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Arteriovenous Malformations (CNS)
Carlos E. Baccin, MD; James D. Rabinov, MD
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KEY FACTS

  • Terminology

    • Procedure

      • Outcomes

        TERMINOLOGY

        • Definitions

          • Cerebral arteriovenous malformation (AVM):Abnormal vascular tangle supplied by arterial feeder(s), drained by vein(s), without intervening capillary bed
            • Location: May occur anywhere in brain
              • Supratentorial (85%)
              • Posterior fossa (15%)
            • Angioarchitecture of AVM
              • Abnormal vascular tangle termed nidus; has tightly packed vascular channels
                • Intranidal "aneurysm" may be present in > 50%
              • Enlarged feeding arteries
                • 10-15% have flow-related aneurysm(s)
              • Dilated draining veins
                • Venous stenoses/aneurysms due to high flow
              • High-flow fistula component
                • AVM may have nonplexiform AV fistula component
            • Clinical presentation
              • Hemorrhage (53%)
                • 7% rebleeding in 1st year after hemorrhage
                • 2–4% hemorrhage risk/year (nonruptured AVM)
                • Morbidity with hemorrhage (20-30%)
                • Mortality with hemorrhage (5-30%)
              • Seizures (20-46%)
              • Headaches (34%)
              • Progressive neurological deficit (21%)
                • May be caused by mass effect/vascular "steal"
          • Spetzler-Martin grading system (range: 1-5)
            • Based upon scoring 3 AVM parameters
              • AVM nidus size
              • AVM venous drainage
              • Adjacent brain eloquent areas
                • Includes sensorimotor, language, visual cortex, thalamus/hypothalamus, internal capsule, brain stem, cerebellar peduncles, cerebellar nuclei
            • Used to predict treatment outcomes
              • Scores from each parameter added
              • Lower score easier to treat/better outcomes
            • Nidus size and score
              • Small (< 3 cm): 1
              • Medium (3-6 cm): 2
              • Large (> 6 cm): 3
            • Venous drainage and score
              • Superficial: 0
              • Deep: 1
            • Eloquence of adjacent brain and score
              • Noneloquent: 0
              • Eloquent: 1
          • Associated conditions
            • Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
              • Autosomal dominant disorder
              • Prevalence: 1/5,000–8,000
              • AVMs of brain, nose, skin, lungs, GI tract
            • Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
              • Neurocutaneous disorder
              • Angiomas involving leptomeninges, retina, facial dermatomes (port wine stains)
            • Wyburn-Mason syndrome (unilateral retinocephalic vascular malformation or Bonnet-Dechaume-Blanc syndrome)
              • Rare nonhereditary congenital condition
              • Unilateral vascular lesion of optic pathway
              • Brain/retinal AVMs
              • Frequently involves optic nerve/tract/chiasm/basal ganglia; may extend to occipital lobes

        PRE-PROCEDURE

        • Indications

          • Contraindications

            • Pre-Procedure Imaging

              • Getting Started

                PROCEDURE

                • Patient Position/Location

                  • Equipment Preparation

                    • Procedure Steps

                      • Findings and Reporting

                        • Alternative Procedures/Therapies

                          POST-PROCEDURE

                          • Expected Outcome

                            • Things to Do

                              • Things to Avoid

                                OUTCOMES

                                • Problems

                                  • Complications

                                    Selected References

                                    1. See AP et al: Stereotactic radiosurgery of cranial arteriovenous malformations and dural arteriovenous fistulas. Neurosurg Clin N Am. 23(1):133-46, 2012
                                    2. van Beijnum J et al: Treatment of brain arteriovenous malformations: a systematic review and meta-analysis. JAMA. 306(18):2011-9, 2011
                                    3. Loh Y et al: A prospective, multicenter, randomized trial of the Onyx liquid embolic system and N-butyl cyanoacrylate embolization of cerebral arteriovenous malformations. Clinical article. J Neurosurg. 113(4):733-41, 2010
                                    4. Mpotsaris A et al: Interdisciplinary clinical management of high grade arteriovenous malformations and ruptured flow-related aneurysms in the posterior fossa. Interv Neuroradiol. 16(4):400-8, 2010
                                    5. Harrigan MR et al: Handbook of Cerebrovascular Disease and Neurointerventional Technique. Totowa, NJ: Humana Press, 2009
                                    6. Weber W et al: Preoperative embolization of intracranial arteriovenous malformations with Onyx. Neurosurgery. 61(2):244-52, 2007
                                    7. Nagashima H et al: Embolization of arteriovenous malformation. Efficacy and safety of preoperative embolization followed by surgical resection of AVM. Interv Neuroradiol. 10 Suppl 2:54-8, 2004
                                    8. Osborn A et al: Diagnostic Imaging: Brain. 1st ed. Salt Lake City: Amirsys, 2004
                                    9. Spetzler RF et al: A proposed grading system for arteriovenous malformations. J Neurosurg. 65(4):476-83, 1986
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Procedure

