UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Clinical manifestations and diagnosis of echinococcosis

Author
Pedro L Moro, MD, MPH
Section Editor
Peter F Weller, MD, FACP
Deputy Editor
Elinor L Baron, MD, DTMH

INTRODUCTION

Echinococcal disease is caused by infection with the metacestode stage of the tapeworm Echinococcus, which belongs to the family Taeniidae. Four species of Echinococcus produce infection in humans; E. granulosus and E. multilocularis are the most common, causing cystic echinococcosis (CE) and alveolar echinococcosis (AE), respectively. The two other species, E. vogeli and E. oligarthrus, cause polycystic echinococcosis but have only rarely been associated with human infection.

The clinical manifestations and diagnosis of cystic and alveolar echinococcal infection will be reviewed here. The epidemiology, treatment, and prevention of echinococcosis are discussed separately. (See "Epidemiology and control of echinococcosis" and "Treatment of echinococcosis".)

CLINICAL MANIFESTATIONS

The Echinococcus species have different geographic distributions and involve different hosts. E. granulosus and E. multilocularis also differ in their clinical presentation. (See "Epidemiology and control of echinococcosis".)

Echinococcus granulosus — The initial phase of primary infection is always asymptomatic. Many infections are acquired in childhood but do not cause clinical manifestations until adulthood. Latent periods of more than 50 years before symptoms arise have been reported. While approximately 50 percent of detected cases occur in asymptomatic patients, many more cases remain undiagnosed or are found incidentally at autopsy.

The clinical presentation of E. granulosus infection depends upon the site of the cysts and their size. Small and/or calcified cysts may remain asymptomatic indefinitely. However, symptoms due to mass effect within organs, obstruction of blood or lymphatic flow, or complications such as rupture or secondary bacterial infections can result.

                         

