Exclusively epidural arteriovenous fistula in the cervical spine with spinal cord symptoms: case report

OBJECTIVE AND IMPORTANCE: We describe the case of an epidural arteriovenous fistula (AVF) in the cervical spine draining only into the epidural and paravertebral plexus. An entirely epidural AVF having such drainage is extremely rare. CLINICAL PRESENTATION: A 24-year-old man presented with a 4-month history of gradually progressive sensory and motor disturbances of the upper and lower extremities. Magnetic resonance imaging and magnetic resonance angiography revealed a peridural vascular lesion within the canal compressing the spinal cord from C5 to T2. Diagnostic angiography revealed a perimedullary and/or dural high-flow AVF, fed mainly by branches of ascending cervical and deep cervical arteries. The fistula drained into the epidural and paravertebral venous plexus without reflux into intradural venous systems. INTERVENTION: Multiple feeders of the AVF were embolized with a Liquid coil and n-butylcyanoacrylate via a two-step procedure. One week after embolization, the AVF was surgically removed. CONCLUSION: Interesting points of this case were the exclusively epidural location of the lesion, the exclusively epidural drainage of the AVF, and the etiology of the symptoms. Venous drainage of the fistula had no relation to any dural or intradural veins. Initially, spinal cord and nerve root compression by extradural veins with varicose dilation seemed to cause the radiculopathy and/or the myelopathy, and subsequent myelopathy caused by spinal venous hypertension was believed to be the main etiology in this case.     

Tentorial dural arteriovenous fistula presenting symptoms due to mass effect on the dilated draining vein: case report

BACKGROUND: Tentorial dural arteriovenous fistula (AVF) presented symptoms due to mass effect on the dilated draining vein. We report a patient presenting left hemisensory disturbance because of compression of the midbrain by a dilated draining vein of the AVF. The AVF has disappeared completely by drainer clipping after feeder embolization. CASE DESCRIPTION: A 66-year-old woman presented with left hemisensory disturbance due to compression of the midbrain by a dilated draining vein with tentorial dural AVF. On admission, she complained of left hemisensory disturbance. Enhanced computed tomography (CT), magnetic resonance imaging, and magnetic resonance angiogram revealed the midbrain compressed by a mass lesion, which was a draining vein with AVF fed by numerous feeding arteries neighboring the right tentorial edge. The single-photon emission CT (SPECT) did not depict any laterality. CONCLUSION: This is a rare case of a tentorial dural AVF which caused left hemisensory disturbance not by venous congestion, but by a compression of the midbrain by the dilated draining vein, because SPECT showed no laterality. In this case, magnetic resonance angiogram, 3-dimensional CT angiography, and SPECT were useful in the diagnosis and planning the strategy for treatment.     

Atypical dural arteriovenous fistula associated with meningitis

A 49-year-old male presented with hemisensory disturbance and gait unsteadiness following a previous episode of meningitis. He had no contributory medical or head injury history. Magnetic resonance imaging revealed innumerable medullary vessels in the white matter of the left cerebral hemisphere, which had not been recognized in the previous imaging study. Cerebral angiography showed variant superior sagittal sinus (SSS) arteriovenous fistula (AVF) fed by the bilateral middle meningeal and superficial temporal arteries, and drained directly to the cortical veins with marked venous engorgement in the affected hemisphere. The fistulas were located on the cortical veins, apart from the SSS. Initial percutaneous transarterial embolization failed, so the AVF was completely obliterated with a combination of surgical and endovascular techniques. The symptoms ameliorated postoperatively. Meningitis may be an underlying pathology of dural AVF. Variant SSS AVF can be treated with a combination of surgical and endovascular techniques.     

Arteriovenous fistula involving the inferior petroclival vein--case report

A 68-year-old man presented with an extremely rare extracranial arteriovenous fistula (AVF) involving the inferior petroclival vein (IPCV) with retrograde venous drainage into an ophthalmic vein through the anterior condylar confluence and inferior petrosal sinus manifesting as ocular symptoms. The AVF was successfully treated by selective transvenous embolization with platinum coils. AVF involving the IPCV should be recognized as a possible extracranial lesion manifesting as clinical symptoms similar to cavernous sinus dural AVF.     

