Article

PDF
Access to the PDF text
Service d'aide à la décision clinique
Advertising


Free Article !

Neurochirurgie
Volume 59, n° 6
pages 210-213 (décembre 2013)
Doi : 10.1016/j.neuchi.2013.06.003
Received : 11 February 2013 ;  accepted : 9 June 2013
Coincidental vascular anomalies at the foramen magnum: Dural arteriovenous fistula and high flow aneurysm on perimedullary fistula
Coexistence de deux anomalies vasculaires de la jonction cranio-cervicale : fistule durale artério-veineuse et anévrisme de l’artère spinale antérieure
 

V. Gilard a, S. Curey a, E. Tollard b, F. Proust a,
a Department of neurosurgery, Rouen university hospital, 1, rue de Germont, 76031 Rouen cedex, France 
b Department of radiology, Rouen university hospital, 1, rue de Germont, 76031 Rouen cedex, France 

Corresponding author.
Abstract

We report the case of a 59-year-old woman admitted for a sudden headache due to a subarachnoid haemorrhage. On CT scan, the clots predominated into the posterior fossa without high-density in the sylvian or interhemispheric fissures. The vertebral angiography revealed a dural arteriovenous fistula at the foramen magnum associated to an aneurysm of the cervical anterior spinal artery. Due to the high rebleeding risk of a dural shunt, we proposed curative treatment using microsurgical interruption of the intradural draining vein. On the postoperative angiography at 15-day follow-up, the 2 malformations were corrected and the outcome at 6 months was excellent. Based on the literature, we assess this exceptional association and suggest its possible management.

The full text of this article is available in PDF format.
Résumé

Une femme de 59ans est admise en urgence pour une céphalée soudaine liée à une hémorragie sous-arachnoïdienne. Sur la tomodensitométrie cranio-encéphalique sans injection, l’hyperdensité prédominait dans la fosse postérieure au niveau de la jonction craniocervicale. L’artériographie vertébrale révélait une fistuledurale artério-veineuse alimentée par une branche méningée provenant de l’artère vertébrale gauche lors de la traversée durale, la veine efférente dilatée se dirigeait vers le haut en péribulbaire puis pré-protubérantiel. En outre, lors des clichés réalisés à l’injection lente du produit de contraste, un anévrisme fusiforme du segment cervical de l’artère spinale antérieure était diagnostiqué. Un traitement de la fistule artério-veineuse était réalisé par approche sous-occipitale microchirurgicale permettant une interruption par clip de la veine dilatée variqueuse. Sur l’angiographie de contrôle à 15jours, les 2 malformations étaient complètement exclues. À 6 mois, la patiente a repris la totalité de ces activités socioprofessionnelles.

The full text of this article is available in PDF format.

Keywords : Dural arteriovenous fistula, Craniocervical junction, Anterior spinal artery, Aneurysm, Subarachnoid haemorrhage

Mots clés : Fistule durale artério-veineuse, Jonction craniocervicale, Artère spinale antérieure, Anévrisme, Hémorragie sous-arachnoidienne


Introduction

Dural arteriovenous fistulas (DAVFs) at the foramen magnum (FM) are located in the dura mater of the foramen magnum supplied by meningeal branches from the vertebral artery, the occipital artery or the ascending pharyngeal artery [1, 2]. In contrast to spinal DAVFs essentially responsible for myelopathy, the common characteristics of this location at the craniocervical junction were the subarachnoid haemorrhage (SAH) as a diagnostic circumstance and a high flow status [3, 4, 5, 6, 7, 8, 9, 10, 11]. The association with other vascular anomalies and the management at this location have rarely been reported in the literature [9, 12]. In one patient, the reported coexistence of a DAFV at the FM with an aneurysm on the anterior spinal artery (ASA) prompted us to investigate the relationship between both malformations.

Case report

A 59-year-old woman, with no previous medical history, was admitted to the emergency department for a sudden headache. On clinical examination, she presented with an isolated meningismus without focal signs. On CT scan without contrast (Fig. 1) performed in emergency, the blood predominated in the peribulbar cistern of the posterior fossa without reflux into the fourth ventricle. No high-density area was observed in the sylvian or interhemispheric fissures. The SAH was classified as Fisher scale type III. The diagnosis of SAH grade I according to the World Federation of Neurosurgery Scale (WFNS) was confirmed. The CT angiogram of the head and neck showed extensive SAH in the upper cervical region as well as the basal cisterns and dilated vessel anterior to the medulla oblongata with an oblique course. No vascular malformation was observed. Comprehensive cerebral and spinal cord angiography was performed. Injection of the left vertebral artery revealed a DAVF at the FM fed by meningeal branches from the vertebral artery at the dural penetration level and drained intracranially to the superior petrous sinus via a tortuous enlarged vein (Fig. 2B). Careful injection of the right vertebral artery showed a fusiform aneurysm measuring 2mm in diameter located on the ASA at the C1 level (Fig. 2A), which depicted the DAVF at the FM. Thus, the ASA fed the DAVF via the left radiculopial artery of C1. These findings were consistent with a category V of a DAVF at the FM fed by the left vertebral artery and the high cervical ASA via the left radiculopial artery of C1.



