R. Al-Shahi, J. J. Bhattacharya, D. G. Currie, V. Papanastassiou, V. Ritchie, R. C. Roberts, R. J. Sellar, C. P. Warlow, Sivms Collaborators



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Background and Purpose – The rarity of intracranial vascular malformations (IVMs) and the infrequency of their outcomes make large, prolonged cohort studies the best means to evaluate their frequency and prognosis. Methods – The Scottish Intracranial Vascular Malformation Study (SIVMS) is a prototype prospective, population-based study of adults resident in Scotland and diagnosed for the first time with an IVM after January 1, 1999. We evaluated the design of SIVMS using 2 complete years of data for adults with arteriovenous malformations ( AVMs) of the brain. Results – A collaborative network of clinicians, radiologists, and pathologists, combined with coding of hospital discharge data and death certificates, recruited a cohort distributed in proportion to the Scottish population. Coding ( with International Classification of Diseases, 10th Revision [ICD-10] codes Q28.2 and I60.8) had a sensitivity of 72% ( 95% CI, 61% to 80%) and a positive predictive value of 46% ( 95% CI, 38% to 55%) for detecting incident brain AVMs. Adults who were detected by coding alone were significantly ( P < 0.05) younger, more likely to present with hemorrhage, more frequently investigated with catheter angiography, and more likely to be treated. Adults recruited from tertiary referral centers were significantly more likely to be investigated with catheter angiography and to be treated. Using catheter angiography as a diagnostic requirement for brain AVMs significantly biases the cohort toward younger adults presenting with hemorrhage and receiving treatment. Conclusions-Population-based studies of IVM frequency and prognosis should use multiple overlapping sources of case ascertainment, and such studies of brain AVMs should not require catheter angiography to be the diagnostic standard.