eMedicine Specialties > Neurology > Pediatric Neurology
Vein of Galen Malformation
Updated: Aug 20, 2008
Introduction
Background
The vein of Galen is located under the cerebral hemispheres and drains the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa. The vein of Galen aneurysmal malformation is a choroidal type of arteriovenous malformation involving the vein of Galen forerunner and is distinct from an arteriovenous malformation with venous drainage into a dilated, but already formed, vein of Galen.
Aneurysmal malformations of the vein of Galen (VGAM) typically result in high-output congestive heart failure or may present with developmental delay, hydrocephalus, and seizures.1
Pathophysiology
Vein of Galen malformation (VGAM) results from an aneurysmal malformation with an arteriovenous shunting of blood. The congenital malformation develops during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski; thus, VGAM is actually a misnomer. The vein of Markowski actually drains into the vein of Galen.
VGAM usually causes high-output heart failure in the newborn resulting from the decreased resistance and high blood flow in the lesion. Associated findings include cerebral ischemic changes such as strokes or steal phenomena that result in progressive hemiparesis. Hemorrhage from the malformation can occur, although this is not a common finding. Finally, the malformation may result in mass effects, causing progressive neurological impairment. Alternatively, the malformation may cause obstruction of the cerebrospinal fluid (CSF) outflow and result in hydrocephalus.2
Frequency
United States
The incidence of the vein of Galen malformation is unknown.
International
The incidence of the vein of Galen malformation is unknown.
Mortality/Morbidity
- Infants often die if the high-output congestive heart failure is the presenting feature.
- Macrocephaly usually improves following shunting for hydrocephalus.
Race
VGAM occurs in all races.
Sex
Boys and girls are affected equally.
Age
VGAM is a congenital malformation; therefore, it may present at birth or in early childhood.
Clinical
History
- Direct communication between a cerebral artery and a cerebral vein results from a congenital vascular malformation. The vein of Galen abnormality is the most frequent arteriovenous malformation in neonates.
- It commonly presents in the neonatal period, although it may present later, in early childhood. Typically, in the neonatal period, VGAM presents with congestive heart failure, a cranial bruit, and marked carotid pulses.
Physical
- Congestive heart failure
- Neonates may present with tachypnea, respiratory distress, and cyanosis.
- They often require ventilatory support and institution of aggressive management of heart failure.
- Hydrocephalus
- Hydrocephalus may be the presenting feature in older infants.
- A cause should be sought in neonates with macrocephaly.
- Infants may have hydrocephalus, in which case prominent scalp veins or "sunset" eye findings are noted.
- Developmental delay: Signs of hydrocephalus and congestive heart failure should be looked for in infants with developmental delay.
- In early childhood, symptoms include headache, convulsive seizures, hydrocephalus, and cardiac failure.
Causes
Although any vessel may be affected, the vein of Galen is the most frequently affected. Congenital malformation develops during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski.
More on Vein of Galen Malformation |
 Overview: Vein of Galen Malformation |
| Differential Diagnoses & Workup: Vein of Galen Malformation |
| Treatment & Medication: Vein of Galen Malformation |
| Follow-up: Vein of Galen Malformation |
| Multimedia: Vein of Galen Malformation |
| References |
| Next Page » |
References
Golombek SG, Ally S, Woolf PK. A newborn with cardiac failure secondary to a large vein of Galen malformation. South Med J. May 2004;97(5):516-8. [Medline].
Incorpora G, Pavone P, Platania N, et al. Vein of Galen malformation and infantile spasms. J Child Neurol. Mar 1999;14(3):196-8. [Medline].
Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery. Nov 2006;59(5 Suppl 3):S184-94; discussion S3-13. [Medline].
Lylyk P, Vinuela F, Dion JE, et al. Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg. Mar 1993;78(3):438-45. [Medline].
Moriarity JL, Steinberg GK. Surgical obliteration for vein of Galen malformation: a case report. Surg Neurol. Oct 1995;44(4):365-9; discussion 369-70. [Medline].
Iizuka Y, Kakihara T, Suzuki M, Komura S, Azusawa H. Endovascular remodeling technique for vein of Galen aneurysmal malformations--angiographic confirmation of a connection between the median prosencephalic vein and the deep venous system. J Neurosurg Pediatrics. Jan 2008;1(1):75-8. [Medline].
Feletti A, Denaro L, Marton E, d'Avella D, Longatti P. Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review. Minim Invasive Neurosurg. Oct 2007;50(5):285-91. [Medline].
De Koning TJ, Gooskens R, Veenhoven R, et al. Arteriovenous malformation of the vein of Galen in three neonates: emphasis on associated early ischaemic brain damage. Eur J Pediatr. 156(3):228-9. [Medline].
Porzionato A, Macchi V, Parenti A, De Caro R. Vein of Galen aneurysm: anatomical study of an adult autopsy case. Clin Anat. Sep 2004;17(6):458-62. [Medline].
Squires LA, Thomas S, Betz BW, Cottingham S. Vein of Galen malformation with diencephalic syndrome: a clinical pathologic report. J Child Neurol. Nov 1998;13(11):575-7. [Medline].
Further Reading
Keywords
vein of Galen, vein of Galen malformation, VGAM, aneurysmal malformations, vein of Markowski, high-output heart failure, high-output congestive heart failure, cerebral ischemic changes, strokes, steal phenomena, progressive hemiparesis, obstruction of the cerebrospinal fluid, hydrocephalus, congenital malformation, seizures, developmental delay
