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Type 3 Arteriovenous Malformation (AVM)
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Juvenile arteriovenous malformation (AVM); intramedullary extramedullary AVM
              • Definitions

                • Direct arterial/venous communications without capillary bed involving cord
                • General spine vascular malformation classification
                  • Type 1: Dural arteriovenous fistula (DAVF)
                    • Most common type (up to 80%)
                  • Type 2: Intramedullary glomus-type AVM (similar to brain AVM)
                  • Type 3: Juvenile-type AVM (large, complex, intramedullary extramedullary AVM)
                    • Rarest of spinal AVMs (7%)
                  • Type 4: Intradural extra-/perimedullary AVF

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nonvascular Interventions

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Rubin MN et al: Vascular diseases of the spinal cord. Neurol Clin. 31(1):153-81, 2013
                                              2. Schirmer CM et al: Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision. J Neurosurg Pediatr. 10(1):44-9, 2012
                                              3. Spiotta AM et al: Combined endovascular and surgical resection of a giant lumbosacral arteriovenous malformation in a patient with Cobb syndrome. J Neurointerv Surg. 3(3):293-6, 2011
                                              4. Johnson WD et al: Variety of spinal vascular pathology seen in adult Cobb syndrome. J Neurosurg Spine. 10(5):430-5, 2009
                                              5. Backes WH et al: Advances in spinal cord MR angiography. AJNR Am J Neuroradiol. 29(4):619-31, 2008
                                              6. Mull M et al: Value and limitations of contrast-enhanced MR angiography in spinal arteriovenous malformations and dural arteriovenous fistulas. AJNR Am J Neuroradiol. 28(7):1249-58, 2007
                                              7. Menku A et al: Successful surgical excision of juvenile-type spinal arteriovenous malformation in two stages following partial embolization. Minim Invasive Neurosurg. 48(1):57-62, 2005
                                              8. Rodesch G et al: Classification of spinal cord arteriovenous shunts: proposal for a reappraisal--the Bicetre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery. 51(2): 374-9; discussion 379-80, 2002
                                              9. Saraf-Lavi E et al: Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced MR angiography: sensitivity, specificity, and prediction of vertebral level. AJNR Am J Neuroradiol. 23(5): 858-67, 2002
                                              10. Spetzler RF et al: Modified classification of spinal cord vascular lesions. J Neurosurg. 96(2 Suppl): 145-56, 2002
                                              11. Van Dijk JM et al: Multidisciplinary management of spinal dural arteriovenous fistulas: clinical presentation and long-term follow-up in 49 patients. Stroke. 33(6): 1578-83, 2002
                                              12. Bemporad JA et al: Magnetic resonance imaging of spinal cord vascular malformations with an emphasis on the cervical spine. Neuroimaging Clin N Am. 11(1): viii, 111-29, 2001
                                              13. Ferch RD et al: Spinal arteriovenous malformations: a review with case illustrations. J Clin Neurosci. 8(4): 299-304, 2001
                                              14. Mascalchi M et al: Spinal vascular malformations: MR angiography after treatment. Radiology. 219(2): 346-53, 2001
                                              15. Rosenow J et al: Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: case report. Neurosurgery. 46(5): 1240-4; discussion 1244-5, 2000
                                              16. Hasegawa M et al: The efficacy of CT arteriography for spinal arteriovenous fistula surgery: technical note. Neuroradiology. 41(12): 915-9, 1999
                                              17. Mandzia JL et al: Spinal cord arteriovenous malformations in two patients with hereditary hemorrhagic telangiectasia. Childs Nerv Syst. 15(2-3): 80-3, 1999
                                              18. Bao YH et al: Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 40(1):75-81, 1997
                                              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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Juvenile arteriovenous malformation (AVM); intramedullary extramedullary AVM
                                                          • Definitions

                                                            • Direct arterial/venous communications without capillary bed involving cord
                                                            • General spine vascular malformation classification
                                                              • Type 1: Dural arteriovenous fistula (DAVF)
                                                                • Most common type (up to 80%)
                                                              • Type 2: Intramedullary glomus-type AVM (similar to brain AVM)
                                                              • Type 3: Juvenile-type AVM (large, complex, intramedullary extramedullary AVM)
                                                                • Rarest of spinal AVMs (7%)
                                                              • Type 4: Intradural extra-/perimedullary AVF

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Nonvascular Interventions

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Rubin MN et al: Vascular diseases of the spinal cord. Neurol Clin. 31(1):153-81, 2013
                                                                                          2. Schirmer CM et al: Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision. J Neurosurg Pediatr. 10(1):44-9, 2012
                                                                                          3. Spiotta AM et al: Combined endovascular and surgical resection of a giant lumbosacral arteriovenous malformation in a patient with Cobb syndrome. J Neurointerv Surg. 3(3):293-6, 2011
                                                                                          4. Johnson WD et al: Variety of spinal vascular pathology seen in adult Cobb syndrome. J Neurosurg Spine. 10(5):430-5, 2009
                                                                                          5. Backes WH et al: Advances in spinal cord MR angiography. AJNR Am J Neuroradiol. 29(4):619-31, 2008
                                                                                          6. Mull M et al: Value and limitations of contrast-enhanced MR angiography in spinal arteriovenous malformations and dural arteriovenous fistulas. AJNR Am J Neuroradiol. 28(7):1249-58, 2007
                                                                                          7. Menku A et al: Successful surgical excision of juvenile-type spinal arteriovenous malformation in two stages following partial embolization. Minim Invasive Neurosurg. 48(1):57-62, 2005
                                                                                          8. Rodesch G et al: Classification of spinal cord arteriovenous shunts: proposal for a reappraisal--the Bicetre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery. 51(2): 374-9; discussion 379-80, 2002
                                                                                          9. Saraf-Lavi E et al: Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced MR angiography: sensitivity, specificity, and prediction of vertebral level. AJNR Am J Neuroradiol. 23(5): 858-67, 2002
                                                                                          10. Spetzler RF et al: Modified classification of spinal cord vascular lesions. J Neurosurg. 96(2 Suppl): 145-56, 2002
                                                                                          11. Van Dijk JM et al: Multidisciplinary management of spinal dural arteriovenous fistulas: clinical presentation and long-term follow-up in 49 patients. Stroke. 33(6): 1578-83, 2002
                                                                                          12. Bemporad JA et al: Magnetic resonance imaging of spinal cord vascular malformations with an emphasis on the cervical spine. Neuroimaging Clin N Am. 11(1): viii, 111-29, 2001
                                                                                          13. Ferch RD et al: Spinal arteriovenous malformations: a review with case illustrations. J Clin Neurosci. 8(4): 299-304, 2001
                                                                                          14. Mascalchi M et al: Spinal vascular malformations: MR angiography after treatment. Radiology. 219(2): 346-53, 2001
                                                                                          15. Rosenow J et al: Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: case report. Neurosurgery. 46(5): 1240-4; discussion 1244-5, 2000
                                                                                          16. Hasegawa M et al: The efficacy of CT arteriography for spinal arteriovenous fistula surgery: technical note. Neuroradiology. 41(12): 915-9, 1999
                                                                                          17. Mandzia JL et al: Spinal cord arteriovenous malformations in two patients with hereditary hemorrhagic telangiectasia. Childs Nerv Syst. 15(2-3): 80-3, 1999
                                                                                          18. Bao YH et al: Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 40(1):75-81, 1997