Authors
M. ABDESSALAMI (1), G. RASSCHAERT (1), L. DUEZ (1), C. SALEM (1), P. EISENDRATH (1), T. SERSTE (2) / [1] CHU Saint-Pierre, Brussels, Belgium, Hepato Gastroenterology, [2] CHU Saint-Pierre, Brussels, Belgium, Hépato-Gastroenterologie
Introduction Alveolar echinococcosis (AE) is caused by the larval stage of the fox tapeworm Echinococcus multilocularis. This rare parasitic infection can be associated with biliary complications (biliary strictures, dilations and fistulas) and may eventually lead to secondary biliary cirrhosis. Apart from partial hepatectomy, treatment options include long term antiparasitic chemotherapy. Biliary damage occurs in about 30% of cases and is potentially treated with therapeutic endoscopic procedures. Case report A 30-year-old Caucasian male, presented at our institution with ascites. He was treated 3 years ago in Russia for an undisclosed affection of the liver leading to a partial right hepatectomy. Exact information on his medical record was difficult to obtain. Numerous stellate angiomata and tensed ascites were observed during clinical examination. A chest X-ray suggested the presence of an organized pleural effusion. The blood panel demonstrated mild cholestasis (alkaline phosphatase 1.3 times the upper limit of the normal), mild hyperbilirubinemia (3.1mg/dL), thrombocytopenia (65.000 platelets per µL) and a prothrombin time of 66.9%. Viral serology showed the presence of HBs Ag without detectable DNA levels. Magnetic resonance cholangiopancreatography (MRCP) revealed a highly dysmorphic liver. The post-hepatectomy status of liver segments VI and VII was confirmed. Multiple cystic lesions (uncountable, about 5 to 10 mm) with thick walls, without bile duct communication, were found throughout the liver parenchyma. Multiple biliary duct stenosis and dilations, both intrahepatic and extrahepatic were described on MRCP. These abnormalities mimicked sclerosing cholangitis and were mainly located on right liver. A 2 cm long filiform subhilar bile duct stricture required insertion of 3 plastic biliary stents (12 and 15 cm, 8.5 French) via endoscopic retrograde cholangiopancreatography (ERCP). This endoscopic procedure confirmed the sclerosing cholangitis-like shape of the right intrahepatic bile tree and disclosed a biliary fistula communicating with the right pleural cavity. Three different parasite serology tests for AE performed on a blood sample were positive: a haemagglutination essay, a western blot and an ELISA EM18 (Kit Bordier®). Western blotting was used as confirmatory test. Moreover, the ELISA that was highly positive, is known to be the most specific for AE. Liver biopsy was performed and found to be compatible with the diagnosis of secondary biliary cirrhosis and microscopic cholangitis. The final diagnosis of AE and secondary biliary cirrhosis was retained. A long term treatment with albendazole 800mg daily was initiated. Pleural effusion and ascites were controlled after biliary drainage and proper diuretic prescription. Median and long term clinico-biological evolution is currently under investigation and the hypothesis of a future liver transplant as a definitive cure is not ruled out. Discussion Echinococcus multilocularis is distributed throughout the Northern Hemisphere. It remains a rare disease in Western Europe, compared to endemic regions such as Russia where our patient was born. During the course of the disease, AE can mimic a neoplastic lesion with progressive growth and dissemination from the liver towards several organs. Treatment options include surgery, as the preferred first line therapy (including liver transplantation), and long-term chemotherapy with benzimidazoles. Despite treatment, biliary complications still occur in one third of the patients, significantly influencing the outcome. The bile ducts and the intrahepatic biliary tree can be compressed or even destroyed by the growing parasite. The clinical presentation of these biliary complications encompass cholangitis lesions (defined as abnormal ERCP or MRCP findings that could evocate sclerosing cholangitis), common bile duct stenosis, biliary fistulas and secondary biliary cirrhosis. AE is a severe infectious disease with significant morbidity and mortality despite substantial increases in diagnostic and therapeutic improvements. Conclusion This case report of hepatic AE with biliary strictures and fistulas leading to secondary biliary cirrhosis, illustrates the whole spectrum of biliary complications that potentially characterize the natural course of this rare infectious disease. References 1. Torgerson PR, Schweiger A, Deplazes P,et al. Alveolar echinococcosis: from a deadly disease to a well-controlled infection. Relative survival and economic analysis in Switzerland over the last 35 years. J Hepatol 2008; 49: 72-77 2. Kadry Z, Renner EC, Bachmann LM,et al. Evaluation of treatment and long-term follow-up in patients with hepatic alveolar echinococcosis. Br J Surg 2005; 92: 1110-1116