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Echinococcosis
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Hydatidosis, hydatid disease, hydatid cyst disease
            • Definitions

              • Disease caused by cyst stage of infestation by tapeworm Echinococcus species
              • Liver, lung involvement most common
                • Bone (0.5-4%) → spine involved in 50% of cases
              • Multiple echinococcal species produce disease; complex life cycle
                • Echinococcus granulosus: Causative agent of hydatid disease in man/other mammals
                  • Dog is definite host; parasitized by adult tapeworm
                    • Gravid proglottids shed by tapeworm, disintegrate in dog intestine
                    • Eggs passed in feces
                  • Mammals act as intermediate host after ingestion of eggs (typically sheep, cattle)
                    • Eggs hatch in intestine, penetrate gut wall, travel via lymphatic/blood vessels throughout body
                    • Cyst develops into infective protoscolices in 1-2 years
                  • Dogs then ingest protoscolices on death of intermediate host (predation/scavenging)
                    • Cyst wall digested in gut, allowing development of adult worms
                • E. multilocularis: Causative agent of alveolar hydatid disease in man/other mammals
                  • Fox is most important definitive host
                  • Cyst grows invasively by external budding, diffuse growth through infected organ
                    • Presents as ramifying, porous, necrotic mass
                • E. vogeli: Polycystic form in Central and South America
                  • Bush dog is definitive host

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Nonvascular Interventions

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Saul D et al: Of Cestodes and men: surgical treatment of a spinal hydatid cyst. J Neurol Surg A Cent Eur Neurosurg. 81(1):86-90, 2020
                                              2. Padayachy LC et al: Hydatid disease (Echinococcus) of the central nervous system. Childs Nerv Syst. 34(10):1967-71, 2018
                                              3. Agnihotri M et al: Hydatid disease of the spine: a rare case. J Craniovertebr Junction Spine. 8(2):159-60, 2017
                                              4. Neumayr A et al: Spinal cystic echinococcosis--a systematic analysis and review of the literature: part 1. Epidemiology and anatomy. PLoS Negl Trop Dis. 7(9):e2450, 2013
                                              5. Neumayr A et al: Spinal cystic echinococcosis--a systematic analysis and review of the literature: part 2. Treatment, follow-up and outcome. PLoS Negl Trop Dis. 7(9):e2458, 2013
                                              6. Thaler M et al: Severe kyphoscoliosis after primary Echinococcus granulosus infection of the spine. Eur Spine J. 19(9):1415-22, 2010
                                              7. Doganay S et al: Role of conventional and diffusion-weighted magnetic resonance imaging of spinal treatment protocol for hydatid disease. J Spinal Cord Med. 32(5):574-7, 2009
                                              8. Güneçs M et al: Multiple intradural spinal hydatid disease: a case report and review of literature. Spine (Phila Pa 1976). 34(9):E346-50, 2009
                                              9. Lakhdar F et al: Spinal intradural extramedullary hydatidosis: report of three cases. Neurosurgery. 65(2):372-6; discussion 376-7, 2009
                                              10. Sasani M et al: Spontaneous drainage of an asymptomatic recurrent hydatid cyst of the sacrum. Spine (Phila Pa 1976). 34(7):E269-71, 2009
                                              11. Viljoen H et al: Hydatid disease of the spine. Spine (Phila Pa 1976). 33(22):2479-80, 2008
                                              12. El-Arousy MH et al: Cerebrospinal echinococcosis: serodiagnosis using different hydatid cyst fluid antigens. J Egypt Soc Parasitol. 35(1):193-204, 2005
                                              13. El Kohen A et al: Multiple hydatid cysts of the neck, the nasopharynx and the skull base revealing cervical vertebral hydatid disease. Int J Pediatr Otorhinolaryngol. 67(6):655-62, 2003
                                              14. Hadjipavlou AG et al: Effectiveness and pitfalls of percutaneous transpedicle biopsy of the spine. Clin Orthop. (411):54-60, 2003
                                              15. Karadereler S et al: Primary spinal extradural hydatid cyst in a child: case report and review of the literature. Eur Spine J. 11(5):500-3, 2002
                                              16. Hassan FO et al: Primary pelvic hydatid cyst: an unusual cause of sciatica and foot drop. Spine. 26(2):230-2, 2001
                                              17. Sener RN et al: Multiple, primary spinal-paraspinal hydatid cysts. Eur Radiol. 11(11):2314-6, 2001
                                              18. Stabler A et al: Imaging of spinal infection. Radiol Clin North Am. 39(1):115-35, 2001
                                              19. Bruschi F et al: Immunochemical and molecular characterization of vertebral hydatid fluid. Scand J Infect Dis. 31(3):322-3, 1999
                                              20. Savas R et al: Spinal cord compression due to costal Echinococcus multilocularis. Comput Med Imaging Graph. 23(2):85-8, 1999
                                              21. Mazyad MA et al: Spinal cord hydatid cysts in Egypt. J Egypt Soc Parasitol. 28(3):655-8, 1998
                                              22. Normelli HC et al: Vertebral hydatid cyst infection (Echinococcus granulosus): a case report. Eur Spine J. 7(2):158-61, 1998
                                              23. von Sinner WN et al: Case report 833: Primary spinal echinococcosis (Echinococcus granulosus) of lumbosacral spine with destruction of the left pedicles of L3-5 and extension of a large paraspinal cystic mass into the spinal canal. Skeletal Radiol. 23(3):220-3, 1994
                                              24. Sharif HS: Role of MR imaging in the management of spinal infections. AJR Am J Roentgenol. 158(6):1333-45, 1992
                                              25. Dernevik L et al: Management of dumbbell tumours. Reports of seven cases. Scand J Thorac Cardiovasc Surg. 24(1):47-51, 1990
                                              26. Richards KS et al: Effect of albendazole on human hydatid cysts: an ultrastructural study. HPB Surg. 2(2):105-12; discussion 112-3, 1990
                                              27. Charles RW et al: Echinococcal infection of the spine with neural involvement. Spine. 13(1):47-9, 1988
                                              28. Bhargava S: Radiology--including computed tomography--of parasitic diseases of the central nervous system. Neurosurg Rev. 6(3):129-37, 1983
                                              29. Braithwaite PA et al: Vertebral hydatid disease: radiological assessment. Radiology. 140(3):763-6, 1981
                                              30. Kaufman DM et al: Infectious agents in spinal epidural abscesses. Neurology. 30(8):844-50, 1980
                                              31. Karvounis PC et al: Intradural spinal echinococcus simulating lumbar disc protrusion. Neurochirurgia (Stuttg). 20(2):58-60, 1977
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Hydatidosis, hydatid disease, hydatid cyst disease
                                                        • Definitions

