eMedicine Specialties > Neurology > Pediatric Neurology

Vein of Galen Malformation: Differential Diagnoses & Workup

Author: Raj D Sheth, MD, Division Chief, Division of Pediatric Neurology, Department of Pediatrics, Nemours Alfred I duPont Hospital for Children
Contributor Information and Disclosures

Updated: Aug 20, 2008

Differential Diagnoses

Abnormal Neonatal EEG
Intracranial Hemorrhage
Arteriovenous Malformations
Mental Retardation
Cavernous Sinus Syndromes
Neonatal Seizures
Cerebral Palsy
Pseudotumor Cerebri
Cerebral Venous Thrombosis
Epilepsy in Children with Mental Retardation
Hydrocephalus

Other Problems to Be Considered

Autism/pervasive development disorder

Workup

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.

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

References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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

Contributor Information and Disclosures

Author

Raj D Sheth, MD, Division Chief, Division of Pediatric Neurology, Department of Pediatrics, Nemours Alfred I duPont Hospital for Children
Raj D Sheth, MD 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
Disclosure: Nothing to disclose.

Medical Editor

Ann M Neumeyer, MD, Clinic Director, Instructor, Departments of Neurology and Pediatrics, Massachusetts General Hospital, Harvard Medical School
Ann M Neumeyer, MD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, and Massachusetts Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Department of Pediatrics, Division of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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