Dural arteriovenous fistula involving the superior sagittal sinus following sinus thrombosis--case report

A 57-year-old woman presented with a dural arteriovenous fistula (AVF) involving the superior sagittal sinus (SSS) based upon serial radiological examinations. Her chief complaints were headache and vomiting. Cerebral angiography and magnetic resonance (MR) venography revealed the sinus thrombosis involving the SSS, the bilateral transverse sinuses (TSs), and the right sigmoid sinus. Her symptoms disappeared after anticoagulant therapy. Follow-up MR venography revealed almost complete recanalization of the occluded sinuses, followed by restenosis of the SSS and the left TS and occlusion of the right TS without symptoms. She developed transient right hemiparesis 13 months after the initial onset. Cerebral angiography revealed a dural AVF involving the SSS with cortical reflux into the left frontoparietal region. The dural AVF was occluded by transarterial and transvenous embolization. Her symptom disappeared during the follow-up period.     

Endosurgical repair of an iatrogenic facial arteriovenous fistula due to percutaneous trigeminal balloon rhizotomy

A 56-year-old woman with right-sided trigeminal neuralgia (TN), who underwent technically uneventful percutaneous balloon rhizotomy, developed significant bilateral pulsatile tinnitus on the first post-operative day. Although the patient reported significantly improved neuralgia, auscultation revealed a right facial bruit. Magnetic resonance angiography (MRA) of the face and brain demonstrated prominent right facial and jugular venous vascularity. Catheter angiography confirmed the suspected facial arteriovenous fistula (AVF). A transarterial approach was used to explore the AVF which arose from a laceration of the right internal maxillary artery and which fistulized directly with the pterygoid venous plexus. Endosurgical repair utilizing three non-fibered platinum coils was done under conscious sedation at the same setting as the diagnostic angiogram. Angiographically, the fistula was obliterated, and the patient's bruit and tinnitus immediately resolved. Follow-up MRA at 3.5 months was normal, and, the patient had no clinical symptoms of recurrent AVF.In conclusion facial AVF can complicate percutaneous trigeminal rhizotomy. Iatrogenic facial AVF can be repaired via an endovascular approach.     

Congestive myelopathy due to cervical perimedullary arteriovenous fistula evaluated by apparent diffusion coefficient values - case report

A 22-year-old woman presented with a cervical perimedullary arteriovenous fistula (AVF) manifesting as right upper and lower extremity weakness. T2-weighted magnetic resonance (MR) imaging showed intramedullary hyperintensity believed to be caused by venous congestion. Preoperative diffusion-weighted MR imaging showed increased apparent diffusion coefficient (ADC) value. Spinal angiography demonstrated an AVF fed mainly by the right C-5 radicular artery. Complete obliteration of AVF was achieved by endovascular embolization and microsurgical shunt occlusion. The ADC value was normalized and her neurological deficits improved after endovascular surgery, whereas T2-weighted MR imaging still demonstrated the lesion. The high preoperative ADC value probably indicated reversible vasogenic edema and immediate normalization of the ADC value suggests a good clinical outcome.     

Characteristics and treatment of dural and perimedullary arteriovenous fistula at the craniocervical junction presenting with subarachnoid hemorrhage

