Pediatric Arteriovenous Malformations of the Neck: A Case Presentation and Review of Literature

Congenital high-flow AVMs are pathologic connections between arteries and veins anywhere upstream of capillary level. These can either be direct fistulous connections or more commonly contain an intervening nidus—a convoluted network of blood vessels with poorly differentiated endothelial cells. Natural history and symptomatology largely depend on extent and location of the lesion. Venous hypertension as a result of chronic arterialization of the draining veins is a major source of early morbidity. Distal ischemia is a later manifestation. Endovascular treatment has evolved into a mainstay of treatment. Although direct arteriovenous fistulas can be cured by use of proximal occluding devices, AVMs with a nidus require infiltration with a liquid embolic agent delivered super selectively via a coaxial microcatheter-based system. Without this, all attempts at more proximal inflow control are futile. Given their convoluted and evolving angioarchitecture, AVMs readily render themselves to creativity and technical innovation during treatment. Experience and expertise with various therapeutic modalities remain the most important determinants of clinical outcomes.