Fig. 1


Fig. 1. 

Admission CT scan without contrast showing a subarachnoid hemorrage classified as Fisher scale type III in the peribulbar cistern of the posterior fossa without reflux into the fourth ventricle nor blood in the sylvian or interhemispheric fissures.

Scanner sans injection réalisé à l’admission montrant une hémorragie sous-arachnoïdienne grade III de la classificaton de Fisher prédominant dans la citerne péribulbaire sans contamination ventriculaire ou de la vallée sylvienne.

Zoom



Fig. 2


Fig. 2. 

Preoperative angiography. A. Showing 1: a 5mm aneurysm of the anterior spinal artery. B. Showing 1: the draining vein; 2: the nidus of the arteriovenous fistula of the craniocervical junction next to the V3-V4 segments.

Angiographie préopératoire. A. Montrant 1 : un anévrisme de 5mm de l’artère spinale antérieure B. Montrant 1 : la veine de drainage de la fistule durale artério-veineuse ; 2 : le nidus de la fistule durale artério-veineuse de la jonction craniocervicale en regard des segments V3-V4.

Zoom

The surgical treatment of the malformation was performed 17 days after the bleeding. The patient was placed in semirecumbent position. A lateral suboccipital craniectomy with haemilaminectomy of C1 allowed a limited longitudinal incision of the dura mater in a curve leading to an adequate exposition of the left vertebral artery at its dural penetration. Via this dorsal view, a distended arterialized vein was exposed just distal to the dural penetration of the left vertebral artery. This vein coursed obliquely upwards to the anterior surface of the medulla oblongata and the protuberance. Using an indocyanine green video-angiography, the disappearance of the DAVF by temporary clipping of the draining vein was confirmed. A 5mm straight clip was applied interrupting the intracranial venous part of the malformation just distal to the left vertebral artery (Fig. 3). We also confirmed a change in the colour of the arterialized vein from red to blue, but the entire course of the vein could not be observed due to its anterior location to the brainstem. The patient subsequently improved recovering a normal physiological status. The postoperative angiography confirmed the complete exclusion of the DAVF and of the ASA aneurysm (Fig. 4A and B). At discharge, there was no complication. At 6 months clinical follow-up, the patient's quality of life was excellent and the patient returned to a normal life without restriction. No recurrence of the fistulas was confirmed on magnetic resonance imaging at 6 months after the surgery.



Fig. 4


Fig. 4. 

Postoperative angiography. A. Showing the complete exclusion of the anterior spinal artery aneurysm. B. Showing the absence of arteriovenous fistula of the craniocervical junction.

Angiographie postopératoire. A. Montrant l’exclusion complète de l’artère spinale antérieure. B. Montrant l’absence de fistule de la jonction craniocervicale.

Zoom



Fig. 3


Fig. 3. 

Operative view showing. A. The anterior spinal artery. B. The medulla oblongata. C. The arteriovenous fistula. D. The spinal root of accessory nerve. E. C1 posterior rootlet.

Vue peropératoire montrant. A. L’artère spinale antérieure. B. Le bulbe rachidien. C. La fistule durale artério-veineuse. D. La racine du nerf accessoire. E. La racine de C1.

Zoom

Discussion

The coexistence of vascular anomalies associated to DAVFs is a presumed assertion. The incidence of coexisting vascular anomalies was estimated for cerebral DAVFs to be approximately 20% [13], and for the spinal DAVFs 2% essentially in the thoracolumbar region [14]. Nevertheless, for the cervical spinal DAVF, a rate of 41.7% of an associated complex vascular anomaly has been reported [12]. Similarly at the craniocervical junction, a rate of 37.5% with an associated vascular anomaly was recently published [9]. At the craniocervical junction, the incidence of vascular anomalies coexistence is probably underestimated because of the difficulties to perform complete angiographic explorations. As in our case, the associated vascular lesion on the ASA was revealed by a careful slow injection of a contrast agent into the contralateral vertebral artery.

