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Volume 20, Issue 1
  • ISSN: 1573-4056
  • E-ISSN: 1875-6603
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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.

© 2024 The Author(s). Published by Bentham Open.
© 2024 The Author(s). Published by Bentham Open. This is an open access article published under CC BY 4.0 https://creativecommons.org/licenses/by/4.0/legalcode
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2024-01-01
2024-11-26

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References

  1. Garcia-PagánJ.C. BuscariniE. JanssenH.L.A. LeebeekF.W.G. PlessierA. Rubbia-BrandtL. SenzoloM. SchoutenJ.N.L. TripodiA. VallaD.C. EASL Clinical Practice Guidelines: Vascular diseases of the liver.J. Hepatol.201664117920210.1016/j.jhep.2015.07.04026516032
     [Google Scholar]
  2. de FranchisR. BoschJ. Garcia-TsaoG. ReibergerT. RipollC. AbraldesJ.G. AlbillosA. BaigesA. BajajJ. BañaresR. Baveno VII – Renewing consensus in portal hypertension.J. Hepatol.202111610.1016/j.jhep.2021.12.02235120736
     [Google Scholar]
  3. QiX. HanG. GuoX. De StefanoV. XuK. LuZ. XuH. MancusoA. ZhangW. HanX. VallaD.C. FanD. Review article: The aetiology of primary Budd-Chiari syndrome - differences between the West and China.Aliment. Pharmacol. Ther.20164411-121152116710.1111/apt.1381527734511
     [Google Scholar]
  4. SeijoS. PlessierA. HoekstraJ. Dell’EraA. MandairD. RifaiK. TrebickaJ. MorardI. LasserL. AbraldesJ.G. Darwish MuradS. HellerJ. HadengueA. PrimignaniM. EliasE. JanssenH.L.A. VallaD.C. Garcia-PaganJ.C. Good long-term outcome of Budd-Chiari syndrome with a step-wise management.Hepatology.20135751962196810.1002/hep.2630623389867
     [Google Scholar]
  5. VallaD.C. Budd–Chiari syndrome/hepatic venous outflow tract obstruction.Hepatol. Int.201812S1Suppl. 116818010.1007/s12072‑017‑9810‑528685257
     [Google Scholar]
  6. EASL Clinical Practical Guidelines on the Management of Acute (Fulminant) Liver Failure J. Hepatol.2017661047108110.1016/j.jhep.2016.12.00328417882
     [Google Scholar]
  7. ParekhJ. MateiV.M. Canas-CotoA. FriedmanD. LeeW.M. Budd‐chiari syndrome causing acute liver failure: A multicenter case series.Liver Transpl.201723213514210.1002/lt.2464327656864
     [Google Scholar]
  8. Garcia-PagánJ.C. HeydtmannM. RaffaS. PlessierA. MuradS. FabrisF. VizziniG. AbraldesJ.G. OlliffS. NicoliniA. LucaA. PrimignaniM. JanssenH.L.A. VallaD. EliasE. BoschJ. TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients.Gastroenterology.2008135380881510.1053/j.gastro.2008.05.05118621047
     [Google Scholar]
  9. GiriS. KaleA. ShuklaA. Efficacy and safety of transjugular intrahepatic portosystemic shunt in budd-chiari syndrome: A systematic review and meta-analysis.J. Vasc. Interv. Radiol.202233111301131210.1016/j.jvir.2022.07.022
     [Google Scholar]
  10. HemachandranN. Shalimar AcharyaS. KediaS. GunjanD. SarayaA. SharmaR. GamanagattiS. Long-term outcomes of endovascular interventions in more than 500 patients with budd–chiari syndrome.J. Vasc. Interv. Radiol.20213216169.e110.1016/j.jvir.2020.08.03533218919
     [Google Scholar]
  11. WongjarupongN. YoungS. HuynhR.K. LakeJ. LimN. Long-term improvement in liver function following transjugular intrahepatic portosystemic shunt in patients with budd–chiari syndrome.J. Clin. Exp. Hepatol.20221261474147910.1016/j.jceh.2022.07.25136340317
     [Google Scholar]
  12. PlessierA. SibertA. ConsignyY. HakimeA. ZappaM. DenningerM.H. CondatB. FargesO. ChagneauC. de LedinghenV. FrancozC. SauvanetA. VilgrainV. BelghitiJ. DurandF. VallaD. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome.Hepatology.20064451308131610.1002/hep.2135417058215