Dural and perimedullary arteriovenous fistula (AVF) at the craniocervical junction tend to cause subarachnoid hemorrhage (SAH). However, their natural history and clinical manifestations still remain to be elucidated. From 2003 to 2009, we encountered 5 cases of dural and perimedullary AVF presented with SAH. They were all male, ranging in age from 53 to 85 year-old (mean: 68 year-old). Rebleeding occurred in 1 patient on day 11. Outcome estimated by modified Rankin Scale did not change remarkably from 2.6 on admission to 2.4 at 3 months later on average. Cerebral angiography and 3D-CT angiography disclosed feeders originating from radicular or intracranial vertebral arteries which drained into the epidural venous plexus or spinal meningeal veins. One patient died of systemic complication during his clinical course. Thus we performed open surgery in the remaining 4 patients. Of these, we failed to occlude feeders completely in the initial surgery without intraoperative digital subtraction angiography (DSA) in 2 patients. Following this treatment we performed coil embolization and repeated open surgery with the aid of intraoperative DSA, respectively. In 1 patient out of the remaining 2 patients, we utilized intraoperative DSA to confirm complete disappearance of AVF composed of multiple feeders. These observations show that SAH caused by dural and perimedullary AVF at the craniocervical junction should be mainly treated by open surgery with the aid of intraoperative DSA in order to accomplish obliteration of the feeders because, otherwise, we might fail to confirm complete disappearance of AVF.     

Spontaneous spinal epidural arteriovenous fistulae in neurofibromatosis type-1

BACKGROUND: NF-1 is one of the most common autosomal-dominantly inherited genetic disorders with an incidence of approximately 1:3500. We report a case and review the literature to characterize spontaneous spinal AVF that occur in neurofibromatosis (NF-1). CASE REPORT: A 51-year-old woman presented with NF-1 and progressive radiculomyelopathy. Angiography revealed an AVF terminating in a giant intraspinal epidural varix extending paraspinally through the C3/4 neural foramen. Trapping of the AVF attempted 18 years earlier prevented endovascular access for embolization, and vigorous bleeding made direct surgical resection impossible. Therefore, as palliation, arterial feeding collaterals were occluded, and surgically exposed tortuous veins were packed with coils. Laminectomies and partial resection of the epidural varix resulted in subtotal occlusion with clinical improvement. CONCLUSION: The spinal AVF associated with NF-1 appears to show dominant venous drainage to the intraspinal extradural and paraspinal venous plexus without evidence of intradural drainage. The vertebral artery is typically the origin of the fistula. A giant venous varix and numerous collateral feeders to the vertebral artery may give an AVM-like appearance. Clinically, the fistulae produce a syndromic triad including symptoms of NF-1, progressive radiculomyelopathy, and a bruit. Treatment is direct attack on the fistula by either surgery or embolization. If, however, a direct approach cannot be chosen, occlusion of feeding vessels combined with laminectomies can result in long-term symptomatic improvement.     

A case of dural arteriovenous fistula of the inferior petrosal sinus successfully treated by transarterial and transvenous embolizations

A 69-year-old male was admitted with chemosis and exophthalmos of his right eye. Angiograms revealed a dural arteriovenous fistula (AVF) involving the right inferior petrosal sinus. The AVF was fed by the right occipital and ascending pharyngeal arteries and drained into the cavernous sinus and right superior ophthalmic vein from the right inferior petrosal sinus. He was treated by transarterial embolization with polyvinyl alcohol in order to reduce the shunt-flow through the fistula. Then he was treated by transvenous embolization with GDC coils five days after the arterial embolization. Symptoms in his right eye have completely disappeared. Transvenous embolization combined with transarterial embolization is a useful and safe approach in the management of AVF involving the inferior petrosal sinus.     

Vertebral arteriovenous fistula that developed in the same place as a previous ruptured aneurysm: a case report

