Guidelines for treatment of cystic and alveolar echinococcosis in humans. WHO Informal Working Group on Echinococcosis.

Summarized in this article are recent experiences in the treatment of human cystic echinococcosis (CE) and alveolar echinococcosis (AE) of the liver caused by the metacestode stages of Echinococcus granulosus and E. multilocularis, respectively. For CE, surgery remains the first choice for treatment with the potential to remove totally the parasite and completely cure the patient. However, chemotherapy with benzimidazole compounds (albendazole or mebendazole) and the recently developed PAIR procedure (puncture-aspiration-injection-re-aspiration) with concomitant chemotherapy offer further options for treatment of CE cases. Chemotherapy is not yet satisfactory: cure can be expected in about 30% of patients and improvement in 30-50%, after 12 months' follow-up. AE is generally a severe disease, with over 90% mortality in untreated patients. Radical surgery is recommended in all operable cases but has to be followed by chemotherapy for at least 2 years. Inoperable cases and patients who have undergone nonradical resection or liver transplantation require continuous chemotherapy for many years. Long-term chemotherapy may significantly prolong survival, even for inoperable patients with severe AE. Liver transplantation may be indicated as a life-saving measure for patients with severe liver dysfunction, but is associated with a relatively high risk of proliferation of intraoperatively undetected parasite remnants. Details of indications, contraindications, treatment schedules and other aspects are discussed.