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Vein of Galen Aneurysmal Malformation
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Vein of Galen malformation (VGAM), vein of Galen aneurysm, galenic varix
              • Definitions

                • High-flow arteriovenous (AV) fistula between deep choroidal arteries & median prosencephalic vein (MPV) of Markowski
                  • Not actually aneurysm of true vein of Galen, which fails to form because of fistula

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Echocardiographic Findings

                          • Angiographic Findings

                            • Imaging Recommendations

                              DIFFERENTIAL DIAGNOSIS

                                PATHOLOGY

                                • General Features

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Berenstein A et al: Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Neurosurgery. 84(2):469-78, 2019
                                                2. Duran D et al: Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 21(4):367-74, 2018
                                                3. Agarwal H et al: Vein of Galen aneurysmal malformation-clinical and angiographic spectrum with management perspective: an institutional experience. J Neurointerv Surg. 9(2):159-64, 2016
                                                4. Meila D et al: Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications. Acta Neurochir (Wien). 158(7):1279-84, 2016
                                                5. Saliou G et al: Decreased duperior sagittal sinus diameter and jugular bulb narrowing are associated with poor clinical outcome in vein of Galen arteriovenous malformation. AJNR Am J Neuroradiol. 37(7):1354-8, 2016
                                                6. Yan J et al: The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis. J Neurointerv Surg. ePub, 2016
                                                7. Chow ML et al: Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg. 7(6):443-8, 2015
                                                8. Meila D et al: Cranial Doppler ultrasound in vein of Galen malformation. Neuroradiology. 57(2):211-9, 2015
                                                9. Zaidi HA et al: Multimodal treatment strategies for complex pediatric cerebral arteriovenous fistulas: contemporary case series at Barrow Neurological Institute. J Neurosurg Pediatr. 1-10, 2015
                                                10. Mortazavi MM et al: Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 12(3):293-306, 2013
                                                11. Deloison B et al: Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. Ultrasound Obstet Gynecol. 40(6):652-8, 2012
                                                12. Jagadeesan BD et al: Susceptibility-weighted imaging: a new tool in the diagnosis and evaluation of abnormalities of the vein of Galen in children. AJNR Am J Neuroradiol. 33(9):1747-51, 2012
                                                13. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                                14. Suazo L et al: Measurement of blood flow in arteriovenous malformations before and after embolization using arterial spin labeling. Interv Neuroradiol. 18(1):42-8, 2012
                                                15. Jea A et al: The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatr Neurosurg. 46(2):141-5, 2010
                                                16. Pearl M et al: Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. Childs Nerv Syst. 26(10):1367-79, 2010
                                                17. Lasjaunias PL et al: The management of vein of Galen aneurysmal malformations. Neurosurgery. 59(5 Suppl 3):S184-94; discussion S3-13, 2006
                                                18. Jones BV et al: Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up. AJNR Am J Neuroradiol. 23(10):1717-24, 2002
                                                19. Charbonneau F et al: Effects of antihypertensive agents on endothelial dysfunction: rationale for the brachial artery normalization of forearm function study. Am J Cardiol. 82(10A):34S-36S, 1998
                                                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

                                                              • Vein of Galen malformation (VGAM), vein of Galen aneurysm, galenic varix
                                                            • Definitions

                                                              • High-flow arteriovenous (AV) fistula between deep choroidal arteries & median prosencephalic vein (MPV) of Markowski
                                                                • Not actually aneurysm of true vein of Galen, which fails to form because of fistula

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic Findings

                                                                      • Echocardiographic Findings

                                                                        • Angiographic Findings

                                                                          • Imaging Recommendations

                                                                            DIFFERENTIAL DIAGNOSIS

                                                                              PATHOLOGY

                                                                              • General Features

                                                                                • Gross Pathologic & Surgical Features

                                                                                  • Microscopic Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Berenstein A et al: Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Neurosurgery. 84(2):469-78, 2019
                                                                                              2. Duran D et al: Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 21(4):367-74, 2018
                                                                                              3. Agarwal H et al: Vein of Galen aneurysmal malformation-clinical and angiographic spectrum with management perspective: an institutional experience. J Neurointerv Surg. 9(2):159-64, 2016
                                                                                              4. Meila D et al: Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications. Acta Neurochir (Wien). 158(7):1279-84, 2016
                                                                                              5. Saliou G et al: Decreased duperior sagittal sinus diameter and jugular bulb narrowing are associated with poor clinical outcome in vein of Galen arteriovenous malformation. AJNR Am J Neuroradiol. 37(7):1354-8, 2016
                                                                                              6. Yan J et al: The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis. J Neurointerv Surg. ePub, 2016
                                                                                              7. Chow ML et al: Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg. 7(6):443-8, 2015
                                                                                              8. Meila D et al: Cranial Doppler ultrasound in vein of Galen malformation. Neuroradiology. 57(2):211-9, 2015
                                                                                              9. Zaidi HA et al: Multimodal treatment strategies for complex pediatric cerebral arteriovenous fistulas: contemporary case series at Barrow Neurological Institute. J Neurosurg Pediatr. 1-10, 2015
                                                                                              10. Mortazavi MM et al: Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 12(3):293-306, 2013
                                                                                              11. Deloison B et al: Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. Ultrasound Obstet Gynecol. 40(6):652-8, 2012
                                                                                              12. Jagadeesan BD et al: Susceptibility-weighted imaging: a new tool in the diagnosis and evaluation of abnormalities of the vein of Galen in children. AJNR Am J Neuroradiol. 33(9):1747-51, 2012
                                                                                              13. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                                                                              14. Suazo L et al: Measurement of blood flow in arteriovenous malformations before and after embolization using arterial spin labeling. Interv Neuroradiol. 18(1):42-8, 2012
                                                                                              15. Jea A et al: The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatr Neurosurg. 46(2):141-5, 2010
                                                                                              16. Pearl M et al: Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. Childs Nerv Syst. 26(10):1367-79, 2010
                                                                                              17. Lasjaunias PL et al: The management of vein of Galen aneurysmal malformations. Neurosurgery. 59(5 Suppl 3):S184-94; discussion S3-13, 2006
                                                                                              18. Jones BV et al: Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up. AJNR Am J Neuroradiol. 23(10):1717-24, 2002
                                                                                              19. Charbonneau F et al: Effects of antihypertensive agents on endothelial dysfunction: rationale for the brachial artery normalization of forearm function study. Am J Cardiol. 82(10A):34S-36S, 1998