BACKGROUND: Aneurysms of the extracranial vertebral artery (VA) and vertebral arteriovenous fistulas (VAVFs) are relatively rare diseases. The most frequent cause of both diseases is trauma. Atraumatic lesions are less common. Presented here is a case of atraumatic AVF of the extracranial VA that developed in the same location as a previous ruptured aneurysm of the ipsilateral VA that was originally treated by proximal occlusion 11 years earlier. METHODS: A 40-year-old woman presented with a massive hematoma in the upper posterior neck region caused by the rupture of an extracranial VA aneurysm. Proximal occlusion of the VA was performed by use of a detachable balloon. She enjoyed good health for 11 years, then she noticed a pulsatile bruit. Angiograms revealed an AVF between the left VA that was fed by collateral circulation and the paravertebral venous plexus. Incidentally found were soft tissue masses in the left retroauricular and the right suboccipital regions. Also, skull X-ray films showed multiple bony defects. Biopsy of the subcutaneous mass was performed in the hope of obtaining clues as to which pathological processes had weakened the artery. RESULTS: As direct transarterial access to the fistula was out of the question, the fistulous compartment of the paravertebral venous plexus was tightly packed with multiple platinum coils effected by the transfemoral approach. A histological examination of the specimen revealed features of a neurofibroma, and a diagnosis of neurofibromatosis Type 1 was established. CONCLUSIONS: In this case, transvenous embolization of the VAVF was successfully performed. The fragility of the arterial wall, related to neurofibromatosis Type 1, was considered to contribute to the development of the aneurysm and AVF.     

Myelopathy caused by tentorial dural arteriovenous fistula: a rare case report

A 51-year-old man with myelopathy due to intracranial dural arteriovenous fistula (dural AVF) is reported. At age 46, the patient experienced subarachnoid hemorrhage caused by rupture of the dural AVF and underwent embolization of the lesion at another hospital. At this time, the patient complained of numbness in his legs and showed paraplegic gait disturbance. MRI scan revealed swelling of the cervical spinal cord. Cerebral angiograms demonstrated the recurrence of tentorial dural AVF fed by bilateral meningohypopheseal trunks and the right posterior inferior cerebellar artery. Arteriovenous shunt (AV shunt) flow was drained into the anterior spinal vein. It seemed that the swelling of the spinal cord and myelopathy was caused by venous hypertension of spinal veins. After surgical interruption of the right petrosal vein which connected dAVF with cerebellar veins, AV shunt was obliterated successfully. Postoperative cerebral angiograms showed disappearance of dural AVF. The patient became ambulant and his cervical spinal cord appeared normal on the postoperative MRI scan. Surgical interruption of the draining vein was simple, effective and essential treatment.     

The validity of intraoperative angiography for the treatment of spinal arteriovenous fistula

STUDY DESIGN: Retrospective study of patients with spinal arteriovenous fistula (AVF) who underwent surgical treatment. OBJECTIVE: To evaluate the validity of the intraoperative angiography (IA) for the surgical treatment of spinal AVF. SUMMARY OF BACKGROUND DATA: Owing to the development of interventional techniques, endovascular embolization has become the treatment of choice for AVF, but it is not applicable for every spinal AVF owing to anatomic complexity of the spinal cord vessels. To get effective occlusion of the AVF, IA has been routinely used in the management of cerebral vascular diseases, but report of its use for spinal AVF is rare. METHODS: Since 2004, 4 consecutive cases of spinal AVF (3 males and 1 female, 3 thoracic, and 1 thoracolumbar) were involved in this study. The mean age at the time of operation was 62.3 years (range from 48 to 76 y). Types of AVFs and surgical techniques were reviewed retrospectively and the outcomes were assessed using the Japanese Orthopedic Association scoring system. RESULTS: AVFs in 3 patients were diagnosed as the dural type and that in the remaining patient as the perimedullary type; all feeding arteries were derived from the ninth to 10th intercostal arteries. Preoperative angiography demonstrated that the feeding arteries in 2 patients with a dural AVF were the branches of Adamkiewicz artery and in another dural AVF case, the Adamkiewicz artery could not be determined, therefore, endovascular embolization was not feasible. Including a patient with perimedullary AVF, a microsurgical clipping combined with IA was selected as the treatment. Complete occlusion of the fistula was achieved in all cases, the mean preoperative Japanese Orthopedic Association score of 4.5 improved to 6 at the final follow-up, and no perioperative complications were observed during the follow-up period. CONCLUSIONS: The favorable clinical results in our spinal AVF cases confirmed that IA ensures safe and accurate occlusion of the fistula. This technique provides satisfactory surgical results for spinal AVFs.     

