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CASE REPORT
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 899-902

Dural arteriovenous fistula with spinal perimedullary venous drainage


Interventional Neuroradiology Department, Beijing Neurosurgical Institute and Beijing Tiantan Hospital, Capital Medical University, Beijing, China

Date of Submission15-Sep-2011
Date of Decision22-Oct-2011
Date of Acceptance08-Nov-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
Zhongxue Wu
Beijing Neurosurgical Institute, No.6, Tiantan Xili, Dongcheng, Beijing, 100050
China
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 » Abstract 

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.


Keywords: Dural arteriovenous fistula, medulla, spinal cord vein


How to cite this article:
Lv X, Yang X, Li Y, Jiang C, Wu Z. Dural arteriovenous fistula with spinal perimedullary venous drainage. Neurol India 2011;59:899-902

How to cite this URL:
Lv X, Yang X, Li Y, Jiang C, Wu Z. Dural arteriovenous fistula with spinal perimedullary venous drainage. Neurol India [serial online] 2011 [cited 2014 Apr 16];59:899-902. Available from: http://www.neurologyindia.com/text.asp?2011/59/6/899/91374



 » Introduction Top


Dural arteriovenous fistulas (DAVFs) with a perimedullary venous drainage account for 5% of all intracranial DAVFs. [1] The clinical presentations include subarachnoid hemorrhage (SAH) and progressive myelopathy. [1],[2],[3] Similar to spinal DAVFs, intracranial DAVFs also lead to venous congestion of the spinal cord. According to the modified classification of Djindjian and Merland, [4] these intracranial DAVFs are Type V fistulas. Blood flow through the fistula runs via an anastomotic pathway between the pontomesencephalic venous system and both anterior and posterior perimedullary veins [1],[2] to the coronal venous plexus, which becomes dilated and tortuous. In this report we discuss the characteristics of six patients with DAVFs with drainage directly into the perimedullary veins.


 » Case Reports Top


Data analysis

Between 2008 and 2011, six consecutive patients with DAVFs with drainage directly into the perimedullary venous system were referred to the department of Interventional Neuroradiology of Beijing Tiantan Hospital. The clinical data and angiographic findings in these six cases were reviewed retrospectively.

Clinical data

The mean age was 48.2 years (range 18-73 years) and included five men and one woman. Computed tomography (CT) was performed in the five patients with a posterior fossa SAH. Myelography or spinal magnetic resonance (MR) imaging was the first examination performed in one patient who presented with progressive myelopathy, sensorimotor deficits with bladder symptoms. In this case, T1-weighted MR images of the spinal cord showed perimedullary flow voids; in one patient, T2-weighted images showed perimedullary flow voids and a central hyperintense signal of the cervical spinal cord.

Angiographic data

In the five patients with 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. The DAVFs were fed by dural branches of the external carotid, internal carotid, and vertebral arteries [Table 1]. The initial venous drainage reached the cervical spinal perimedullary vein (three patients), the anterior spinal vein (two patients), or both anterior and posterior spinal veins (one patient). The cervical spinal perimedullary veins drained into the cervical epidural venous plexus between the C-4 and C-7 levels. 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 [Figure 1]. All six patients were successfully treated with Onyx or NBCA embolization. Angiographic cure of the fistula resulted in clinical cure in three patients and stabilization in three patients.
Figure 1: Case 6. (a): Sagittal T2-weighted MR image showing intramedullary high signal intensity in the cervical spinal cord. b: MR image shows engorged perimedullary veins. c: Lateral view of the right internal artery shows the fistula point identified as a tentorial branch of the right internal carotid artery. (d): Selective angiography of the left external carotid artery shows that the middle meningeal artery also supplies the DAVF. (e): Fluoroscopic image after Onyx embolization via the left middle meningeal artery. (f): Demonstrates the postoembolization angiogram with a complete obstruction of the fistula (g and h): 5-month follow-up MR images show no intramedullary high signal intensity and engorged perimedullary veins.

