Medical Director The Ohio State Medical Center Columbus, Ohio, United States
Case Diagnosis: Thalamic intraparenchymal hemorrhage
Case Description: A 31-year-old male with congenital Vein of Galen malformation (VoGM) and recurrent strokes was admitted following a spontaneous thalamic intraparenchymal hemorrhage with resultant right hemiparesis, inferior quadrantanopia, and severe subcortical aphasia. In the past, he has undergone multiple rounds of gamma knife radiosurgery with palliative embolization of the fistulous connections in the left ACA, right PCA, and left MCA. Despite this, he developed a repeat left thalamic IPH. He underwent repeat embolization of the fistulous connection in the right PCA, left ACA, left MCA, and left internal carotid artery. Post-operative MRI brain also revealed chronic left thalamic hematoma with small acute cortical infarcts in the left parietal and occipital lobes and small subcortical infarcts in the left parietal lobe.
Discussions: Vein of Galen malformations are uncommon and typically present in the pediatric age group as high output cardiac failure, hydrocephalus, and/or developmental delay. It is due to a cerebral arteriovenous fistula of the median prosencephalic vein (a precursor of the vein of Galen) that fails to regress and become aneurysmal. Prior to endovascular intervention, there was 100% mortality without treatment and 90% mortality following surgical treatment. Radiosurgery is typically limited to patients that are not candidates for other modalities. Despite radiosurgery, the VoGM in this patient persisted and resulted in recurrent thalamic IPH thought to be due venous hypertension.
Conclusions: We will review the Vein of Galen malformation anatomy, pathophysiology, and appropriate treatment and associated guidelines. Additionally, we will review thalamic subcortical aphasia.