - differences between the West and China. Aliment Pharmacol Ther 2016; 44(11-12): 1152-67.
[http://dx.doi.org/10.1111/apt.13815] [PMID: 27734511]
[http://dx.doi.org/10.1111/apt.13815] [PMID: 27734511]
[4]
Seijo S, Plessier A, Hoekstra J, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology 2013; 57(5): 1962-8.
[http://dx.doi.org/10.1002/hep.26306] [PMID: 23389867]
[http://dx.doi.org/10.1002/hep.26306] [PMID: 23389867]
[5]
Valla DC. Budd–Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int 2018; 12(S1) (Suppl. 1): 168-80.
[http://dx.doi.org/10.1007/s12072-017-9810-5] [PMID: 28685257]
[http://dx.doi.org/10.1007/s12072-017-9810-5] [PMID: 28685257]
[7]
Parekh J, Matei VM, Canas-Coto A, Friedman D, Lee WM. Budd‐chiari syndrome causing acute liver failure: A multicenter case series. Liver Transpl 2017; 23(2): 135-42.
[http://dx.doi.org/10.1002/lt.24643] [PMID: 27656864]
[http://dx.doi.org/10.1002/lt.24643] [PMID: 27656864]
[8]
Garcia-Pagán JC, Heydtmann M, Raffa S, et al. TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients. Gastroenterology 2008; 135(3): 808-15.
[http://dx.doi.org/10.1053/j.gastro.2008.05.051] [PMID: 18621047]
[http://dx.doi.org/10.1053/j.gastro.2008.05.051] [PMID: 18621047]
[9]
Giri S, Kale A, Shukla A. Efficacy and safety of transjugular intrahepatic portosystemic shunt in budd-chiari syndrome: A systematic review and meta-analysis. J Vasc Interv Radiol 2022; 33(11): 1301-12.
[http://dx.doi.org/10.1016/j.jvir.2022.07.022]
[http://dx.doi.org/10.1016/j.jvir.2022.07.022]
[10]
Hemachandran N, Shalimar , Acharya S, et al. Long-term outcomes of endovascular interventions in more than 500 patients with budd–chiari syndrome. J Vasc Interv Radiol 2021; 32(1): 61-69.e1.
[http://dx.doi.org/10.1016/j.jvir.2020.08.035] [PMID: 33218919]
[http://dx.doi.org/10.1016/j.jvir.2020.08.035] [PMID: 33218919]
[11]
Wongjarupong N, Young S, Huynh RK, Lake J, Lim N. Long-term improvement in liver function following transjugular intrahepatic portosystemic shunt in patients with budd–chiari syndrome. J Clin Exp Hepatol 2022; 12(6): 1474-9.
[http://dx.doi.org/10.1016/j.jceh.2022.07.251] [PMID: 36340317]
[http://dx.doi.org/10.1016/j.jceh.2022.07.251] [PMID: 36340317]
[12]
Plessier A, Sibert A, Consigny Y, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006; 44(5): 1308-16.
[http://dx.doi.org/10.1002/hep.21354] [PMID: 17058215]
[http://dx.doi.org/10.1002/hep.21354] [PMID: 17058215]
[13]
Kawai H, Osawa Y, Matsuda M, et al. Sphingosine‐1‐phosphate promotes tumor development and liver fibrosis in mouse model of congestive hepatopathy. Hepatology 2022; 76(1): 112-25.
[http://dx.doi.org/10.1002/hep.32256] [PMID: 34855990]
[http://dx.doi.org/10.1002/hep.32256] [PMID: 34855990]
[14]
Sonavane AD, Amarapurkar DN, Rathod KR, Punamiya SJ. Long term survival of patients undergoing tips in budd-chiari syndrome. J Clin Exp Hepatol 2019; 9(1): 56-61.
[http://dx.doi.org/10.1016/j.jceh.2018.02.008] [PMID: 30765940]
[http://dx.doi.org/10.1016/j.jceh.2018.02.008] [PMID: 30765940]
[15]
Zahn A, Gotthardt D, Weiss KH, et al. Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt. BMC Gastroenterol 2010; 10(1): 25.
