eMedicine Specialties > Neurology > Pediatric Neurology

Vein of Galen Malformation: Treatment & Medication

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

Treatment

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.

Medication

No specific medical therapy is available for VGAM.

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
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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].

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Further Reading

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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

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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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