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。     

Intracranial dural fistula as a cause of diffuse MR enhancement of the cervical spinal cord

Spinal MR findings are reported in a patient with progressive myelopathy and intracranial dural arteriovenous fistula draining into spinal veins. Associated with previously reported abnormalities on T1 weighted and T2 weighted images, postcontrast T1 weighted images disclosed diffuse intense enhancement of the cervical cord itself. This enhancement decreased after endovascular treatment.     

Dural arteriovenous fistulae causing pseudotumour cerebri syndrome in an elderly man.

The clinical presentation of dural arteriovenous fistulae (DAVF) is dependent on their location and the nature of their venous drainage. The latter plays a critical part in determining whether or not the fistula gives rise to intracranial hypertension, which is present in only a minority of cases. We present a case of the pseudotumour cerebri syndrome in an elderly man with bilateral intracranial DAVF supplied by the occipital arteries. Cerebral angiography was required for definitive diagnosis, and to characterise the abnormal venous drainage. The pathophysiology of intracranial hypertension in DAVF is discussed.     

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。     

硬脑膜动-静脉瘘合并髓静脉扩张的临床及影像学表现(附1例报告及文献复习)

目的报道1例硬脑膜动-静脉瘘(DAVF)合并髓静脉扩张病例的临床资料,探讨DAVF合并髓静脉扩张的临床及影像学表现。方法回顾性收集1例DAVF合并髓静脉扩张患者的临床及影像学资料,并结合文献复习进行分析。结果患者以症状性癫痫起病。头颅MRI表现为脑白质内迂曲、扩张血管影,并继发性脑梗死和脑水肿;全脑数字减影血管造影(DSA)提示右侧颈内动脉造影静脉期上矢状窦前1/3不显影,静脉晚期颅内多发髓静脉迂曲增粗,经室管膜下静脉-大脑内静脉回流,右侧颈外动脉造影示DAVF形成,大脑上静脉逆向回流,左侧颈内动脉造影示左侧发育性静脉畸形。经血管内介入栓塞DAVF治疗后患者病情改善。结论DAVF合并髓静脉扩张少见,可继发脑实质病变进而出现神经功能缺损症状与体征;影像学表现为头颅MRI提示髓静脉扩张涉及的疾病谱较多,需仔细鉴别;MRI和DSA联合检查能更好评价脑实质和血管情况。     

Hypointensity of draining veins on susceptibility-weighted magnetic resonance images might indicate normal venous flow and a lower risk of intracerebral hemorrhage in patients with intracranial arteriovenous shunt(s)

Patients with intracranial arteriovenous shunt(s) have a risk of intracerebral hemorrhage (ICH). We investigated the signal intensity of draining veins on susceptibility-weighted imaging (SWI) and the status of venous drainage shown by digital subtraction angiography (DSA). We then evaluated whether the signal intensity of draining veins on SWI is related to normal venous flow (NVF) and/or ICH. We analyzed SWI and DSA in 10 consecutive patients with intracranial arteriovenous shunt(s). Opacification of draining veins in the normal venous phase by DSA was judged as NVF. We evaluated the relationship between the intensity of draining veins on SWI and the presence of NVF before and after treatment. The relationship between the intensity of draining veins on SWI and the presence of ICH surrounding the draining veins was also evaluated. Of 10 patients with untreated arteriovenous shunt(s), two had arteriovenous malformation and eight had a dural arteriovenous fistula with cortical venous reflux. We analyzed 26 draining veins before treatment. In preoperative analysis, draining veins with hypointensity were significantly more likely to show NVF than were draining veins with isointensity or hyperintensity (45.5% vs. 0.0%, P = 0.007). While 69.2% of the areas surrounding draining veins with isointensity or hyperintensity showed ICH, no veins with hypointensity showed ICH (P = 0.011, odds ratio 0.036; 95% confidence interval 0.0017–0.80). In conclusion, draining veins with hypointensity on SWI may contain NVF, despite arteriovenous shunting. The areas surrounding these veins might have a lower risk of ICH because of less venous hypertension.     