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Fri Aug 26 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Frider B, Larrieu E, Odriozola M. Long-term outcome of asymptomatic liver hydatidosis. J Hepatol 1999; 30:228.
  2. Moro PL, Gilman RH, Verastegui M, et al. Human hydatidosis in the central Andes of Peru: evolution of the disease over 3 years. Clin Infect Dis 1999; 29:807.
  3. Santivanez S, Garcia HH. Pulmonary cystic echinococcosis. Curr Opin Pulm Med 2010; 16:257.
  4. Arinc S, Kosif A, Ertugrul M, et al. Evaluation of pulmonary hydatid cyst cases. Int J Surg 2009; 7:192.
  5. Arroud M, Afifi MA, El Ghazi K, et al. Lung hydatic cysts in children: comparison study between giant and non-giant cysts. Pediatr Surg Int 2009; 25:37.
  6. Dopchiz MC, Elissondo MC, Andresiuk MV, et al. Pediatric hydatidosis in the south-east of the Buenos Aires province, Argentina. Rev Argent Microbiol 2009; 41:105.
  7. Turgut AT, Altinok T, Topçu S, Koşar U. Local complications of hydatid disease involving thoracic cavity: imaging findings. Eur J Radiol 2009; 70:49.
  8. Dziri C, Haouet K, Fingerhut A, Zaouche A. Management of cystic echinococcosis complications and dissemination: where is the evidence? World J Surg 2009; 33:1266.
  9. Bhatia G. Echinococcus. Semin Respir Infect 1997; 12:171.
  10. Baden LR, Elliott DD. Case records of the Massachusetts General Hospital. Weekly Clinicopathological exercises. Case 4-2003. A 42-year-old woman with cough, fever, and abnormalities on thoracoabdominal computed tomography. N Engl J Med 2003; 348:447.
  11. Ben-Hamda K, Maatouk F, Ben-Farhat M, et al. Eighteen-year experience with echinococcosus of the heart: clinical and echocardiographic features in 14 patients. Int J Cardiol 2003; 91:145.
  12. Díaz-Menéndez M, Pérez-Molina JA, Norman FF, et al. Management and outcome of cardiac and endovascular cystic echinococcosis. PLoS Negl Trop Dis 2012; 6:e1437.
  13. Kahlfuß S, Flieger RR, Roepke TK, Yilmaz K. Diagnosis and treatment of cardiac echinococcosis. Heart 2016; 102:1348.
  14. Nourbakhsh A, Vannemreddy P, Minagar A, et al. Hydatid disease of the central nervous system: a review of literature with an emphasis on Latin American countries. Neurol Res 2010; 32:245.
  15. Göğüş C, Safak M, Baltaci S, Türkölmez K. Isolated renal hydatidosis: experience with 20 cases. J Urol 2003; 169:186.
  16. Ali-Khan Z, Rausch RL. Demonstration of amyloid and immune complex deposits in renal and hepatic parenchyma of Alaskan alveolar hydatid disease patients. Ann Trop Med Parasitol 1987; 81:381.
  17. Gelman R, Brook G, Green J, et al. Minimal change glomerulonephritis associated with hydatid disease. Clin Nephrol 2000; 53:152.
  18. Zlitni M, Ezzaouia K, Lebib H, et al. Hydatid cyst of bone: diagnosis and treatment. World J Surg 2001; 25:75.
  19. Chaabouni M, Ben Zina Z, Ben Ayez H, et al. [Hydatid orbital cyst: a unique intra-orbital locality. A case report]. J Fr Ophtalmol 1999; 22:329.
  20. Sinav S, Demirci A, Sinav B, et al. A primary intraocular hydatid cyst. Acta Ophthalmol (Copenh) 1991; 69:802.
  21. Suffee T, Chader H, Foulet F, et al. A suspicious subcutaneous tumor. Clin Infect Dis 2015; 61:1707, 1759.
  22. A Suspicious Subcutaneous Tumor. Clin Infect Dis 2015; 61:1707.
  23. McManus DP, Zhang W, Li J, Bartley PB. Echinococcosis. Lancet 2003; 362:1295.
  24. Kern P, Bardonnet K, Renner E, et al. European echinococcosis registry: human alveolar echinococcosis, Europe, 1982-2000. Emerg Infect Dis 2003; 9:343.
  25. Ammann RW, Eckert J. Cestodes. Echinococcus. Gastroenterol Clin North Am 1996; 25:655.
  26. Torgerson PR, Schweiger A, Deplazes P, et al. Alveolar echinococcosis: from a deadly disease to a well-controlled infection. Relative survival and economic analysis in Switzerland over the last 35 years. J Hepatol 2008; 49:72.
  27. Morar R, Feldman C. Pulmonary echinococcosis. Eur Respir J 2003; 21:1069.
  28. Barbieri M, Severi MA, Pírez MI, et al. Use of specific antibody and circulating antigen serum levels in the hydatid immunodiagnosis of asymptomatic population. Int J Parasitol 1994; 24:937.