     [Google Scholar]
  13. KawaiH. OsawaY. MatsudaM. TsunodaT. YanagidaK. HishikawaD. OkawaraM. SakamotoY. ShimagakiT. TsutsuiY. YoshidaY. YoshikawaS. HashiK. DoiH. MoriT. YamazoeT. YoshioS. SugiyamaM. OkuzakiD. KomatsuH. InuiA. Tamura-NakanoM. OyamaC. ShindouH. KusanoH. KageM. IkegamiT. YanagaK. KantoT. Sphingosine‐1‐phosphate promotes tumor development and liver fibrosis in mouse model of congestive hepatopathy.Hepatology.202276111212510.1002/hep.3225634855990
     [Google Scholar]
  14. SonavaneA.D. AmarapurkarD.N. RathodK.R. PunamiyaS.J. Long term survival of patients undergoing tips in budd-chiari syndrome.J. Clin. Exp. Hepatol.201991566110.1016/j.jceh.2018.02.00830765940
     [Google Scholar]
  15. ZahnA. GotthardtD. WeissK.H. RichterG. SchmidtJ. StremmelW. SauerP. Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt.BMC Gastroenterol.20101012510.1186/1471‑230X‑10‑2520193077
     [Google Scholar]
  16. RössleM. SiegerstetterV. EuringerW. OlschewskiM. KromeierJ. KurzK. LangerM. The use of a polytetrafluoroethylene-covered stent graft for transjugular intrahepatic portosystemic shunt (TIPS): Long-term follow-up of 100 patients.Acta Radiol.200647766066610.1080/0284185060080632416950701
     [Google Scholar]
  17. AlqahtaniS.A. SchneiderC. SimsO.T. GurakarA. TamimH. BonderA. SaberiB. Liver transplantation for budd-chiari syndrome in the MELD Era.Transplant. Direct.2022812e140710.1097/TXD.000000000000140736398192
     [Google Scholar]
  18. BureauC. PaganJ.C.G. LayrarguesG.P. MetivierS. BellotP. PerreaultP. OtalP. AbraldesJ.G. PeronJ.M. RousseauH. BoschJ. VinelJ.P. Patency of stents covered with polytetrafluoroethylene in patients treated by transjugular intrahepatic portosystemic shunts: Long-term results of a randomized multicentre study.Liver Int.200727674274710.1111/j.1478‑3231.2007.01522.x17617116
     [Google Scholar]
  19. PerarnauJ.M. Le GougeA. NicolasC. d’AlterocheL. BorentainP. SalibaF. MinelloA. AntyR. Chagneau-DerrodeC. BernardP.H. AbergelA. Ollivier-HourmandI. GournayJ. AyoubJ. GaboritC. RuschE. GiraudeauB. STIC-TIPS group Covered vs. uncovered stents for transjugular intrahepatic portosystemic shunt: A randomized controlled trial.J. Hepatol.201460596296810.1016/j.jhep.2014.01.01524480619
     [Google Scholar]
  20. Shalimar SharmaSanchit GamanagattiShivanand R Acute-on-chronic liver failure in budd-chiari syndrome: Profile and predictors of outcomeDig. Dis. Sci.20206592719272910.1007/s10620‑019‑06005‑7
     [Google Scholar]
  21. VermaA. SharmaG. MohanS. SaraswatV.A. BaijalS.S. 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.200831S2Suppl. 219719910.1007/s00270‑007‑9198‑z18046604
     [Google Scholar]
  22. CastroI. RíosJ.J. IniestaN. PerezI. RoblesA. GilA. Acute and fulminant Budd-Chiari syndrome in a well-anticoagulated patient with primary antiphospholipid syndrome.Lupus.2005141297998010.1191/0961203305lu2243xx16425582
     [Google Scholar]
  23. KavanaghP.M. RobertsJ. GibneyR. MaloneD. HegartyJ. McCormickP.A. 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.200419101135113910.1111/j.1440‑1746.2004.03427.x15377290
     [Google Scholar]
  24. GaspariniD. Del FornoM. SponzaM. BrancaB. ToniuttoP. MarzioA. PirisiM. Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd–Chiari syndrome.Eur. J. Gastroenterol. Hepatol.200214556757110.1097/00042737‑200205000‑0001711984158
     [Google Scholar]
