Outcomes of multimodality therapy in pediatric patients with ruptured and unruptured brain arteriovenous malformations

Background: Brain arteriovenous malformations (BAVMs) are a frequent cause of pediatric hemorrhagic stroke, which frequently results in significant morbidity and mortality. 
Objective: To analyze the results of multimodality treatment for a consecutive series of pediatric patients with ruptured and unruptured BAVMs at a single institution. 
Methods: Forty patients <18 years of age were retrospectively reviewed. Results were divided by hemorrhage status, ie, ruptured or unruptured, and the intended curative treatment modality, ie, surgical resection or stereotactic radiosurgery. 
Results: Twenty-seven patients (68%) presented with hemorrhage, and 13 patients (32%) presented without hemorrhage. Among ruptured patients, 19 (70%) underwent surgery and 8 (30%) underwent stereotactic radiosurgery. In surviving patients who presented with hemorrhage, 23 of 26 (88%) had a modified Rankin Scale (mRS) score of 0 to 2 at the last follow-up, and 24 of 26 (92%) obtained radiographic cure. For unruptured BAVMs, all 6 patients with grade I to III BAVM obtained radiographic cure and had an mRS score of 0 to 1 at the last follow-up, whereas 1 of 5 patients (20%) with grade IV and V BAVM had BAVM obliteration and a mean mRS score of 1.8 at the last follow-up. In a total of 93.6 years of follow-up from date of presentation to last clinical follow-up, there was 1 hemorrhage (1.1%/y). Of 30 patients with radiographic obliteration, 2 patients had radiographic recurrence (7% incidence). 
Conclusion: The majority of ruptured patients had an mRS score of 0 to 2 at the last follow-up and obtained radiographic cure. Unruptured patients with grade I to III BAVMs had superior outcomes compared with those with grade IV and V AVMs. Treatment of grade I to III BAVMs appears safe, and additional study is needed to determine optimal strategies for the management of unruptured grade IV and V BAVMs. Abbreviations: ARUBA, A Randomized Trial of Unruptured Brain Arteriovenous Malformations BAVM, brain arteriovenous malformation RBAS, radiosurgery-based brain arteriovenous malformation grading system mRS, modified Rankin Scale SAIVM, Scottish Audit of Intracranial Vascular Malformations SRS, stereotactic radiosurgery WFNS, World Federation of Neurosurgical Societies.