脊髓动静脉畸形血管内栓塞治疗

目的：经血管内栓塞治疗脊髓动静脉畸形（ＡＶＭｓ）。方法：本组１８例脊髓ＡＶＭｓ，１４例采用干燥硬脑膜粒栓塞，２例采用α－氰基丙烯酸正丁酯（ＮＢＣＡ）胶粘合剂经微导管超选择性栓塞，另２例用３个０真丝线段栓塞。结果：病灶完全栓塞１５例，栓塞８０％～９０％３例。肢体瘫痪症状明显改善１６例，其中２例有手术切除史者症状未改善，平均随访３２个月，有１４例症状完全恢复正常，２例多支供血者经再次栓塞治疗后症状改善，另２例无变化。结论：血管内治疗脊髓ＡＶＭｓ优于手术治疗，应首先采用此法     

脊髓动静脉畸形、硬脊膜动静脉瘘、硬脊膜外动静脉瘘与脊髓周围动静脉瘘的鉴别

正脊髓血管病比较少见,但是脊髓动静脉畸形(spinal cord arteriovenous marformation,SAVM)、硬脊膜动静脉瘘(spinal dural arteriovenous fistula,SDAVF)、硬脊膜外动静脉瘘(spinal epidural arteriovenous fistula,SEDAVF)、脊髓周围动静脉瘘(spinal perimedullary arteriovenous fistula,SPAVF)四种类型,诊断容易混淆,也容易误诊。这四种脊髓血     

脊髓血管畸形合并胸腔血管畸形

脊髓血管畸形的临床发病率较低,仅为颅内血管畸形或脊髓新生物的1/10左右.本病可能与其它组织的血管异常同时存在,如皮肤血管痣(Cobb综合征),皮肤、粘膜病变,和Klipple-Trenaunay-Weber综合征等.本文报告的与胸腔血管畸形同时存在的脊髓血管畸形,我们尚未见诸报道.本文还结合文献就脊髓血管畸形导致脊髓功能损害的病理生理作了阐述.     

Quantification of intracerebral steal in patients with arteriovenous malformation

Eleven patients with angiographically and/or pathologically proved arteriovenous malformations (AVMs) were studied using dynamic, single-photon-emission computed tomography (DSPECT). Quantification of regional cerebral blood flow in structurally normal areas remote from the AVM disclosed areas of decreased flow compared with normal controls in eight of 11 patients examined. Areas of hypoperfusion correlated with altered function as manifested by epileptogenic foci and impaired cognitive function. Dynamic, single-photon-emission computed tomography provides a noninvasive technique to monitor quantitatively hemodynamic changes associated with AVMs. Our findings suggest that such changes are present in the majority of patients with AVMs and that they may be clinically significant. The potential application of regional cerebral blood flow imaging by DSPECT in the management of patients with AVMs is discussed.     

Intramedullary spinal arteriovenous malformation in a boy of familial cerebral cavernous hemangioma

Object The coexistence of spinal arteriovenous malformation (AVM) and a familial cerebral cavernous hemangioma (CCH) is extremely rare. Methods A 9-year-old boy suddenly developed severe paraplegia and urinary dysfunction. Spinal magnetic resonance imaging (MRI) scan revealed a cervical and upper thoracic intramedullary lesion. Due to acute neurological dysfunction, the patient underwent emergency surgical exploration. An intramedullary vascular lesion was found and excised. Pathologically, AVM was noted. After the surgery, the boy was ambulatory with left lower limb stiffness. MRI scan of the brain revealed multiple cerebral cavernous hemangioma. Symptomatic multiple CCH in his mother and grandmother were also noted. Conclusions We concluded that the presence of spinal AVM should be suspected if the patient with familial CCH develops the signs of space-occupying lesion of the spinal cord, facilitating early diagnosis of the spinal AVM.     