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Table 1: Clinical and angiographic characteristics in 6 cases of DAVF with spinal venous drainage

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 » Discussion Top


Intracranial DAVFs with spinal perimedullary venous drainage are rare lesions with distinctive clinical, radiological, and therapeutic aspects. These lesions were classified as Type V in the Djindjian and Merland classification of dural intracranial AV fistulas. As in all dural fistulas, treatment is directed toward closure of the draining vein, either by surgical or endovascular techniques. Surgery of these disorders requires extensive exploration of the skull base with inherent risks; [5] therefore, endovascular closure of the fistula is preferred. Endovascular therapy is a safe effective method in the treatment of these fistulas and should be the first option. [4] All our patients and most of the cases in the literature [6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] were treated with endovascular embolization alone. High-quality six-vessel angiography facilitates understanding of complex anatomy of the tiny and tortuous supplying vessels. Careful navigation of the microcatheter, to avoid dissection of small arteries, to a position as close as possible to the fistula site in a wedge position with flow control allows deep penetration of the liquid embolic agent with occlusion of the draining vein. After angiographic closure of the fistula, clinical results are variable. Some patients may show remarkable improvement, whereas others remain unchanged, despite normalization of MR imaging findings. [1],[4],[11]

Because the rarity of this type of DAVF, only a few comprehensive reviews have been published: clinical presentation, prognosis, and therapeutic options. [11] A series of 12 patients reveals the relation between the clinical presentation and the venous drainage of DAVFs: the patients who had myelopathy had extensive spinal venous drainage descending towards the lower thoracic spinal cord and the cauda equine. [1] In our series also patients who presented with posterior fossa SAH had venous drainage limited to the cervical spinal cord. In our series male gender was predominant. Most patients presented with clinical features after the fourth decade of life. Patients with this type of DAVFs present with two distinct clinical syndromes: SAH and myelopathy. No age and gender differences have been observed between the two groups.

Diagnosis of a DAVF with spinal perimedullary venous drainage is challenging because the presenting symptoms are usually related to dysfunction of the spinal cord, not of the brain. Repeated spinal angiograms are usually performed before the diagnosis is finally made by cerebral angiography. [17] Comprehensive spinal angiographic studies could not locate the DAVFs, even bilateral vertebral angiography initially failed to demonstrate the fistulas. However, diagnosis was established with external carotid angiography. Cerebral angiography with catheterization of internal carotid, external carotid, and vertebral arteries was the key to the diagnosis. It showed the DAVFs with spinal venous drainage located in the posterior fossa. Drainage of the DAVFs through the enlarged spinal perimedullary veins could be followed downward along the entire spinal cord. While normally there are numerous medullary-radicular veins draining the blood from the spinal cord to the epidural space in the cervical and thoracic areas. [18],[19] With regard to digital subtraction angiography (DSA) for SAH investigation, it is recommended now to inject both external carotids when bilateral ICA and vertebral arteries have failed to show the cause of SAH. Also, when external carotids are also normal, then spinal MRI is also indicated.

Diagnosis of a DAVF presenting with myelopathy is more challenging than of those presenting with SAH. Bilateral vertebral angiography initially failed to demonstrate the dural fistula causing myelopathy, and diagnosis could be established with carotid artery angiography.