Initially, we had interpreted the lesion as a fusiform aneurysm on the ASA. This interpretation is subject to discussion. In reality, this lesion may be included in a perimedullary arteriovenous fistula (PAVF) located on the anterior surface of the upper cervical spine. Moreover, the spinal aneurysm could be located on the lateral spinal artery as previously described by Lasjaunias et al. [15]. Arterial aneurysms have been observed in 80% of these associated PAVFs [9]. The development of the aneurysm may result from the combination of haemodynamic and genetic factors. The PAVF may cause arterial change due to the high flow related to the arterial feeders supplying the shunt [16]. Moreover, the arterial susceptibility could be related to the embryologic homology of this PAVF to the presence of the DAVF at the same metameric level. Indeed, this combination of both vascular malformations confirmed the hypothesis of spinal arteriovenous metameric syndrome as previously reported by Rodesch et al. [17].

The incidence of SAH as a diagnostic circumstance was estimated to be approximately 45% for these DAVFs of the craniocervical junction [18]. The mechanism underlying the occurrence of SAH has not been completely understood [5, 10, 18, 19], although several characteristics have been associated with this clinical event:

the spine location on cervical or craniocervical junction in contrast to the thoracolumbar exceptionally responsible for this condition;
the variceal enlargement of perimedullary veins;
the rostral direction of the intracranial venous drainage.

The venous hypertension, determining the arterialization of the medullary veins and the valveless pial coronal venous plexus with an increase in venous pressure [20], is probably involved in the occurrence of bleeding. However, this angioarchitectural factor, insufficient for the thoracolumbar region, becomes effective for the DAVFs located at the junction. In some case series [1, 5, 9, 12], the drainage pathways appeared as a small size despite the feeding arteries with a high flow as in the case of the vertebral artery and a slight opacification illustrating the low flow into the intracranial sinus in the posterior fossa. Nevertheless, the risk of rebleeding for the cerebral DAVFs has been estimated at approximately 35% for the DAVFs with retrograde cortical venous drainage within 2 weeks after the initial bleeding [21]. This risk could be applied for these DAVFs with retrograde rostral venous drainage into the posterior fossa and we recommend treatment at an early stage of these malformations in order to avoid the possible occurrence of major complications that could be caused by new bleeding.

The treatment of DAVFs at FM remains controversial between endovascular or microsurgical exclusion [4, 5, 12, 14, 22, 23]. Moreover, the microsurgical interruption of venous drainage has currently been fortunate because endovascular access is not easy for microcatheterization into these small tortuous arteries. Furthermore, the high risk of possible emboli related to the complex microanastomotic network, as well as the transvenous embolization is not suitable as this includes the high possibility of recanalization after incomplete occlusion. Moreover, the lateral suboccipital approach by a linear incision permitted a reliable exposure of the vertebral artery and the venous draining to achieve blockage. As in our case, the treatment of the venous aspect provided the treatment of both vascular anomalies under partial intraoperative control by indocyanine green video-angiography.

Conclusion

The coexistence of vascular anomalies at the FM should be carefully investigated by complete craniocervical angiography. The metameric syndrome of PAVF and DAVFs suggests a possible common genetic origin. The successful interruption of the venous side of the DAVF provided the complete treatment of both malformations.

Disclosure of interest

The authors declare that they have no conflicts of interest concerning this article.

