Hydatid Cysts Treatment & Management
- Author: Imad S Dandan, MD; Chief Editor: BS Anand, MD more...
Medical management differs for CE and AE. In CE, surgery remains the primary treatment and the only hope for complete cure. Better forms of chemotherapy and newer methods, such as the puncture, aspiration, injection, and reaspiration (PAIR) technique are now available but need to be tested. Currently, indications for these modes of therapy are restricted. In AE, radical surgical excision is coupled with chemotherapy in operable cases and long-term aggressive chemotherapy for partially resected or unresectable lesions. In CE, one should consider the risks and benefits, indications, and contraindications for each case before making a decision regarding the type and timing of surgery.
- Chemotherapy in CE
- Indications: Chemotherapy is indicated in patients with primary liver or lung cysts that are inoperable (because of location or medical condition), patients with cysts in 2 or more organs, and peritoneal cysts.
- Contraindications: Early pregnancy, bone marrow suppression, chronic hepatic disease, large cysts with the risk of rupture, and inactive or calcified cysts are contraindications. A relative contraindication is bone cysts because of the significantly decreased response.
- Chemotherapeutic agents: Two benzimidazoles are used, albendazole and mebendazole. Albendazole is administered in several 1-month oral doses (10-15 mg/kg/d) separated by 14-day intervals. New data for continuous treatment are emerging from China. The optimal period of treatment ranges from 3-6 months, with no further increase in the incidence of adverse effects if this period is prolonged. Mebendazole is also administered for 3-6 months orally in dosages of 40-50 mg/kg/d. Limited data are available on the weekly use of praziquantel, an isoquinoline derivative, at a dose of 40 mg/kg/wk, especially in cases in which intraoperative spillage has occurred. Albendazole has been found ineffective in the treatment of primary liver cysts in patients who are surgical candidates.
- Monitoring: Monitor patients for adverse effects of agents every 2 weeks with a CBC count and liver enzyme evaluation for the first 3 months and then every 4 weeks. Monitoring albendazole and mebendazole serum levels is desirable, but few laboratories are capable of performing this measurement. Imaging studies are required for follow-up on the morphologic status of the cyst.
- Outcome from medical treatment of CE: Response rates in 1000 treated patients showed that 30% had cyst disappearance (cure), 30-50% had a decrease in the size of the cyst (improvement), and 20-40% had no changes. Also, younger adults responded better than older adults.
- Chemotherapy in AE
- Indications: Chemotherapy with benzimidazoles is used perioperatively for approximately 2 years in patients in whom radical resection is feasible because of possible undetected residual parasite tissue. In patients who undergo a partial resection, patients who are inoperable, or patients who have had a liver transplant, long-term chemotherapy is required (3-10 y).
- Contraindications: Because chemotherapy is the only treatment in certain cases, contraindications are limited to early pregnancy and severe leukopenia. Chemotherapeutic agents and patient monitoring are the same as with CE, but the length of treatment is different.
- Outcome: A significant increase in 10-year survival rates exists in patients receiving chemotherapy compared to patients who are not (85-90% vs 10%, respectively).
- PAIR in CE: The Puncture Aspiration Injection Reaspiration (PAIR) technique is performed using either ultrasound or CT guidance, involves aspiration of the cyst contents via a special cannula, followed by injection of a scolicidal agent for at least 15 minutes, and then reaspiration of the cystic contents. This is repeated until the return is clear. The cyst is then filled with isotonic sodium chloride solution. Perioperative treatment with a benzimidazole is mandatory (4 d prior to the procedure and 1-3 mo after).
- The PAIR technique can be performed on liver, bone, and kidney cysts but should not be performed on lung and brain cysts. The cysts should be larger than 5 cm in diameter and type I or II according to the Gharbi ultrasound classification of liver cysts (ie, type I is purely cystic; type II is purely cystic plus hydatid sand; type III has the membrane undulating in the cystic cavity; and type IV is the peripheral or diffuse distribution of coarse echoes in a complex and heterogeneous mass). PAIR can be performed on type III cysts as long as it is not a honeycomb cyst.
- Indications: Inoperable patients; patients refusing surgery; patients with multiple cysts in segment I, II, and III of the liver; and relapse after surgery or chemotherapy are indications for the PAIR technique.
- Contraindications: Early pregnancy, lung cysts, inaccessible cysts, superficially located cysts (risk of spillage), type II honeycomb cysts, type IV cysts, and cysts communicating with the biliary tree (risk of sclerosing cholangitis from the scolecoidal agent) are contraindications for the PAIR technique.
- Outcome: The reduced cost and shorter hospital stay associated with PAIR compared to surgery make it desirable. The risk of spillage and anaphylaxis is considerable, especially in superficially located cysts, and transhepatic puncture is recommended. Sclerosing cholangitis (chemical) and biliary fistulas are other risks. Experience is still limited, but early reports are supportive of this technique if the indications are followed.