[http://dx.doi.org/10.1186/1471-230X-10-25] [PMID: 20193077]
[http://dx.doi.org/10.1186/1471-230X-10-25] [PMID: 20193077]
[16]
Rössle M, Siegerstetter V, Euringer W, et al. The use of a polytetrafluoroethylene-covered stent graft for transjugular intrahepatic portosystemic shunt (TIPS): Long-term follow-up of 100 patients. Acta Radiol 2006; 47(7): 660-6.
[http://dx.doi.org/10.1080/02841850600806324] [PMID: 16950701]
[http://dx.doi.org/10.1080/02841850600806324] [PMID: 16950701]
[17]
Alqahtani SA, Schneider C, Sims OT, et al. Liver transplantation for budd-chiari syndrome in the MELD Era. Transplant Direct 2022; 8(12): e1407.
[http://dx.doi.org/10.1097/TXD.0000000000001407] [PMID: 36398192]
[http://dx.doi.org/10.1097/TXD.0000000000001407] [PMID: 36398192]
[18]
Bureau C, Pagan JCG, Layrargues GP, et al. Patency of stents covered with polytetrafluoroethylene in patients treated by transjugular intrahepatic portosystemic shunts: Long-term results of a randomized multicentre study. Liver Int 2007; 27(6): 742-7.
[http://dx.doi.org/10.1111/j.1478-3231.2007.01522.x] [PMID: 17617116]
[http://dx.doi.org/10.1111/j.1478-3231.2007.01522.x] [PMID: 17617116]
[19]
Perarnau JM, Le Gouge A, Nicolas C, et al. Covered vs. uncovered stents for transjugular intrahepatic portosystemic shunt: A randomized controlled trial. J Hepatol 2014; 60(5): 962-8.
[http://dx.doi.org/10.1016/j.jhep.2014.01.015] [PMID: 24480619]
[http://dx.doi.org/10.1016/j.jhep.2014.01.015] [PMID: 24480619]
[20]
Shalimar , Sharma Sanchit, Gamanagatti Shivanand R, et al. Acute-on-chronic liver failure in budd-chiari syndrome: Profile and predictors of outcome Dig Dis Sci 2020; 65(9): 2719-29.
[http://dx.doi.org/10.1007/s10620-019-06005-7]
[http://dx.doi.org/10.1007/s10620-019-06005-7]
[21]
Verma A, Sharma G, Mohan S, Saraswat VA, Baijal SS. TIPS can be lifesaving in acute liver failure associated with portal vein and inferior vena cava thrombosis in a case of budd chiari syndrome due to protein s deficiency. Cardiovasc Intervent Radiol 2008; 31(S2) (Suppl. 2): 197-9.
[http://dx.doi.org/10.1007/s00270-007-9198-z] [PMID: 18046604]
[http://dx.doi.org/10.1007/s00270-007-9198-z] [PMID: 18046604]
[22]
Castro I, Ríos JJ, Iniesta N, Perez I, Robles A, Gil A. Acute and fulminant Budd-Chiari syndrome in a well-anticoagulated patient with primary antiphospholipid syndrome. Lupus 2005; 14(12): 979-80.
[http://dx.doi.org/10.1191/0961203305lu2243xx] [PMID: 16425582]
[http://dx.doi.org/10.1191/0961203305lu2243xx] [PMID: 16425582]
[23]
Kavanagh PM, Roberts J, Gibney R, Malone D, Hegarty J, McCormick PA. Acute Budd-Chiari syndrome with liver failure: The experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol 2004; 19(10): 1135-9.
[http://dx.doi.org/10.1111/j.1440-1746.2004.03427.x] [PMID: 15377290]
[http://dx.doi.org/10.1111/j.1440-1746.2004.03427.x] [PMID: 15377290]
[24]
Gasparini D, Del Forno M, Sponza M, et al. Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd–Chiari syndrome. Eur J Gastroenterol Hepatol 2002; 14(5): 567-71.
