Echinococcosis Treatment & Management

  • Author: Dominique A Vuitton, MD, PhD; Chief Editor: Burke A Cunha, MD   more...
 
Updated: Oct 20, 2011
 

Medical Care

Alveolar echinococcosis is rare and can be severe. All cases merit prolonged follow-up. Refer patients to reference centers to confirm their diagnosis and to obtain advice on therapeutic strategy.

  • Antiparasitic chemotherapy
    • The basic medical treatment is chemotherapy with benzimidazoles (eg, mebendazole, albendazole) at high doses.
    • According to the 1996 WHO Informal Working Group on Echinococcosis and recently updated consensus recommendations,[2] long-term chemotherapy, for several years (and possibly for life), is mandatory in inoperable patients. The decision to withdraw treatment is particularly difficult without objective and irrefutable proof of definitive cure. An attempt at withdrawing benzimidazole treatment may rely on negative FDG-PET scanning findings, including delayed images and negative EM2+ or Em18 serology results. Follow-up with careful PET scanning and serology should be performed first 3 months after treatment interruption and then yearly for 10 years.
    • Complementary and continuous chemotherapy with, preferably, albendazole is mandatory for at least 2 years following surgery. Careful follow-up examinations in these patients must continue for at least 10 years. In all cases of palliative operations, either surgical or ultrasonographically guided, chemotherapy is mandatory and follows the same therapeutic schedule as for patients who have not undergone surgery.
    • Intravenous amphotericin B (preferably as lipid emulsion) may be used as a rescue chemotherapy in patients resistant or intolerant to benzimidazoles. Pilot trials with interferon-gamma and nitazoxanide were unsuccessful. Interferon-alpha has yet to be tested in a pilot trial.
    • Administer chemotherapy for at least 2 years after radical liver transplantation and for life in patients who demonstrate evidence of parasitic remnants in the liver area and/or of distant metastases outside the liver.
  • Other medication
    • Additional medical treatment includes antibiotics and antifungal agents for bacterial and fungal superinfection of the lesions, cholangitis and/or septicemia (mostly gram-negative bacteria, antibiotic-resistant Streptococcus faecalis, Pseudomonas aeruginosa, and Candida species after surgical interventions), and chronic cholestasis (including vitamin K and D supplementation).
    • Use propranolol to prevent digestive bleeding related to portal hypertension.
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Surgical Care

Except in cases of limited lesions located in the left liver lobe, attempt surgical procedures only if the team is trained and equipped for major liver surgery.

  • Radical surgery
    • If operation is feasible and if resection of the entire parasitic lesion from other affected organs is possible, surgery is the first treatment choice.
    • Recurrence has been observed after liver resections judged radical by the surgeon based on macroscopic evidence.
  • Palliative surgery
    • When radical surgery is impossible, one option is nonradical liver resection to reduce the parasitic mass and to increase the chances of effective chemotherapy. These palliative resections, as well as other types of palliative surgery performed to treat complications of disease (especially biliary obstruction), may generate specific complications (eg, recurrent biliary obstruction with cholangitis, septicemia, intrahepatic gallstones leading to secondary biliary cirrhosis). Long-term follow-up of patients shows that palliative or debulking surgery should be avoided.
    • The current recommendation is to avoid palliative surgical procedures because of the relative efficacy of medical treatment, the use of interventional radiology and/or endoscopy, and a possible further indication of liver transplantation in patients with severe disease.
  • Liver transplantation
    • Consider liver transplantation in very advanced cases. Since 1986, more than 50 liver transplantations have been performed in patients with alveolar echinococcosis. The overall associated survival rate is lower than that of other indications for liver transplantation but is acceptable. Survival periods of more than 20 years have been observed in patients with alveolar echinococcosis who have undergone liver transplantation, even in those with residual or recurrent lesions.
    • Recurrences and metastases are common after transplantation (even those judged radical) in immunosuppressed patients. Benzimidazole treatment is mandatory as soon as possible after transplantation in all patients; such therapy can stop the progression of residual or recurrent lesions.
  • Interventional radiology or endoscopy
    • Consider ultrasonographically guided percutaneous procedures (eg, internal/external biliary drainage, abscess drainage, stenting) to alleviate intrahepatic complications.
    • Consider endosonography-guided permanent stenting of the bile duct in patients with biliary obstruction.
    • Consider using endoscopic sclerosis of esophageal varices to prevent hemorrhages due to portal hypertension.
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Consultations

  • Before any therapeutic decision, especially if major hepatectomy or liver transplantation is considered, carefully assess for distant metastasis (ie, brain, lung, bone localizations). Include appropriate morphologic examinations and consultations. Discuss the case with liver and infectious disease specialists, radiologists, and all involved surgeons.
  • Neurosurgery and thoracic surgery may be indicated if brain or lung metastasis is complicated and/or accessible to surgery. These decisions and procedures require appropriate consultations.
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Diet

Patients require no special diet, except those with chronic cholestasis.

