脊髓圆锥部的髓内动静脉畸形并发同侧腰骶部的动静脉瘘

目的增强临床医师对脊髓圆锥部的髓内动静脉畸形(SAVM)并发同侧腰骶部的动静脉瘘(硬脊膜动静脉瘘和髓周动静脉瘘)的认识。方法回顾性统计分析4年来北京宣武医院共收治的6例圆锥部的SAVM并发同侧腰骶部的动静脉瘘的患者,分析其临床与影像学特征、治疗方式及预后转归。结果所有患者均在全脊髓血管造影后得到确诊。单纯手术治疗3例,先栓塞后手术1例,先手术后栓塞2例,术后症状全部改善。6例随访,未见症状加重及复发。结论早期发现、正确诊断、早期治疗是成功的关健。从头颈到骶椎完整的全脊髓造影对诊断至关重要。治疗的关键是既要完整去除圆锥部的畸形又要切断硬脊膜的血管,如硬脊膜支或根动脉对瘘口的供血,而保留正常的脊髓静脉引流。     

脊髓硬脊膜动静脉瘘的临床特点(附2例报道和222例文献分析)

目的探讨脊髓硬脊膜动静脉瘘的发病机制和临床特征,以增进对该病的认识,减少误诊误治。方法报道2例临床病例,附1例手术和病理所见。结合文献中222例报道,进行临床分析。结果脊髓硬脊膜动静脉瘘是脊髓动静脉性血管病变的一种类型,临床表现为进行性、上行性的双下肢运动、感觉、大小便功能障碍,由于该病早期的症状和体征无特异性,易造成误诊,脊髓MRI和脊髓血管造影有特征性表现。结论对急性、亚急性病程的脊髓病变患者的MRI图像认真分析,并进行选择性脊髓血管造影检查,有利于早期确诊本病。早期诊断、早期治疗可以较好地改善临床症状。     

硬脊膜动静脉瘘的临床诊断与显微手术治疗分析

目的分析和总结硬脊膜动静脉瘘的临床诊断及显微手术切除治疗经验。 方法回顾性分析2010年1月至2014年1月在平煤神马医疗集团总医院确诊并行显微手术切除治疗的硬脊膜动静脉瘘患者8例,并从临床症状、影像学资料及显微手术切除治疗效果总结了临床诊断及治疗的经验。 结果所有病例均经半椎板入路行动静脉瘘显微手术切除术,术后复查脊髓血管造影,均未发现异常的瘘口及迂曲扩张的引流静脉显影。随访24个月,其中痊愈5例,好转2例,有效率87.5%。 结论MRI是筛选硬脊膜动静脉瘘的无创手段,脊髓血管造影是确定诊断的金标准。显微手术方法简单,疗效确切,可作为治疗硬脊膜动静脉瘘的首选方法。     

2例罕见硬脊膜外动静脉瘘的诊治及误诊原因分析

目的 探讨硬脊膜外动静脉瘘的诊治方法,分析误诊原因和对策。方法 回顾性分析2017年9月至2018年12月收治的2例罕见硬脊膜外动静脉瘘的临床资料,总结临床特点,并分析其误诊原因。结果 2例均表现为进行性双下肢乏力伴大小便功能障碍,外院DSA无法确诊而转入我院。我们结合神经系统体征定位诊断,在病变定位部位采用延长脊髓血管造影时间的方法发现胸5节段硬脊膜外动静脉瘘,伴硬脊膜外静脉湖形成;运用介入栓塞供血动脉和瘘口,再行开放性手术灼闭硬脊膜外静脉湖;1例治愈,另1例因误诊2年余仅部分神经功能恢复。结论 硬脊膜外动静脉瘘是一种临床罕见的脊髓血管病,以椎管内静脉高压或脊髓压迫的形式造成脊髓功能障碍,因为临床表现缺乏特异性,容易误诊和漏诊;可通过结合神经系统体征定位、延长选择性脊髓动脉造影时间或进行脊髓主要供血动脉无关血管的造影,可提高确诊率;介入治疗和复合手术均能达到良好治疗效果,临床症状的改善程度与确诊时间呈负相关。     