[http://dx.doi.org/10.1097/00042737-200205000-00017] [PMID: 11984158]
[http://dx.doi.org/10.1097/00042737-200205000-00017] [PMID: 11984158]
[25]
Hastings GS, O’Connor DK, Pais SO. Transjugular intrahepatic portosystemic shunt placement as a bridge to liver transplantation in fulminant Budd-Chiari syndrome. J Vasc Interv Radiol 1996; 7(4): 616.
[http://dx.doi.org/10.1016/S1051-0443(96)70818-7] [PMID: 8855550]
[http://dx.doi.org/10.1016/S1051-0443(96)70818-7] [PMID: 8855550]
[26]
Kuo PC, Johnson LB, Hastings G, et al. Fulminant hepatic failure from the Budd-Chiari syndrome. A bridge to transplantation with transjugular intrahepatic portosystemic shunt. Transplantation 1996; 62(2): 294-6.
[http://dx.doi.org/10.1097/00007890-199607270-00024] [PMID: 8755831]
[http://dx.doi.org/10.1097/00007890-199607270-00024] [PMID: 8755831]
[27]
Shrestha R, Durham JD, Wachs M, et al. Use of transjugular intrahepatic portosystemic shunt as a bridge to transplantation in fulminant hepatic failure due to Budd-Chiari syndrome. Am J Gastroenterol 1997; 92(12): 2304-6.
[PMID: 9399778]
[PMID: 9399778]
[28]
Aslam N, Riely CA, Waters B. Intraperitoneal rupture of ectopic varices: Two case reports and a review of literature. Am J Med Sci 2008; 335(2): 160-2.
[http://dx.doi.org/10.1097/MAJ.0b013e318068b273] [PMID: 18277129]
[http://dx.doi.org/10.1097/MAJ.0b013e318068b273] [PMID: 18277129]
[29]
Kitagawa S, Sato T, Yamazaki K, et al. Spontaneous hemoperitoneum due to rupture of the paraumbilical vein successfully treated with balloon-occluded retrograde transvenous obliteration. Clin J Gastroenterol 2013; 6(1): 75-9.
[http://dx.doi.org/10.1007/s12328-012-0349-7] [PMID: 26181408]
[http://dx.doi.org/10.1007/s12328-012-0349-7] [PMID: 26181408]
[30]
Oey RC, de Wit K, Moelker A, et al. Variable efficacy of TIPSS in the management of ectopic variceal bleeding: A multicentre retrospective study. Aliment Pharmacol Ther 2018; 48(9): 975-83.
[http://dx.doi.org/10.1111/apt.14947] [PMID: 30136292]
[http://dx.doi.org/10.1111/apt.14947] [PMID: 30136292]
[31]
Bañares R, Nevens F, Larsen FS, et al. Extracorporeal albumin dialysis with the molecular adsorbent recirculating system in acute-on-chronic liver failure: The RELIEF trial. Hepatology 2013; 57(3): 1153-62.
[http://dx.doi.org/10.1002/hep.26185] [PMID: 23213075]
[http://dx.doi.org/10.1002/hep.26185] [PMID: 23213075]
[32]
Dominik A, Stange J. similarities, differences, and potential synergies in the mechanism of action of albumin dialysis using the mars albumin dialysis device and the cytosorb hemoperfusion device in the treatment of liver failure. Blood Purif 2020; 50(1): 119-28.
[http://dx.doi.org/10.1159/000508810]
[http://dx.doi.org/10.1159/000508810]
[33]
Tomescu D, Popescu M, David C, Sima R, Dima S. Haemoadsorption by CytoSorb® in patients with acute liver failure: A case series. Int J Artif Organs 2021; 44(8): 560-4.
[http://dx.doi.org/10.1177/0391398820981383] [PMID: 33302765]
[http://dx.doi.org/10.1177/0391398820981383] [PMID: 33302765]
[34]
Scharf C, Liebchen U, Paal M, et al. Successful elimination of bilirubin in critically ill patients with acute liver dysfunction using a cytokine adsorber and albumin dialysis: A pilot study. Sci Rep 2021; 11(1): 10190.
[http://dx.doi.org/10.1038/s41598-021-89712-4] [PMID: 33986443]
[http://dx.doi.org/10.1038/s41598-021-89712-4] [PMID: 33986443]
