Pediatric Congenital Cerebrovascular Anomalies

J Neuroimaging. 2019 Mar;29(2):165-181. doi: 10.1111/jon.12575. Epub 2018 Oct 31.


Congenital cerebrovascular anomalies in the pediatric age group are myriad with diverse etiologies. The purpose of this paper is to provide an imaging overview of congenital vascular malformations and vascular tumors, as these conditions are varied and the characteristic vascular abnormality may even suggest the underlying systemic condition in helping to guide further management. For example, the identification of an arterial anomaly such as agenesis/hypoplasia/duplication may warrant further evaluation for an associated syndrome. Recognition of other pertinent findings, such as persistent carotid-vertebrobasilar anastomoses for instance, would be particularly important for the planning of potential embolization/coiling of associated vascular malformation and/or aneurysm. Awareness of congenital dural sinus anomalies, including hypoplasia, aplasia, and or duplication, prevents the incorrect or overdiagnosis of sinus thrombosis. Moreover, multiple developmental venous anomalies in a patient may require further investigation for its familial association. In a similar context, there may be familial association with multiple cavernous malformations and children with central nervous system vascular malformations may have an association with other vascular conditions, eg, hereditary hemorrhagic telangiectasia, etc. An accurate description of vascular lesions is critical for understanding the natural history of the disease process and ultimately for guiding treatment. An important example of this includes Sturge-Weber syndrome, which is often incorrectly viewed as an arteriovenous malformation, but actually a type of venous malformation. Another example which is important to recognize includes the differentiation between an infantile hemangioma and a vascular malformation, particularly as the course of the pathology and the treatment is so different between the two.

Keywords: PHACES; arteriovenous shunts; cerebrofacial vascular metameric syndromes; infantile hemangioma; vein of Galen malformation.

Publication types

  • Review

MeSH terms

  • Central Nervous System Vascular Malformations / diagnostic imaging*
  • Cerebral Arteries / abnormalities*
  • Cerebral Arteries / diagnostic imaging
  • Cerebral Veins / abnormalities*
  • Cerebral Veins / diagnostic imaging
  • Child
  • Humans
  • Neuroimaging