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Washington University Experience | VASCULAR | Vein of Galen Aneurysmal Malformation | 1A0 Case 1 History
Case 1 History ---- The patient was a 4-day-old male baby born full term following an uncomplicated pregnancy. After birth, the neonate was noted to have a heart murmur; a workup revealed right ventricular enlargement with increased pressure, moderate tricuspid regurgitation and a patent ductus arteriosus with bidirectional flow. A bruit was also noted over the anterior fontanelle. A head ultrasound showed a Vein of Galen malformation with prominent adjacent vessels. Though he was hemodynamically stable upon arrival at SLCH, he soon developed signs of heart failure, requiring respiratory and pressor support. An MRI with MRA/MRV confirmed a large Vein of Galen malformation. The supplying vessels to this malformation were multiple and consisted of branches of the superficial temporal as well as the middle meningeal arteries, which supplied the enlarged posterior cerebral artery vessel and the medial and lateral posterior choroidal arteries, arising from the anterior and middle cerebral arteries. Calcifications were identified along the white matter and gray-white junction. Transarterial coil embolization was attempted by Interventional neuroradiology; however, during the procedure, there was complete arrest of venous blood flow, development of thrombosis with probable intracranial hemorrhage, and the procedure was aborted. A subsequent head CT showed a decrease in the gray-white matter differentiation suggesting global hypoxic damage. Clinically no reflexes were noted. After extensive conversations between the family and clinicians, it was decided to withdraw care. ---- At autopsy the weight of the fixed brain is 327 g. A 6 cm segment of the vein of Galen appeared intact and without transmural tears and contained an intravenous coil with surrounding laminated blood clot that extended into the straight sinus. The brainstem was slightly compressed from the presence of a 3x3 cm blood clot in the region of the brainstem-diencephalon. Coronal sections of the cerebral hemispheres revealed an attenuated and focally thinned cortical ribbon. The underlying periventricular white matter was attenuated and the central white matter shows patches of brown discoloration. The basal ganglia and thalami showed focal hemorrhage and cystic change. The corpus callosum was markedly thinned. The ventricular system is compressed, contained clotted blood, and was lined by dusky ependyma. Transverse sections of the brainstem showed diffuse tan-brown discoloration of both the gray and white matter. Radial sections of the cerebellum showed normal foliar architecture with tan-brown discoloration of the white matter. The cerebellar cortex is unremarkable, but the dentate nucleus shows red-brown discoloration consistent with hemorrhage. ---- In summary, gross and microscopic evaluation of the CNS showed malformation of the vein of Galen with thrombosis which also extended into the straight sinus. There was widespread recent and remote hypoxic/ischemic damage to both gray and white matter at all CNS levels. These changes likely represent the result of the hemodynamic alterations resulting from the combination of the vein of Galen malformation, secondary high output cardiac failure and, in the case of recent infarcts, recent thrombosis of the great vein.