硬脊膜动静脉瘘的手术治疗

目的探讨硬脊膜动静脉瘘的诊断及总结手术治疗的经验。方法回顾性分析13例经脊髓血管造影确诊的硬脊膜动静脉瘘患者的临床资料，均行手术夹闭瘘口，其中经全椎板切除入路9例，经半椎板切除入路4例。结果瘘口位于上胸段2例，中胸段3例，下胸段6例，腰段2例。全部病例手术后行脊髓血管造影复查，均未见异常瘘口及迂曲引流静脉。随访2—36个月，13例中症状基本消失、痊愈5例，症状改善、好转7例，无变化1例。结论脊髓血管造影可以准确定位瘘口位置，是诊断硬脊膜动静瘘的金标准。手术夹闭瘘口方法简单，夹闭瘘口确切可靠，效果肯定，可作为硬脊膜动静脉瘘的首选治疗方法。     

24例胸段硬脊膜动静脉瘘的DSA诊断及手术治疗

目的总结胸段硬脊膜动静脉瘘的DSA诊断经验及手术治疗体会。方法选取近5年我院经脊髓数字减影血管造影(DSA)确诊的胸段硬脊膜动静脉瘘(SDAVF)24例,回顾性分析其临床及随访资料。结果本组患者分别经全椎板入路(4例)及半椎板入路(20例),紧靠硬脊膜下电凝并切断瘘结构向脊髓表面的引流静脉,术后予以扩容、血液稀释、改善循环、减轻水肿等药物治疗,24例患者均于刀口拆线后复查造影,证实脊髓表面引流静脉未再显影,术后3个月脊髓MRI检查及6~18个月随访,大部分患者脊髓表面静脉流空影消失,水肿减退,临床症状明显改善。结论胸段硬脊膜动静脉瘘临床症状多变,查体定位不准确,需根据脊髓MRI发现异常,并行脊髓DSA确诊,瘘口多偏于一侧,经半椎板入路多可显露并切断引流静脉,是治疗本病的首选方法,早期明确诊断、准确定位并行手术治疗,是预后的关键。     

硬脊膜动静脉瘘的治疗

作从1983年1月到1996年1月共收治硬脊膜动静脉瘘24例，全部病人均表现为进行性脊髓损害。除1例(术中证实)外，均经选择性脊髓血管造影证实。9例病人治疗前后行MRI检查。24例中，单纯栓塞5例，单纯手术14例．栓塞 手术4例，未治1例。经治疗的23例病人中痊愈7例．改善12例，无变化8例．加重1例。中对硬脊膜动静脉瘘的影像学特点和治疗进行了讨论。     

显微手术联合术中血管造影治疗硬脊膜动静脉瘘

目的 探讨显微手术联合术中脊髓血管造影检查治疗脊髓硬脊膜动静脉瘘的疗效和应用价值。方法 回顾性分析四川大学华西医院神经外科2013年5月—2017年9月,采用显微手术闭塞瘘口同时行术中脊髓血管造影检查治疗的52例脊髓硬脊膜动静脉瘘患者的临床资料。结果 在术中脊髓血管造影辅助下,52例患者术中达到100%的解剖治愈率。术后第7 d 43例患者(82. 7%)的脊髓功能评分改善。术后50例患者(96. 2%)随访6个月,Aminoff评分显示28例患者(56%)治愈,18例患者(36%)脊髓功能改善,4例患者(8%)脊髓功能无变化;无症状加重者。结论 显微手术闭塞瘘口能有效治疗脊髓硬脊膜动静脉瘘,术中脊髓血管造影对准确判断是否完全闭塞瘘口和多个瘘口的存在有着极大地帮助。     

手术夹闭治疗硬脊膜动静脉瘘(附6例报道)