References

Guo L.M., Zhou H.Y., Xu J.W., Wang G.S., Tian X., Wang Y., and al. Dural arteriovenous fistula at the foramen magnum presenting with subarachnoid hemorrhage: case reports and literature review Eur J Neurol 2010 ;  17 (5) : 684-691 [cross-ref]
Rhoton A.L. The foramen magnum Neurosurgery 2000 ;  47 (Suppl. 3) : S155-S193
Hanabusa K., Morikawa A., Tashiro H., Yamanaka M. [Craniocervical junction dural arteriovenous malformation presenting with subarachnoid hemorrhage: report of two cases] No Shinkei Geka 1999 ;  27 (3) : 237-241
Kim M.S., Han D.H., Han M.H., Oh C.W. Posterior fossa hemorrhage caused by dural arteriovenous fistula: case reports Surg Neurol 2003 ;  59 (6) : 512-516[Discussion p. 16–7].  [cross-ref]
Kinouchi H., Mizoi K., Takahashi A., Nagamine Y., Koshu K., Yoshimoto T. Dural arteriovenous shunts at the craniocervical junction J Neurosurg 1998 ;  89 (5) : 755-761 [cross-ref]
Kulwin C., Bohnstedt B.N., Scott J.A., Cohen-Gadol A. Dural arteriovenous fistulas presenting with brainstem dysfunction: diagnosis and surgical treatment Neurosurg Focus 2012 ;  32 (5) : E10
Reinges M.H., Thron A., Mull M., Huffmann B.C., Gilsbach J.M. Dural arteriovenous fistulae at the foramen magnum J Neurol 2001 ;  248 (3) : 197-203 [cross-ref]
Rivierez M., Gazengel J., Chiras J., Dorwling-Carter D., Debussche D., Dubbs M., and al. [Vertebro-dural arteriovenous fistulae of the foramen magnum with perimedullary venous drainage] Neurochirurgie 1991 ;  37 (3) : 179-184
Sato K., Endo T., Niizuma K., Fujimura M., Inoue T., Shimizu H., and al. Concurrent dural and perimedullary arteriovenous fistulas at the craniocervical junction: case series with special reference to angioarchitecture J Neurosurg 2013 ;  118 (2) : 451-459 [cross-ref]
Takahashi K., Hayashi S., Ootani T., Sera M., Negishi M. [Dural arteriovenous fistula manifesting as subarachnoid hemorrhage at the craniocervical junction. A case report] No Shinkei Geka 2008 ;  36 (10) : 901-906
Takami T., Ohata K., Nishio A., Nishikawa M., Goto T., Tsuyuguchi N., and al. Microsurgical interruption of dural arteriovenous fistula at the foramen magnum J Clin Neurosci 2005 ;  12 (5) : 580-583 [cross-ref]
Kim D.J., Willinsky R., Geibprasert S., Krings T., Wallace C., Gentili F., and al. Angiographic characteristics and treatment of cervical spinal dural arteriovenous shunts AJNR Am J Neuroradiol 2010 ;  31 (8) : 1512-1515 [cross-ref]
Gross B.A., Ropper A.E., Du R. Cerebral dural arteriovenous fistulas and aneurysms Neurosurg Focus 2012 ;  32 (5) : E2
Krings T., Geibprasert S. Spinal dural arteriovenous fistulas AJNR Am J Neuroradiol 2009 ;  30 (4) : 639-648 [cross-ref]
Lasjaunias P., Valle B., Person H. The lateral spinal artery of the upper cervical spinal cord JNS 1985 ;
Kai Y., Hamada J., Morioka M., Yano S., Mizuno T., Kuratsu J. Arteriovenous fistulas at the cervicomedullary junction presenting with subarachnoid hemorrhage: six case reports with special reference to the angiographic pattern of venous drainage AJNR Am J Neuroradiol 2005 ;  26 (8) : 1949-1954
Rodesch G., Hurth M., Alvarez H., Tadie M., Lasjaunias P. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal: the Bicetre experience with 155 consecutive patients treated between 1981 and 1999 Neurosurgery 2002 ;  51 (2) : 374-379[Discussion 79-80].  [cross-ref]
Aviv R.I., Shad A., Tomlinson G., Niemann D., Teddy P.J., Molyneux A.J., and al. Cervical dural arteriovenous fistulae manifesting as subarachnoid hemorrhage: report of two cases and literature review AJNR Am J Neuroradiol 2004 ;  25 (5) : 854-858
Fassett D.R., Rammos S.K., Patel P., Parikh H., Couldwell W.T. Intracranial subarachnoid hemorrhage resulting from cervical spine dural arteriovenous fistulas: literature review and case presentation Neurosurg Focus 2009 ;  26 (1) : E4
Hassler W., Thron A., Grote E.H. Hemodynamics of spinal dural arteriovenous fistulas. An intraoperative study J Neurosurg 1989 ;  70 (3) : 360-370 [cross-ref]
Duffau H., Lopes M., Janosevic V., Sichez J.P., Faillot T., Capelle L., and al. Early rebleeding from intracranial dural arteriovenous fistulas: report of 20 cases and review of the literature J Neurosurg 1999 ;  90 (1) : 78-84 [cross-ref]
Kawaguchi T., Nakatani M., Kawano T. Study of dural arteriovenous fistula drains into leptomeningeal vein without sinus interposition Interv Neuroradiol 2004 ;  10 (Suppl. 1) : 127-134
Ringer A.J., Salud L., Tomsick T.A. Carotid cavernous fistulas: anatomy, classification, and treatment Neurosurg Clin N Am 2005 ;  16 (2) : 279-295[viii].  [inter-ref]



© 2013  Elsevier Masson SAS. All Rights Reserved.
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Close
Article Outline
You can move this window by clicking on the headline