The Echinococcoses: Diagnosis, Clinical Management and Burden of Disease

The echinococcoses are chronic, parasitic diseases that are acquired after ingestion of infective taeniid tapeworm eggs from certain species of the genus Echinococcus. Cystic echinococcosis (CE) occurs worldwide, whereas, alveolar echinococcosis (AE) is restricted to the northern hemisphere, and neotropical echinococcosis (NE) has only been identified in Central and South America. Clinical manifestations and disease courses vary profoundly for the different species of Echinococcus. CE presents as small to large cysts, and has commonly been referred to as 'hydatid disease', or 'hydatidosis'. A structured stage-specific approach to CE management, based on the World Health Organization (WHO) ultrasound classification of liver cysts, is now recommended. Management options include percutaneous sterilization techniques, surgery, drug treatment, a 'watch-and-wait' approach or combinations thereof. In contrast, clinical manifestations associated with AE resemble those of a 'malignant', silently-progressing liver disease, with local tissue infiltration and metastases. Structured care is important for AE management and includes WHO staging, drug therapy and long-term follow-up for at least a decade. NE presents as polycystic or unicystic disease. Clinical characteristics resemble those of AE, and management needs to be structured accordingly. However, to date, only a few hundreds of cases have been reported in the literature. The echinococcoses are often expensive and complicated to treat, and prospective clinical studies are needed to better inform case management decisions.