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Neurology > Pediatric Neurology
Vein of Galen Malformation
Article Last Updated: Aug 20, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Raj D Sheth, MD, Division Chief, Division of Pediatric Neurology, Department of Pediatrics, Nemours Alfred I duPont Hospital for Children
Raj D Sheth is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society
Editors: Ann M Neumeyer, MD, Clinic Director, Instructor, Departments of Neurology and Pediatrics, Massachusetts General Hospital, Harvard Medical School; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic; Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital; Amy Kao, MD, Assistant Professor, Department of Neurology, Department of Pediatrics, Division of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital
Author and Editor Disclosure
Synonyms and related 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
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.
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.
Abnormal Neonatal EEG
Arteriovenous Malformations
Cavernous Sinus Syndromes
Cerebral Palsy
Cerebral Venous Thrombosis
Epilepsy in Children with Mental Retardation
Hydrocephalus
Intracranial Hemorrhage
Mental Retardation
Neonatal Seizures
Pseudotumor Cerebri
Other Problems to be Considered
Autism/pervasive development disorder
Imaging Studies
- Cranial ultrasound
- This will help to localize or identify the lesion.
- Doppler studies can help further to understand the hemodynamics of the lesion.
- Cranial MRI and/or CT scan with and without contrast administration
- These studies will help confirm the diagnosis and define the degree of involvement. Imaging studies in infants will also help determine whether the patient has accompanying hydrocephalus (see Media files 1-4).
- MR angiography can help to delineate the vascular supply (see Media file 5).
- Cranial angiography: In patients being considered for surgery or for occlusive therapy, cranial angiography is required to define the extent of aneurysmal dilatation and details for arterial feeders (see Media file 6).
- Cardiac ultrasound: This study may be indicated to assess left ventricular function.
Other Tests
- ECG to look for evidence of congestive heart failure
Histologic Findings
Lesion shows thin capillaries or veins connecting directly to the arteries.
Medical Care
- Cardiac management of high-output heart failure is essential. A pediatric cardiologist should be consulted to manage high-output failure, if present. Often patients need to be intubated.
- Seizures should be managed with antiepileptic medications. Usually, neonate patients are given phenobarbital and phenytoin. Please see the articles Complex Partial Seizures and Neonatal Seizures.
- Head circumference measurements should be obtained regularly and monitored carefully to detect hydrocephalus.
- Assessment of the child's development is an important part of medical care.
Surgical Care
- Neurosurgical procedures to relieve hydrocephalus are important.3 A ventriculoperitoneal shunt may be required in some infants.4, 5, 6, 7
- Vaso-occlusive therapy, including selective catheterization and therapeutic embolization of feeding arteries in the vein of Galen malformation, can be performed (see Media file 7).
- Only a small number of patients with arteriovenous aneurysm have been reported.
- Death usually results from cardiac failure or cerebral decompression.
- A few patients have been cured by surgical ligation of the arterial feeders from the posterior and middle cerebral arteries and plication of the aneurysm.
- Some malformations thrombose spontaneously.
Consultations
- Neurosurgical evaluation should be obtained urgently to assess the extent and location of the VGAM and to treat hydrocephalus.
- A cardiologist should evaluate the patient for congestive heart failure.
- A neonatologist should help coordinate care and decide whether intubation and ventilatory support are required.
- The infant should be assessed in a facility where interventional neuroradiologist expertise is available; should embolization be an option, this specialist can help with specific care.
Diet
Diet is determined by the presence of congestive heart failure.
Activity
Patients are usually very sick and activity is inherently limited.
No specific medical therapy is available for VGAM.
Further Inpatient Care
- Infants who undergo surgical ligation or selective embolization should be reimaged to assess results of the therapeutic intervention.
- Head circumference measurements should be obtained in patients who undergo ventriculoperitoneal shunt to treat hydrocephalus.
Further Outpatient Care
- Patient should be monitored for the development of hydrocephalus.
Transfer
- Patients diagnosed with VGAM should be transferred to a tertiary neonatal intensive care unit where a pediatric neurologist, an interventional neuroradiologist, and a neurosurgeon are available for management of the infant.
Complications
- Over half the patients with VGAM have a malformation that cannot be corrected. Patients frequently die in the neonatal period or in early infancy.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Ernesto Tiznado-Garcia, MD, to the development and writing of this article.
| Media file 1:
Cerebral MRI showing large flow void in the central region with enlarged straight sinus. |
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Media type: MRI
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| Media file 2:
Coronal MRI of head showing large central vein of Galen malformation with moderate degree of hydrocephalus. |
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Media type: MRI
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| Media file 3:
Cranial MRI showing flow void in the sagittal plain and drainage to the straight and transverse sinuses. |
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Media type: MRI
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| Media file 4:
Sagittal cerebral MRI with gadolinium showing the relationship of a vein of Galen malformation to the corpus callosum. |
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Media type: MRI
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| Media file 5:
MRI venogram showing vein of Galen malformation with draining veins. |
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Media type: MRI
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| Media file 6:
Venogram showing the draining vasculature for the vein of Galen malformation. |
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Media type: X-RAY
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| Media file 7:
Skull radiograph showing coils that have been placed during an intravascular embolization of a vein of Galen malformation. Note the ventriculoperitoneal shunt catheter in the anterior head region to relieve hydrocephalus. |
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Media type: X-RAY
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- 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].
Vein of Galen Malformation excerpt Article Last Updated: Aug 20, 2008
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