- Interventional procedures in AE: Patients with AE require interventional procedures when radical complete resective surgery is not possible. Local complications may occur. These interfere with the function of the organ and may be alleviated by certain interventional procedures. These procedures can be performed endoscopically or under ultrasound or CT guidance. Dilatation, stenting, drainage of collections, and sclerosis of esophageal varices are some examples.
- Indications: These include hyperbilirubinemia, vena cava thrombosis, portal vein thrombosis, necrotic collections, and bleeding esophageal varices.
- Contraindications: Postinterventional chemotherapy is not possible, and the risk of spreading the parasite is high.
The indications and type of surgery are different for CE and AE.
- Cystic echinococcosis
- Indications: Large liver cysts with multiple daughter cysts; superficially located single liver cysts that may rupture (traumatically or spontaneously); liver cysts with biliary tree communication or pressure effects on vital organs or structures; infected cysts; and cysts in lungs, brain, kidneys, eyes, bones, and all other organs are indications for surgery.
- Contraindications: General contraindications to surgical procedures (eg, extremes of age, pregnancy, severe preexisting medical conditions); multiple cysts in multiple organs; cysts that are difficult to access; dead cysts; calcified cysts; and very small cysts are contraindications.
- Choice of surgical technique: Radical surgery (total pericystectomy or partial affected organ resection, if possible), conservative surgery (open cystectomy), or simple tube drainage of infected and communicating cysts are choices for surgical technique. The more radical the procedure, the lower the risk of relapses but the higher the risk of complications. Patient care must be individualized accordingly.
- Description of surgical procedure
- The basic steps of the procedure are eradication of the parasite by mechanical removal, sterilization of the cyst cavity by injection of a scolicidal agent, and protection of the surrounding tissues and cavities.
- Scolicidal agents include formalin, hydrogen peroxide, hypertonic saline, chlorhexidine, absolute alcohol, and cetrimide. A variety of complications have been described with all scolicidal agents, but in the authors' experience, 0.5% cetrimide solution provides the best protection with the least complications. Other scolicidal agents are 70-95% ethanol and 15-20% hypertonic saline solutions. A report by Ochieng'-Mitula and Burt in 1996 on the injection of ivermectin in the hydatid cysts of infected gerbils revealed severely damaged cysts with no viable protoscoleces. Further evaluation of this scolicidal agent is needed.
- At surgery, the exact location of the cyst is identified and correlated with the radiologic findings. The surrounding tissues are protected by covering them with cetrimide-soaked pads. The cyst is then evacuated using a strong suction device, and cetrimide is injected into the cavity. This procedure is repeated until the return is completely clear. Cetrimide is instilled and allowed to sit for 10 minutes, after which it is evacuated, and the cavity is irrigated with isotonic sodium chloride solution. This ensures both mechanical and chemical evacuation and destruction of all cyst contents. During this process, care is taken to ensure no spillage occurs to prevent seeding and secondary infestation.
- The cavity is then filled with isotonic sodium chloride solution and closed. Rarely, omentum is needed to fill the cavity. The cyst fluid is inspected for bile staining at the end of the evacuation and irrigation process. The inside of the cyst is inspected, and any bile duct communication is sutured. In case of infected cysts with biliary communication, closed suction drainage is required. Regardless of whether an open or laparoscopic approach is chosen, these basic principles must be followed in order to ensure the safety of the procedure.
- Medical requirements: The medical staff at the treating center should have experience with treating CE. Concomitant treatment with benzimidazoles (albendazole or mebendazole) has been reported to reduce the risk of secondary echinococcosis. Treatment is started 4 days preoperatively and lasts for 1 month.
- Alveolar echinococcosis
- Indications: The indication is resectability of the liver lesion (assessed by imaging techniques preoperatively).
- Contraindications: These are inoperable lesions, extensive lesions, and lesions extending outside the liver and involving other organs.
- Choice of surgical technique: Radical surgery with complete excision of the lesion is the only chance for cure. In certain cases, total hepatectomy with transplantation has been performed as long as no extra hepatic disease is present. Reemergence of the parasite in the transplanted liver and distant metastasis occur under immunosuppression. Partial resections of unresectable masses are considered to decrease the parasite load to aid the chemotherapeutic agents.
- Medical requirements: Surgical staff experienced in major liver resections and medical staff experienced in the administration of chemotherapy to persons with AE are required. Perform liver transplantations in centers where a well-coordinated and experienced team is available.
Consultants are needed in different contexts to help in the management of CE and AE.
- Although an infectious disease consultant is needed to help in the administration and monitoring of chemotherapeutic agents, the interventional radiologist has different roles in CE and AE.
- In certain cases of CE, the PAIR procedure is needed.
- In certain instances involving AE, interventions are required to improve organ function.
Wang K, Zhang X, Jin Z, Ma H, Teng Z, Wang L. Modelling and analysis of the transmission of echinococcosis with application to Xinjiang Uygur Autonomous Region of China. J Theor Biol. 2013 May 10. [Medline].