  29. Dhar P, Chaudhary A, Desai R, et al. Current trends in the diagnosis and management of cystic hydatid disease of the liver. J Commun Dis 1996; 28:221.
  30. Safioleas M, Misiakos E, Manti C, et al. Diagnostic evaluation and surgical management of hydatid disease of the liver. World J Surg 1994; 18:859.
  31. Pant CS, Gupta RK. Diagnostic value of ultrasonography in hydatid disease in abdomen and chest. Acta Radiol 1987; 28:743.
  32. Salama H, Farid Abdel-Wahab M, Strickland GT. Diagnosis and treatment of hepatic hydatid cysts with the aid of echo-guided percutaneous cyst puncture. Clin Infect Dis 1995; 21:1372.
  33. Suwan Z. Sonographic findings in hydatid disease of the liver: comparison with other imaging methods. Ann Trop Med Parasitol 1995; 89:261.
  34. WHO Informal Working Group. International classification of ultrasound images in cystic echinococcosis for application in clinical and field epidemiological settings. Acta Trop 2003; 85:253.
  35. http://www.medicalweb.it/aumi/echinonet/ (Accessed on December 04, 2012).
  36. Gharbi HA, Hassine W, Brauner MW, Dupuch K. Ultrasound examination of the hydatic liver. Radiology 1981; 139:459.
  37. Hosch W, Stojkovic M, Jänisch T, et al. MR imaging for diagnosing cysto-biliary fistulas in cystic echinococcosis. Eur J Radiol 2008; 66:262.
  38. Xynos E, Pechlivanides G, Tzortzinis A, et al. Hydatid disease of the liver. Diagnosis and surgical treatment. HPB Surg 1991; 4:59.
  39. el-Tahir MI, Omojola MF, Malatani T, et al. Hydatid disease of the liver: evaluation of ultrasound and computed tomography. Br J Radiol 1992; 65:390.
  40. Kervancioglu R, Bayram M, Elbeyli L. CT findings in pulmonary hydatid disease. Acta Radiol 1999; 40:510.
  41. al Karawi MA, el-Shiekh Mohamed AR, Yasawy MI. Advances in diagnosis and management of hydatid disease. Hepatogastroenterology 1990; 37:327.
  42. Marani SA, Canossi GC, Nicoli FA, et al. Hydatid disease: MR imaging study. Radiology 1990; 175:701.
  43. von Sinner WN, Rifai A, te Strake L, Sieck J. Magnetic resonance imaging of thoracic hydatid disease. Correlation with clinical findings, radiography, ultrasonography, CT and pathology. Acta Radiol 1990; 31:59.
  44. Morris DL, Buckley J, Gregson R, Worthington BS. Magnetic resonance imaging in hydatid disease. Clin Radiol 1987; 38:141.
  45. Taourel P, Marty-Ane B, Charasset S, et al. Hydatid cyst of the liver: comparison of CT and MRI. J Comput Assist Tomogr 1993; 17:80.
  46. Tüzün M, Altinörs N, Arda IS, Hekimoğlu B. Cerebral hydatid disease CT and MR findings. Clin Imaging 2002; 26:353.
  47. Riganò R, Profumo E, Ioppolo S, et al. Immunological markers indicating the effectiveness of pharmacological treatment in human hydatid disease. Clin Exp Immunol 1995; 102:281.
  48. Riganò R, Ioppolo S, Ortona E, et al. Long-term serological evaluation of patients with cystic echinococcosis treated with benzimidazole carbamates. Clin Exp Immunol 2002; 129:485.
  49. Zarzosa MP, Orduña Domingo A, Gutiérrez P, et al. Evaluation of six serological tests in diagnosis and postoperative control of pulmonary hydatid disease patients. Diagn Microbiol Infect Dis 1999; 35:255.
  50. Biava MF, Dao A, Fortier B. Laboratory diagnosis of cystic hydatic disease. World J Surg 2001; 25:10.
  51. Ortona E, Riganò R, Buttari B, et al. An update on immunodiagnosis of cystic echinococcosis. Acta Trop 2003; 85:165.
  52. Force L, Torres JM, Carrillo A, Buscà J. Evaluation of eight serological tests in the diagnosis of human echinococcosis and follow-up. Clin Infect Dis 1992; 15:473.
  53. Babba H, Messedi A, Masmoudi S, et al. Diagnosis of human hydatidosis: comparison between imagery and six serologic techniques. Am J Trop Med Hyg 1994; 50:64.
  54. Sbihi Y, Rmiqui A, Rodriguez-Cabezas MN, et al. Comparative sensitivity of six serological tests and diagnostic value of ELISA using purified antigen in hydatidosis. J Clin Lab Anal 2001; 15:14.
  55. Siles-Lucas MM, Gottstein BB. Molecular tools for the diagnosis of cystic and alveolar echinococcosis. Trop Med Int Health 2001; 6:463.