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。     

Spinal dural arteriovenous fistulae: exacerbation after myelography with metrizamide

Metrizamide dorsal myelography was performed in two patients with minor to moderate sensorimotor paraparesis. Direct and indirect myelographic signs of spinal arteriovenous aneurysm were seen and spinal cord angiography showed thoracic dural arteriovenous fistulae (AVF) in both cases. Within 24 hours following myelography, clear neurological worsening occurred, associated with cephalalgia, nausea and transient diplopia in one case, leading to paraplegia in a few days. Paraplegia was complete six months after surgery in one case, and had resolved after embolization of fistula in the other patient. The mechanism of neurological worsening may include: substraction of cerebrospinal fluid, sitting position during and after myelography and local increase of metrizamide concentration secondary to impaired resorption caused by the fistula. Water-soluble myelography is of invaluable aid in the diagnosis of dural AVF and must be followed by early angiography, thus allowing prompt therapeutic embolization.     

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。     

Conus perimedullary arteriovenous fistula in a child: unusual angio-architectural features and pathophysiology

INTRODUCTION: The perimedullary arteriovenous fistulas are located on the pial surface and are usually supplied by spinal medullary arteries, that is, either by the anterior or posterior spinal arteries, with no intervening nidus between the feeder arteries and the venous drainage. The clinical findings are, more commonly, caused by progressive radiculomedullary ischemic processes secondary to steal vascular mechanism. As the vascular supply to the spinal cord and to the arteriovenous fistulas (AVF) is not shared with one another, the vascular steal phenomenon cannot be implicated in this case's physiopathology. Most probably, the mass effect caused by the giant venous dilatation was the pathophysiological mechanism involved in this lesion CASE REPORT: The authors describe the case of a 6-year-old girl with an intradural ventral arteriovenous fistula, with a giant venous dilatation, fed directly by L2 and L3 radiculomedullary arteries at the conus medullaris. There was no arterial supply to the fistula from the anterior or posterior spinal arteries. Selective spinal angiography showed an arteriovenous fistula supplied directly by two radiculomedullary arteries, with a large draining vein caudally. Interposing the arterial and venous vessels was a giant venous aneurysmal dilatation located ventral to the conus medullaris and extending from L3 to T6. The patient was successfully treated by a surgical approach through a laminotomy from L3 to T11. CONCLUSION: The type IV-C spinal arteriovenous malformations or perimedullary AVFs are rare lesions predominately described at the conus medullaris with various types of angio-architecture and controversial treatment.     

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。     

Dural arteriovenous fistulas with intracranial hemorrhage: diagnostic and therapeutic aspects

Seven patients presented with intracranial hemorrhage due to arteriovenous dural fistula. Six patients showed intracerebral hemorrhage combined with subdural hematoma and intraventricular hemorrhage in one case respectively, and one patient had infratentorial subarachnoid hemorrhage. Location of the fistulae was frontobasal (n=2), tentorium (n=2), transverse sinus (n=2), and superior sagittal sinus (n=1). Angiography revealed reflux into cortical veins in all cases. Therapy was surgery in both cases with fistula of the anterior cranial fossa with good results. An endovascular intraarterial therapy was performed in a case with circumscribed fistula of the superior sagittal sinus, this patient developed a second dural fistula during follow-up. Two patients with tentorial fistulae had primary endovascular treatment complicated by infarction of both thalami in one case and a recurrence of the fistula in the other. In the last case the fistula was closed by surgery. Out of two patients with widespread fistulae of the transverse sinus one made a good clinical recovery and the other remained unchanged. In the first case definite closure of a remnant of the fistula was refused, in the second no further therapy was recommended.     

吲哚菁绿术中荧光造影在脊髓硬脊膜动静脉瘘手术中的应用

目的 探引哚菁绿术中荧光造影术在脊髓硬脊膜动静脉瘘手术中的作用.方法 9例脊髓硬脊膜动静咏瘘患者,均经脊髓DSA确诊,行后正中全椎板切开,术中吲哚菁绿荧光造影明确供血动脉、引流静脉及瘘口,夹闭瘘口并选择性切除静脉血管畸形.结果 畸形血管位于颈段1例、胸段4例、胸腰段2例、腰段1例、胸腰骶段1例;介入栓塞不充分后转手术1例,介入栓塞微导管难以到位转手术8例;术后MRI显示髓周异常迂曲畸形血管消失,脊髓缺血水肿好转.术后症状消失2例,改善6例,无变化1例.结论 吲哚菁绿术中荧光造影能够明确供血动脉、引流静脉及瘘口情况,有效地提高了脊髓硬脊膜动静脉瘘手术的疗效.

