Clinical
Presentation: A 6-month-old female presented with left eye proptosis.
Imaging Findings:
CT: Enlarged vein of Galen and dilated left superior ophthalmic vein. Several large vessels were seen in the basal cistern and left sylvian fissure. Cerebral Angiogram: Vein of Galen aneurysmal malformation with multiple feeding vessels from the posterior choroidal arteries and the pericallosal arteries. Venous hypertension with retrograde drainage of the superficial cerebral veins toward the basal venous system rather than to the superior sagittal sinus. Retrograde filling of the superior ophthalmic veins, particularly on the left. MR: 3 1/2 years later: status post-coiling of the vein of Galen malformation. Absent right transverse and sigmoid sinus. Thin superior sagital sinus. Multiple tortuous collateral vessels including a large left superior ophthalmic vein.
Figure 1A: CT - vein of Galen.
Figure 1B: CT - dilated left superior ophthalmic vein.
Figure 1C: CT - collateral vessels in basal cistern.
Figure 2A: Angio - AP right common carotid.
Figure 2B: Angio - lateral right common carotid.
Figure 2C: Angio - AP left vertebral artery.
Figure 2D: Angio - lateral left vertebral artery.
Figure 3A: MR - sagittal T1 image showing vein of Galen and coiling material (arrow).
Figure 3B: MR - axial 3D time-of-flight image showing coiled vein of Galen malformation.
Figure 3C: MR - axial T2 image showing dilated left superior ophthalmic vein.
Diagnosis: Vein of Galen malformation
Discussion: The vein of Galen drains the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa. A vein of Galen malformation is due to an aneurysmal congenital malformation of the persistent embryonic prosencephalic vein of Markowski. This vein drains into the vein of Galen. The vein of Galen malformation is the most common arteriovenous malformation in neonates and usually causes high-output congestive heart failure secondary to decreased resistance and high blood flow through the shunt. The malformation may also present with developmental delay, hydrocephalus, and seizures.
Initial imaging used is cranial ultrasound, typically to localize or identify the lesion in a newborn with heart failure and a cranial bruit. An MRI or CT of the head may be obtained to define the degree of involvement. Cranial angiography is reserved for patients being considered for surgical or occlusive therapy. Over half of the patients with a vein of Galen malformation have a malformation that cannot be corrected. Patients frequently die in the neonatal period or in early infancy.
Medical therapy is used to manage the high-output cardiac failure and seizures, if present. Surgically placed ventriculoperitoneal shunts may be helpful in patients with hydrocephalus. Selective catheterization and therapeutic embolization of the feeding arteries of the vein of Galen malformation is the treatment of choice if possible. If there is no evidence of gross cerebral parenchymal damage on imaging, and medical therapy of cardiac failure fails, urgent endovascular therapy can reduce the expected almost absolute mortality without severe morbidity. Endovascular therapy can provide good outcomes even in the highest risk neonate with cardiac failure. Multiple embolizations in multiple stages are usually necessary.
The patient in this case presented with left eye proptosis secondary to an enlarged collateral superior ophthalmic vein. She was started on Lasix for high output heart failure discovered on admission and underwent three separate embolizations to her vein of Galen malformation over the next 18 months. Lasix was discontinued after 6 months. She has no known developmental delay and is meeting all milestones as a well 4-year-old child.
Landis MS. Vein of Galen malformation. CMAJ. 2006 Oct 24;175(9):1059. [Medline]
Golombek SG, Ally S, Woolf PK. A newborn with cardiac failure secondary to a large vein of Galen malformation. South Med J. 2004 May;97(5):516-8. [Medline]
Mitchell PJ, Rosenfeld JV, Dargaville P, Loughnan P, Ditchfield MR, Frawley G, Tress BM. Endovascular management of vein of Galen aneurysmal malformations presenting in the neonatal period. AJNR Am J Neuroradiol. 2001 Aug;22(7):1403-9. [Medline]
Lylyk P, Vinuela F, Dion JE, Duckwiler G, Guglielmi G, Peacock W, Martin N. Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg. 1993 Mar;78(3):438-45. [Medline]
