脊髓血管畸形的临床特征研究

目的:分析研究脊髓血管畸形的临床特征。方法:收集６２例ＭＲＩ、ＤＳＡ和(或)手术病理证实的脊髓血管畸形患者。结果;髓内隐匿型ＡＶＭ无明显的性别差异,余各类型男性多于女性;髓周ＡＶＭ、髓内ＡＶＦ青少年多见,硬膜型ＡＶＦ多见中老年;髓内ＡＶＭ多位于颈髓及胸腰髓,ＡＶＦ多位于胸腰段区域;髓内ＡＶＭ急性起病多见,ＡＶＦ多慢性起病,进行性加重。结论:根据临床症状、起病特征可初步判断病变类型,争取早期正确诊断、早期治疗。     

A case of cervical myelopathy due to dural arteriovenous fistula at the craniocervical junction]

A 70-year-old woman noted paresthesia ascending from both legs to her thighs 27 months previously. She also suffered from urinary urgency and incontinence. Thereafter, weakness in both legs developed and gradually became worse. At the time of admission, a neurological examination revealed diffuse atrophy and mild spasticity in all four extremities, bilateral mild weakness in both upper extremities, and severe weakness in both lower extremities. Her superficial sensation was moderately impaired below the Th 3 level on her right side, and below the Th 4 level on her left side along with a mildly decreased sense of vibration in her left leg. Marked hyperreflexia in all four extremities and bilateral pathological reflexes were also observed. Pollakisurea, urinary incontinence and constipation were also present. Cervical MRI showed a swelling of the spinal cord at the C3 to C7 levels. Inside the spinal cord, low signal intensity lesions were seen on the T1-weighted MRI, and high signal intensity lesions were observed on the T2-weighted MRI, and the rim of the cervical cord was also enhanced by gadolinium-DTPA. MR angiography revealed enlarged and tortuous vessels at the craniocervical junction, thus suggesting the presence of a dural arteriovenous fistula (AVF). Vertebral arteriography demonstrated abnormal vessels at the spinomedullary junction supplied by the right vertebral artery, which drained into the anterior and posterior spinal veins. After surgically treating the dural AVF, the swelling of the spinal cord, the abnormal signals on MRI, and the clinical symptoms all markedly improved. Although most of the spinal dural AVF were located at the thoracic and lumbar levels, the present case was considered to be a very rare case of dural AVF, since it was located at the craniocervical junction and thus led to the development of cervical myelopathy.     

Venous manifestations of spinal arteriovenous fistulas

Spinal dural AVFs, the most common type of spinal arteriovenous malformations, are symptomatic because of venous hypertension and congestion. This has been referred to as venous congestive myelopathy. The typical MRI findings that reflect venous congestive myelopathy include peripheral T2 hypointensity that outlines a T2 hyperintensity within a swollen spinal cord. Enlarged perimedullary vessels are typically present. Contrast-enhanced MRA has become instrumental in localizing the site of these fistulas. Spinal epidural AVFs and the perimedullary spinal cord AVFs may also present with a congestive myelopathy and have similar findings on MRI. Angiography remains the gold standard for characterization of the angioarchitecture of spinal vascular malformations. [figure: see text] Multidisciplinary treatment planning is mandatory and requires knowledge of the natural history of these vascular lesions.     

Intracranial dural arteriovenous fistulas with or without cerebral sinus thrombosis: analysis of 69 patients

