Echinococcosis Hydatid Cyst Treatment & Management

  • Author: Enrico Brunetti, MD; Chief Editor: Burke A Cunha, MD   more...
 
Updated: Oct 19, 2011
 

Medical Care

  • Two benzimidazolic drugs, mebendazole and albendazole, are the only anthelmintics effective against cystic echinococcosis. Albendazole and mebendazole are well tolerated but show different efficacy.
    • Albendazole is significantly more effective than mebendazole in the treatment of liver cysts. Benzimidazole treatment alone requires prolonged administration over many weeks, with an unpredictable outcome in terms of response rates in individuals.[24]
    • Treatment with albendazole in E granulosus infection can result in an apparent cure in as many as 30% of patients, with a further 40-50% of patients showing objective evidence of response when observed short term. Patients who do not show obvious initial evidence of response may be found to be cured when observed over several years.
  • Duration of therapy and doses are also important. Albendazole efficacy increases with courses of up to 3 months in the more common cyst sites.
  • Patients once received these drugs in cycles of 4 weeks separated by 1-2 weeks without drugs. This regimen is no longer advocated given the parasitostatic activity of benzimidazoles and their safety as shown by cumulative data from several retrospective studies. Continuous treatment is preferred and has been administered for periods of up to 2 years without significant side effects.[25, 13] The safety profile shows that liver function abnormalities are common, although they rarely limit treatment, while occasional hematologic changes affecting white cells may be more serious. The safety data supply the rationale for monitoring patients during treatment.
  • Overall, albendazole has been demonstrated to be a useful advance in the management of cystic echinococcosis when used as sole treatment or as an adjunct to surgery or other treatments.
  • Praziquantel has recently been suggested, administered additionally once per week in a dose of 40 mg/kg during treatment with albendazole. However, available data are limited.[26]
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Surgical Care

  • Surgery was the only treatment available before the introduction of anthelmintic drugs. It is considered the first choice of treatment for echinococcosis but is associated with considerable mortality (up to 2% in some series, increasing with second and further operations), morbidity,[27] and recurrence rates (2-25%). Given the more frequent detection of early and asymptomatic E granulosus liver lesions, a widened indication for chemotherapy exists.
  • Several procedures have been described for the treatment of hepatic echinococcal cysts, ranging from simple puncture to liver resection and transplantation, although the most commonly used technique is total or partial cystopericystectomy.
  • Usually, radical surgery (total pericystectomy or partial hepatectomy) is indicated for liver cysts. Conservative surgery (open endocystectomy with or without omentoplasty) or palliative surgery (simple tube drainage of infected cysts or communicating cysts) is also an option. More radical interventions have higher intraoperative risks but less numerous relapses. With the inclusion of chemotherapy prior to or after surgery, less-aggressive surgery may be possible.
  • Surgery for pulmonary cysts includes extrusion of cysts using Barrett technique (intact endocystectomy without preliminary aspiration), pericystectomy, and lobectomy.[28]
  • Peripheral and unilobar echinococcal cysts, regardless of how complicated they are, can also be treated with laparoscopic surgery using partial cystopericystectomy and drainage. When surgery cannot be avoided, presurgical use of albendazole reduces risk of recurrence and facilitates surgery by reducing intracystic pressure.

Percutaneous treatment

Minimally invasive treatment involves the following:

  • The puncture of echinococcal cysts has long been discouraged because of risks of anaphylactic shock and spillage of the fluid; however, as experience with ultrasonography-guided interventional techniques has increased since the early 1980s, an increasing number of articles have reported its effectiveness and safety in treating abdominal, especially liver, echinococcal cysts. A recent systematic review of the literature found that the overall fatality rate due to lethal anaphylaxis from puncture of echinococcal cysts is 0.03% (2 in 5943 procedures) for procedures and 0.04% (2 in 5517 cysts) for cysts respectively[23] .
  • One study of 446 patients who were treated surgically for cystic echinococcosis (CE) found an increased incidence in anaphylatic shock in younger patients (P < 0.001) and in patients with pulmonary cysts. The authors suggest taking precautions such as reducing intracystic pressure, preventing antigen from contacting other tissues where it might trigger anaphylaxis, and resecting the cyst completely when feasible.[29]
  • Under albendazole coverage, cysts are punctured under ultrasonographic or CT guidance either with a needle or with a catheter according to their size. The presence of an anesthesiologist who intervenes in case of allergic manifestations or anaphylactic shock is mandatory. Usually, a small quantity of fluid is first aspirated and examined by light microscope to observe for the presence of viable protoscolices. If they are present, the cyst is aspirated completely.
  • At this point, exclude possible connections of the cyst with the biliary tree by means of injection of contrast medium in the cavity. If no connections are evident, a scolecoidal agent, usually hypertonic sodium chloride solution or ethanol, is injected and left for a variable period (usually 5-30 min) and then reaspirated. The destruction of protoscolices can be observed in fluid sample aspirated after the injection of a scolecoidal agent. This sequence is termed PAIR (puncture, aspiration, injection, reaspiration). As happens with drug therapy, positive responses include both a decrease in cyst size and a progressive change in echo pattern (generally solidification).[30, 31, 32]
  • From a diagnostic standpoint, PAIR is the only method that helps provide a direct diagnosis of the parasitic nature of the cysts. Neither imaging modalities nor serology is sufficient to exclude the diagnosis. PAIR is also an effective alternative to chemotherapy alone because it has a higher efficacy and avoids the problem of drug resistance. It also shortens the time of treatment and final recovery. PAIR is a valuable alternative to surgery in terms of cost containment and hospitalization time.[33, 34, 35, 36, 37, 38] In types I and II (Gharbi classification), CE1 and CE3a (WHO-IWGE classification) echinococcal cysts with no or incomplete response to therapy, PAIR is an effective therapeutic tool in the management of human cystic echinococcosis.
  • Increasing evidence shows that CE2 cysts (multivesiculated, type I in Gharbi classification) and CE3b (predominantly solid with daughter cysts)[13] tend to relapse after PAIR,[39] so other percutaneous treatments should be adopted, if indicated, for this type of cyst.
  • Reserve PAIR for use in highly specialized centers where teams are well prepared to deal with possible complications.
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Consultations

  • Consult a surgeon to discuss the opportunity of surgical intervention.
  • Consult a radiologist for injection of contrast medium in the cyst after fluid aspiration if PAIR is scheduled. Contrast injection in the cyst allows the physician to exclude connections of the cyst with the biliary tree. Contact of scolecoidal agents, such as alcohol and hypertonic sodium chloride solution, with the biliary epithelium may lead to cholangitis. Attempts to inject albendazole directly into the cysts have yielded interesting results in animal studies but are still methodologically weak in human studies. Most recently, a glucose solution as a scolecoidal agent has been used with good results in vitro.[40] Studies in vivo should confirm the safety of this approach before it can be applied to humans.
  • Consult an anesthesiologist for assistance in case of anaphylactic shock or anaphylactoid reactions if PAIR is scheduled.
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Contributor Information and Disclosures
Author

Enrico Brunetti, MD  Assistant Professor, Department of Infectious Diseases, Division of Infectious and Tropical Diseases, University of Pavia, Italy; Staff Physician, Department of Infectious Diseases, IRCCS S Matteo Hospital Foundation, Pavia, Italy

Enrico Brunetti, MD is a member of the following medical societies: American Society of Tropical Medicine and Hygiene and European Society of Clinical Microbiology and Infectious Diseases

Disclosure: Nothing to disclose.

Coauthor(s)

Carlo Filice, MD  Chief of Ultrasound Unit, Associate Professor, Department of Internal Medicine, IRCCS S Matteo Hospital, University of Pavia, Italy

Disclosure: Nothing to disclose.

Specialty Editor Board

Mark R Wallace, MD, FACP, FIDSA  Clinical Professor of Medicine, Florida State University College of Medicine; Head of Infectious Disease Fellowship Program, Orlando Regional Medical Center

Mark R Wallace, MD, FACP, FIDSA is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Tropical Medicine and Hygiene, and Infectious Diseases Society of America

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.

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WHO Informal Working Group on Echinococcosis standardized ultrasound classification of echinococcal cysts. Image courtesy of World Health Organization (WHO).
CE1 cyst in the right segments of the liver in a young Albanian boy
CE3a echinococcal cyst of the liver. Note the folding endocyst ("waterlily sign") typical of CE3a cysts.
CE3b cyst in the right lobe of the liver. The cyst is predominantly solid with a few daughter cysts
CE4 cyst in the liver. The cyst is completely solid and inactive.
CE3b cyst in the muscles of posterior thigh (scanned with a convex ultrasound probe).
CE4 cysts in the peritoneum in a patient with disseminated echinococcosis (longitudinal scan).
Viable scolices (note rostellar hooklets).
CT scan of peritoneal, disseminated echinococcosis
MR scan of echinococcal cyst in the right psoas muscle, infiltrating the adjacent vertebral body
Ultrasound scan of a CE3b subcutaneous cyst located in the lumbar area. The cyst was the subcutaneous extension of a cyst located in the spine that had been previously operated on.
MR of the spine showing CE involvement of CE3,CE4,CE5 vertebral bodies, spinous processes and subcutaneous tissue (same patient as # )
CT scan showing CE infiltration of vertebral bodies and destruction of left peduncles
 
 
 
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