脑动静脉畸形合并出血相关影响因素分析

目的 通过全脑DSA造影检测脑动静脉畸形血管构筑特征,分析探讨脑动静脉畸形合并出血的相关影响因素.方法 回顾性分析珠江医院神经外科收治的199例脑动静脉畸形患者的影像学资料,其中出血组107例,非出血组92例.采用单因素和多因素Logistic回归分析方法,分析畸形血管团大小、位置,供血动脉数目、位置,引流静脉数目、引流方向,合并动脉瘤数目、大小、与动脉瘤的位置关系,有无合并瘤样改变等因素与畸形血管团破裂出血的关系.结果 单因素分析表明微型(0.5～1.0 cm)和小型(1.0～2.0 cm)畸形血管团、深部畸形血管团、深部供血动脉、1～2支引流静脉、深部引流静脉、合并多个动脉瘤、合并小动脉瘤(＜5 mm)和位于供血动脉端动脉瘤是畸形血管团破裂出血的危险因素,合并瘤样变则降低出血的风险,供血动脉数目与出血无关.多因素Logistic回归分析发现畸形血管团破裂出血独立危险因素是微型和小型畸形血管团,深部引流静脉及合并供血动脉端动脉瘤.结论 微型、小型畸形血管团,深部引流静脉,供血动脉端动脉瘤与畸形血管团破裂出血密切相关.

Abstract：

Objective To explore the influencing factors of bleeding in patients with cerebral arteriovenous malformation (AVM) through observing the angioarchitecture of AVM with cerebral DSA.Methods The clinical and imaging data of 199 patients with AVM, including 107 with bleeding and 92without bleeding, were retrospectively analyzed. The relationships between bleeding and such factors as the site and size of masses, the type and site of supplying arteries, the number and pathway of venous drainage, having vascular tumor-like changes or not, the relation between AVM and aneurysm in terms of location, and the number and size of concurrent aneurysms were determined by univariate analyses and Binary logistic regression with SPSS13.0. Results Univariate analysis indicated that such factors as micro-sized (0.5-1.0 cra) and small-sized (1.0-2.0 cra) masses ofAVM, AVM underdeep location,having exclusively deep draining veins, fewer draining veins and deep feeding arteries, concurrent small aneurysms (＜5 mm), aneurysms at the end of the blood-supply artery were positively correlated to hemorrhagic presentation. The number of feeding arteries and the number of concurrent aneurysms were not correlated with hemorrhagic presentation. Having vascular tumor-like changes can decreased the risk of bleeding. When stepwise multiple logistic regression analysis was employed, only micro-sized and small-sized masses of AVM, having deep feeding arteries, and aneurysms at the end of the blood-supply artery were dependent predictors of hemorrhagic presentation. Conclusion Micro-sized and small-sized masses of AVM, having deep draining veins and aneurysms at the end of the blood-supply artery are the most powerful risk predictors for hemorrhagic AVM presentation.     

Cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions

OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: Cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, Israel. PATIENTS: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, Arnold-Chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.     

Comparison of an animal model of arteriovenous malformation with human arteriovenous malformation

This study assessed the blood flow and histological changes of an animal model of arteriovenous malformation (AVM) over 84 days in 71 rats, and compared the histological findings to 17 specimens of human AVM. Carotid–jugular fistula blood flow positively correlated with time. The maximum flow rate occurred at 42 days, at which time the nidus was considered mature and was histologically similar to human AVMs. Morphological similarities between the model and human AVM vessels included heterogeneously thickened walls, splitting of the elastic lamina, thickened endothelial layers, endothelial cushions, lack of tight junctions, loss of endothelial continuity, endothelial–subendothelial adherent junctions, and luminally directed filopodia. These findings support the theory that vascular changes in human AVMs are secondary to increased flow and provide a basis for using this model in studies of AVMs.     

De novo thalamic arteriovenous malformation in a boy with a brainstem cavernous malformation

Brain arteriovenous malformations (bAVMs) have long been considered to be congenital, developing between the third and eighth weeks of embryogenesis. However, cases reporting their de novo formation suggest that these lesions can develop after birth and have challenged this concept. We present a case of a 6-year-old boy with a history of a brainstem cavernous malformation diagnosed after birth who later developed a de novo bAVM. The de novo occurrence of this bAVM distant to the site of the cavernous malformation and a prior negative catheter angiography contributes to the uncertainty of the dynamics and pathophysiology of bAVMs.     

Application of fluorescein sodium videoangiography in surgery for spinal arteriovenous malformation

We present our recent experience with fluorescein sodium videoangiography (FLVA) in the intra-operative evaluation of a patient with conus medullaris arteriovenous malformation (AVM). To our knowledge this is the first report in the literature of use of FLVA in the surgery of spinal AVM. Intra-operative FLVA was done to identify an early filling vessel and to obliterate the site of fistulous connection. This was correlated and confirmed with simultaneous indocyanine green videoangiography (ICGVA). The conus and cauda equina roots could be appreciated and manipulated in relation to this fluorescence. Obliteration was confirmed with FLVA and correlated with ICGVA. There was no untoward reaction to the dye injection. We conclude that FLVA is a useful adjunct in the surgical treatment of conus medullaris AVMs since it is a real time, noninvasive, radiation-free, easily reproducible technique allowing surgical manipulation through the operating oculars with simultaneous visualization of surrounding critical structures.     