OBJECTIVES: To compare the characteristics of dural arteriovenous fistulas (AVFs) with or without cerebral sinus thrombosis (CST), and to analyse the determinants of aggressive manifestations in patients with dural AVF. METHODS: We investigated 69 patients aged 51.4 (SD 15) years who were diagnosed as having dural AVF. According to the location of the lesion and venous drainage pattern, dural AVF was classified into three sites (cavernous sinus, large sinus, and other) and five types (by Cognard's method). Aggressive manifestations of dural AVF were defined as intracranial haemorrhage, venous infarction, seizure, altered mental status, and intracranial hypertension. The diagnosis of CST was based on cerebral angiography. Logistic regression methods were used to analyse the determinants of aggressive manifestation in patients with dural AVF. RESULTS: CST was found in 39% of the patients with dural AVF. It was located at almost either the sinus around the dural AVF or the downstream venous flow pathways of the dural AVF. There was no significant difference with regard to sex, location, or type of dural AVF between patients with dural AVF with and without CST. The location "other sinuses" and the type of dural AVF "IIb/IIa+b/III/IV/V" were significantly related to aggressive manifestations of dural AVF (odds ratio 19 (p = 0.001) and 5.63 (p = 0.033), respectively). Presence of CST in patients with dural AVF had an odds ratio of 4.25 (p = 0.12) for development of aggressive manifestations. CONCLUSIONS: CST affects two fifths of patients with dural AVF. The location and type of dural AVF are major determinants of aggressive manifestations in patients with dural AVF.     

A case of spinal dural arteriovenous fistula draining to the ventral coronal venous plexus]

Here we report a case of spinal dural areteriovenous fistula (AVF) draining to the anterior spinal vein. An 80-year-old female presented with progressive weakness of lower extremities. MRI showed spinal enlargement at the Th10 to L1 with high intensity signals on T2-weighted image and multiple flow voids on the dorsal and ventral surface of the spinal cord. Angiogram of the left L2 lumbar artery demonstrated a hairpin-shaped vessel with ascending and descending limbs, mimicking radiculomedullary artery. Oblique view angiogram of the left L2 lumbar artery showed that radiculomedullary vein drained to the dilated anterior spinal vein, which then drained cranially and caudally on the anterior and posterior surface of the spinal cord. The patient underwent T9-L2 laminectomy. Several large tortuous dilated veins in the subarachnoid space were found. Examination of the inner surface of the dura revealed an arterialized vein that began at the level of L2 and coursed superiorly. The arterialized vein was coagulated and interrupted. The postoperative angiogram demonstrated the obliteration of the fistula. Postoperative MRI returned to normal with complete disappearance of T2 high signal, cord enlargement. In most spinal dural AVF, the venous drainage is predominantly upward on the posterior surface of the spinal cord. The spinal dural AVF draining to the anterior spinal vein is atypical, and cause difficulty in differentiating the anterior spinal artery from the anterior spinal vein. Oblique view angiogram may be helpful to differentiate the anterior spinal vein from anterior spinal artery.     

Venous congestive myelopathy: Three autopsy cases showing a variety of clinicopathologic features

We describe three patients with progressive myelopathy, in whom autopsy revealed spinal cord pathology compatible with that of venous congestive myelopathy (VCM) associated with dural arteriovenous fistula (AVF), formerly known as angiodysgenetic necrotizing myelopathy (Foix–Alajournine syndrome). In these three patients, common symptoms were gait disturbance and sensory disturbance of the extremities, and these symptoms slowly worsened. The clinical diagnoses varied and included spinal cord intramedullary tumor, cervical spondylosis and multiple sclerosis. At autopsy, all the patients showed enlarged, tortuous venous vessels on the dorsal surfaces of the spinal cord at the affected levels. In the affected spinal cord parenchyma, necrotic lesions manifested by various degrees of neuronal loss and gliosis, with increased numbers of hyalinized vessels, were evident. The presence or absence of associated spinal dural AVF could not be identified histopathologically. Even with the help of modern neurological examination methods, early and accurate clinical diagnosis of VCM is sometimes difficult. When encountering patients with progressive myelopathy, VCM, although recognized as rare, should be considered as an important differential diagnosis.     

