Case presentation: V.R.P., 2 days old, male, gestational age of 39 weeks and 6 days, born by c-section,
weighing 2.970kg, breech presentation, Apgar score 8 and 9. In the home postpartum
period, evolved with several seizures, which motivated the family to seek medical
attention. The initial patient assessment and the physical examination showed normal
vital signs, capillary glycemia of 76mg/dL, good general condition, intensity 4–5
on the Wong-Baker Scale, isochoric and reactive pupils, no signs of meningeal irritation
and presence of symmetric superficial and deep reflexes. Three hours later, he had
3 focal seizures with retained awareness and automatisms, which were reversed with
a loading dose of phenytoin. The transcranial doppler showed germinal matrix hemorrhage
grade 3 on the Papile Scale. On the next day, presented with 5 new seizures with automatisms,
when a loading dose of phenobarbital was used, followed by a maintenance dose. The
EEG showed no alterations and the cranial CT without contrast revealed hyperdense
content next to the brain scythe and the tentorium and between the cortical gyri's
grooves on the frontal lobe, bilaterally, as well as extrinsic compression in the
III ventricle. An external ventricular derivation catheter was placed on an urgent
basis to treat the hydrocephalus. After the procedure, the patient had clinical improvement.
The requested cranial MRI exhibited malformation at the vein of Galen territory.
Discussion: The vein of Galen aneurysmal malformation (VGAM) is a rare anomaly responsible for
less than 1% of all congenital vascular malformations. It is characterized by the
formation of multiple arteriovenous fistulas between the choroidal circulation and
the median vein of the prosencephalon, an embryonic vessel precursor of the vein of
Galen that dilates. About 94% of the cases are diagnosed in the neonatal period, the
first manifestation being heart failure and hydrocephalus. Transarterial embolization
is the treatment of choice, which can be delayed until the patient is 6 years old,
when the formation of the venous sinus is complete.
Final comments: There are few studies concerning the VGAM, in spite of its high mortality rates in
late diagnosed patients. Therefore, this report is important, since it brings information
about the condition and stimulates future research on the matter.
