Arteriovenous malformation of the vein of Galen in the pediatric patient: a case report

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.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
18 September 2023