Etter Manina M, Guzman Raphael, Psychogios Marios-Nikos, Soleman Jehuda
Department of Neurosurgery, University Hospital of Basel, Spitalstrasse 21, 4031, Basel, Switzerland.
Division of Pediatric Neurosurgery, University Children's Hospital of Basel, Basel, Switzerland.
Childs Nerv Syst. 2025 Mar 17;41(1):132. doi: 10.1007/s00381-025-06784-7.
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.
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.
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.
软脑膜动静脉瘘是一种罕见的脑血管畸形，主要发生于儿童群体。其症状范围广泛，从偶然发现到颅内出血不等。然而，准确诊断和最佳治疗除其他因素外，还需要动态成像模式和多学科管理。
我们描述了一例9岁患者，表现为急性自发性头痛和淡漠。磁共振成像（MRI）显示右侧颞叶脑实质内出血，无潜在血管病变迹象。进行了脑血管造影，发现疑似软脑膜动静脉瘘。该患者计划接受手术清除血肿并切除软脑膜瘘，术中进行血管造影控制。在清除血肿并切除软脑膜瘘后，术中脑血管造影显示一个在术前初始血管造影中未发现的额外瘘点。扩大开颅范围并切除剩余的瘘。最终术中血管造影证实软脑膜瘘已完全切除。
儿童软脑膜动静脉瘘是罕见、复杂且具有挑战性的动静脉病变。准确诊断和多学科管理至关重要。然而，在诊断流程和治疗方法上仍缺乏共识。因此，我们报告我们的病例，并提出儿童颅内血管破裂性异常的诊断和治疗方案。
