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Washington University Experience | VASCULAR | Vein of Galen Aneurysmal Malformation | 2A0 Case 2 History
Case 2 History ---- This patient was a 38 week-gestational age male infant born by a scheduled Cesarean section to a 31 year-old Gravida 1 Para 0 mother. The pregnancy was initially uncomplicated but, at 35 weeks, the fetus was found to have cardiomegaly and a vein of Galen malformation by ultrasound examination findings which were then confirmed by fetal MRI studies. Apgars at birth were 7 at 1 minute and 8 at 5 minutes, however shortly thereafter, he developed respiratory distress due to severe congestive heart failure and required dobutamine drip along with intubation and mechanical ventilation. Echocardiographic studies showed severe pulmonary hypertension with a dilated right ventricle. A large patent ductus arteriosus with a right to left shunt was also observed along with a severely dilated superior vena and innominate veins. A head CT scan showed a vein of Galen malformation and a straight sinus that was markedly enlarged. Also noted were enlarged jugular and sigmoid sinuses and multiple enlarged draining veins entering the vein of Galen. There was evidence of hydrocephalus and a suspicion for right frontal and parietal infarcts. A cerebral angiogram was performed which showed a large vein of Galen malformation with arterial supply from at least the posterior choroidal arteries, right anterior choroidal arteries, right middle cerebral artery and right anterior cerebral artery branches. The angiography results were communicated to the parents and discussed the possibility of proceeding with coiling the cerebral vein of Galen. Given the size and nature of his lesion, the chances of relieving his heart failure with the coiling procedure was still very low and considering the overall risk associated with interventional procedure and the poor prognosis, the parents opted not to proceed with the procedure and re-direction of care was offered and accepted. He was pronounced dead on 10/19. ---- At autopsy the weight of the unfixed brain was 290 g (normal=318 +/-106 g). The superficial leptomeningeal veins in the parieto-occipital region on the superior aspect and the straight sinus are markedly engorged. Coronal sections of the cerebral hemispheres showed a normal cerebral cortical ribbon on the left side; however, the right cerebral hemisphere showed marked variation noted within the cortical ribbon as well as the underlying white matter accompanied by significant thinning of the corpus callosum and hydrocephalus ex vacuo. ---- Subacute infarcts were seen in multiple brain regions including the right parietooccipital areas, basal ganglia, thalamus and white matter. The patient received a diagnosis of vein of Galen malformation (arteriovenous fistula) with global hypoxic/ischemic encephalopathy.