Torgerson PR. The emergence of echinococcosis in central Asia. Parasitology. 2013 May 10. 1-7. [Medline].
Moldovan R, Neghina AM, Calma CL, Marincu I, Neghina R. Human cystic echinococcosis in two south-western and central-western Romanian counties: A 7-year epidemiological and clinical overview. Acta Trop. 2012 Jan. 121(1):26-9. [Medline].
Kapan S, Turhan AN, Kalayci MU, Alis H, Aygun E. Albendazole is not effective for primary treatment of hepatic hydatid cysts. J Gastrointest Surg. 2008 May. 12(5):867-71. [Medline].
Ochieng'-Mitula PJ, Burt MD. The effects of ivermectin on the hydatid cyst of Echinococcus granulosus after direct injection at laparotomy. J Parasitol. 1996 Feb. 82(1):155-7. [Medline].
Mamarajabov S, Kodera Y, Karimov S, Abdiev S, Sabirov B, Krotov N, et al. Surgical Alternatives for Hepatic Hydatid Disease. Hepatogastroenterology. 2011 Oct 12. 58(112):[Medline].
Elsebaie SB, El-Sebae MM, Esmat ME, Nasr MM, Kamel MM. Modified endocystectomy versus pericystectomy in echinococcus granulosus liver cysts: a randomized controlled study, and the role of specific anti-hydatid IgG4 in detection of early recurrence. J Egypt Soc Parasitol. 2006 Dec. 36(3):993-1006. [Medline].
Filippou D, Tselepis D, Filippou G, Papadopoulos V. Advances in liver echinococcosis: diagnosis and treatment. Clin Gastroenterol Hepatol. 2007 Feb. 5(2):152-9. [Medline].
Flisser A. Larval cestodes. Collier L, Balows A, Sussman M, eds. Topley and Wilson's Microbiology and Microbial Infections. Parasitology. 9th ed. New York, NY: Oxford University Press; 1998. Vol 5: 539-60.
Gargouri M, Ben Amor N, Ben Chehida F, Hammou A, Gharbi HA, Ben Cheikh M, et al. Percutaneous treatment of hydatid cysts (Echinococcus granulosus). Cardiovasc Intervent Radiol. 1990 Jun-Jul. 13(3):169-73. [Medline].
Heath DD. Immunology of echinococcus infections. Thompson R, Lymbery AJ, eds. Echinococcus and Hydatid Disease. Wallingford, Oxon: CAB International; 1995. 183-200.
Kjossev KT, Losanoff JE. Hydatid cysts of the liver: TN(R)C classification. Int Surg. 1998 Oct-Dec. 83(4):311-3. [Medline].
Liu D, Lightowlers MW, Rickard MD. Evaluation of a monoclonal antibody-based competition ELISA for the diagnosis of human hydatidosis. Parasitology. 1992 Apr. 104 ( Pt 2):357-61. [Medline].
Liu YH, Wang XG, Chen YT, Yao YQ. Computer tomography of liver in alveolar echinococcosis treated with albendazole. Trans R Soc Trop Med Hyg. 1993 May-Jun. 87(3):319-21. [Medline].
Polo JR, Garcia-Sabrido JL. Sclerosing cholangitis associated with hydatid liver disease. Arch Surg. 1989 May. 124(5):637. [Medline].
Schröder R, Robotti G. New aspects in the management of alveolar echinococcosis involving the liver. World J Surg. 1986 Dec. 10(6):968-73. [Medline].
Tan A, Yakut M, Kaymakçioglu N, Ozerhan IH, Cetiner S, Akdeniz A. The results of surgical treatment and percutaneous drainage of hepatic hydatid disease. Int Surg. 1998 Oct-Dec. 83(4):314-6. [Medline].
Taylor BR, Langer B. Current surgical management of hepatic cyst disease. Adv Surg. 1997. 31:127-48. [Medline].
Taylor DH, Morris DL. In vitro culture of Echinococcus multilocularis: protoscolicidal action of praziquantel and albendazole sulphoxide. Trans R Soc Trop Med Hyg. 1988. 82(2):265-7. [Medline].
Todorov T, Mechkov G, Vutova K, Georgiev P, Lazarova I, Tonchev Z, et al. Factors influencing the response to chemotherapy in human cystic echinococcosis. Bull World Health Organ. 1992. 70(3):347-58. [Medline].
von Sinner WN. New diagnostic signs in hydatid disease; radiography, ultrasound, CT and MRI correlated to pathology. Eur J Radiol. 1991 Mar-Apr. 12(2):150-9. [Medline].
Williams JF, Perez Esandi MV, Oriol R. Evaluation of purified lipoprotein antigens of Echinococcus granulosus in the immunodiagnosis of human infection. Am J Trop Med Hyg. 1971 Jul. 20(4):575-9. [Medline].