Abstract
Background
Alveolar echinococcosis (AE) of the liver, caused by the larval stage of the fox tapeworm Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. It is one of the rare parasitic diseases for which a parasitolytic drug is not yet available, and AE is lethal in the absence of appropriate therapeutic management. Complete surgical resection of the parasite at an early stage of infection provides favourable prospects for cure, but, due to a long clinical latency, many cases are diagnosed at an advanced stage, so that partial liver resection can be performed in only 35% of patients. Benzimidazole (BZM) treatment is given in inoperable cases but these compounds are only parasitostatic, and lifelong therapy is required. During the past 20 years some centres have considered liver transplantation (LT) for the treatment of incurable AE.
Methods
Our review summarizes the results of this experience based on a series of 47 European patients who received transplants between 1985 and 2002, tries to specify the real place of LT for AE, and underlines the measures that could be undertaken in the future to improve the results.
Results
Five-year survival was 71%. Five-year survival without recurrence was 58%. Major technical difficulties related either to previous laparotomies or to the loco-regional involvement were observed. The nine early deaths concerned AE patients with a long past-history of symptomatic AE (iterative cholangitis, secondary biliary cirrhosis). Five late deaths were directly related to ongoing AE, located in the brain in three cases, a very rare AE location that was not investigated before LT in these patients.
Conclusions
In general, the pre-LT screening for distant AE metastases appeared insufficient in this series. Heavy immunosuppressive schemes, absence or delayed re-introduction of BZM after LT have clearly played a role in this unfavourable course. This unique experience indicates that, despite major technical difficulties, LT for incurable AE is feasible and could be discussed in very symptomatic cases. Before LT, interventional radiology should be preferred to repeated laparotomies. Pre-LT and post-LT BZM treatment is mandatory. A careful evaluation of possible distant metastases should be done before the decision for LT is made. After LT, the possibility of an ongoing AE must be permanently kept in mind. This could be reduced by lightening the immunosuppressants, carefully following the specific circulating antibodies, and applying a systematic radiological evaluation, not only to the graft but also to the lungs and the brain.
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References
Bresson-Hadni S, Vuitton DA (2001) Echinococcoses. Rev Prat 51:2091–2098
Bresson-Hadni S, Vuitton DA, Bartholomot B, et al (2000) A twenty-year history of alveolar echinococcosis: analysis of a series of 117 patients from eastern France. Eur J Gastroenterol Hepatol 12:327–336
Chapuis Y, Houssin D, Brouzes S, et al (1987) Hepatic transplantation in alveolar echinococcosis. 3 attempts. Chirurgie 113:634–640
Mboti B, Van de Stadt J, Carlier Y, et al (1996) Long-term disease-free survival after liver transplantation for alveolar echinococcosis. Acta Chir Belg 96:229–232
Bresson-Hadni S, Franza A, Miguet JP, et al (1991) Orthotopic liver transplantation for incurable alveolar echinococcosis of the liver: report of 17 cases. Hepatology 13:1061–1070
Ammann RW, Ilitsch N, Marincek B, et al (1994) Effect of chemotherapy on the larval mass and the long-term course of alveolar echinococcosis. Hepatology 19:735–742
Koch S, Bresson-Hadni S, Miguet JP, et al (2003) Experience of liver transplantation for incurable alveolar echinococcosis: a 45 case European collaborative report. Transplantation 75:856–863
Bresson-Hadni S, Laplante JJ, Lenys D, et al (1994) Seroepidemiological screening of E. multilocularis infection in 7,884 subjects of a European endemic area of alveolar echinococcosis. Am J Trop Med Hyg 51:837–846
Gillet M, Miguet JP, Mantion G, et al (1988) Orthotopic liver transplantation in alveolar echinococcosis of the liver: analysis of a series of six patients. Transplant Proc 20:573–576
Slim K, Chipponi J, Pezet D, et al (1996) Récidive d'une échinococcose alvéolaire après transplantation hépatique. Lyon Chir 92:301–303
Bresson-Hadni S, Koch S, Beurton I, et al (1999) Primary disease recurrence after liver transplantation for alveolar echinococcosis: long-term evaluation in 15 patients. Hepatology 30:857–864
Gottstein B, Schantz PM, Wilson JF (1985) Serological screening of Echinococcus multilocularis infections with ELISA. Lancet i:1097–1098
Gottstein B, Tschudi K, Eckert J, et al (1989) Em2 Elisa for the follow-up of alveolar echinococcosis after complete surgical resection of liver lesions. Trans R Soc Trop Med Hyg 83:389–393
Bresson-Hadni S, Miguet JP, Lenys D, et al (1992) Recurrence of alveolar echinococcosis in the liver graft after liver transplantation. Hepatology 16:279–280
Liance M, Bresson-Hadni S, Vuitton DA, et al (1992) Effects of cyclosporin A on the course of murine alveolar echinococcosis and on specific cellular and humoral responses against Echinococcus multilocularis. Int J Parasitol 22:23–28
Ammann RW, Hoffmann AF, Grimm F, et al (1998) Long-term mebendazole therapy may be parasitocidal in alveolar echinococcosis. J Hepatol 29:994–998
Wang X, Liu Y, Yu D (1996) Continuous therapy with albendazole for hepatic alveolar echinococcosis associated with obstructive jaundice. Clin J Intern Med 35:261–264
WHO Informal Working group on Echinococcosis (1996) Guidelines for treatment of cystic and alveolar echinococcosis in humans. Bull World Health Organ 74:231–242
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The authors wrote this report in collaboration with a European group of clinicians, which included the following participants. France: Jean-Pierre Crumbach, Sabine Kurz, Liver Transplant Unit, CHU Besançon; Anne Minello, Patrick Hillon, CHU Dijon and Liver Transplant Unit, CHU Besançon; Didier Samuel, Henri Bismuth, Centre Hepato-biliaire, Hopital Paul-Brousse, Paris; Maxime Audet, Philippe Wolf, Daniel Jaeck, Strasbourg; Patrick Boissel, Nancy; Filomena Conti, Yves Chapuis, Hopital Cochin, Paris; Jean Chipponi, Clermont-Ferrand; Michel Pouyet, Hôpital de la Croix Rousse, Lyons; Martine Neau-Cransac, Jean-Yves Lacut, Jacques Carles, Bordeaux; Belgium: Jean Van de Stadt, Yves Carlier, Brussels; Switzerland: Philippe Morel, Gilles Mentha, Petro Majno, Geneva; Germany: Markus Golling, Gerd Otto, Heidelberg; Juergen Klempnauer, Karl Oldhafer, Hanover; Ewert Schutte-Frohlinde, Peter Vorwald, Munich; Olaf Guckelberger, Berlin; Carl Allers, Frankfurt; Johannes Scheele, Jena; Tobias Beckurts, Cologne
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Bresson-Hadni, S., Koch, S., Miguet, JP. et al. Indications and results of liver transplantation for Echinococcus alveolar infection: an overview. Langenbecks Arch Surg 388, 231–238 (2003). https://doi.org/10.1007/s00423-003-0394-2
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DOI: https://doi.org/10.1007/s00423-003-0394-2