Epidemiology of Cerebral Arteriovenous Malformations in Children
Incidence and Prevalence
1.4% in 3200 autopsies: In a large autopsy series, the overall frequency of detection of AVMs was 1.4% (46 among 3,200 unselected brain tumor cases) (3).
1.1 per 100,000/year: In another report, the annual incidence of symptomatic AVMs was 1.1 per 100,000 (6).
0.02% prevalence in children: The pediatric age group comprises 12–18% of all AVMs from major centers, and the overall prevalence in children is about 0.02% of the pediatric population (7–10).
Mean age at diagnosis 30–40 years: Most AVMs present in adulthood, with a mean age at presentation of approximately 30-40 years. About 20% of all symptomatic AVMs present before 15 years of age (11).
None: There is no sex predilection for pediatric AVMs.
Possibly more frequent in Asians: AVMs are about one-seventh as common as saccular aneurysms; they may be more frequent in Asian populations (12).
Relationships to Other Disease States and Syndromes
Hemorrhagic telangiectasia: The association of AVMs with HHT has been documented In one study, 35% of cases were associated with HHT and 23% of persons had multiple AVMs; the mean age at presentation was 35 years (6).
RASA1 mutation: The RASA1 mutation, resulting in familial AVMs and/or cutaneous capillary malformations, has been associated with symptomatic cerebral AVMs in a small number of families (13).