link
Bookmarks
Vein of Galen Malformation Treatment
Carlos E. Baccin, MD; James D. Rabinov, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
0

KEY FACTS

  • Terminology

    • Pre-Procedure

      • Procedure

        • Post-Procedure

          TERMINOLOGY

          • Definitions

            • Vein of Galen aneurysmal malformation (VGAM): Arteriovenous malformation (AVM) resulting in aneurysmal dilatation of vein of Galen
              • Rare, dramatic choroidal form of embryonic AVM
                • Supplied by choroidal arteries
                • Midline location in choroidal fissure at quadrigeminal plate/velum interpositum cisterns
              • Persistent median prosencephalic vein of Markowski (MPV): Precursor of vein of Galen
                • Vein becomes aneurysmal
                • Drains via falcine sinus to superior sagittal sinus
              • Arteriovenous (AV) fistula between deep choroidal arteries/median prosencephalic vein
              • Various morphologies
                • Choroidal type: Myriad varying-sized complex shunts from pericallosal artery/posterior cerebral artery (PCA)/anterior choroidal artery (AChA)
                • Mural type: Fewer shunts (PCA/collicular arteries)
              • Variable shunt size, number, complexity depending on type; commensurate MPV dilatation
              • Clinical presentation of progressively worsening cardiac failure leading to multiorgan failure
            • Vein of Galen aneurysmal dilatation (VGAD): Aneurysmal dilatation of "true" vein of Galen caused by adjacent brain AVM; different from VGAM
              • AVM localized in subpial space (supra- or infratentorially) drains into usual tributaries of vein of Galen; results in venous overload/dilatation
              • Vein of Galen fully developed; drains not only AVM but also adjacent brain parenchyma
              • Dilatation of vein of Galen variable
                • Depends on any outflow obstruction due to stenoses/thrombosis where vein of Galen joins straight sinus
              • No melting brain syndrome, no mental retardation, no jugular bulb dysmaturation
                • Higher risk of hemorrhage than VGAM
            • Vein of Galen varix (also known as Galenic varix)
              • No AV malformation or fistula
              • 2 types
                • Transient dilatation in neonates
                  • Caused by cardiac failure of another etiology
                  • Persists for few days postnatally, disappears
                  • Asymptomatic; disappearance parallels cardiac improvement
                • Complex developmental venous anomalies/postthrombotic collateral circulation drain entire cerebral hemisphere into deep venous system
                  • Causes dilatation of vein of Galen
                  • Patients may show symptoms of venous ischemia from insufficient venous drainage
            • Dural arteriovenous shunts with aneurysmal dilatation of vein of Galen
              • Acquired dural lesions develop in wall of vein of Galen or at venous-sinus junction
                • Straight sinus almost always thrombosed
                • Most common in adults

          PRE-PROCEDURE

          • Indications

            • Contraindications

              • Pre-Procedure Imaging

                • Getting Started

                  PROCEDURE

                  • Patient Position/Location

                    • Procedure Steps

                      • Alternative Procedures/Therapies

                        POST-PROCEDURE

                        • Expected Outcome

                          • Things to Do

                            • Things to Avoid

                              OUTCOMES

                              • Complications

                                Selected References

                                1. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                2. Berenstein A et al: Vein of Galen Malformations in Neonates: New Management Paradigms for Improving Outcomes. Neurosurgery. Epub ahead of print, 2011
                                3. Rao VR et al: Pediatric aneurysms and vein of Galen malformations. J Pediatr Neurosci. 6(Suppl 1):S109-17, 2011
                                4. Papanagiotou P et al: Vein of galen aneurysmal malformation treated with Onyx. Arch Neurol. 66(7):906-7, 2009
                                5. Alvarez H et al: Vein of galen aneurysmal malformations. Neuroimaging Clin N Am. 17(2):189-206, 2007
                                6. Cekirge HS et al: Intrasaccular combination of metallic coils and onyx liquid embolic agent for the endovascular treatment of cerebral aneurysms. J Neurosurg. 105(5):706-12, 2006
                                7. Gailloud P et al: Confirmation of communication between deep venous drainage and the vein of galen after treatment of a vein of Galen aneurysmal malformation in an infant presenting with severe pulmonary hypertension. AJNR Am J Neuroradiol. 27(2):317-20, 2006
                                8. Lasjaunias P et al: Management of vein of Galen aneurysm malformations. Neurosurgery. 59(5):184-94, 2006
                                9. Gailloud P et al: Diagnosis & management of vein of Galen aneurysmal malformation. J Perinatol. 25(8):542-51, 2005
                                10. Bhattacharya JJ et al: Vein of galen malformations. J Neurol Neurosurg Psychiatry. 74 Suppl 1:i42-4, 2003
                                11. Halbach VV et al: Endovascular treatment of mural-type vein of Galen malformations. J Neurosurg. 89(1):74-80, 1998
                                Related Anatomy
                                Loading...
                                Related Differential Diagnoses
                                Loading...
                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Pre-Procedure