Delayed Dural Arteriovenous Fistula after Microvascular Decompression for Hemifacial Spasm

Dural arteriovenous fistula (AVF) is very rare, acquired lesion that may present with intracranial hemorrhage or neurological deficits. The etiology is not completely understood but dural AVF often has been associated with thrombosis of the involved dural sinuses. To our knowledge, this is the first well documented intracranial hemorrhage case caused by dural AVF following microvascular decompression for hemifacial spasm. A 49-year-old male patient had left microvascular decompression of anterior inferior cerebellar artery via retrosigmoid suboccipital craniotomy. The patient was in good condition without any residual spasm or surgery-related complications. However, after 10 months, he suffered sudden onset of amnesia and dysarthria. Computed tomography and magnetic resonance imaging revealed the presence of dural AVF around the left transverse-sigmoid sinus. The dural AVF was treated with Onyx® (ev3) embolization. At the one-year follow up visit, there were no evidence of recurrence and morbidity related to dural AVF and its treatment. This case confirms that the acquired etiology of dural AVF may be associated with retrosigmoid suboccipital craniotomy for hemifacial spasm, even though it is an extremely consequence of this procedure.     

Endovascular Therapy of a Craniocervical Pial AVF Fed by the Anterior Spinal Artery

Though pial arteriovenous fistulae (PAVF) are an uncommon cerebrovascular disorder, their presentation with subarachnoid hemorrhage (SAH) is not rare. PAVF near the craniocervical junction are rare and may have a worse outcome. These fistulae are often fed from either the carotid and/or the vertebrobasilar systems, but are rarely fed by the anterior spinal artery. We report the case of a young man presenting with SAH. Cerebral angiography revealed 2 AVF, a symptomatic PAVF located at the craniocervical junction and fed from the anterior spinal artery and incidental dural AVF (DAVF) originate from the left occipital and middle meningeal arteries. These fistulae were treated with different endovascular techniques, including Onyx and platinum coil embolization into the feeding arteries of the DAVF and PAVF, respectively.     

A case of spinal dural AVF treated by endovascular embolization]

Here we report a case of spinal dural arteriovenous fistula(AVF) treated by endovascular embolization. A 58-year-old female presented with progressive intermittent claudication and numbness of the lower extremities. MRI showed swelling of the spinal cord with intramedullary high signal intensity on T2-weighted image and intramedullary enhancement, suggested spinal cord myelopathy. Myelography demonstrated the dilated serpentine vessels in the subarachnoid space and focal filling defect. Angiography showed spinal dural AVF fed by bilateral lateral sacral artery. The draining vein was posterior spinal vein. Endovascular embolization using liquid material was performed under general anesthesia. The injection of glue included the distal feeding artery, the shunt itself and the initial part of draining vein. A complete cure was achieved, with a normal postoperative angiogram. MRI returned to normal with complete disappearance of T2 high signal, cord enlargement and enhancement by contrast medium. It was suggested that venous congestion induced the transient spinal ischemia, manifested as intermittent claudication. Endovascular embolization using liquid material was safe and quite effective for spinal dural AVF.     

Nerve root prolapse into a spinal arachnoid cyst—An unusual cause of radiculopathy

Arachnoid cysts are rare lesions of the spine and can present with myelopathy, radiculopathy, local pain or a combination of these symptoms. Nerve root prolapse into an arachnoid cyst causing radiculopathy has not been reported before. We report a nerve root prolapse into a spinal arachnoid cyst presenting clinically as radiculopathy. An 18-year-old female patient presented with mid-back pain, right anterior thigh pain and hip flexor weakness. Magnetic resonance imaging (MRI) and computerized tomography (CT) myelography revealed an arachnoid cyst at T12-L1 level on the right side. At surgery, a nerve root was seen prolapsing into an extradural arachnoid cyst. The nerve root was replaced back into dural sac and the dural defect closed. At 20 months of follow-up, the patient continues to be asymptomatic with no evidence of recurrence on imaging. Replacing the prolapsed nerve root into the dural sac with meticulous closure of the dural defect could lead to good clinical outcome. We propose a modification to the popular classification of these lesions to better rationalize their surgical management. Classification of extradural arachnoid spinal cysts (Nabors's type 1) should be based on the presence or absence of dural communication. Sacral meningoceles (Nabors’ type 1B) should be excluded from the classification as they have free communication with the thecal sac and are not true spinal cysts.     

