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(Stroke. 2009;40:1980.)
© 2009 American Heart Association, Inc.

Original Contributions

The Presentation and Clinical Course of Intracranial Developmental Venous Anomalies in Adults

A Systematic Review and Prospective, Population-Based Study

Jennifer M.L. Hon, BA; Jo J. Bhattacharya, FRCR; Carl E. Counsell, MRCP; Vakis Papanastassiou, FRCS(SN); Vaughn Ritchie, MB; Richard C. Roberts, FRCP; Robin J. Sellar, FRCR; Charles P. Warlow, FRCP; Rustam Al-Shahi Salman, FRCP(Edin) on behalf of the SIVMS Collaborators ^*

From the Division of Clinical Neurosciences (J.M.L.H., R.J.S., C.P.W., R.A.-S.S.), University of Edinburgh, Western General Hospital, Edinburgh, UK; the Institute of Neurological Sciences (J.J.B., V.P.), Southern General Hospital, Glasgow, UK; the Department of Neurology (C.E.C.), Aberdeen Royal Infirmary, Aberdeen, UK; Fauldhouse Health Centre (V.R.), Fauldhouse, Edinburgh, UK; and the Department of Neurology (R.C.R.), Ninewells Hospital and Medical School, Dundee, UK.

Correspondence to Rustam Al-Shahi Salman, FRCP(Edin), Bramwell Dott Building, Division of Clinical Neurosciences, Western General Hospital, Edinburgh, EH4 2XU, UK. E-mail Rustam.Al-Shahi{at}ed.ac.uk

Background and Purpose— Reported risks of hemorrhage from intracranial developmental venous anomalies (DVAs) vary, so we investigated this in a systematic review and population-based study.

Methods— We systematically reviewed the literature (Ovid Medline and Embase to November 7, 2007) and selected studies of 20 participants with 1 DVA(s) that described their clinical presentation and/or their clinical course over a specified follow-up period. We also identified every adult first diagnosed with a DVA in Scotland from 1999 to 2003 and followed them in a prospective, population-based study.

Results— Of 2068 articles detected by the literature search, 15 met our inclusion criteria and described clinical presentation, 8 of which also described the clinical course of DVAs. In the 15 studies of 714 people first presenting with a DVA, 61% were incidental findings, the mode of presentation was unclear in 23%, 6% presented with nonhemorrhagic focal neurological deficit, 6% had caused symptomatic hemorrhage, 4% were associated with epileptic seizure, and <1% were associated with infarction. In studies of the clinical course of 422 people with a DVA, the hemorrhage rate after first presentation ranged from 0% to 1.28% per year. In the population-based study of 93 adults with DVAs, 98% were incidental, 1% presented with symptomatic hemorrhage, and 1% presented with an infarct, but there were no symptomatic hemorrhages or infarcts in 492 person-years of follow-up (0% per person-year; 95% CI, 0% to 0.7%).

Conclusions— Intracranial DVAs have a benign presentation and clinical course.

Key Words: hemorrhagic • intracranial developmental venous anomaly • stroke • vascular malformations