 
 » References Top

1.Brunereau L, Gobin YP, Meder JF, Cognard C, Tubiana JM, Merland JJ. Intracranial dural arteriovenous fistulas with spinal venous drainage: Relation between clinical presentation and angiographic findings. AJNR Am J Neuroradiol 1996;17:1549-54.  Back to cited text no. 1
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2.Cognard C, Gobin YP, Pierot L, Bailly AL, Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: Clinical and angiographic correlation with a revised classification of venous drainage. Radiology 1995;194:671-80.  Back to cited text no. 2
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3.Hurst RW, Grossman RI. Peripheral spinal cord hypointensity on T2-weighted MR images: a reliable imaging sign of venous hypertensive myelopathy. AJNR Am J Neuroradiol 2000;21:781-6.  Back to cited text no. 3
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4.Gobin YP, Rogopoulos A, Aymard A, Khayata M, Reizine D, Chiras J, et al. Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage. J Neurosurg 1992;77:718-23.  Back to cited text no. 4
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5.Pannu Y, Shownkeen H, Nockels RP, Origitano TC. Obliteration of a tentorial dural arteriovenous fistula causing spinal cord myelopathy using the cranio-orbito zygomatic approach. Surg Neurol 2004;62:463-7.  Back to cited text no. 5
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6.A kkoc Y, Atamaz F, Oran I, Durmaz B. Intracranial dural arteriovenous fistula draining into spinal perimedullary veins: A rare cause of myelopathy. J Korean Med Sci 2006;21:958-62.  Back to cited text no. 6
    
7.Li J, Ezura M, Takahashi A, Yoshimoto T. Intracranial dural arteriovenous fistula with venous reflux to the brainstem and spinal cord mimicking brainstem infarction--case report. Neurol Med Chir (Tokyo) 2004;44:24-8.  Back to cited text no. 7
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8.Mahagne MH, Rogopoulos A, Paquis P, Guilhem D, Lonjon M, Grellier P, et al. Intracranial dural arteriovenous fistula with spinal venous drainage. Rev Neurol (Paris) 1992;148:789-92.   Back to cited text no. 8
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9.Ohnishi H, Deguchi J, Yamada M, Kuroiwa T. Cavernous arteriovenous fistula presenting myelopathy: A case report. No Shinkei Geka 2003;31:1119-23.   Back to cited text no. 9
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10.Oishi H, Horinaka N, Shmizu T, Ozaki Y, Arai H. A case of intracranial dural arteriovenous fistula presenting with brainstem infarction. No Shinkei Geka 2005;33:1095-9.   Back to cited text no. 10
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11.Ricolfi F, Manelfe C, Meder JF, Arrué P, Decq P, Brugiéres P, et al. Intracranial dural arteriovenous fistulae with perimedullary venous drainage: Anatomical, clinical and therapeutic considerations. Neuroradiology 1999;41:803-12.  Back to cited text no. 11
    
12.Satoh M, Kuriyama M, Fujiwara T, Tokunaga K, Sugiu K. Brain stem ischemia from intracranial dural arteriovenous fistula: Case report. Surg Neurol 2005;64:341-5.  Back to cited text no. 12
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13.Seda LF Jr, Pieruccetti MA, Freitas JM, Listik S, Pereira CA. Intracranial dural arteriovenous fistula draining into spinal cord veins: Case report. Arq Neuropsiquiatr 2002;60:856-60.   Back to cited text no. 13
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14.Slaba S, Smayra T, Hage P, Okais N, Atallah N. An unusual cause of acute myelopathy: A dural arteriovenous fistula at the craniocervical junction. J Med Liban 2000;48:168-72.   Back to cited text no. 14
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16.Wiesmann M, Padovan CS, Pfister HW, Yousry TA. Intracranial dural arteriovenous fistula with spinal medullary venous drainage. Eur Radiol 2000;10:1606-9.  Back to cited text no. 16
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17.Chen CJ, Chen CM, Lin TK. Enhanced cervical MRI in identifying intracranial dural arteriovenous fistulae with spinal perimedullary venous drainage. Neuroradiology 1998;40:393-7.  Back to cited text no. 17
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18.Brunet E, Tache R, Lafitte F, Piotin M, Miaux Y, Martin-Duverneuil N, et al. Intracranial dural arteriovenous fistula with perimedullary venous drainage. J Neuroradiol 1998;25:103-10.  Back to cited text no. 18
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19.Wrobel CJ, Oldfield EH, Di Chiro G, Tarlov EC, Baker RA, Doppman JL. Myelopathy due to intracranial dural arteriovenous fistulas draining intrathecally into spinal medullary veins: Report of three cases. J Neurosurg 1988;69:934-9.  Back to cited text no. 19
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