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Vascular Malformations
Vein of Galen Aneurysmal Malformation
Vein of Galen Aneurysmal Malformation
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Vein of Galen aneurysm

          • Definitions

            • Arteriovenous fistula (AVF) between deep choroidal arteries and embryonic median prosencephalic vein (MPV) of Markowski
              • High flow through MPV prevents formation of vein of Galen
            • Vein of Galen aneurysmal malformation (VGAM) is actually misnomer but still commonly used to describe this type of fistula

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • MR Findings

                • 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. De Luca C et al: An ACVRL1 gene mutation presenting as vein of Galen malformation at prenatal diagnosis. Am J Med Genet A. 182(5):1255-8, 2020
                                      2. Mochizuki Y et al: Clinical course and management of vein of Galen varix of the neonate: a case report and literature review. Pediatr Neurosurg. 54(4):281-7, 2019
                                      3. Spada C et al: Heart failure caused by VGAM: a lesson for diagnosis and treatment from a case and literature review. J Matern Fetal Neonatal Med. 1-7, 2019
                                      4. Duran D et al: Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 21(4):367-74, 2018
                                      5. Marinaş MC et al: A severe case of aneurysmal malformation of the vein of Galen, cardiac failure and intrauterine fetal death. Curr Health Sci J. 44(1):76-9, 2018
                                      6. Vivanti A et al: Loss of function mutations in EPHB4 are responsible for vein of Galen aneurysmal malformation. Brain. 141(4):979-88, 2018
                                      7. Pires P et al: Prenatal diagnosis of Galen vein aneurysm using ultrasonography and magnetic resonance imaging and perinatal and long-term neurological outcomes: a case series. Rev Bras Ginecol Obstet. 39(6):309-14, 2017
                                      8. Saliou G et al: Pseudofeeders on fetal magnetic resonance imaging predict outcome in vein of Galen malformations. Ann Neurol. 81(2):278-86, 2017
                                      9. Zhou LX et al: Diagnosis of vein of Galen aneurysmal malformation using fetal MRI. J Magn Reson Imaging. 46(5):1535-9, 2016
                                      10. Kwong Y et al: Fetal MRI demonstrating vein of Galen malformations in two successive pregnancies-a previously unreported occurrence. Childs Nerv Syst. 31(7):1033-5, 2015
                                      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. Gün I et al: Is prenatal volumetric evaluation of aneurysm of the vein of Galen important in the prediction of adverse fetal outcome? J Clin Ultrasound. 38(8):443-5, 2010
                                      13. Heuer GG et al: Diagnosis and treatment of vein of Galen aneurysmal malformations. Childs Nerv Syst. 26(7):879-87, 2010
                                      14. Kalra V et al: Fetal MR diagnosis of vein of Galen aneurysmal malformation. Pediatr Radiol. 40 Suppl 1:S155, 2010
                                      15. McSweeney N et al: Management and outcome of vein of Galen malformation. Arch Dis Child. 95(11):903-9, 2010
                                      16. Vijayaraghavan SB et al: Prenatal diagnosis of thrombosed aneurysm of vein of Galen. Ultrasound Obstet Gynecol. 27(1):81-3, 2006
                                      17. Paternoster DM et al: Prenatal diagnosis of vein of Galen aneurysmal malformations. Fetal Diagn Ther. 18(6):408-11, 2003
                                      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
                                      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 aneurysm

                                              • Definitions

                                                • Arteriovenous fistula (AVF) between deep choroidal arteries and embryonic median prosencephalic vein (MPV) of Markowski
                                                  • High flow through MPV prevents formation of vein of Galen
                                                • Vein of Galen aneurysmal malformation (VGAM) is actually misnomer but still commonly used to describe this type of fistula

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • 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. De Luca C et al: An ACVRL1 gene mutation presenting as vein of Galen malformation at prenatal diagnosis. Am J Med Genet A. 182(5):1255-8, 2020
                                                                          2. Mochizuki Y et al: Clinical course and management of vein of Galen varix of the neonate: a case report and literature review. Pediatr Neurosurg. 54(4):281-7, 2019
                                                                          3. Spada C et al: Heart failure caused by VGAM: a lesson for diagnosis and treatment from a case and literature review. J Matern Fetal Neonatal Med. 1-7, 2019
                                                                          4. Duran D et al: Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 21(4):367-74, 2018
                                                                          5. Marinaş MC et al: A severe case of aneurysmal malformation of the vein of Galen, cardiac failure and intrauterine fetal death. Curr Health Sci J. 44(1):76-9, 2018
                                                                          6. Vivanti A et al: Loss of function mutations in EPHB4 are responsible for vein of Galen aneurysmal malformation. Brain. 141(4):979-88, 2018
                                                                          7. Pires P et al: Prenatal diagnosis of Galen vein aneurysm using ultrasonography and magnetic resonance imaging and perinatal and long-term neurological outcomes: a case series. Rev Bras Ginecol Obstet. 39(6):309-14, 2017
                                                                          8. Saliou G et al: Pseudofeeders on fetal magnetic resonance imaging predict outcome in vein of Galen malformations. Ann Neurol. 81(2):278-86, 2017
                                                                          9. Zhou LX et al: Diagnosis of vein of Galen aneurysmal malformation using fetal MRI. J Magn Reson Imaging. 46(5):1535-9, 2016
                                                                          10. Kwong Y et al: Fetal MRI demonstrating vein of Galen malformations in two successive pregnancies-a previously unreported occurrence. Childs Nerv Syst. 31(7):1033-5, 2015
                                                                          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. Gün I et al: Is prenatal volumetric evaluation of aneurysm of the vein of Galen important in the prediction of adverse fetal outcome? J Clin Ultrasound. 38(8):443-5, 2010
                                                                          13. Heuer GG et al: Diagnosis and treatment of vein of Galen aneurysmal malformations. Childs Nerv Syst. 26(7):879-87, 2010
                                                                          14. Kalra V et al: Fetal MR diagnosis of vein of Galen aneurysmal malformation. Pediatr Radiol. 40 Suppl 1:S155, 2010
                                                                          15. McSweeney N et al: Management and outcome of vein of Galen malformation. Arch Dis Child. 95(11):903-9, 2010
                                                                          16. Vijayaraghavan SB et al: Prenatal diagnosis of thrombosed aneurysm of vein of Galen. Ultrasound Obstet Gynecol. 27(1):81-3, 2006
                                                                          17. Paternoster DM et al: Prenatal diagnosis of vein of Galen aneurysmal malformations. Fetal Diagn Ther. 18(6):408-11, 2003
                                                                          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