Alveolar Echinococcosis (Alveolar Hydatid Disease)

Abstract Alveolar echinococcosis (AE), an infection with the larval form of Echinococcus multilocularis, is reported from many regions in the Northern Hemisphere. The liver is primarily affected, and patients typically present with advanced disease when surgical cure is no longer an option. Diagnosis and staging are based on ultrasound, computed tomography (CT) scan, and magnetic resonance imaging. Serology has a confirmatory role, and selected tests can be used to follow response to therapy and detect relapse. Therapy includes surgery and anti-parasitic therapy, depending on the stage and extent of disease at presentation. AE has similar features and management principles as malignancy. Endoscopic dilatation and stenting show promise. Liver transplantation should only be considered as a last resort in the very late stages of disease; recurrence rates are high after liver transplantation. Control and eradication of E. multilocularis is difficult to achieve due to the sylvatic parasite cycle.