Venous congestive myelopathy of the cervical spinal cord: An autopsy case showing a rapidly progressive clinical course

We report a rapidly progressive myelopathy in a 74‐year‐old Japanese man who was admitted to our hospital with a 4‐month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord. At autopsy, ischemic changes were observed in the upper‐to‐middle cervical cord segments, with so‐called arterialized veins in the subarachnoid space. No neoplastic lesions were found within or outside the brain and spinal cord. These pathological findings were essentially those of venous congestive myelopathy (VCM) associated with dural arteriovenous fistulae (AVF), formerly known as Foix–Alajouanine syndrome. VCM associated with dural AVF, which is now considered to be treatable in the early stages, is rare found in the cervical spinal cord. The present autopsy case, with MRI findings, provides further information that might be useful for recognition and diagnosis.     

Atypical spinal dural arteriovenous fistula with supply from the lateral sacral artery.

We report a dural arteriovenous fistula (AVF) that developed at a site on the midline dorsal surface of the dura mater that had been damaged by repeated lumbar punctures. A 61-year-old male patient had undergone repeated lumbar punctures and discectomy for severe lumbago 40 years before the present admission. After surgery, the lumbago symptoms resolved. However, 30 years after the operation, he started to experience dysaesthesia, motor weakness in both legs, and urinary disturbance. Physical examination revealed bilateral leg weakness, diminished deep tendon reflexes in the patellar and Achilles tendons bilaterally, and decreased superficial sensation below L1. Magnetic resonance imaging revealed swelling with intramedullary high intensity and multiple flow voids around the conus and spinal cord on T(2)-weighted images, and adhesive arachnoiditis. Spinal angiography revealed an AVF between the left lateral sacral artery and the S1 radicular vein at the site of the previous operation. Surgery was conducted to carry out excision of the dural AVF at the shunting point, the arterialized intradural vein, and lysis of the arachnoiditis. This case of dural AVF may have been caused by repeated lumbar punctures.     

Dural arteriovenous malformations of the skull base

Dural arteriovenous malformations (AVMs) are interesting lesions, which can present as diagnostic and therapeutic challenges. Such AVMs were initially described as located solely in the dural venous sinuses, but during the last decade researchers have discovered them in other locations such as the tentorium and foramen magnum. It is understandable that they can develop in other locations because numerous arteriovenous shunts normally occur all over the dura. Early reports emphasize that they are congenital; however, some investigators have proposed that they are acquired lesions arising after sinus thrombosis and trauma. They also have variable clinical features. From 1975 to 2000 we treated 400 AVMs, of which 46 were dural. Of these 46 dural AVMs, 18 could be classified as skull base AVMs. We restricted the designation of skull base AVMs to those AVMs located in the tentorium or adjacent to bones of the skull base. Two of these AVMs actually had their vascular nidus located in the bone. The common dural sinus AVMs were excluded from this category. The presenting clinical features of these skull base AVMs ranged from headache and pulsatile tinnitus to intracranial hemorrhage. They were treated by a combination of endovascular and microsurgical treatment. The majority of patients had a good clinical outcome. Skull base dural AVMs are an interesting clinical entity. Their clinical course, treatment and final outcome are variable depending on their location. They should be managed by a multidisciplinary approach.     

