Liver International ISSN 1478-3223
Letters to the Editor DOI:10.1111/liv.12828 Liver Int. 2015; 35: 2339
Time to resize the role of liver transplant for Budd–Chiari syndrome To the Editor: I read with interest the wide long-term one centre experience on liver transplant (LT) for Budd–Chiari Syndrome (BCS) recently published in Liver International (1). Together with other previous data, the article confirms that myeloproliferative neoplasms are not a contraindication for LT in BCS, since they do not seem to significantly affect the outcome after LT. However, as often happening with rare diseases, the experiences published are hardly sufficiently powered to reach adequate statistically significance. The role of LT in BCS should probably be that of salvage treatment, after all the other therapeutic attempts have failed. In fact, TIPS has been shown to be effective as BCS treatment and can be successful also in the technically difficult case of extension of thrombosis to the portal vein tree, with survival rates similar to those after LT (2, 3). Moreover, albeit without validated criteria, BCS management generally follows indication criteria that needs to be up-dated. In fact, following the so called step-wise strategy, longterm experiences reported poor outcome in the subgroup treated with only medical therapy (4). A reasoning model of BCS physiopathology is that not only impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development but also causes hepatic fibrosis development and liver failure through chronic ischaemic liver damage. The ischaemic liver cirrhosis (ILC) theory, albeit not evidence-based and requiring validation, postulated that cirrhosis development could arise from chronic micro-vascular ischaemia. One model of ILC theory is BCS. In fact, a substantial rate of patients with BCS finally develops cirrhosis, in the absence of known causative factors but chronic liver ischaemia. By reverting liver congestion through TIPS or other invasive treatment, liver function is expected to improve (5). Recently, I reported a proposal of a new algorithm for the management of BCS (4), in which medical
Liver International (2015) © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
therapy is suggested only for patients without any sign of portal hypertension, whether early interventional treatment is suggested when either any symptom or sign of portal hypertension appears, with the aim of preventing hepatic fibrosis development, disease progression and finally improving outcome. LT should only be considered as a salvage treatment. Acknowledgements
Financial support: None declared. Conflict of interest: The authors do not have any disclosures to report.
Andrea Mancuso1,2 1 Medicina Interna 1, ARNAS Civico - Di Cristina Benfratelli, Palermo, Italy 2 Epatologia e Gastroenterologia, Ospedale Niguarda Ca Granda, Milano, Italy
References 1. Potthoff A, Attia D, Pischke S, et al. Long-term Outcome of Liver Transplant Patients with Budd-Chiari Syndrome Secondary to Myeloproliferative Neoplasms. Liver Int 2015; 35: 2042–9. 2. Mancuso A, Fung K, Mela M, et al. TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience. J Hepatol 2003; 38: 751–4. 3. Mancuso A, Watkinson A, Tibballs J, Patch D, Burroughs AK. Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins. Gut 2003; 52: 438. 4. Mancuso A. An Update on Management of Budd-Chiari Syndrome: the Issues of Timing and Choice of Treatment. Eur J Gastro Hepatol 2015; 27: 200–3. 5. Mancuso A. Cirrhosis development probably arises from chronic micro-vascular ischemia. Med Hypotheses 2014; 82: 243–4.
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Letters to the Editor DOI:10.1111/liv.12828 Liver Int. 2015; 35: 2339
Time to resize the role of liver transplant for Budd–Chiari syndrome To the Editor: I read with interest the wide long-term one centre experience on liver transplant (LT) for Budd–Chiari Syndrome (BCS) recently published in Liver International (1). Together with other previous data, the article confirms that myeloproliferative neoplasms are not a contraindication for LT in BCS, since they do not seem to significantly affect the outcome after LT. However, as often happening with rare diseases, the experiences published are hardly sufficiently powered to reach adequate statistically significance. The role of LT in BCS should probably be that of salvage treatment, after all the other therapeutic attempts have failed. In fact, TIPS has been shown to be effective as BCS treatment and can be successful also in the technically difficult case of extension of thrombosis to the portal vein tree, with survival rates similar to those after LT (2, 3). Moreover, albeit without validated criteria, BCS management generally follows indication criteria that needs to be up-dated. In fact, following the so called step-wise strategy, longterm experiences reported poor outcome in the subgroup treated with only medical therapy (4). A reasoning model of BCS physiopathology is that not only impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development but also causes hepatic fibrosis development and liver failure through chronic ischaemic liver damage. The ischaemic liver cirrhosis (ILC) theory, albeit not evidence-based and requiring validation, postulated that cirrhosis development could arise from chronic micro-vascular ischaemia. One model of ILC theory is BCS. In fact, a substantial rate of patients with BCS finally develops cirrhosis, in the absence of known causative factors but chronic liver ischaemia. By reverting liver congestion through TIPS or other invasive treatment, liver function is expected to improve (5). Recently, I reported a proposal of a new algorithm for the management of BCS (4), in which medical
Liver International (2015) © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
therapy is suggested only for patients without any sign of portal hypertension, whether early interventional treatment is suggested when either any symptom or sign of portal hypertension appears, with the aim of preventing hepatic fibrosis development, disease progression and finally improving outcome. LT should only be considered as a salvage treatment. Acknowledgements
Financial support: None declared. Conflict of interest: The authors do not have any disclosures to report.
Andrea Mancuso1,2 1 Medicina Interna 1, ARNAS Civico - Di Cristina Benfratelli, Palermo, Italy 2 Epatologia e Gastroenterologia, Ospedale Niguarda Ca Granda, Milano, Italy
References 1. Potthoff A, Attia D, Pischke S, et al. Long-term Outcome of Liver Transplant Patients with Budd-Chiari Syndrome Secondary to Myeloproliferative Neoplasms. Liver Int 2015; 35: 2042–9. 2. Mancuso A, Fung K, Mela M, et al. TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience. J Hepatol 2003; 38: 751–4. 3. Mancuso A, Watkinson A, Tibballs J, Patch D, Burroughs AK. Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins. Gut 2003; 52: 438. 4. Mancuso A. An Update on Management of Budd-Chiari Syndrome: the Issues of Timing and Choice of Treatment. Eur J Gastro Hepatol 2015; 27: 200–3. 5. Mancuso A. Cirrhosis development probably arises from chronic micro-vascular ischemia. Med Hypotheses 2014; 82: 243–4.
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