Nationwide survey of pediatric intracranial arteriovenous shunts in Japan: Japanese Pediatric Arteriovenous Shunts Study (JPAS)

Abstract

OBJECTIVEThis nationwide study was organized to evaluate the clinical characteristics, especially the annual detected rate, of pediatric intracranial arteriovenous (AV) shunts such as brain AV malformations (BAVMs), pial AV fistulas (PAVFs), vein of Galen aneurysmal malformations (VGAMs), and dural AV fistulas (DAVFs) in Japan.METHODSParticipation invitation emails for the study were sent to all members of the Japanese Society for Neuroendovascular Therapy and the Japanese Society for Pediatric Neurosurgery, totaling 4123 physicians. Excel files were attached to the emails to collect clinical data on patients aged 5 years old or younger with intracranial AV shunts at each hospital in the 5-year period from 2012 to 2016.RESULTSRepresentative physicians from 209 hospitals returned Excel files containing the patient data. Thirty-four hospitals reported on 72 patients with intracranial AV shunts. Among this cohort, 24 patients (33%) had BAVMs, 22 (31%) had PAVFs, 16 (22%) had VGAMs, and 10 (14%) had DAVFs. Male predominance (70%) was observed among all types of AV shunts. Most PAVFs (73%), VGAMs (88%), and DAVFs (100%) were diagnosed in patients younger than 1 year, especially neonates, whereas most BAVMs (92%) were diagnosed in children older than 1 year. In the patients older than 1 year, BAVMs were relatively common, and VGAMs and DAVFs were rare.CONCLUSIONSThe annual detected rates of BAVMs, PAVFs, VGAMs, and DAVFs in patients 5 years old or younger were 0.0775, 0.0710, 0.0517, and 0.0323 per 100,000 persons, respectively. In neonates and infants, VGAM, DAVF, and PAVF were relatively common, but BAVMs were extremely rare.