                                    • Procedure

                                      • Post-Procedure

                                        TERMINOLOGY

                                        • Definitions

                                          • Vein of Galen aneurysmal malformation (VGAM): Arteriovenous malformation (AVM) resulting in aneurysmal dilatation of vein of Galen
                                            • Rare, dramatic choroidal form of embryonic AVM
                                              • Supplied by choroidal arteries
                                              • Midline location in choroidal fissure at quadrigeminal plate/velum interpositum cisterns
                                            • Persistent median prosencephalic vein of Markowski (MPV): Precursor of vein of Galen
                                              • Vein becomes aneurysmal
                                              • Drains via falcine sinus to superior sagittal sinus
                                            • Arteriovenous (AV) fistula between deep choroidal arteries/median prosencephalic vein
                                            • Various morphologies
                                              • Choroidal type: Myriad varying-sized complex shunts from pericallosal artery/posterior cerebral artery (PCA)/anterior choroidal artery (AChA)
                                              • Mural type: Fewer shunts (PCA/collicular arteries)
                                            • Variable shunt size, number, complexity depending on type; commensurate MPV dilatation
                                            • Clinical presentation of progressively worsening cardiac failure leading to multiorgan failure
                                          • Vein of Galen aneurysmal dilatation (VGAD): Aneurysmal dilatation of "true" vein of Galen caused by adjacent brain AVM; different from VGAM
                                            • AVM localized in subpial space (supra- or infratentorially) drains into usual tributaries of vein of Galen; results in venous overload/dilatation
                                            • Vein of Galen fully developed; drains not only AVM but also adjacent brain parenchyma
                                            • Dilatation of vein of Galen variable
                                              • Depends on any outflow obstruction due to stenoses/thrombosis where vein of Galen joins straight sinus
                                            • No melting brain syndrome, no mental retardation, no jugular bulb dysmaturation
                                              • Higher risk of hemorrhage than VGAM
                                          • Vein of Galen varix (also known as Galenic varix)
                                            • No AV malformation or fistula
                                            • 2 types
                                              • Transient dilatation in neonates
                                                • Caused by cardiac failure of another etiology
                                                • Persists for few days postnatally, disappears
                                                • Asymptomatic; disappearance parallels cardiac improvement
                                              • Complex developmental venous anomalies/postthrombotic collateral circulation drain entire cerebral hemisphere into deep venous system
                                                • Causes dilatation of vein of Galen
                                                • Patients may show symptoms of venous ischemia from insufficient venous drainage
                                          • Dural arteriovenous shunts with aneurysmal dilatation of vein of Galen
                                            • Acquired dural lesions develop in wall of vein of Galen or at venous-sinus junction
                                              • Straight sinus almost always thrombosed
                                              • Most common in adults

                                        PRE-PROCEDURE

                                        • Indications

                                          • Contraindications

                                            • Pre-Procedure Imaging

                                              • Getting Started

                                                PROCEDURE

                                                • Patient Position/Location

                                                  • Procedure Steps

                                                    • Alternative Procedures/Therapies

                                                      POST-PROCEDURE

                                                      • Expected Outcome

                                                        • Things to Do

                                                          • Things to Avoid

                                                            OUTCOMES

                                                            • Complications

                                                              Selected References

                                                              1. Recinos PF et al: Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 23(1):165-77, 2012
                                                              2. Berenstein A et al: Vein of Galen Malformations in Neonates: New Management Paradigms for Improving Outcomes. Neurosurgery. Epub ahead of print, 2011
                                                              3. Rao VR et al: Pediatric aneurysms and vein of Galen malformations. J Pediatr Neurosci. 6(Suppl 1):S109-17, 2011
                                                              4. Papanagiotou P et al: Vein of galen aneurysmal malformation treated with Onyx. Arch Neurol. 66(7):906-7, 2009
                                                              5. Alvarez H et al: Vein of galen aneurysmal malformations. Neuroimaging Clin N Am. 17(2):189-206, 2007
                                                              6. Cekirge HS et al: Intrasaccular combination of metallic coils and onyx liquid embolic agent for the endovascular treatment of cerebral aneurysms. J Neurosurg. 105(5):706-12, 2006
                                                              7. Gailloud P et al: Confirmation of communication between deep venous drainage and the vein of galen after treatment of a vein of Galen aneurysmal malformation in an infant presenting with severe pulmonary hypertension. AJNR Am J Neuroradiol. 27(2):317-20, 2006
                                                              8. Lasjaunias P et al: Management of vein of Galen aneurysm malformations. Neurosurgery. 59(5):184-94, 2006
                                                              9. Gailloud P et al: Diagnosis & management of vein of Galen aneurysmal malformation. J Perinatol. 25(8):542-51, 2005
                                                              10. Bhattacharya JJ et al: Vein of galen malformations. J Neurol Neurosurg Psychiatry. 74 Suppl 1:i42-4, 2003
                                                              11. Halbach VV et al: Endovascular treatment of mural-type vein of Galen malformations. J Neurosurg. 89(1):74-80, 1998