目的探讨显微手术夹闭硬脊膜动静脉瘘的方法和疗效。方法回顾分析6例经脊髓血管造影明确的硬脊膜动静脉瘘,采用后正中入路显微镜下探查硬脊膜动静脉瘘口,电灼硬膜上瘘口,夹闭引流静脉近端并观察手术疗效。结果术后双下肢麻木无力,大小便障碍等临床症状明显改善3例,好转2例,无变化1例。无手术死亡、截瘫及其他严重并发症。结论术前精确定位,采用显微手术直接夹闭硬脊膜动静脉瘘口,可取得良好疗效。     

经单侧椎板开窗夹闭硬脊膜动静脉瘘

目的　总结经单侧椎板开窗入路夹闭硬脊膜动静脉瘘的经验。方法　回顾性分析了 5 6例经脊髓MR和脊髓血管造影确诊的硬脊膜动静脉瘘患者经单侧椎板开窗夹闭瘘口的临床资料。结果　 5 4例患者术后行脊髓血管造影复查 ,显示瘘口全部消失。 38例患者术后 6个月行脊髓MR复查 ,显示脊髓周围的血管流空影完全消失 ,T2 像髓内高信号影消失或明显减少。 5 4例患者获随访 ,随访时间 3～ 36个月 ,2 4例症状完全消失 ,2 7例症状改善 ,3例无变化。结论　经单侧椎板开窗夹闭瘘口的手术方法是硬脊膜动静脉瘘的首选治疗方法。     

表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘

目的对表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘发病机制进行分析,预测出血因素,避免漏诊,指导治疗。方法对5例表现为蛛网膜下腔出血的高颈段硬脊膜动静脉瘘的临床资料进行总结。结果5例瘘口均位于枕大孔区～颈2节段,由椎动脉脊膜支供血．通过髓周静脉引流。所有5例引流静脉均向颅内引流,有不同程度扩张,3例伴有静脉瘤样改变。手术后复合瘘口均消火,4例治愈,1例死亡。结论高颈段硬脊膜动静脉瘘向颅内引流并伴有引流静脉扩张易出血,颅内压增高可能是诱发出血的因素。血管造影应全面,避免小部分蛛网膜下腔出血的患者造影假阴性。手术夹闭瘘口并切断近端引流静脉是可靠的治疗方法。     

Spinal dural arteriovenous fistulas—An underdiagnosed disease

Background Spinal dural arteriovenous fistulas (SDAVF) are rare and present with non–specific symptoms. The diagnosis is difficult and it is therefore conceivable that patients may not be recognized. Methods We reviewed the intake forms of patients who had been admitted to the spinal cord injury ward of a rehabilitation center in the period 1980–2004 to identify possible patients with an undiagnosed SDAVF. Clinical and radiological data were evaluated in selected cases. Results In 20 of 1429 newly admitted patients to the rehabilitation center (in 614 of whom trauma was not the cause), we restudied the CT myelograms, MRI scans or spinal angiograms and in two of these we found an undiagnosed SDAVF, and one cerebral dural arteriovenous fistula. One of these three was diagnosed with SDAVF 8 years after the admission to the rehabilitation center; the other two patients had never been diagnosed with SDAVF. In 9 patients a diagnosis of SDAVF had already been established by the time they were admitted to the spinal cord unit. In 20 other patients the admission diagnosis was a vascular lesion or 'progressive myelopathy' but appropriate radiological studies had been destroyed or had never been performed. Conclusion Our results suggest that spinal dural arteriovenous fistulas are an underdiagnosed condition.     

硬脊膜动静脉瘘(附1例临床分析)

目的探讨硬脊膜动静脉瘘(SDAVF)的病因、发病机制、临床表现、诊断及治疗。方法回顾性分析了1例患者的相关临床资料。结果本例患者以双下肢渐进性麻木、无力1年余,加重伴大小便障碍9个月为主要临床表现,脊髓MRI显示T9~L1髓内以长T2长T1为主之异常信号,超选择DSA造影可见位于入LT12椎管处一硬脊膜动静脉之瘘口,成功地进行了经单侧椎板开窗夹闭瘘口术。结论SDAVF为一具有直接的动静脉交通性病变,常伴有小型畸形团,多发生于中年男性,误诊率高,主要表现为渐进性的肢体麻木、无力及大小便障碍,MRI有助于诊断,但仍须DSA确诊。阻断连接瘘口与冠状静脉丛的引流静脉是治疗SDAVF的有效方法,显微外科手术效果可靠、复发率低,尽早地确诊和治疗是取得良好疗效的根本前提。     

