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Case Seventy Eight - Vein of Galen Malformation

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Clinical History: One-day-old child with high-flow congestive heart failure.

Findings: Large malformation seen as decreased signal on T1 and T2 weighted images. The location is extra-axial, with prominent dilatation of the Vein of Galen, the straight sinus, the confluence of sinuses, transverse sinuses, and the sigmoid sinus. Turbulent flow within the malformed vessels produces a mixed signal intensity.

MRA of the cerebral vessels display a prominent Vein of Galen with multiple arteries leading to the malformation.

Diagnosis: Vein of Galen Malformation.

Discussion: There exists two types of VOGM. Both types of malformations can present with cranial bruit, focal neurological deficit, seizure, and hemorrhage.

The first type, which this case displays, is due to single or multiple arteries (usually choroidal or quadrigeminal arteries) flowing directly into the vein of Galen, resulting in a shunt that leads to high-flow congestive heart failure. Patients usually present at birth, and often have macrocephaly with hydrocephalus.

The second type is due to a parenchymal AVM in the thalamus or midbrain, which can present as developmental delay with ocular symptoms.

References:

  1. Osbourne A. Diagnostic Neuroradiology. 1994; Mosby, Chicago. 320-329.

  2. Seidenwurm D, Berenstein A, Kowalsla H. Vein of Galen Malformation:
    Correlation of Clinical Presentation, Arteriography and MR Imaging. AJNR; 1991.

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Submitted by:
Patrick Tessman , MS III
C. F. Lanzieri, M.D.