  56. Parija SC. A review of some simple immunoassays in the serodiagnosis of cystic hydatid disease. Acta Trop 1998; 70:17.
  57. Rogan MT, Craig PS, Zeyhle E, et al. Evaluation of a rapid dot-ELISA as a field test for the diagnosis of cystic hydatid disease. Trans R Soc Trop Med Hyg 1991; 85:773.
  58. Romia SA, Youssef ME, Handoussa AE, et al. Dot-ELISA as a diagnostic test in hydatid disease. J Egypt Soc Parasitol 1992; 22:603.
  59. Pappas MG, Schantz PM, Cannon LT Sr, Wahlquist SP. Dot-ELISA for the rapid serodiagnosis of human hydatid disease. Diagn Immunol 1986; 4:271.
  60. Mamuti W, Sako Y, Nakao M, et al. Recent advances in characterization of Echinococcus antigen B. Parasitol Int 2006; 55 Suppl:S57.
  61. Maddison SE, Slemenda SB, Schantz PM, et al. A specific diagnostic antigen of Echinococcus granulosus with an apparent molecular weight of 8 kDA. Am J Trop Med Hyg 1989; 40:377.
  62. Poretti D, Felleisen E, Grimm F, et al. Differential immunodiagnosis between cystic hydatid disease and other cross-reactive pathologies. Am J Trop Med Hyg 1999; 60:193.
  63. Kaddah MH, Maher KM, Hassanein HI, et al. Evaluation of different immunodiagnostic techniques for diagnosis of hydatidosis in Egypt. J Egypt Soc Parasitol 1992; 22:653.
  64. Siracusano A, Ioppolo S, Notargiacomo S, et al. Detection of antibodies against Echinococcus granulosus major antigens and their subunits by immunoblotting. Trans R Soc Trop Med Hyg 1991; 85:239.
  65. Ito A, Ma L, Schantz PM, et al. Differential serodiagnosis for cystic and alveolar echinococcosis using fractions of Echinococcus granulosus cyst fluid (antigen B) and E. multilocularis protoscolex (EM18). Am J Trop Med Hyg 1999; 60:188.
  66. Ioppolo S, Notargiacomo S, Profumo E, et al. Immunological responses to antigen B from Echinococcus granulosus cyst fluid in hydatid patients. Parasite Immunol 1996; 18:571.
  67. Moro PL, Bonifacio N, Gilman RH, et al. Field diagnosis of Echinococcus granulosus infection among intermediate and definitive hosts in an endemic focus of human cystic echinococcosis. Trans R Soc Trop Med Hyg 1999; 93:611.
  68. Gavidia CM, Gonzalez AE, Zhang W, et al. Diagnosis of cystic echinococcosis, central Peruvian Highlands. Emerg Infect Dis 2008; 14:260.
  69. Parija SC. Recent trends in the serodiagnosis of hydatid disease. Southeast Asian J Trop Med Public Health 1991; 22 Suppl:371.
  70. Ramzy RM, Helmy H, El Zayyat EA, et al. An enzyme-linked immunosorbent assay for detection of IgG1 antibodies specific to human cystic echinococcosis in Egypt. Trop Med Int Health 1999; 4:616.
  71. Virginio VG, Hernández A, Rott MB, et al. A set of recombinant antigens from Echinococcus granulosus with potential for use in the immunodiagnosis of human cystic hydatid disease. Clin Exp Immunol 2003; 132:309.
  72. Lawn SD, Bligh J, Craig PS, Chiodini PL. Human cystic echinococcosis: evaluation of post-treatment serologic follow-up by IgG subclass antibody detection. Am J Trop Med Hyg 2004; 70:329.
  73. Verastegui M, Moro P, Guevara A, et al. Enzyme-linked immunoelectrotransfer blot test for diagnosis of human hydatid disease. J Clin Microbiol 1992; 30:1557.
  74. Craig PS, Bailey W, Nelson GS. A specific test for the identification of cyst fluid samples from suspected human hydatid infections. Trans R Soc Trop Med Hyg 1986; 80:256.
  75. Ravinder PT, Parija SC. Countercurrent immunoelectrophoresis test for detection of hydatid antigen in the fluid from hydatid cysts: a preliminary report. Acta Trop 1997; 66:169.
  76. Devi Chandrakesan S, Parija SC. Latex agglutination test (LAT) for antigen detection in the cystic fluid for the diagnosis of cystic echinococcosis. Diagn Microbiol Infect Dis 2003; 45:123.
  77. Wang Y, Bradshaw H, Rogan MT, Craig PS. Rapid dot-ELISA for the detection of specific antigens in the cyst fluid from human cases of cystic echinococcosis. Ann Trop Med Parasitol 2002; 96:691.
  78. Devi CS, Parija SC. A new serum hydatid antigen detection test for diagnosis of cystic echinococcosis. Am J Trop Med Hyg 2003; 69:525.
