Neurovascular disease and syndromes: Diagnosis and therapy in children

Handb Clin Neurol. 2021;176:305-323. doi: 10.1016/B978-0-444-64034-5.00015-8.


The pediatric neurovascular disease runs the chronologic spectrum with dramatic changes in the presentation, evaluation, and treatment from the prenatal, perinatal, and infant periods through childhood and adolescence. These diseases are often dynamic throughout this period and the dynamic continues throughout life. There are four major categories: high-flow arteriovenous shunting lesions, arterial aneurysms, low-flow vascular lesions, and vascular occlusive disease. The high-flow lesions can be subdivided into a vein of Galen malformation, non-Galenic arteriovenous fistula, dural sinus malformations and fistula, and arteriovenous malformation. Low-flow vascular lesions include cerebral cavernous malformation, developmental venous anomaly, and capillary telangiectasia. The cerebrovascular occlusive disease can be divided between arterial occlusive disease and cerebral venous sinus thrombosis. The presentation of each of these entities can be very similar, especially in younger children; however, imaging and laboratory analysis can establish the diagnosis leading to the most appropriate therapy. A multidisciplinary team, dedicated to treating pediatric cerebrovascular disease, is important in delivering the best outcomes in these complex diseases. Given the relative rarity of pediatric presentation of cerebrovascular disease, many apply adult concepts to children. A better understanding of the diseases and their difference from adults makes a critical difference in selecting the correct approach.

Keywords: AV fistula; AVM; Cavernous malformation; Pediatric aneurysm; Pediatric stroke; Vein of Galen; Venous thrombosis.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Arteriovenous Fistula*
  • Arteriovenous Malformations*
  • Child
  • Cranial Sinuses
  • Humans
  • Infant
  • Intracranial Arteriovenous Malformations*
  • Syndrome