Matushita Hamilton, Caldas Jose Guilherme Mendes Pereira, Texeira Manoel Jacobsen
Pediatric Neurosurgery Division, Department of Neurosurgery, University of São Paulo School of Medicine, São Paulo, SP, Brazil.
Childs Nerv Syst. 2012 Feb;28(2):253-64. doi: 10.1007/s00381-011-1566-y. Epub 2011 Sep 6.
Perimedullary arteriovenous fistulas (PMAVFs) are rare spinal lesions and even more uncommon in children.
The aim of this study was to document rare occurrences of this type of arteriovenous malformation in six children treated at our institution.
The clinical data, radiological findings, and treatment in six cases of PMAVFs were reviewed. Six patients with PMAVFs were managed at our institution over a 5-year period. The patients (four girls and two boys), ranging in age from 6 to 15 years, presented with initially fluctuating, and eventually permanent and progressive, sudden-onset paraparesis, sensory disturbances, and sphincter dysfunction. The duration of symptoms before diagnosis ranged from 1 week to 13 years.
All the patients underwent magnetic resonance imaging and spinal selective angiography, which demonstrated the characteristic imaging of an arteriovenous fistula. Embolization of the arteriovenous fistula was initially attempted in three patients with successful occlusion of the fistula in two. For the remaining cases, open surgery was performed, with complete occlusion of the fistula. There was no morbidity, regardless of the treatment performed. All the patients experienced neurological improvement after treatment.
No specific clinical or radiological characteristic of PMAVFs in the pediatric population was observed when our series was compared with a general series. Early diagnosis and timing of the therapeutic intervention seemed to avoid the development of irreversible ischemic myeloradiculopathy and prevented hemorrhage. Treatment for PMAVFs is difficult to standardize because these are extremely rare lesions with different angioarchitecture configurations.
髓周动静脉瘘（PMAVF）是一种罕见的脊柱病变，在儿童中更为少见。
本研究旨在记录在我们机构接受治疗的6例儿童中这种类型动静脉畸形的罕见病例。
回顾了6例PMAVF患者的临床资料、影像学表现及治疗情况。在5年期间，我们机构共收治了6例PMAVF患者。患者（4名女孩和2名男孩）年龄在6至15岁之间，最初表现为间歇性，最终发展为持续性和进行性的突发双下肢轻瘫、感觉障碍及括约肌功能障碍。诊断前症状持续时间从1周至13年不等。
所有患者均接受了磁共振成像和脊髓选择性血管造影，显示出动静脉瘘的特征性影像学表现。3例患者最初尝试进行动静脉瘘栓塞，其中2例成功闭塞了瘘口。其余病例则进行了开放手术，瘘口完全闭塞。无论采用何种治疗方法，均未出现并发症。所有患者治疗后神经功能均有改善。
将我们的系列病例与一般病例系列进行比较时，未观察到儿童PMAVF有特定的临床或影像学特征。早期诊断和治疗干预时机似乎可避免不可逆缺血性脊髓神经根病的发生并预防出血。PMAVF的治疗难以标准化，因为这些是极为罕见的病变，具有不同的血管结构构型。