Case of cervical paraspinal arteriovenous fistulae with a huge intracanalicular varix successfully treated with transvenous embolization

Paraspinal arteriovenous fistulas (AVFs), with the fistulas draining into the epidural veins alone, are relatively rare and few cases have been reported until now. We reported a case of cervical paraspinal AVFs draining only into the epidural venous plexus without reflux into the intradural venous system. The patient showed myelopathy due to direct compression of the spinal cord by a large varix. A 57-year-old man presented with gait disturbance. Neurological findings on admission revealed that tetraparesis, sensory disturbance of four extremitas below the C5 level and bladder bowel dysfunction. Magnetic resonance imaging and computed tomography at the cervical level disclosed remarkable compression of the spinal cord by a large venous pouch at the C6 level. Digital subtraction angiography (DSA) revealed paraspinal arteriovenous fistulas fed by bilateral C6 radicular arteries, the right ascending cervical arteries, and the right deep cervical artery in the right C6 intervertebral foramen. Three-staged transarterial embolization was performed by selective catheterization of the multiple feeders with n-butylcyanoacrylate, followed by transvenous embolization. During transvenous embolization, motor evoked potential (MEP) monitoring was performed. After retrograde catheterization of the epidural venous plexus, the large varix was occluded with Guglielmi detachable coils. The AV fistulas were completely occluded without any change in MEP monitoring during the procedure. The patient's gait improved well after the procedure and follow up DSA six months later showed no recurrence of the paraspinal AVFs.     

Cavernous sinus dural arteriovenous fistula associated with the development of sphenoidal ridge meningioma--case report

A 76-year-old man presented with a cavernous sinus (CS) dural arteriovenous fistula (AVF) associated with the development of a meningioma without venous sinus occlusion. Initial digital subtraction angiography did not reveal the CS dural AVF, which appeared simultaneously with the enlargement of the meningioma and lead to right oculomotor nerve paresis. In this case, the development of meningioma possibly increased the vascular tumor bed and affected the venous hemodynamic return, thus leading to the dural AVF.     

Intracranial pial arteriovenous fistula

A 33-year-old woman presented with a rare intracranial pial arteriovenous fistula manifesting as monoparesis and hypesthesia of the right lower extremity. Computed tomography demonstrated an approximately 10-mm diameter subcortical hematoma in the left postcentral gyrus. Two months after suffering the ictus, angiography demonstrated a pial arteriovenous fistula in the late arterial phase fed by the left paracentral artery and drained into the left precentral vein. No nidus or dural arteriovenous fistula was detected. Left parietal craniotomy was performed and the pial arteriovenous fistula was extirpated by electrocoagulation. Intraoperative angiography demonstrated disappearance of the fistula. She experienced no postoperative neurological deterioration, but hypesthesia of the right leg persisted. Obliteration of the pial arteriovenous fistula was reconfirmed by postoperative angiography. She suffered no rebleeding episodes during the 36-month follow-up period. Pial arteriovenous fistula causing mild symptoms should be treated by flow disconnection because the direct arteriovenous shunt and attendant high blood flow usually results in huge venous varices. To determine whether direct surgery or endovascular treatment is appropriate, the position and shape of the lesion must be known.     

Extradural arteriovenous fistulas involving the vertebral artery in neurofibromatosis Type 1

Spinal extradural arteriovenous fistulas (AVFs) are rare lesions that may be associated with neurofibromatosis Type 1 (NF1). In these patients, the shunt typically occurs between the V(2) segment of the vertebral artery and the epidural venous plexus. Previously, reported cases have been treated either by using endovascular embolization or, sporadically, by open surgery. In surgical reports, proximal deafferentation or manipulation of the venous portion of the shunt--including suture, resection, or open embolization of the epidural ectasia--was attempted with variable results. The authors report on a case of a young patient with NF1 who underwent emergency surgical disconnection of a cervical extradural AVF after previously unsuccessful endovascular and surgical therapy. The lesion drained into a giant intrathecal varix, causing severe myelopathy. After surgery, the patient recovered almost completely. This experience clarified the surgical anatomy of these malformations and showed that, when surgery is necessary, the optimal treatment providing complete and permanent cure of this condition is direct closure of the epidural shunt pedicle.     

