Abstract
BACKGROUND: Pial arteriovenous fistulas are rare cerebrovascular malformations, predominantly occurring in the pediatric population. The spectrum of symptoms is broad, ranging from incidental findings to intracranial hemorrhage. However, accurate diagnosis and optimal treatment require, among other factors, dynamic imaging modalities and interdisciplinary management. CASE PRESENTATION: We describe a case of a 9-year-old patient presenting with acute spontaneous headache and apathy. MRI revealed a right temporal intraparenchymal hemorrhage, without signs of an underlying vascular pathology. Cerebral angiography was performed, revealing a suspected pial arteriovenous fistula. The patient was scheduled for surgical hematoma removal and resection of the pial fistula, with intraoperative angiographic control. After hematoma removal and resection of the pial fistula, intraoperative cerebral angiography revealed an additional fistula point that had not been appreciated on the initial preoperative angiography. The craniotomy was extended and the remaining fistula was resected. Final intraoperative angiography confirmed complete resection of the pial fistula. CONCLUSION: Pediatric pial arteriovenous fistulas are rare, complex, and challenging arteriovenous lesions. Accurate diagnosis and an interdisciplinary management are essential. However, consensus on the diagnostic workflow and treatment approach remains lacking. Therefore, we report our case and propose a diagnostic and therapeutic workup for ruptured vascular intracranial anomalies in children.