                                                          • Disease caused by cyst stage of infestation by tapeworm Echinococcus species
                                                          • Liver, lung involvement most common
                                                            • Bone (0.5-4%) → spine involved in 50% of cases
                                                          • Multiple echinococcal species produce disease; complex life cycle
                                                            • Echinococcus granulosus: Causative agent of hydatid disease in man/other mammals
                                                              • Dog is definite host; parasitized by adult tapeworm
                                                                • Gravid proglottids shed by tapeworm, disintegrate in dog intestine
                                                                • Eggs passed in feces
                                                              • Mammals act as intermediate host after ingestion of eggs (typically sheep, cattle)
                                                                • Eggs hatch in intestine, penetrate gut wall, travel via lymphatic/blood vessels throughout body
                                                                • Cyst develops into infective protoscolices in 1-2 years
                                                              • Dogs then ingest protoscolices on death of intermediate host (predation/scavenging)
                                                                • Cyst wall digested in gut, allowing development of adult worms
                                                            • E. multilocularis: Causative agent of alveolar hydatid disease in man/other mammals
                                                              • Fox is most important definitive host
                                                              • Cyst grows invasively by external budding, diffuse growth through infected organ
                                                                • Presents as ramifying, porous, necrotic mass
                                                            • E. vogeli: Polycystic form in Central and South America
                                                              • Bush dog is definitive host

