Primary disease recurrence after liver transplantation for alveolar echinococcosis: Long-term evaluation in 15 patients

✍ Scribed by Solange Bresson-Hadni; Stéphane Koch; Isabelle Beurton; Dominique-Angèle Vuitton; Brigitte Bartholomot; Stephan Hrusovsky; Bruno Heyd; Danièle Lenys; Anne Minello; Marie C. Becker; Claire Vanlemmens; Georges-André Mantion; Jean-Philippe Miguet

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 298 KB
• Volume: 30
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.510300426

✦ Synopsis

Between 1986 and 1991, 21 patients received liver grafts in our center for incurable alveolar echinococcosis (AE). The aim of this study was to analyze the long-term results in 15 of these 21 patients who survived more than 1 year after undergoing a liver transplantation (LT). The follow-up, mainly aimed at the diagnosis of recurrence, consisted of repeated radiological and specific immunological investigations. The role of pre-and post-LT benzimidazole (BZM) therapy was also evaluated. Among the 15 patients, 8 patients had a palliative LT related to previously known pulmonary AE metastases and/or inextirpable abdominal parasitic foci. In the 7 remaining patients, LT was considered curative. In June 1998, the mean follow-up duration was 96 months (range: 28-138 months). Five late deaths occurred, 2 of them were directly related to residual AE. A reinfection of the graft was observed in 4 patients. Preoperative BZM therapy seemed useful in preventing or delaying the parasitic recurrence. Post-LT BZM was able to stabilize and even to reduce residual AE. The anti-Em2 enzyme-linked immunosorbent assay (ELISA), which is the standard test used in patient follow-up after partial liver resection for AE, did not appear useful in detecting recurrence here; however, an ELISA, using a crude heterologous antigen (Echinococcus granulosus) allowed early diagnosis of residual AE. In conclusion, primary disease recurrence is not rare after LT for AE. Immunosuppressive therapy may favor larval growth in extrahepatic sites; therefore, an extensive extrahepatic radiological check-up has to be performed before LT. BZM therapy seems to stabilize residual foci. Anti-Eg immunoglobulin G (IgG) follow-up is the most useful test for early diagnosis of parasite recurrence. (HEPATOL-OGY 1999;30:857-864.) Alveolar echinococcosis (AE) is a rare parasitic disease caused by the intrahepatic growth of the taenia Echinococcus multilocularis larvae. The main endemic regions for human AE are Central Europe (Southern Germany, Western Austria, Eastern France), Russia, Turkey, Japan (Hokkaı ¨do), the Kurile Islands, China, and North America (Alaska, Northern Canada). In France, the Franche-Comte ´region is the most endemic region for AE. [1][2][3] One hundred seventeen consecutive patients were followed up in our Liver Unit between 1972 and 1993. 4 Humans are accidental intermediate hosts. They become infected either by eating wild vegetables contaminated by fox feces or by touching the animals themselves. The hepatic lesions of AE generally consist of a unique mass of fibrous tissue with numerous cavities, ranging in size from a few millimeters to a few centimeters. Extrahepatic locations, particularly pulmonary metastases, are observed in 20% of patients. [4][5][6] Although this taenia is similar to that of Echinococcus granulosus, which causes hydatid cysts of the liver, the 2 diseases are completely different. In contrast to E. granulosus, E. multilocularis larvae grow indefinitely; they produce liver necrosis and induce a major granulomatous reaction. Lesions are never limited by a fibrous shell and AE is often compared with a slow-growing liver cancer. Invasion of the bile ducts and the vessels and necrosis in the central part of the parasitic tissue lead to very severe complications such as cholangitis, liver abscesses, septic shock, portal hypertension, biliary cirrhosis, and parasitic Budd-Chiari syndrome, which can seriously affect not only the quality of life of these middle-aged patients, but also their survival. In fact, the disease is always fatal unless identified in time and then completely removed by partial hepatectomy. Long-term benzimidazole (BZM)-carbamates treatment may slow down the larval progression but does not prevent the complications related to the fibrous scars along the biliary and vascular tracts. [7][8][9] Patients with incurable AE can benefit from a liver transplantation (LT). 10 A recent European inquiry indicates that over the past 10 years 41 LTs have been performed for incurable AE. 11 Half of these patients came from our liver transplant unit: between 1986 to 1998, 21 of 257 LTs were performed for AE. This disease represents 8% of the indications for an LT in our center. The short-term results of LT for this rare indication have previously been reported. 10,12 The aim of this study was to analyze the long-term results in 15 patients with AE who survived more than 1 year after LT, focusing on the problem of the possible persistence of the parasitic larvae and thus potential recurrence of the disease, and to assess the particularities of the host-parasite interplay in these patients who underwent LT.