  25. HastingsG.S. O’ConnorD.K. PaisS.O. Transjugular intrahepatic portosystemic shunt placement as a bridge to liver transplantation in fulminant Budd-Chiari syndrome.J. Vasc. Interv. Radiol.19967461610.1016/S1051‑0443(96)70818‑78855550
     [Google Scholar]
  26. KuoP.C. JohnsonL.B. HastingsG. PaisS.O. PlotkinJ.S. OrensJ.B. HowellC.D. LewisW.D. BartlettS.T. Fulminant hepatic failure from the Budd-Chiari syndrome. A bridge to transplantation with transjugular intrahepatic portosystemic shunt.Transplantation.199662229429610.1097/00007890‑199607270‑000248755831
     [Google Scholar]
  27. ShresthaR. DurhamJ.D. WachsM. BilirB.M. KamI. TrouillotT. EversonG.T. Use of transjugular intrahepatic portosystemic shunt as a bridge to transplantation in fulminant hepatic failure due to Budd-Chiari syndrome.Am. J. Gastroenterol.19979212230423069399778
     [Google Scholar]
  28. AslamN. RielyC.A. WatersB. Intraperitoneal rupture of ectopic varices: Two case reports and a review of literature.Am. J. Med. Sci.2008335216016210.1097/MAJ.0b013e318068b27318277129
     [Google Scholar]
  29. KitagawaS. SatoT. YamazakiK. OhmuraT. KarinoY. ToyotaJ. HasegawaT. SakaiW. MoritaR. Spontaneous hemoperitoneum due to rupture of the paraumbilical vein successfully treated with balloon-occluded retrograde transvenous obliteration.Clin. J. Gastroenterol.201361757910.1007/s12328‑012‑0349‑726181408
     [Google Scholar]
  30. OeyR.C. de WitK. MoelkerA. AtalikT. van DeldenO.M. MaleuxG. ErlerN.S. TakkenbergR.B. de ManR.A. NevensF. van BuurenH.R. Variable efficacy of TIPSS in the management of ectopic variceal bleeding: A multicentre retrospective study.Aliment. Pharmacol. Ther.201848997598310.1111/apt.1494730136292
     [Google Scholar]
  31. BañaresR. NevensF. LarsenF.S. JalanR. AlbillosA. DollingerM. SalibaF. SauerbruchT. KlammtS. OckengaJ. ParesA. WendonJ. BrünnlerT. KramerL. MathurinP. de la MataM. GasbarriniA. MüllhauptB. WilmerA. LalemanW. EefsenM. SenS. ZipprichA. TenorioT. PavesiM. SchmidtH.H.J. MitznerS. WilliamsR. ArroyoV. RELIEF study group Extracorporeal albumin dialysis with the molecular adsorbent recirculating system in acute-on-chronic liver failure: The RELIEF trial.Hepatology.20135731153116210.1002/hep.2618523213075
     [Google Scholar]
  32. DominikA. StangeJ. 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.202050111912810.1159/000508810
     [Google Scholar]
  33. TomescuD. PopescuM. DavidC. SimaR. DimaS. Haemoadsorption by CytoSorb® in patients with acute liver failure: A case series.Int. J. Artif. Organs.202144856056410.1177/039139882098138333302765
     [Google Scholar]
  34. ScharfC. LiebchenU. PaalM. Becker-PennrichA. IrlbeckM. ZollerM. SchroederI. Successful elimination of bilirubin in critically ill patients with acute liver dysfunction using a cytokine adsorber and albumin dialysis: A pilot study.Sci. Rep.20211111019010.1038/s41598‑021‑89712‑433986443
     [Google Scholar]
/content/journals/cmir/10.2174/1573405620666230908111803
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TIPS with a Twist – The Real Life Management of a Case of Budd-Chiari-related Acute Liver and Subsequent Multiple Organ Failure
Curr. Med. Imaging 20, E080923220829 (2024); https://doi.org/10.2174/1573405620666230908111803
/content/journals/cmir/10.2174/1573405620666230908111803
/content/journals/cmir/10.2174/1573405620666230908111803
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  • Article Type:     Case Report
Keyword(s): Acute liver failure; Budd chiari; Case report; Gastric variceal bleeding; Multiple organ failure; TIPS

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