Acute spinal cord infarction: vascular steal in arteriovenous malformation.

Central nervous system arteriovenous malformations typically present with chronic neurologic impairment. An 8-year-old boy presented with acute spinal cord infarction associated with a spinal arteriovenous malformation. Vascular steal phenomenon suggested by spinal angiography happens to underly the pathogenic mechanism.     

Disturbances of sexual potency in patients with basilar impression and Arnold-Chiari malformation.

Sexual impotence is common in patients with basilar impression and/or Arnold-Chiari malformation. There is no evidence of hypogonadism and testicular biopsy is usually normal. An autonomic disturbance is postulated.     

Acute flank pain, an unusual first symptom of a spinal arteriovenous malformation.

This case report describes a 46-year-old man whose first symptom was an attack of acute flank pain, followed by the gradual onset neurological symptoms. We demonstrated a small nidus and serpentine signal-void area in the spinal cord by MRI and diagnosed a glomus type of spinal arteriovenous malformation (AVM). Flank pain is a rare initial finding in an adult with spinal AVM. In cases with acute flank pain, neurologists should consider spinal AVM as a differential diagnosis.     

Interhemispheric transfer of language in patients with left frontal cerebral arteriovenous malformation

Cerebral arteriovenous malformations (AVMs) are frequently evaluated before therapeutic embolization by superselective injection of anesthetics into individual arterial branches so as to determine whether permanent occlusion would affect eloquent function. In Experiment 1, we used this adaptation of the Wada procedure to study three right-handed adult patients with left frontal cerebral AVMs by injecting vessels in Wernicke's and Broca's areas, respectively, and assessing language functions. The results showed that superselective testing in the inferior division of the left MCA in all three patients produced a dense Wernicke's aphasia. Injections into the left frontal regions, however, resulted in right paresis in all patients, but no language deficits including no loss of fluency. In Experiment 2, Patient 2 underwent fMRI activation for spontaneous word-list generation using multi-slice echo planar BOLD techniques at 1.5 Tesla. A voxel-by-voxel comparison of rest vs activation for each task was performed with a Z-score threshold of 2.5 SD for activated voxels. There was activation in the right hemisphere in the insula, frontal operculum pars opercularis, and inferior frontal gyrus, an area homologous to Broca's area in the left hemisphere. There was also activation in the left hemisphere in the Rolandic region, but language function was unaffected during Wada testing in this area. These data suggested that features of expressive language were no longer controlled by the left frontal lobe where the AVM was located, and provided new evidence for interhemispheric re-organization under conditions of chronic neurovascular disease.     

Spontaneous spinal epidural hematoma secondary to extradural arteriovenous malformation in a child: a case-based update

Introduction

The aim of this study was to report a rare pediatric case of spontaneous spinal epidural hematoma (SSEH) mimicking Guillain-Barré syndrome (GBS), secondary to an epidural arteriovenous malformation (AVM). Furthermore, a case-based update and insight into the entity is attempted.

Methods

An 8-year-old male presented with progressing severe lower limb weakness and no traumatic history. Presentation was mimicking GBS with ascending symptoms. Magnetic resonance (MR) scan revealed a dorsal epidural mass, extending from C6–C7 to T2, compressing the spinal cord. Emergency laminoplasties and surgical evacuation of the hematoma were performed. An up-to-date review of reported SSEH cases in children was conducted, with emphasis on underlying vascular malformations (epidural AVMs in particular). Pathogenesis, predisposing factors, imaging, diagnosis, treatment and outcome are discussed.

Results

The hematoma was successfully evacuated. A vascular membrane on the dura was peeled off and sent for histopathology. There was no evidence of intradural vascular penetration. The patient improved postoperatively and was able to walk with support 7 months later. Histology revealed closely packed thin-walled angiomatous structures with wide lumens (filled with red blood cells) with walls composed of collagen and smooth muscle fibers, findings consistent with AVM.

Conclusions

Non-traumatic SSEH is rare in the pediatric population. Although vascular malformations are suspected, they are extremely rarely identified histopathologically. This case represents one of the very few reports of pediatric SSEH caused by a histologically proven, purely epidural AVM. High index of clinical suspicion and low threshold for MR can lead to timely diagnosis and prompt treatment with good functional outcome.