Abstract：

Objective To evaluate the clinical significance of intraoperative indocyanine green (ICG)videoangiography in surgical management of spinal dural ateriovenots fistulae (dAVFs).Method In this retrospective analysis we examined nine cases of dAVFs, diagnosed by complete spinal angiography,in which laminoplasty were performed through posterior approach.An operating microscope - integrated light source containing infrared excitation light illuminated the operating field and was used to visualize an intravenous bolus of ICG.The locations of fistulae, feeding arteries and draininig veins were identified and compared before and after surgical obhteration by intraoperative ICG videoangiography.Results In the nine cases the dAVFs involved one cervical cord, four thoracic cord,two thoracic lumbar cord, one lumbar cord and one thoracic and lumbosacral cord.One of them used to take an unsuccessful endovascular embolization, while the rest of them were given the operation right after diagnosed by the spinal angiography.Microscope-based ICG videoangiography identified the fistulous point(s),feeding arteries and draining veins in all nine cases,as confirmed by postoperative MRI which showed complete obliteration of the dAVFs with improved spinal blood supply and reduced spinal cord edema.After the operation the clini cal symptoms were nearly disappered in two cases, improved in six cases, and present no obvious changes in one case.Conclusions Intraoerative ICG videoangiography provides real -time information about the precise location of spinal dAVFs,the feeding arteries and the draining veins, as well as additional feeding aiteries unrevealed by the preoperative spinal angiography and residue pathologic blood vessels during the operation, which efficiently improves the surgical outcomes and prognosis.     

Dural arteriovenous fistula with spinal perimedullary venous drainage

The purpose of this study was to investigate the characteristics of six patients with dural arteriovenous fistula (DAVF) with drainage directly into the perimedullary venous system. In five patients with subarachnoid hemorrhage (SAH), cerebral angiography revealed a DAVF with spinal venous drainage located at the petrosal sinus in one, at the tentorium in one, and at the craniocervical junction in four. In the patient with myelopathy, angiographic exploration began with a spinal angiogram. Bilateral vertebral angiography initially failed to demonstrate the fistula, and a tentorial DAVF was established with carotid artery angiography. Patients had no myelopathy when the venous drainage was limited to the cervical cord; myelopathy was present when the venous drainage descended toward the conus medullaris. Diagnosis of a DAVF presenting with myelopathy is more challenging than of those presenting with SAH.     

颅内硬脑膜动静脉瘘引起严重脊髓症状(3例报告)

目的 :结合 3例硬脑膜动静脉瘘 (IDAVF)伴脊髓表面引流的影像学、临床表现、病程特点 ,探讨引起脊髓症状的原因。方法 :分析 3例IDAVF伴脊髓表面引流的影像学特点、临床表现、病程发展 ,结合文献加以分析和讨论。结果 :临床症状主要表现为颅内症状者 1例 ,脊髓症状者 2例。结论 :IDAVF伴脊髓表面引流的临床表现与脊髓根静脉的引流出路有关。硬脑膜动静脉瘘伴脊髓症状的诊断较困难 ,特别是仅有脊髓症状者。年轻人中慢性脊髓病变 ,急性加重时应注意颅内血管造影可做出诊断     

Intracranial dural arteriovenous fistula draining into spinal cord veins: case report

We present an usual case of intracranial dural arteriovenous fistula with perimedullary and spinal cord venous plexus drainage and discuss its etiological, physiopathological, diagnostic and therapeutic aspects.     

Diffusion-Weighted Magnetic Resonance Imaging of Spontaneous Venous Infarction and Cortical Hemorrhage Complicating Dural Arteriovenous Fistula

Dural arteriovenous fistula (DAVF) is an abnormal arteriovenous shunt that occurs in the dura matter within or near a dural sinus. The clinical manifestations vary. The authors report herein a rare case of type III DAVF presenting with coexisting intracranial hemorrhage (ICH) and venous infarction evidenced by computed tomography (CT) and diffusion-weighted magnetic resonance imaging. Conventional angiography proved that the right middle meningeal artery and the occipital artery supplied the DAVF, with direct drainage into the cortical veins and superior sagittal sinus.     

