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Cavernous Malformation
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Cavernous malformation (CM)
            • Synonyms

              • Cavernous angioma, cavernous hemangioma, cryptic vascular malformation
            • Definitions

              • Vascular lesion with lobulated, thin sinusoidal vascular channels and no interspersed neural tissue

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • 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. Awad IA et al: Cavernous angiomas: deconstructing a neurosurgical disease. J Neurosurg. 131(1):1-13, 2019
                                            2. Zafar A et al: Familial cerebral cavernous malformations. Stroke. 50(5):1294-301, 2019
                                            3. Otten M et al: Natural history of spinal cavernous malformations. Handb Clin Neurol. 143:233-9, 2017
                                            4. Badhiwala JH et al: Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data. J Neurosurg Spine. 21(4):662-76, 2014
                                            5. Pearl MS et al: Angiographic detection and characterization of "cryptic venous anomalies" associated with spinal cord cavernous malformations using flat-panel catheter angiotomography. Neurosurgery. 71(1 Suppl Operative):125-32, 2012
                                            6. Tong X et al: Clinical presentation and surgical outcome of intramedullary spinal cord cavernous malformations. J Neurosurg Spine. 16(3):308-14, 2012
                                            7. Consiglieri GD et al: Utility of the CO(2) laser in the microsurgical resection of cavernous malformations. World Neurosurg. 79(5-6):714-8, 2013
                                            8. Kivelev J et al: A proposed grading system of brain and spinal cavernomas. Neurosurgery. 69(4):807-13; discussion 813-4, 2011
                                            9. Maslehaty H et al: Symptomatic spinal cavernous malformations: indication for microsurgical treatment and outcome. Eur Spine J. 20(10):1765-70, 2011
                                            10. Mitha AP et al: Surgical approaches to intramedullary cavernous malformations of the spinal cord. Neurosurgery. 68(2 Suppl Operative):317-24; discussion 324, 2011
                                            11. Labauge P et al: Outcome in 53 patients with spinal cord cavernomas. Surg Neurol. 70(2):176-81; discussion 181, 2008
                                            12. Lee ST et al: Identification of an Arg35X mutation in the PDCD10 gene in a patient with cerebral and multiple spinal cavernous malformations. J Neurol Sci. 267(1-2):177-81, 2008
                                            13. Kharkar S et al: The natural history of conservatively managed symptomatic intramedullary spinal cord cavernomas. Neurosurgery. 60(5):865-72; discussion 865-72, 2007
                                            14. Jallo GI et al: Clinical presentation and optimal management for intramedullary cavernous malformations. Neurosurg Focus. 21(1):e10, 2006
                                            15. Musunuru K et al: Widespread central nervous system cavernous malformations associated with cafe-au-lait skin lesions. Case report. J Neurosurg. 99(2):412-5, 2003
                                            16. Sandalcioglu IE et al: Intramedullary spinal cord cavernous malformations: clinical features and risk of hemorrhage. Neurosurg Rev. 26(4):253-6, 2003
                                            17. Chen DH et al: Cerebral cavernous malformation: novel mutation in a Chinese family and evidence for heterogeneity. J Neurol Sci. 196(1-2):91-6, 2002
                                            18. Nagib MG et al: Intramedullary cavernous angiomas of the spinal cord in the pediatric age group: a pediatric series. Pediatr Neurosurg. 36(2):57-63, 2002
                                            19. Balaban H et al: Multiple spinal intramedullary cavernous angioma: case report. Clin Neurol Neurosurg. 103(2):120-2, 2001
                                            20. Clatterbuck RE et al: The nature and fate of punctate (type IV) cavernous malformations. Neurosurgery. 49(1):26-30; discussion 30-2, 2001
                                            21. Deutsch H et al: Pediatric intramedullary spinal cavernous malformations. Spine. 26(18):E427-31, 2001
                                            22. Mottolese C et al: Central nervous system cavernomas in the pediatric age group. Neurosurg Rev. 24(2-3):55-71; discussion 72-3, 2001
                                            23. Sure U et al: Endothelial proliferation, neoangiogenesis, and potential de novo generation of cerebrovascular malformations. J Neurosurg. 94(6):972-7, 2001
                                            24. Zacharia TT et al: Co-existing spinal cord and brain cavernous angiomas. J Assoc Physicians India. 49:835-7, 2001
                                            25. Deutsch H et al: Spinal intramedullary cavernoma: clinical presentation and surgical outcome. J Neurosurg. 93(1 Suppl):65-70, 2000
                                            26. Chabert E et al: Intramedullary cavernous malformations. J Neuroradiol. 26(4):262-8, 1999
                                            27. Maraire JN et al: De novo development of a cavernous malformation of the spinal cord following spinal axis radiation. Case report. J Neurosurg. 90(2 Suppl):234-8, 1999
                                            28. Moriarity JL et al: The natural history of cavernous malformations. Neurosurg Clin N Am. 10(3):411-7, 1999
                                            29. Porter RW et al: Cavernous malformations of the brainstem: experience with 100 patients. J Neurosurg. 90(1):50-8, 1999
                                            30. Vishteh AG et al: Patients with spinal cord cavernous malformations are at an increased risk for multiple neuraxis cavernous malformations. Neurosurgery. 45(1):30-2; discussion 33, 1999
                                            31. Zevgaridis D et al: Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien). 141(3):237-45, 1999
                                            32. Duke BJ et al: Cavernous angiomas of the cauda equina: case report and review of the literature. Surg Neurol. 50(5):442-5, 1998
                                            33. Huffmann BC et al: Treatment strategies and results in spinal vascular malformations. Neurol Med Chir (Tokyo). 38 Suppl:231-7, 1998
                                            34. Naim-Ur-Rahman et al: Intramedullary ossified cavernous angioma of the spinal cord: case report. Br J Neurosurg. 12(3):267-70, 1998
                                            35. Padovani R et al: Cavernous angiomas of the spinal district: surgical treatment of 11 patients. Eur Spine J. 6(5):298-303, 1997
                                            36. Harrison MJ et al: Symptomatic cavernous malformations affecting the spine and spinal cord. Neurosurgery. 37(2):195-204; discussion 204-5, 1995
                                            37. Spetzger U et al: Cavernous angiomas of the spinal cord clinical presentation, surgical strategy, and postoperative results. Acta Neurochir (Wien). 134(3-4):200-6, 1995
                                            38. Turjman F et al: MRI of intramedullary cavernous haemangiomas. Neuroradiology. 37(4):297-302, 1995
                                            39. Canavero S et al: Spinal intramedullary cavernous angiomas: a literature meta-analysis. Surg Neurol. 41(5):381-8, 1994
                                            40. Acciarri N et al: Surgical treatment of spinal cavernous angiomas. J Neurosurg Sci. 37(4):209-15, 1993
                                            41. Acciarri N et al: Spinal cavernous angioma: a rare cause of subarachnoid hemorrhage. Surg Neurol. 37(6):453-6, 1992
                                            42. Bourgouin PM et al: Multiple occult vascular malformations of the brain and spinal cord: MRI diagnosis. Neuroradiology. 34(2):110-1, 1992
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Cavernous malformation (CM)
                                                      • Synonyms