A Case of Dural Arteriovenous Fistula of Superior Sagittal Sinus after Tamoxifen Treatment for Breast Cancer

We are reporting an unusual case of dural arteriovenous fistula (AVF) of the superior sagittal sinus (SSS) after tamoxifen treatment for breast cancer. A 30-year-old female arrived at the emergency room with a sudden headache and left sided weakness and sensory loss. In her past medical history, she was diagnosed with breast cancer 1 year prior, and subsequently underwent a breast conserving mastectomy with whole breast radiation and adjuvant chemotherapy with tamoxifen. At the time of admission, computed tomography showed a small acute intracerebral hemorrhage at the right parietal cortex, and magnetic resonance imaging showed that a dural AVF at the SSS with a prominent and tortuous venous enhancement along the centrum semiovale was present. Cerebral angiography showed that the dural AVF at the mid-portion of the SSS with meningeal arterial feeding vessels entering the wall of the SSS, then draining through the dilated cortical veins. Our patient had no signs of active malignancy or any abnormalities in her coagulation profile, so it can be concluded that the tamoxifen was the likely cause of the SSS thrombosis and dural AVF. The dural AVF was treated by an endovascular coil embolization for the arterialized segment of the SSS. The patient dramatically recovered favorably from left side motor and sensory deficit. The best clinical approach is to screen potential patients of tamoxifen hormonal therapy and educate them on the sign and symptoms of life threatening thromboembolic events while taking tamoxifen.     

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.     

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.     

A case of visual perseveration attack caused by transverse-sigmoid sinus dural arteriovenous fistula]

A case of visual perseveration attack in the right superior quadrantic visual field caused by left transverse-sigmoid sinus dural arteriovenous fistula (AVF) was reported. A 75-year old right-handed man noticed that when he saw an object, the image sometimes persisted even after he looked away or the object was removed. The palinoptic images always appeared in the right superior quadrant visual field, consisted of a row of multiple objects all identical to the real one in shape, and were accompanied by photopsia. The palinoptic images disappeared within a few minutes. He was neurologically normal and showed no hemianopia or quadrantanopia on admission. EEG showed no epileptic discharge. CT scanning of the brain revealed a small high density area in the left occipital lobe. MRI demonstrated hyperintensity in the left inferior occipital lobe corresponding to the lower part of Brodmann's areas 18 and 19 on T2 weighted image and flow-voids caused by the dilated left occipital artery. 99mTc-ECD SPECT disclosed a decrease of regional cerebral blood flow in the left occipital lobe. Cerebral angiography revealed dural AVF fed by three branches of the left occipital artery and another branch of the left ascending pharyngeal artery with retrograde drainage into the left transverse sinus, sigmoid sinus, and dilated cortical veins. The left transverse and sigmoid sinuses were occluded. Visual perseveration disappeared following the treatment of the dural AVF by transarterial embolization. Because the lesions on MRI and SPECT improved after the treatment, these lesions were considered to represent not infarction but vasogenic edema due to venous congestion. We emphasized the role of the left inferior occipital lesion including the secondary visual cortex (Brodmann's areas 18 and 19) as the cause of visual perseveration in the right superior quadrantic visual field.     

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.     

Dural arteriovenous malformations of the skull base

Abstract

Dural arteriovenous malformations (AVMs) are interesting lesions, which can present as diagnostic and therapeutic challenges. Such AVMs were initially described as located solely in the dural venous sinuses, but during the last decade researchers have discovered them in other locations such as the tentorium and foramen magnum. It is understandable that they can develop in other locations because numerous arteriovenous shunts normally occur all over the dura. Early reports emphasize that they are congenital; however, some investigators have proposed that they are acquired lesions arising after sinus thrombosis and trauma. They also have variable clinical features. From 1975 to 2000 we treated 400 AVMs, of which 46 were dural. Of these 46 dural AVMs, 18 could be classified as skull base AVMs. We restricted the designation of skull base AVMs to those AVMs located in the tentorium or adjacent to bones of the skull base. Two of these AVMs actually had their vascular nidus located in the bone. The common dural sinus AVMs were excluded from this category. The presenting clinical features of these skull base AVMs ranged from headache and pulsatile tinnitus to intracranial hemorrhage. They were treated by a combination of endovascular and microsurgical treatment. The majority of patients had a good clinical outcome. Skull base dural AVMs are an interesting clinical entity. Their clinical course, treatment and final outcome are variable depending on their location. They should be managed by a multidisciplinary approach.