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Activity

Activity modification is not indicated, but chronic inflammation and cytokine production usually result in fatigue, which typically limits activity and may greatly impair quality of life.

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Dominique A Vuitton, MD, PhD  Coordinator of International Affairs, WHO Collaborating Center for Prevention and Treatment of Echinococcosis, Professor Emeritus in Clinical Immunology, University of Franche-Comté, Besançon, France

Dominique A Vuitton, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Specialty Editor Board

John M Leedom, MD  Professor Emeritus of Medicine, Keck School of Medicine of the University of Southern California

John M Leedom, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society for Microbiology, Infectious Diseases Society of America, International AIDS Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

John W King, MD  Professor of Medicine, Chief, Section of Infectious Diseases, Director, Viral Therapeutics Clinics for Hepatitis, Louisiana State University Health Sciences Center; Consultant in Infectious Diseases, Overton Brooks Veterans Affairs Medical Center

John W King, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Federation for Medical Research, American Society for Microbiology, Association of Subspecialty Professors, Infectious Diseases Society of America, and Sigma Xi

Disclosure: emedicine $50.00 Author of chapter; MERCK None Other

Eleftherios Mylonakis, MD  Clinical and Research Fellow, Department of Internal Medicine, Division of Infectious Diseases, Massachusetts General Hospital

Eleftherios Mylonakis, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Microbiology, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Chief Editor

Burke A Cunha, MD  Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital

Burke A Cunha, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Additional Contributors

Thanks to all colleagues who are actively working within the framework of the WHO-Collaborating Centre for Prevention and Treatment of Human Echinococcosis, and more generally within the framework of the WHO-Informal Working Group on Echinococcosis.

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Foxes are the definitive hosts of the cestode Echinococcus multilocularis. Courtesy of Dominique A. Vuitton, MD, PhD.
Macroscopic aspect of alveolar echinococcosis lesions in the liver. Courtesy of Bernadette Kantelip, MD.
Ultrasonographic, CT scan, and perioperative aspect of a typical lesion of alveolar echinococcosis with central necrosis. Courtesy of Jean-Philippe Miguet, MD.
Microtus larvalis (common vole) is one of the most common intermediate hosts of Echinococcus multilocularis in Europe. Courtesy of Patrick Giraudoux, PhD.
Sonogram of a typical form of alveolar echinococcosis of the liver, discovered at a screening in China. Courtesy of Dominique A. Vuitton, MD, PhD; Brigitte Bartholomot, MD; and Philip S. Craig, PhD.
Sonogram of an abortive form of alveolar echinococcosis of the liver, discovered at a screening in China. Courtesy of Dominique A. Vuitton, MD, PhD; Brigitte Bartholomot, MD; and Philip S. Craig, PhD.
Pathognomonic aspect of alveolar echinococcosis lesions invading the adrenal gland (resembling a honeycomb) that shows a necrotic area in the contiguous left liver lesion; MRI showing multiple parasitic cysts smaller than 1 cm in diameter appearing in high signal intensity on T2-weighted sequence. Courtesy of Brigitte Bartholomot, MD.
Brain metastasis of alveolar echinococcosis. Courtesy of Jean-Philippe Miguet, MD.
Skin metastasis of alveolar echinococcosis. Courtesy of Solange Bresson-Hadni, MD, PhD.
Histologic features of alveolar echinococcosis vesicles and periparasitic granuloma in humans, periodic acid-Schiff staining of the laminated layer. Courtesy of Bernadette Kantelip, MD.
Fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan aspect of active alveolar echinococcosis. White-yellow colors show a very high FDG uptake due to the periparasitic granulomatous infiltration and/or active germinal layer of Echinococcus multilocularis, and green-gray colors show the absence of the FDG uptake by inactive parasitic lesions (mostly necrotic). Courtesy of Solange Bresson-Hadni, MD, PhD, and Oleg Blagosklonov, MD, PhD.
Fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan aspect of inactive alveolar echinococcosis. No abnormal FDG uptake by the parasitic lesions after several years of albendazole treatment. Courtesy of Solange Bresson-Hadni, MD, PhD, and Oleg Blagosklonov, MD, PhD.
 
 
 
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