Alveolar Echinococcosis (AE) Guidelines

Updated: Nov 08, 2019
  • Author: Dominique A Vuitton, MD, PhD; Chief Editor: Burke A Cunha, MD  more...
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Guidelines Summary

Guidelines for the treatment of alveolar echinococcosis (AE) were published in 1996 [45] and updated in 2010 [46] by the WHO-Informal Working Group on Echinococcosis. The most recent comprehensive reviews on the clinical aspects of alveolar echinococcosis were published by Kern et al in 2017 [12] and Wen et al in 2019. [26] Although the level of evidence is not high (no prospective controlled studies for this rare disease), the current guidelines are based on sound retrospective evaluation of treatment in reference centers, as follows:

  • Combined surgical and nonsurgical interventions contribute to the treatment of patients with alveolar echinococcosis.
  • Therapeutic decisions should be made by a multidisciplinary team, with a multimodality imaging approach, taking the number, size, and anatomical location of lesions into account, as well as the general status and the specific situation of the patient.
  • Surgery should be used only when it may reasonably be curative and accompanied by administration of albendazole for 2 years, regardless of the “safety margin” and the opinion of the surgeon at operation.
  • Ex-vivo lesion resection with autotransplantation (ELRA) may be a technical solution to extend the indications of hepatic resection and to avoid liver allotransplantation more prone to disease recurrence because of the antirejection treatment.
  • All patients who cannot benefit from radical resection should be treated with lifelong albendazole therapy.
  • Percutaneous procedures are best indicated for bacterial/fungal infection of the central necrotic cavity, which is often present in advanced cases.
  • Perendoscopic procedures are best indicated for compression or obstruction of the bile ducts by the parasitic lesion, with resulting jaundice and/or cholangitis. To prevent recurrence of cholangitis and/or biliary obstruction, it is necessary to remove all intrahepatic gallstones and parasitic debris, to perform intensive lavage of the bile ducts with isotonic saline, to administer adapted antibiotics, and to use multiple plastic stents.
  • Palliative surgery should be reserved for extremely selected patients.