A 68-year-old man of dural arteriovenous fistula at the cranio-cervical junction with dysesthesia ascending from his both toes]

We reported a 68-year-old man of dural arteriovenous fistula at the cranio-cervical junction with dysesthesia ascending from his both toes. He recognized dysesthesia at his both toes 10 months previously. Thereafter dysesthesia ascended to his girdle which was stronger as far as his girdle and gait disturbance developed. Somatosensory evoked potential (SEP) revealed delayed central conduction time. Cervical MRI showed a swelling of the spinal cord and intramedullary hyperintense lesion from the C2 to C7 level on the T2-weighted image. Moreover flow void behind the mudulla oblongata on the T2-weighted MRI was outstanding. Angiogram through right ascending pharyngeal artery revealed enlarged and tortuous anterior and posterior spinal veins at the early arterial phase. We diagnosed as dural arteriovenous fistula (AVF) and conducted intraarterial embolization. After treatment, the swelling and hyperintense lesion of the cervical spinal cord improved on MRI, and flow void behind medulla oblongata was extinguished. Gait disturbance also improved. We think that the valves of veins in the spinal cord are responsible for the tendency of higher venous pressure in outer circumference, which results in the symptom dominating in the lower extremities. We recommend that dural AVF at the cranio-cervical junction should be considered as a differential diagnosis in case with the similar clinical course to our case.     

Spinal extradural arteriovenous fistulas with retrograde intradural venous drainage: Diagnostic features in digital subtraction angiography and time-resolved magnetic resonance angiography

Spinal extradural arteriovenous fistulas (AVFs) may be more difficult to prospectively identify than dural AVFs because they are less common than dural AVFs. The primary purpose was to further characterize the diagnostic imaging of spinal extradural AVFs with intradural retrograde venous drainage. The magnetic resonance (MR) imaging and angiographic results of 23 patients with suspected spinal dural AVFs were analyzed in order to distinguish dural and extradural AVFs. The diagnostic accuracy of MR angiography was retrospectively compared between dural and extradural AVFs. All 23 patients showed high intensity in the spinal cord on T2-weighted MR images. Eighteen out of 23 patients were diagnosed with dural AVFs, while the remaining 5 were diagnosed with extradural AVFs by angiography. Extradural AVFs were fed by a branch of the segmental artery to the vertebral body, characterized by a fistula located in the ventral extradural space, and drained retrogradely via an epidural venous pouch into intradural veins. The segmental artery was localized within 1 vertebral level using MRA in 12 out of 18 patients (67%) with dural AVFs and in 1 out of 5 patients (20%) with extradural AVFs (p = 0.09). The reasons behind the lower accuracy was mainly the image misinterpretation. Congestion of the spinal cord in spinal extradural AVFs with intradural retrograde venous drainage was similar to that in dural AVFs, whereas its angioarchitecture differed from that of dural AVFs. A clearer understanding of the imaging features of extradural AVFs is important for improving the diagnostic accuracy and clarifying treatment targets.     

Surgical treatment of spinal dural arteriovenous fistulas

Increased understanding of the epidemiology, pathophysiology, and anatomy of vascular lesions affecting the spinal cord over the past 2 decades has permitted identification of subdivisions based on distinct biological features. The dural arteriovenous fistula (AVF) is the most common type of spinal vascular malformation and the most amenable to treatment. Venous congestion, the cause of myelopathy in these lesions, is effectively treated by interruption of the medullary vein as it enters the subarachnoid space between the dural nidus and the coronal venous plexus. Embolization of an untreated dural AVF in an acutely deteriorating patient stabilizes the neurological deficit and provides adequate time for definitive surgical treatment. The outcome after treatment of dural AVFs depends not only on the type and location of the lesion but also on the preoperative neurologic function. Patients who are ambulatory before treatment are usually ambulatory after treatment. Optimal outcome depends on early diagnosis and intervention.