                                        • Outcomes

                                          TERMINOLOGY

                                          • Definitions

                                            • Cerebral arteriovenous malformation (AVM):Abnormal vascular tangle supplied by arterial feeder(s), drained by vein(s), without intervening capillary bed
                                              • Location: May occur anywhere in brain
                                                • Supratentorial (85%)
                                                • Posterior fossa (15%)
                                              • Angioarchitecture of AVM
                                                • Abnormal vascular tangle termed nidus; has tightly packed vascular channels
                                                  • Intranidal "aneurysm" may be present in > 50%
                                                • Enlarged feeding arteries
                                                  • 10-15% have flow-related aneurysm(s)
                                                • Dilated draining veins
                                                  • Venous stenoses/aneurysms due to high flow
                                                • High-flow fistula component
                                                  • AVM may have nonplexiform AV fistula component
                                              • Clinical presentation
                                                • Hemorrhage (53%)
                                                  • 7% rebleeding in 1st year after hemorrhage
                                                  • 2–4% hemorrhage risk/year (nonruptured AVM)
                                                  • Morbidity with hemorrhage (20-30%)
                                                  • Mortality with hemorrhage (5-30%)
                                                • Seizures (20-46%)
                                                • Headaches (34%)
                                                • Progressive neurological deficit (21%)
                                                  • May be caused by mass effect/vascular "steal"
                                            • Spetzler-Martin grading system (range: 1-5)
                                              • Based upon scoring 3 AVM parameters
                                                • AVM nidus size
                                                • AVM venous drainage
                                                • Adjacent brain eloquent areas
                                                  • Includes sensorimotor, language, visual cortex, thalamus/hypothalamus, internal capsule, brain stem, cerebellar peduncles, cerebellar nuclei
                                              • Used to predict treatment outcomes
                                                • Scores from each parameter added
                                                • Lower score easier to treat/better outcomes
                                              • Nidus size and score
                                                • Small (< 3 cm): 1
                                                • Medium (3-6 cm): 2
                                                • Large (> 6 cm): 3
                                              • Venous drainage and score
                                                • Superficial: 0
                                                • Deep: 1
                                              • Eloquence of adjacent brain and score
                                                • Noneloquent: 0
                                                • Eloquent: 1
                                            • Associated conditions
                                              • Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
                                                • Autosomal dominant disorder
                                                • Prevalence: 1/5,000–8,000
                                                • AVMs of brain, nose, skin, lungs, GI tract
                                              • Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
                                                • Neurocutaneous disorder
                                                • Angiomas involving leptomeninges, retina, facial dermatomes (port wine stains)
                                              • Wyburn-Mason syndrome (unilateral retinocephalic vascular malformation or Bonnet-Dechaume-Blanc syndrome)
                                                • Rare nonhereditary congenital condition
                                                • Unilateral vascular lesion of optic pathway
                                                • Brain/retinal AVMs
                                                • Frequently involves optic nerve/tract/chiasm/basal ganglia; may extend to occipital lobes

                                          PRE-PROCEDURE

                                          • Indications

                                            • Contraindications

                                              • Pre-Procedure Imaging

                                                • Getting Started

                                                  PROCEDURE

                                                  • Patient Position/Location

                                                    • Equipment Preparation

                                                      • Procedure Steps

                                                        • Findings and Reporting

                                                          • Alternative Procedures/Therapies

                                                            POST-PROCEDURE

                                                            • Expected Outcome

                                                              • Things to Do

                                                                • Things to Avoid

                                                                  OUTCOMES

                                                                  • Problems

                                                                    • Complications

                                                                      Selected References

                                                                      1. See AP et al: Stereotactic radiosurgery of cranial arteriovenous malformations and dural arteriovenous fistulas. Neurosurg Clin N Am. 23(1):133-46, 2012
                                                                      2. van Beijnum J et al: Treatment of brain arteriovenous malformations: a systematic review and meta-analysis. JAMA. 306(18):2011-9, 2011
                                                                      3. Loh Y et al: A prospective, multicenter, randomized trial of the Onyx liquid embolic system and N-butyl cyanoacrylate embolization of cerebral arteriovenous malformations. Clinical article. J Neurosurg. 113(4):733-41, 2010
                                                                      4. Mpotsaris A et al: Interdisciplinary clinical management of high grade arteriovenous malformations and ruptured flow-related aneurysms in the posterior fossa. Interv Neuroradiol. 16(4):400-8, 2010
                                                                      5. Harrigan MR et al: Handbook of Cerebrovascular Disease and Neurointerventional Technique. Totowa, NJ: Humana Press, 2009
                                                                      6. Weber W et al: Preoperative embolization of intracranial arteriovenous malformations with Onyx. Neurosurgery. 61(2):244-52, 2007
                                                                      7. Nagashima H et al: Embolization of arteriovenous malformation. Efficacy and safety of preoperative embolization followed by surgical resection of AVM. Interv Neuroradiol. 10 Suppl 2:54-8, 2004
                                                                      8. Osborn A et al: Diagnostic Imaging: Brain. 1st ed. Salt Lake City: Amirsys, 2004
                                                                      9. Spetzler RF et al: A proposed grading system for arteriovenous malformations. J Neurosurg. 65(4):476-83, 1986