  79. Gottstein B. An immunoassay for the detection of circulating antigens in human echinococcosis. Am J Trop Med Hyg 1984; 33:1185.
  80. Lanier AP, Trujillo DE, Schantz PM, et al. Comparison of serologic tests for the diagnosis and follow-up of alveolar hydatid disease. Am J Trop Med Hyg 1987; 37:609.
  81. Schantz PM, Wilson JF, Wahlquist SP, et al. Serologic tests for diagnosis and post-treatment evaluation of patients with alveolar hydatid disease (Echinococcus multilocularis). Am J Trop Med Hyg 1983; 32:1381.
  82. Gottstein B, Tschudi K, Eckert J, Ammann R. Em2-ELISA for the follow-up of alveolar echinococcosis after complete surgical resection of liver lesions. Trans R Soc Trop Med Hyg 1989; 83:389.
  83. Deplazes P, Gottstein B. A monoclonal antibody against Echinococcus multilocularis Em2 antigen. Parasitology 1991; 103 Pt 1:41.
  84. Gottstein B. Molecular and immunological diagnosis of echinococcosis. Clin Microbiol Rev 1992; 5:248.
  85. Gottstein B, Jacquier P, Bresson-Hadni S, Eckert J. Improved primary immunodiagnosis of alveolar echinococcosis in humans by an enzyme-linked immunosorbent assay using the Em2plus antigen. J Clin Microbiol 1993; 31:373.
  86. Ma L, Ito A, Liu YH, et al. Alveolar echinococcosis: Em2plus-ELISA and Em18-western blots for follow-up after treatment with albendazole. Trans R Soc Trop Med Hyg 1997; 91:476.
  87. Ito A, Schantz PM, Wilson JF. Em18, a new serodiagnostic marker for differentiation of active and inactive cases of alveolar hydatid disease. Am J Trop Med Hyg 1995; 52:41.
  88. Akira I. Serodiagnosis of alveolar echinococcosis: detection of antibody against EM18 in patients and rodents. Southeast Asian J Trop Med Public Health 1997; 28 Suppl 1:117.
  89. Ito A, Nakao M, Kutsumi H, et al. Serodiagnosis of alveolar hydatid disease by western blotting. Trans R Soc Trop Med Hyg 1993; 87:170.
  90. Ito A, Xiao N, Liance M, et al. Evaluation of an enzyme-linked immunosorbent assay (ELISA) with affinity-purified Em18 and an ELISA with recombinant Em18 for differential diagnosis of alveolar echinococcosis: results of a blind test. J Clin Microbiol 2002; 40:4161.
  91. Jiang L, Wen H, Ito A. Immunodiagnostic differentiation of alveolar and cystic echinococcosis using ELISA test with 18-kDa antigen extracted from Echinococcus protoscoleces. Trans R Soc Trop Med Hyg 2001; 95:285.
  92. Sako Y, Nakao M, Nakaya K, et al. Alveolar echinococcosis: characterization of diagnostic antigen Em18 and serological evaluation of recombinant Em18. J Clin Microbiol 2002; 40:2760.
  93. Fujimoto Y, Ito A, Ishikawa Y, et al. Usefulness of recombinant Em18-ELISA to evaluate efficacy of treatment in patients with alveolar echinococcosis. J Gastroenterol 2005; 40:426.
  94. Gargouri M, Ben Amor N, Ben Chehida F, et al. Percutaneous treatment of hydatid cysts (Echinococcus granulosus). Cardiovasc Intervent Radiol 1990; 13:169.
  95. Khuroo MS, Dar MY, Yattoo GN, et al. Percutaneous drainage versus albendazole therapy in hepatic hydatidosis: a prospective, randomized study. Gastroenterology 1993; 104:1452.
  96. Filice C, Di Perri G, Strosselli M, et al. Parasitologic findings in percutaneous drainage of human hydatid liver cysts. J Infect Dis 1990; 161:1290.
  97. Giorgio A, Tarantino L, Francica G, et al. Unilocular hydatid liver cysts: treatment with US-guided, double percutaneous aspiration and alcohol injection. Radiology 1992; 184:705.
  98. Clavel A, Varea M, Doiz O, et al. Visualization of hydatid elements: comparison of several techniques. J Clin Microbiol 1999; 37:1561.
  99. Brunetti E, Kern P, Vuitton DA, Writing Panel for the WHO-IWGE. Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop 2010; 114:1.
  100. Kern P, Frosch P, Helbig M, et al. Diagnosis of Echinococcus multilocularis infection by reverse-transcription polymerase chain reaction. Gastroenterology 1995; 109:596.
  101. Rinaldi F, Brunetti E, Neumayr A, et al. Cystic echinococcosis of the liver: A primer for hepatologists. World J Hepatol 2014; 6:293.