Abstract
Purpose
This study was performed to evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of patients affected by Budd-Chiari syndrome (BCS).
Materials and methods
From January 1999 to December 2006, 15 patients (seven male and eight female subjects, age range 7–52 years) with BCS uncontrolled by medical therapy were treated with TIPS placement. In seven cases BCS was idiopathic, in four it was caused by myeloproliferative disorders and in four by other disorders. One patient also had portal vein thrombosis. In 5/15 cases TIPS was created through a transcaval approach. Eight patients (53.4%) received a bare stent, and seven (46.6%) received a stent graft. The follow-up lasted a median of 29.4 (range 3.2-68) months.
Results
Technical success was achieved in all patients without major complications. TIPS was very effective in decreasing the portosystemic pressure gradient from 26.2±5.8 to 10±6.2 mmHg. All patients but two were alive at the time of writing. Acute leukaemia was the cause of the single early death and was unrelated to the procedure. The patient with portal vein thrombosis underwent thrombolysis before TIPS, but the vein occluded again after 3 weeks, and the patient died 6 months later. The other patients showed significant improvements in liver function, ascites and symptoms related to portal hypertension. Primary patency was 53.3%, and primary assisted patency was 93.3%. No patient required or was scheduled for liver transplantation.
Conclusions
TIPS is an effective and safe treatment for BCS and may be considered a valuable alternative to traditional surgical portosystemic shunting or liver transplantation.
Riassunto
Obiettivo
Valutare efficacia e sicurezza dello shunt portosistemico intraepatico per via transgiugulare (TIPS) come trattamento nei pazienti affetti da sindrome di Budd-Chiari (SBC).
Materiali e metodi
Dal gennaio 1999 al dicembre 2006, 15 pazienti (7 maschi e 8 femmine, di età compresa tra 7-52 anni) affetti da SBC non controllata dalla terapia medica sono stati sottoposti a TIPS. In 7 casi la SBC era idiopatica, in 4 causata da malattie mieloproliferative ed in 4 da altre cause. In 1 paziente era anche presente trombosi portale. In 5/15 casi la TIPS è stata realizzata mediante puntura transcavale. In otto pazienti (53,4%) è stato posizionato uno stent non ricoperto mentre in sette pazienti (46,6%) è stato utilizzato uno stent-graft. Il follow-up è stato di 3,2-68 mesi (mediana 29,4 mesi).
Risultati
In tutti i pazienti si è ottenuto il successo tecnico in assenza di complicanze maggiori. La TIPS è risultata molto efficace nel ridurre il gradiente pressorio portosistemico da 26,2±5,8 a 10±6,2 mmHg. Attualmente tutti i pazienti, eccetto due, sono vivi. La leucemia acuta è stata la causa dell’unico decesso precoce non correlato alla procedura. Il paziente con trombosi portale è stato sottoposto prima della TIPS a fibrinolisi, ma si è osservata riocclusione della TIPS a distanza di tre settimane con decesso del paziente dopo 6 mesi. Gli altri pazienti hanno mostrato un significativo miglioramento degli indici di funzionalità epatica, dell’ascite e dei sintomi correlati all’ipertensione portale. La pervietà primaria è stata del 53,3% e la pervietà assistita del 93,3%. Nessuno dei pazienti ha richiesto o è stato inserito in lista per un trapianto di fegato.
Conclusioni
La TIPS nella SBC è un trattamento efficace e sicuro e dovrebbe essere considerata una valida alternativa al tradizionale shunt porto-sistemico chirurgico o al trapianto di fegato.