硬脊膜动静脉瘘误诊分析

目的 探讨如何避免误诊硬脊膜动静脉瘘。方法 回顾分析3例误诊的硬脊膜动静脉瘘的临床表现，误诊原因及治疗结果。结果 1例误诊为腰椎间盘突出，2例误诊为前列腺肥大，不仅延误了诊断，还因误治加重了神经功能损害或导致正常器官损害。结论 仔细询问病史和查体，适时行脊髓MRI检查是避免误诊的关键。     

Spinal dural arteriovenous fistula

PURPOSE OF REVIEW: To summarize clinical key points, diagnostic features, and results of imaging and therapy of spinal dural arteriovenous fistula (SDAVF). RECENT FINDINGS: SDAVF accounts for 70% of spinal arteriovenous malformation with an annual incidence of 5-10 cases per million. At least 80% of patients are male, and more than 66% of patients are in the sixth and seventh decade of life indicating preponderance of gender and age. Thrombophilia is not a predisposing factor of disease. Clinical course is predominated by symptoms of congestive myelopathy, but subarachnoid hemorrhage may occur. Double SDAVF is a rare problem in the management of disease. Magnetic resonance imaging has replaced myelography as screening procedure. Contrast-enhanced magnetic resonance angiography and multislice computerized tomographic angiography may facilitate diagnostic procedure, however, spinal angiography is still required to confirm diagnosis. Treatment by permanent occlusion of fistula results in clinical improvement in 70% of cases. Microsurgical shunt interruption has proven secure and reliable. Endovascular shunt embolization has been established as a standardized procedure, but occlusion rates are still lower than in surgical treatment. SUMMARY: Advances have been made in diagnosis and treatment of SDAVF, but the disease is still not completely understood.     

Histological characteristics of arterialized medullary vein in spinal dural arteriovenous fistulas related with clinical findings: report of five cases

The histological features of arterialized medullary vein (MV) in spinal dural arteriovenous fistulas (SDAVF) were studied in five consecutive patients who presented with progressive congestive myelopathy. Retrograde venous filling on preoperative angiography was recognized as being severe in 3 cases and moderate in 2 cases. Direct intradural interruption of the arterialized MV was performed in all patients. The arterialized MV was sampled and examined histologically to determine the percentage of the hyperplasia of venous wall (hypertrophic ratio). Histological examination of arterialized MV showed that hypertrophic alteration of venous wall structure was due to hyperplasia of elastic fibers, ranging from 41 to 82%. Patients with angiographically severe venous hypertension tended to have a higher hypertrophic ratio than patients with moderate venous hypertension. Our observations support the clinical concept that long-standing arterial stress in the spinal venous circulation causes histological alterations of spinal vascular structure associated with the progression of venous hypertension. We suggested that possibly the histological parameter can be used for predicting neurological recovery after occlusion of the fistulas.     

寰枕交界区硬脊膜动静脉瘘的诊断与治疗(2例报告并文献复习)

目的探讨寰枕交界区硬脊膜动静脉瘘诊断与治疗。方法采用多模式影像学方法诊断,枕下后正中入路手术治疗寰枕交界区硬脊膜动静脉瘘患者2例,并结合文献进行分析。结果本组2例寰枕交界区硬脊膜动静脉瘘患者均表现为自发性蛛网膜下腔出血,均经DSA确诊,并经枕下后正中入路成功阻断瘘口后引流静脉。结论寰枕交界区硬脊膜动静脉瘘发病率低,缺乏特征性临床表现,诊断困难;多模态影像、尤其是DSA是确诊的主要方法;开颅手术切断瘘口后引流静脉是相对安全、有较高病灶闭塞率的治疗方法。