Case of the Month May 2015, page 2

The study demonstrates cardiomegaly.  

The following grayscale and color Doppler ultrasound of the brain was then obtained:

For larger images, please click on Baby boy 2 and Baby boy 3

Coronal images demonstrate a large midline and left sided vascular malformation, moderate to severe ventriculomegaly, and parenchymal calcifications in the periventricular region.  

Next, a CT scan of the head was performed.

For larger images, please click on Baby boy 4 , Baby boy 5 and Baby boy 6 .

Axial contrast enhanced and coronal and sagittal unenhanced images reveal an arteriovenous malformation invaginating into the left lateral ventricle.  The arterial input stems from the left anterior cerebral artery with major drainage to the left transverse sinus and to anomalous vessels that course posterior to the midbrain.  Extensive ventriculomegaly and coarse periventricular calcifications are also present.

Diagnosis:  Vein of Galen Malformation.  

A vein of Galen malformation results when a fistula occurs between the deep arteries of the choroidal plexus and the median prosencephalic vein of Markowski.  Formation of a fistula causes increased blood flow through the vein of Markoski and inhibits the normal regression of this embryological structure into the vein of Galen.  This commonly leads to high output congestive heart failure, hydrocephalus, and brain atrophy as seen in our patient.  
< Previous PageVisit our Case of the Month Archive