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References/Bibliografia
Dilawari JB, Bambery P, Chawla Y et al (1994) Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine 73:21–36
Janssen HLA, Pagan JCG, Elias E et al (2003) Budd-Chiari syndrome: a review by an expert panel. J Hepatol 38:364–371
Menon KV, Shah V, LaBerge JM (2004) The Budd-Chiari syndrome. N Engl J Med 350:578–585
Wang ZG, Jones RS (1996) Budd-Chiari syndrome. Curr Probl Surg 33:83–211
Murad SD, Valla DC, De Groen PC et al (2004) Determinants of survival and the effect of portosystemic shunt in patients with Budd-Chiari syndrome. Hepatology 39:500–508
McCarthy PM, Van Heerden JA, Adson MA et al (1985) The Budd-Chiari syndrome. Medical and surgical management of 30 patients. Arch Surg 120:657–662
Slakey D, Klein AS, Venbrux AC, Cameron JC (2001) Budd-Chiari syndrome: current management options. Ann Surg 233:340–352
Klein AS (2006) Management of Budd-Chiari syndrome. Liver Transplantation 12:23–28
Perello A, Pagan JCG, Gilabert R et al (2002) TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology 35:132–139
Ganger DR, Klapman JB, McDonald V et al (1999) Transjugular Intrahepatic Portosystemic Shunt (TIPS) for Budd-Chiari Syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol 94:603–608
Gasparini D, Del Forno M, Sponza M et al (2002) Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd-Chiari syndrome. Eur J Gastroenterol Hepatol 14:567–571
Kugelmas M (1988) Budd-Chiari syndrome. Treatment options and the value of liver transplantation. Hepatogastroenterology 45:1381–1386
Zeitoun G, Escolano S, Hadengue A et al (1999) Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology 30:84–89
Orloff MJ, Orloff MS, Daily PO (1992) Long-term results of treatment of Budd-Chiari syndrome with portal decompression. Arch Surg 127:1182–1187
Pisani Ceretti A, Intra M, Prestipino F et al (1998) Surgical and radiologic treatment of primary Budd-Chiari syndrome. World J Surg 22:48–53
Orloff MJ, Daily PO, Orloff SL et al (2000) A 27-year experience with surgical treatment of Budd-Chiari syndrome. Ann Surg 232:340–352
Srinivasan P, Rela M, Prachalias A et al (2002) Liver transplantation for Budd-Chiari syndrome. Transplantation 73:973–977
Mentha G, Giostra E, Majno P et al (2006) Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol 44:520–528
Yamada R, Sato M, Kawabata M et al (1983) Segmental obstruction of the hepatic inferior vena cava treated by transluminal angioplasty. Radiology 149:91–94
Griffith JF, Mahmoud AE, Cooper S et al (1996) Radiological intervention in Budd-Chiari syndrome: techniques and outcome in 18 patients. Clin Radiol 51:775–784
Bilbao JB, Pueyo JP, Longo JM et al (1997) Interventional Therapeutic Techniques in Budd-Chiari Syndrome. Cardiovasc Intervent Radiol 20:112–119
Khuroo MS, Al-Suhabani H, Al-Sebayel M et al (2005) Budd-Chiari syndrome: Long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol 20:1494–1502
Kerlan R (1998) TIPS technique. Tech Vasc Intervent Radiol 1:68–79
Rossle M, Olschewski M, Siegerstetter V et al (2004) The Budd-Chiari syndrome: outcome after treatment with transjugular intrahepatic portosystemic shunt. Surgery 135:394–403
Ochs A, Sellinger M, Haag K et al (1993) Transjugular intrahepatic portosystemic stent-shunt (TIPS) in treatment of Budd-Chiari syndrome. J Hepatology 18:217–225
Blum U, Rossle M, Haag K et al (1995) Budd-Chiari syndrome: Technical hemodynamic and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology 197:805–811
Hernàndez-Guerra M, Turnes J, Rubinstein P et al (2004) PTFE-covered stent improve TIPS patency in Budd-Chiari syndrome. Hepatology 40:1197–1202
Fanelli F, Salvatori FM, Corona M et al (2006) Stent graft in TIPS: technical and procedural aspects. Radiol Med 111:709–723
Gandini R, Konda D, Simonetti G (2006) Transjugular intrahepatic portosystemic shunt patency and clinical outcome in patients with Budd-Chiari syndrome: covered versus uncovered stents. Radiology 241:298–305
Ryu RK, Durham JD, Krysl J et al (1999) Role of TIPS as a bridge to hepatic transplantation in Budd-Chiari syndrome. J Vasc Interv Radiol 10:799–805
Ong JP, Sands M, Younussi ZM et al (2000) Transjugular Intrahepatic Portosystemic Shunts (TIPS). J Clin Gastroenterol 30:14–28
Eapen CE, Velissaris D, Heydtmann M et al (2006) Favourable medium term outcome following hepatic recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut 55:878–884
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Corso, R., Intotero, M., Solcia, M. et al. Treatment of Budd-Chiari syndrome with transjugular intrahepatic portosystemic shunt (TIPS). Radiol med 113, 727–738 (2008). https://doi.org/10.1007/s11547-008-0288-z
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DOI: https://doi.org/10.1007/s11547-008-0288-z
Keywords
- Budd-Chiari syndrome
- Transjugular intrahepatic portosystemic shunt (TIPS)
- Liver transplantation
- Surgical portosystemic shunt