                                                        • Cavernous angioma, cavernous hemangioma, cryptic vascular malformation
                                                      • Definitions

                                                        • Vascular lesion with lobulated, thin sinusoidal vascular channels and no interspersed neural tissue

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Angiographic Findings

                                                              • 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. Awad IA et al: Cavernous angiomas: deconstructing a neurosurgical disease. J Neurosurg. 131(1):1-13, 2019
                                                                                      2. Zafar A et al: Familial cerebral cavernous malformations. Stroke. 50(5):1294-301, 2019
                                                                                      3. Otten M et al: Natural history of spinal cavernous malformations. Handb Clin Neurol. 143:233-9, 2017
                                                                                      4. Badhiwala JH et al: Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data. J Neurosurg Spine. 21(4):662-76, 2014
                                                                                      5. Pearl MS et al: Angiographic detection and characterization of "cryptic venous anomalies" associated with spinal cord cavernous malformations using flat-panel catheter angiotomography. Neurosurgery. 71(1 Suppl Operative):125-32, 2012
                                                                                      6. Tong X et al: Clinical presentation and surgical outcome of intramedullary spinal cord cavernous malformations. J Neurosurg Spine. 16(3):308-14, 2012
                                                                                      7. Consiglieri GD et al: Utility of the CO(2) laser in the microsurgical resection of cavernous malformations. World Neurosurg. 79(5-6):714-8, 2013
                                                                                      8. Kivelev J et al: A proposed grading system of brain and spinal cavernomas. Neurosurgery. 69(4):807-13; discussion 813-4, 2011
                                                                                      9. Maslehaty H et al: Symptomatic spinal cavernous malformations: indication for microsurgical treatment and outcome. Eur Spine J. 20(10):1765-70, 2011
                                                                                      10. Mitha AP et al: Surgical approaches to intramedullary cavernous malformations of the spinal cord. Neurosurgery. 68(2 Suppl Operative):317-24; discussion 324, 2011
                                                                                      11. Labauge P et al: Outcome in 53 patients with spinal cord cavernomas. Surg Neurol. 70(2):176-81; discussion 181, 2008
                                                                                      12. Lee ST et al: Identification of an Arg35X mutation in the PDCD10 gene in a patient with cerebral and multiple spinal cavernous malformations. J Neurol Sci. 267(1-2):177-81, 2008
                                                                                      13. Kharkar S et al: The natural history of conservatively managed symptomatic intramedullary spinal cord cavernomas. Neurosurgery. 60(5):865-72; discussion 865-72, 2007
                                                                                      14. Jallo GI et al: Clinical presentation and optimal management for intramedullary cavernous malformations. Neurosurg Focus. 21(1):e10, 2006
                                                                                      15. Musunuru K et al: Widespread central nervous system cavernous malformations associated with cafe-au-lait skin lesions. Case report. J Neurosurg. 99(2):412-5, 2003
                                                                                      16. Sandalcioglu IE et al: Intramedullary spinal cord cavernous malformations: clinical features and risk of hemorrhage. Neurosurg Rev. 26(4):253-6, 2003
                                                                                      17. Chen DH et al: Cerebral cavernous malformation: novel mutation in a Chinese family and evidence for heterogeneity. J Neurol Sci. 196(1-2):91-6, 2002
                                                                                      18. Nagib MG et al: Intramedullary cavernous angiomas of the spinal cord in the pediatric age group: a pediatric series. Pediatr Neurosurg. 36(2):57-63, 2002
                                                                                      19. Balaban H et al: Multiple spinal intramedullary cavernous angioma: case report. Clin Neurol Neurosurg. 103(2):120-2, 2001
