Madsen Peter J, Lang Shih-Shan, Pisapia Jared M, Storm Phillip B, Hurst Robert W, Heuer Gregory G
Departments of Neurosurgery and.
J Neurosurg Pediatr. 2013 Oct;12(4):344-50. doi: 10.3171/2013.6.PEDS13110. Epub 2013 Aug 2.
Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature.
A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012.
Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both N-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit.
Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.
软脑膜动静脉瘘（PAVF）是一种罕见的脑血管疾病形式，在儿科人群中往往占比过高。对这组患者的临床特征和预后的研究有限。在此，作者试图通过机构病例和文献综述来更好地描述这一临床实体。
对我们机构的病例进行回顾性研究，以确定2000年至2012年间所有接受PAVF治疗的儿科患者。
确定了5例接受PAVF治疗的患者。患者诊断时的平均年龄为1.9岁，最常见的首发症状是癫痫发作和巨头症。患者主要接受栓塞治疗，3例患者需要使用氰基丙烯酸正丁酯（NBCA）胶和弹簧圈。4例患者的PAVF完全闭塞，随访时儿科总体表现类别评分为1分（n = 3）或2分（n = 1）。有1例患者因心力衰竭死亡。文献综述分析表明，就诊时年龄较小或存在脑出血（ICH）或充血性心力衰竭（CHF）可能预示预后较差。年龄较大的患者更常出现ICH，而年龄较小的患者更常出现CHF。文献中报道的大多数儿科患者接受了血管内栓塞治疗，最常见的是单独使用NBCA胶。文献中的大多数患者（65.4%）预后良好，无神经功能缺损。
软脑膜动静脉瘘是一种严重但罕见的脑血管疾病形式。就诊时患有ICH或CHF的儿科患者或非常年幼的患者预后可能较差。对这些患者的血管内治疗极大地改变了这种疾病的自然病程，但并发症和死亡率表明需要在治疗方面持续深入了解并取得进展。
