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Type 4 Vascular Malformation (AVF)
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Perimedullary arteriovenous fistula (AVF), type 4 spinal vascular malformation, pial AVF, ventral AVF
            • Definitions

              • General spine vascular malformation classification
                • Type 1: Dural arteriovenous fistula (DAVF)
                  • Most common = type 1 (up to 80%)
                • Type 2: Intramedullary glomus-type AVM (similar to brain AVM)
                • Type 3: Juvenile-type AVM (intramedullary, extramedullary)
                  • 2nd most common = intramedullary types 2 and 3 (15-20%)
                • Type 4: Intradural extra-/perimedullary AVF (types A, B, C)
              • Type 4: Direct intradural extramedullary arterial/venous communication from anterior spinal artery (ASA), or posterior spinal artery (PSA) to draining vein without capillary bed
                • Heterogenous group of lesions ranging from slow flow fistula (IVa) to large high flow (IVc)

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic 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

                                            Selected References

                                            1. Gross BA et al: Spinal pial (type IV) arteriovenous fistulae: a systematic pooled analysis of demographics, hemorrhage risk, and treatment results. Neurosurgery. 73(1):141-51; discussion 151, 2013
                                            2. Krings T et al: Spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol. 30(4):639-48, 2009
                                            3. Pattany PM et al: MR angiography of the spine and spinal cord. Top Magn Reson Imaging. 14(6):444-60, 2003
                                            4. Hida K et al: Corpectomy: a direct approach to perimedullary arteriovenous fistulas of the anterior cervical spinal cord. J Neurosurg. 96(2 Suppl): 157-61, 2002
                                            5. 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
                                            6. Spetzler RF et al: Modified classification of spinal cord vascular lesions. J Neurosurg. 96(2 Suppl): 145-56, 2002
                                            7. Mascalchi M et al: Spinal vascular malformations: MR angiography after treatment. Radiology. 219(2): 346-53, 2001
                                            8. Sugiu K et al: Successful embolization of a spinal perimedullary arteriovenous fistula with cellulose acetate polymer solution: technical case report. Neurosurgery. 49(5): 1257-60; discussion 1260-1, 2001
                                            9. Vates GE et al: Conus perimedullary arteriovenous fistula with intracranial drainage: case report. Neurosurgery. 49(2): 457-61; discussion 461-2, 2001
                                            10. Sure U et al: Spinal type IV arteriovenous malformations (perimedullary fistulas) in children. Childs Nerv Syst. 16(8): 508-15, 2000
                                            11. Grote EH et al: Clinical syndromes, natural history, and pathophysiology of vascular lesions of the spinal cord. Neurosurg Clin N Am. 10(1): 17-45, 1999
                                            12. Hida K et al: Results of the surgical treatment of perimedullary arteriovenous fistulas with special reference to embolization. J Neurosurg. 90(4 Suppl): 198-205, 1999
                                            13. Mascalchi M et al: Contrast-enhanced time-resolved MR angiography of spinal vascular malformations. J Comput Assist Tomogr. 23(3): 341-5, 1999
                                            14. Bao YH et al: Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 40(1):75-81, 1997
                                            15. Ricolfi F et al: Giant perimedullary arteriovenous fistulas of the spine: clinical and radiologic features and endovascular treatment. AJNR Am J Neuroradiol. 18(4): 677-87, 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

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Perimedullary arteriovenous fistula (AVF), type 4 spinal vascular malformation, pial AVF, ventral AVF
                                                      • Definitions

                                                        • General spine vascular malformation classification
                                                          • Type 1: Dural arteriovenous fistula (DAVF)
                                                            • Most common = type 1 (up to 80%)
                                                          • Type 2: Intramedullary glomus-type AVM (similar to brain AVM)
                                                          • Type 3: Juvenile-type AVM (intramedullary, extramedullary)
                                                            • 2nd most common = intramedullary types 2 and 3 (15-20%)
                                                          • Type 4: Intradural extra-/perimedullary AVF (types A, B, C)
                                                        • Type 4: Direct intradural extramedullary arterial/venous communication from anterior spinal artery (ASA), or posterior spinal artery (PSA) to draining vein without capillary bed
                                                          • Heterogenous group of lesions ranging from slow flow fistula (IVa) to large high flow (IVc)

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Angiographic 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

                                                                                      Selected References

                                                                                      1. Gross BA et al: Spinal pial (type IV) arteriovenous fistulae: a systematic pooled analysis of demographics, hemorrhage risk, and treatment results. Neurosurgery. 73(1):141-51; discussion 151, 2013
                                                                                      2. Krings T et al: Spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol. 30(4):639-48, 2009
                                                                                      3. Pattany PM et al: MR angiography of the spine and spinal cord. Top Magn Reson Imaging. 14(6):444-60, 2003
                                                                                      4. Hida K et al: Corpectomy: a direct approach to perimedullary arteriovenous fistulas of the anterior cervical spinal cord. J Neurosurg. 96(2 Suppl): 157-61, 2002
                                                                                      5. 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
                                                                                      6. Spetzler RF et al: Modified classification of spinal cord vascular lesions. J Neurosurg. 96(2 Suppl): 145-56, 2002
                                                                                      7. Mascalchi M et al: Spinal vascular malformations: MR angiography after treatment. Radiology. 219(2): 346-53, 2001
                                                                                      8. Sugiu K et al: Successful embolization of a spinal perimedullary arteriovenous fistula with cellulose acetate polymer solution: technical case report. Neurosurgery. 49(5): 1257-60; discussion 1260-1, 2001
                                                                                      9. Vates GE et al: Conus perimedullary arteriovenous fistula with intracranial drainage: case report. Neurosurgery. 49(2): 457-61; discussion 461-2, 2001
                                                                                      10. Sure U et al: Spinal type IV arteriovenous malformations (perimedullary fistulas) in children. Childs Nerv Syst. 16(8): 508-15, 2000
                                                                                      11. Grote EH et al: Clinical syndromes, natural history, and pathophysiology of vascular lesions of the spinal cord. Neurosurg Clin N Am. 10(1): 17-45, 1999
                                                                                      12. Hida K et al: Results of the surgical treatment of perimedullary arteriovenous fistulas with special reference to embolization. J Neurosurg. 90(4 Suppl): 198-205, 1999
                                                                                      13. Mascalchi M et al: Contrast-enhanced time-resolved MR angiography of spinal vascular malformations. J Comput Assist Tomogr. 23(3): 341-5, 1999
                                                                                      14. Bao YH et al: Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 40(1):75-81, 1997
                                                                                      15. Ricolfi F et al: Giant perimedullary arteriovenous fistulas of the spine: clinical and radiologic features and endovascular treatment. AJNR Am J Neuroradiol. 18(4): 677-87, 1997