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Nonvascular Interventions

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Saul D et al: Of Cestodes and men: surgical treatment of a spinal hydatid cyst. J Neurol Surg A Cent Eur Neurosurg. 81(1):86-90, 2020
                                                                                          2. Padayachy LC et al: Hydatid disease (Echinococcus) of the central nervous system. Childs Nerv Syst. 34(10):1967-71, 2018
                                                                                          3. Agnihotri M et al: Hydatid disease of the spine: a rare case. J Craniovertebr Junction Spine. 8(2):159-60, 2017
                                                                                          4. Neumayr A et al: Spinal cystic echinococcosis--a systematic analysis and review of the literature: part 1. Epidemiology and anatomy. PLoS Negl Trop Dis. 7(9):e2450, 2013
                                                                                          5. Neumayr A et al: Spinal cystic echinococcosis--a systematic analysis and review of the literature: part 2. Treatment, follow-up and outcome. PLoS Negl Trop Dis. 7(9):e2458, 2013
                                                                                          6. Thaler M et al: Severe kyphoscoliosis after primary Echinococcus granulosus infection of the spine. Eur Spine J. 19(9):1415-22, 2010
                                                                                          7. Doganay S et al: Role of conventional and diffusion-weighted magnetic resonance imaging of spinal treatment protocol for hydatid disease. J Spinal Cord Med. 32(5):574-7, 2009
                                                                                          8. Güneçs M et al: Multiple intradural spinal hydatid disease: a case report and review of literature. Spine (Phila Pa 1976). 34(9):E346-50, 2009
                                                                                          9. Lakhdar F et al: Spinal intradural extramedullary hydatidosis: report of three cases. Neurosurgery. 65(2):372-6; discussion 376-7, 2009
                                                                                          10. Sasani M et al: Spontaneous drainage of an asymptomatic recurrent hydatid cyst of the sacrum. Spine (Phila Pa 1976). 34(7):E269-71, 2009
                                                                                          11. Viljoen H et al: Hydatid disease of the spine. Spine (Phila Pa 1976). 33(22):2479-80, 2008
                                                                                          12. El-Arousy MH et al: Cerebrospinal echinococcosis: serodiagnosis using different hydatid cyst fluid antigens. J Egypt Soc Parasitol. 35(1):193-204, 2005
                                                                                          13. El Kohen A et al: Multiple hydatid cysts of the neck, the nasopharynx and the skull base revealing cervical vertebral hydatid disease. Int J Pediatr Otorhinolaryngol. 67(6):655-62, 2003
                                                                                          14. Hadjipavlou AG et al: Effectiveness and pitfalls of percutaneous transpedicle biopsy of the spine. Clin Orthop. (411):54-60, 2003
                                                                                          15. Karadereler S et al: Primary spinal extradural hydatid cyst in a child: case report and review of the literature. Eur Spine J. 11(5):500-3, 2002
                                                                                          16. Hassan FO et al: Primary pelvic hydatid cyst: an unusual cause of sciatica and foot drop. Spine. 26(2):230-2, 2001
                                                                                          17. Sener RN et al: Multiple, primary spinal-paraspinal hydatid cysts. Eur Radiol. 11(11):2314-6, 2001
                                                                                          18. Stabler A et al: Imaging of spinal infection. Radiol Clin North Am. 39(1):115-35, 2001
                                                                                          19. Bruschi F et al: Immunochemical and molecular characterization of vertebral hydatid fluid. Scand J Infect Dis. 31(3):322-3, 1999
                                                                                          20. Savas R et al: Spinal cord compression due to costal Echinococcus multilocularis. Comput Med Imaging Graph. 23(2):85-8, 1999
                                                                                          21. Mazyad MA et al: Spinal cord hydatid cysts in Egypt. J Egypt Soc Parasitol. 28(3):655-8, 1998
                                                                                          22. Normelli HC et al: Vertebral hydatid cyst infection (Echinococcus granulosus): a case report. Eur Spine J. 7(2):158-61, 1998
                                                                                          23. von Sinner WN et al: Case report 833: Primary spinal echinococcosis (Echinococcus granulosus) of lumbosacral spine with destruction of the left pedicles of L3-5 and extension of a large paraspinal cystic mass into the spinal canal. Skeletal Radiol. 23(3):220-3, 1994
                                                                                          24. Sharif HS: Role of MR imaging in the management of spinal infections. AJR Am J Roentgenol. 158(6):1333-45, 1992
                                                                                          25. Dernevik L et al: Management of dumbbell tumours. Reports of seven cases. Scand J Thorac Cardiovasc Surg. 24(1):47-51, 1990
                                                                                          26. Richards KS et al: Effect of albendazole on human hydatid cysts: an ultrastructural study. HPB Surg. 2(2):105-12; discussion 112-3, 1990
                                                                                          27. Charles RW et al: Echinococcal infection of the spine with neural involvement. Spine. 13(1):47-9, 1988
                                                                                          28. Bhargava S: Radiology--including computed tomography--of parasitic diseases of the central nervous system. Neurosurg Rev. 6(3):129-37, 1983
                                                                                          29. Braithwaite PA et al: Vertebral hydatid disease: radiological assessment. Radiology. 140(3):763-6, 1981
                                                                                          30. Kaufman DM et al: Infectious agents in spinal epidural abscesses. Neurology. 30(8):844-50, 1980
                                                                                          31. Karvounis PC et al: Intradural spinal echinococcus simulating lumbar disc protrusion. Neurochirurgia (Stuttg). 20(2):58-60, 1977