A dural arteriovenous fistula of the tentorium successfully treated by intravascular embolization.

BACKGROUND: Dural arteriovenous fistulas of the tentorium are rare lesions that often present with intracranial hemorrhage. Definitive treatment is therefore necessary, but transarterial embolization has rarely been curative. CASE DESCRIPTION: A 59-year-old man presenting with sudden onset of severe headache had subarachnoid hemorrhage demonstrated by computed tomography. Left carotid angiography showed a tentorial dural arteriovenous fistula fed by a tentorial branch from the internal carotid artery and by a middle meningeal artery; the fistula drained to the marginal sinus via a dilated varicosity. Transarterial embolization successfully obliterated the fistula, and the patient was discharged with no neurologic deficit. CONCLUSION: This tentorial fistula, that showed extremely rare angiographic features, particularly venous drainage, was embolized successfully. The literature concerning tentorial dural arteriovenous fistulas is discussed in terms of effective therapeutic choice.     

Spinal epidural arteriovenous fistula with perimedullary drainage. Case report and pathomechanical considerations

The classic angiographically demonstrated features of spinal dural arteriovenous fistulas are shunts of radiculomeningeal branches with radicular veins draining exclusively in the direction of perimedullary veins and thereby causing venous congestion. These shunts are located at the point where the radicular vein passes the dura mater. Spinal epidural arteriovenous shunts, however, normally do not drain into the perimedullary veins and are, therefore, asymptomatic, presumably because of a postulated reflux-impeding mechanism between the dural sleeves. The authors report on a patient in whom an epidural arteriovenous shunt showed delayed retrograde drainage into perimedullary veins, leading to the classic clinical (and magnetic resonance imaging-based) findings of venous congestion. Intraoperatively the angiographically established diagnosis was confirmed. Coagulation of both the epidural shunt zone and the radicular vein resulted in complete obliteration of the fistula, as confirmed on repeated angiography. This rare type of fistula should stimulate considerations on the role of valvelike mechanisms normally impeding retrograde flow from the epidural plexus to perimedullary veins and suggest that, in certain pathological circumstances, epidural fistulas can drain retrogradely into perimedullary veins as an infrequent variant of spinal arteriovenous shunts.     

Obliteration of a tentorial dural arteriovenous fistula causing spinal cord myelopathy using the cranio-orbito zygomatic approach

BACKGROUND: Intracranial dural arteriovenous fistulas account for 10 to 15% of all intracranial arteriovenous malformations. Tentorial dural arteriovenous fistulas with spinal medullary venous drainage causing spinal cord myelopathy are very rare, but have been previously described. We describe a case using a cranio-orbito zygomatic approach with intraoperative angiography for the surgical treatment of a tentorial artery dural arteriovenous fistula causing spinal cord myelopathy. CASE PRESENTATION: A 42-year-old male presented complaining of a 1-year history of incoordination and dizziness and a 2-month history of progressive myelopathy with bowel and bladder incontinence. The patient had magnetic resonance imaging (MRI) performed along with cerebral and spinal angiography that revealed a right tentorial artery dural arteriovenous fistula with spinal medullary venous involvement down to T11. Angiographic embolization was attempted, but selective catheterization was unsuccessful. The patient underwent a cranio-orbito zygomatic approach with obliteration of the dural arteriovenous fistula. An intraoperative angiogram confirmed complete obliteration of the dural arteriovenous fistula. CONCLUSION: Intracranial dural arteriovenous fistulas are a rare cause of spinal cord myelopathy. When a patient presents with suspicion of spinal dural fistula and negative spinal angiography, an intracranial origin should be suspected and a cerebral angiogram performed. Skull base approaches along with intraoperative angiography provide an alternative modality for obliteration of the dural arteriovenous fistula nidus, thereby eliminating the venous congestion and hence the spinal cord ischemia.