                                                                                      20. Clatterbuck RE et al: The nature and fate of punctate (type IV) cavernous malformations. Neurosurgery. 49(1):26-30; discussion 30-2, 2001
                                                                                      21. Deutsch H et al: Pediatric intramedullary spinal cavernous malformations. Spine. 26(18):E427-31, 2001
                                                                                      22. Mottolese C et al: Central nervous system cavernomas in the pediatric age group. Neurosurg Rev. 24(2-3):55-71; discussion 72-3, 2001
                                                                                      23. Sure U et al: Endothelial proliferation, neoangiogenesis, and potential de novo generation of cerebrovascular malformations. J Neurosurg. 94(6):972-7, 2001
                                                                                      24. Zacharia TT et al: Co-existing spinal cord and brain cavernous angiomas. J Assoc Physicians India. 49:835-7, 2001
                                                                                      25. Deutsch H et al: Spinal intramedullary cavernoma: clinical presentation and surgical outcome. J Neurosurg. 93(1 Suppl):65-70, 2000
                                                                                      26. Chabert E et al: Intramedullary cavernous malformations. J Neuroradiol. 26(4):262-8, 1999
                                                                                      27. Maraire JN et al: De novo development of a cavernous malformation of the spinal cord following spinal axis radiation. Case report. J Neurosurg. 90(2 Suppl):234-8, 1999
                                                                                      28. Moriarity JL et al: The natural history of cavernous malformations. Neurosurg Clin N Am. 10(3):411-7, 1999
                                                                                      29. Porter RW et al: Cavernous malformations of the brainstem: experience with 100 patients. J Neurosurg. 90(1):50-8, 1999
                                                                                      30. Vishteh AG et al: Patients with spinal cord cavernous malformations are at an increased risk for multiple neuraxis cavernous malformations. Neurosurgery. 45(1):30-2; discussion 33, 1999
                                                                                      31. Zevgaridis D et al: Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien). 141(3):237-45, 1999
                                                                                      32. Duke BJ et al: Cavernous angiomas of the cauda equina: case report and review of the literature. Surg Neurol. 50(5):442-5, 1998
                                                                                      33. Huffmann BC et al: Treatment strategies and results in spinal vascular malformations. Neurol Med Chir (Tokyo). 38 Suppl:231-7, 1998
                                                                                      34. Naim-Ur-Rahman et al: Intramedullary ossified cavernous angioma of the spinal cord: case report. Br J Neurosurg. 12(3):267-70, 1998
                                                                                      35. Padovani R et al: Cavernous angiomas of the spinal district: surgical treatment of 11 patients. Eur Spine J. 6(5):298-303, 1997
                                                                                      36. Harrison MJ et al: Symptomatic cavernous malformations affecting the spine and spinal cord. Neurosurgery. 37(2):195-204; discussion 204-5, 1995
                                                                                      37. Spetzger U et al: Cavernous angiomas of the spinal cord clinical presentation, surgical strategy, and postoperative results. Acta Neurochir (Wien). 134(3-4):200-6, 1995
                                                                                      38. Turjman F et al: MRI of intramedullary cavernous haemangiomas. Neuroradiology. 37(4):297-302, 1995
                                                                                      39. Canavero S et al: Spinal intramedullary cavernous angiomas: a literature meta-analysis. Surg Neurol. 41(5):381-8, 1994
                                                                                      40. Acciarri N et al: Surgical treatment of spinal cavernous angiomas. J Neurosurg Sci. 37(4):209-15, 1993
                                                                                      41. Acciarri N et al: Spinal cavernous angioma: a rare cause of subarachnoid hemorrhage. Surg Neurol. 37(6):453-6, 1992
                                                                                      42. Bourgouin PM et al: Multiple occult vascular malformations of the brain and spinal cord: MRI diagnosis. Neuroradiology. 34(2):110-1, 1992