ORIGINAL ARTICLE Surgical strategies for management of pediatric arteriovenous malformation rupture: the role of initial decompressive craniectomy Melissa A. LoPresti 1,2 & Eric A. Goethe 1,2 & Sandi Lam 3,4 Received: 13 September 2019 /Accepted: 2 January 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020 Abstract Introduction Arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children, which can result in elevated intracranial pressure (ICP) and cerebral edema. We sought to explore the role of initial decompressive craniectomy at time of rupture, followed by interval surgical AVM resection, compared to treatment with initial resection, in clinical outcomes and recovery in children. Methods A retrospective chart review was conducted examining patients age 0–18 with AVM rupture between 2005 and 2018 who underwent resection for ruptured AVM either initially at presentation or underwent initial decompressive craniectomy followed by interval AVM resection. Clinical, radiographic, surgical, and outcome data were examined. Primary outcomes measured included functional status, AVM obliteration rate, AVM recurrence/residual, and re-hemorrhage. Results Thirty-six cases were included; 28 (77.8%) underwent initial AVM resection, and 7 (19.4%) underwent initial decompressive craniectomy with interval resection. The mean time between craniectomy and resection was 66.9 days (SD 59.3). Patients undergoing initial decompressive craniectomy with interval resection were younger (mean age 6.1 vs. 9.8 years, p = 0.05) and had a higher mean hematoma volume (52.9 vs. 22.2 mL, p = 0.01), mean midline shift (5.1 vs. 2.1 mm, p = 0.01), and presence of cisternal effacement (p = 0.01). There were no statistically significant associations between surgical strategy and postoperative outcomes, including complications, radiographic outcomes, complete resection, residual, recurrence, and functional outcomes. Those treated by initial craniectomy followed by interval resection were associated with undergoing additional procedures. Conclusions Children presenting with AVM rupture who require emergent decompression may safely undergo emergent craniectomy with interval AVM resection and cranioplasty without additional risk of morbidity or mortality. This is reasonable in those with elevated intracranial pressure. This strategy may provide time for initial recovery and allow for natural degradation of the hematoma enhancing the plane for interval AVM resection, perhaps improving outcomes. Keywords Pediatric arteriovenous malformation . Arteriovenous malformation rupture . Decompressive craniectomy . Arteriovenous malformation resection Abbreviations AVM Arteriovenous malformation ICH Intracranial hemorrhage GCS Glasgow Coma Scale ICP Intracranial pressure Introduction Arteriovenous malformations (AVMs) are responsible for up to half of intracranial hemorrhages (ICHs) seen in children [3, 8, 14]. Rupture occurs in up to 87% of pediatric AVMs [3, 6, 9, 16] and can be catastrophic, resulting in severe neurological sequelae and mortality rates as high as 25% [6, 8, 10]. The ultimate goal of treatment is obliteration of the AVM for cure [6, 14, 18]. Surgical resection of ruptured AVMs * Sandi Lam slam@luriechildrens.org 1 Department of Neurosurgery, Baylor College of Medicine, Houston, TX, USA 2 Division of Neurosurgery, Texas Children’s Hospital, Houston, TX, USA 3 Ann and Robert H Lurie Children’s Hospital, Chicago, IL, USA 4 Department of Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA Child's Nervous System https://doi.org/10.1007/s00381-020-04501-0