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

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Vein of Galen aneurysmal malformation (VGAM), 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

              • Ultrasonographic Findings

                • CT Findings

                  • MR 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. D'Amico A et al: Outcome of fetal vein Galen aneurysmal malformations: a systematic review and meta-analysis. J Matern Fetal Neonatal Med. 1-6, 2021
                                            2. Arko L et al: Fetal and neonatal MRI predictors of aggressive early clinical course in vein of Galen malformation. AJNR Am J Neuroradiol. 41(6):1105-11, 2020
                                            3. Li TG et al: Diagnosis of foetal vein of galen aneurysmal malformation by ultrasound combined with magnetic resonance imaging: a case series. BMC Med Imaging. 20(1):63, 2020
                                            4. Berenstein A et al: Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Neurosurgery. 84(2):469-78, 2019
                                            5. 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, 2017
                                            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. 9(6):564-70, 2017
                                            7. 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
                                            8. Chow ML et al: Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg. 7(6):443-8, 2015
                                            9. Meila D et al: Cranial Doppler ultrasound in vein of Galen malformation. Neuroradiology. 57(2):211-9, 2015
                                            10. 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
                                            11. 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
                                            12. 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
                                            13. 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
                                            14. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                            15. 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
                                            16. 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
                                            17. 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
                                            18. Lasjaunias PL et al: The management of vein of Galen aneurysmal malformations. Neurosurgery. 59(5 Suppl 3):S184-94; discussion S3-13, 2006
                                            19. 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
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Vein of Galen aneurysmal malformation (VGAM), 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

                                                        • Ultrasonographic Findings

                                                          • CT Findings

                                                            • MR 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. D'Amico A et al: Outcome of fetal vein Galen aneurysmal malformations: a systematic review and meta-analysis. J Matern Fetal Neonatal Med. 1-6, 2021
                                                                                      2. Arko L et al: Fetal and neonatal MRI predictors of aggressive early clinical course in vein of Galen malformation. AJNR Am J Neuroradiol. 41(6):1105-11, 2020
                                                                                      3. Li TG et al: Diagnosis of foetal vein of galen aneurysmal malformation by ultrasound combined with magnetic resonance imaging: a case series. BMC Med Imaging. 20(1):63, 2020
                                                                                      4. Berenstein A et al: Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Neurosurgery. 84(2):469-78, 2019
                                                                                      5. 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, 2017
                                                                                      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. 9(6):564-70, 2017
                                                                                      7. 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
                                                                                      8. Chow ML et al: Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg. 7(6):443-8, 2015
                                                                                      9. Meila D et al: Cranial Doppler ultrasound in vein of Galen malformation. Neuroradiology. 57(2):211-9, 2015
                                                                                      10. 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
                                                                                      11. 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
                                                                                      12. 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
                                                                                      13. 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
                                                                                      14. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                                                                      15. 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
                                                                                      16. 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
                                                                                      17. 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
                                                                                      18. Lasjaunias PL et al: The management of vein of Galen aneurysmal malformations. Neurosurgery. 59(5 Suppl 3):S184-94; discussion S3-13, 2006
                                                                                      19. 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