vein of Galen Aneurysmal Malformation in Children: Challenges of Imaging in An African setting

Abstract

Aneurysmal malformations of the vein of Galen (VGAM) are a rare congenital intracranial vascular disorder. It is known to occur in two main forms. We present two children with classic demonstrations of broadly recognized categories. It typically results in high-output congestive heart failure or may present with developmental delay, hydrocephalus and seizures. Our aim is to demonstrate the challenges of imaging and management in an African setting of a rare congenital intracranial vascular disorder presenting in older African children with infrequently recognized associations using limited imaging techniques. Computed tomography (CT) showed extensive gyriform hyperdensities at the grey-white matter junction, dilatation of a portion of the superior sagittal sinus with erosion of the inner table of the calvarium, multiple abnormal tortuous vessels in the suprasellar region, and a markedly dilated vein of Galen and ventricular system. Magnetic resonance imaging (MRI) showed a tortuous dilated pontomesencephalic vein in the quadrigeminal cistern, dilated vein of Galen and straight sinus. A network of vessels in the quadrigeminal cistern draining into dilated vein of Galen thinning of the
corpus callosum is seen. Chest radiograph and electrocardiographic chart showed evidence of cardiomegaly and cardiac failure in one patient. We review the literature and discuss the challenges of imaging and management in our African setting. The VGAM is a recognized rare anomaly, however, knowing the true prevalence and improving its management in our African environment is limited due to the challenges of available imaging technology, lack of personnel in interventional neuroradiology, and inaccessibility to care of a large poor population.