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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 50  |  Issue : 6  |  Page : 223-226

Craniocervical junction spinal dural arteriovenous fistula presenting with progressive myelopathy and sudden deterioration


1 Department of Surgery, Division of Neurosurgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
2 Department of Nursing, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
3 Department of Medical Image, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
4 Department of Surgery, Division of Neurosurgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University; Department of Surgery, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan

Date of Submission13-Dec-2016
Date of Decision24-Jan-2017
Date of Acceptance24-May-2017
Date of Web Publication08-Dec-2017

Correspondence Address:
Prof. Ann-Shung Lieu
No. 100, Tzyou 1st Road, Kaohsiung 807
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_98_17

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  Abstract 


Spinal dura arteriovenous fistula (SDAVF) is not an uncommon vascular malformation but is rare at craniocervical junction. The initial presentation is usually subarachnoid hemorrhage or cervical myelopathy caused by venous hypertension of the drainage vein. We report a male patient who has sudden onset of paraplegia, anesthesia of bilateral lower limbs, and urine retention. Cervical spinal cord edema and engorgement of posterior and anterior spinal veins were noted on magnetic resonance image study. Angiogram disclosed a SDAVF feeding by meningeal branch of the left vertebral artery and drainage inferiorly through posterior and anterior spinal vein. Complete obliteration of the fistula was achieved by transarterial embolization with n-butyl cyanoacrylate. The muscle power of lower limbs improved after the embolization. In previous studies, functional outcome is most correlated with pretreatment condition. Early diagnosis and treatment of the lesion will prevent permanent neurological deficit.

Keywords: Craniocervical junction, spinal dural arteriovenous fistula, treatment


How to cite this article:
Lin IC, Wu HC, Wu CH, Lin WC, Lieu AS. Craniocervical junction spinal dural arteriovenous fistula presenting with progressive myelopathy and sudden deterioration. Formos J Surg 2017;50:223-6

How to cite this URL:
Lin IC, Wu HC, Wu CH, Lin WC, Lieu AS. Craniocervical junction spinal dural arteriovenous fistula presenting with progressive myelopathy and sudden deterioration. Formos J Surg [serial online] 2017 [cited 2020 Sep 23];50:223-6. Available from: http://www.e-fjs.org/text.asp?2017/50/6/223/220351




  Introduction Top


Spinal dural arteriovenous fistula (SDAVF) is the most common type of spinal vascular malformation and generally occurs in the thoracolumbar region. Craniocervical junction SDAVF is rare and may present with subarachnoid hemorrhage, brain stem congestion, or progressive myelopathy depending on the different venous drainage patterns.[1],[2],[3],[4],[5] Magnetic resonance imaging (MRI) may be the initial evaluation modality, but catheter angiography remains the gold standard for diagnosis. Treatment options include surgical excision or endovascular embolization of the fistula. Here, we report a case of craniocervical junction SDAVF treated with endovascular embolization. Although most SDAVF has a slowly progression course, sudden deterioration may occur as in our case.


  Case Report Top


A 53-year-old male patient had a history of neck soreness with radiation numbness of right arm for 5 months. Cervical spondylosis was the initial impression according to cervical spine X-ray, and he received conservative management. However, progressive weakness of all limbs with frequent falling down episodes occurred 3 days before he visited our emergency department. Neurological examination revealed quadriparesis (muscle power = 4) and Foley catheter was applied for acute urine retention. After admission, muscle power of both upper limbs improved, but sudden deterioration of both lower limbs was recorded (muscle power = 0). Deep tendon reflex showed general areflexia and bilateral Babinski signs were absent.

Cervical spine MRI showed increased signal intensity through C3–C7 cord on the T2-weighted image. T1-weighted image demonstrated signal voids posterior to the spinal cord from C2 to T3 level. After contrast administration, posterior spinal veins and spinal cord at C5C6 level were enhanced [Figure 1]. Digital subtraction angiography disclosed a craniocervical junction spinal dural arteriovenous fistula feeding by meningeal branch of the left vertebral artery and drainage downward through anterior and posterior spinal veins [Figure 2] and [Figure 3]. Under local anesthesia, endovascular embolization of the fistula was performed with n-butyl 2-cyanoacrylate (nBCA) smoothly. Angiography showed complete obliteration of the fistula and disappearance of the serpiginous vessels postembolization [Figure 4]. Muscle power of bilateral lower limbs improved gradually after embolization, and the patient could walk with assistance when discharged 3 weeks after embolization. Foley catheter was removed successfully 4 months after embolization. Follow-up MRI [Figure 5] 1 year after embolization revealed disappearance of the serpentine veins.
Figure 1: (a) T1-weighted magnetic resonance imaging in sagittal view shows signal voids (arrow) posterior to the spinal cord from C2 to T3 level (b) T2-weighted image shows high signal intensity (arrow)of cervical spinal cord through C3–C7 level (c) Enhanced T1-weighted image shows enhancement of cord over C5C6 level (arrow head)

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Figure 2:(a) Catheter angiography of left vertebral artery shows a spinal dural arteriovenous fistula supplied by meningeal branch of left vertebral artery (arrow) and downward drainage through the fistula (arrowhead) (b) T2-weighted image shows high signal intensity (arrow)

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Figure 3: The shunting point (a) meningeal branch (arrowhead) of left vertebral artery and shunting point (arrow) (b) fistula (arrow) (c) downward drainage through anterior and posterior spinal veins (arrowhead and arrow)

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Figure 4: Postembolization with n-butyl 2-cyanoacrylate. Angiogram shows total obliteration of the fistula and disappearance of the drainage vein

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Figure 5: T1-weighted (a) and T2-weighted (b) magnetic resonance imaging 1 year after embolization

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  Discussion Top


SDAVF are the most common and account for 70%–80% of all spinal vascular abnormalities. Mean age at the time of diagnosis is in the fifth and sixth decades with male predominance.[1],[4],[5],[6],[7] Craniocervical junction SDAVF is uncommon and reported to be 2% of SDAVFs.[4]

The exact etiology of SDAVF is still unknown. The pathophysiology is due to direct artery-venous shunting. It causes venous congestion/hypertension and then reduces perfusion pressure of the spinal cord, which leads to spinal cord ischemia and finally progresses to necrotizing myelopathy as the “Foix-Alajouanine syndrome” described.[4] Persistent ischemia of the vulnerable nerves will cause irreversible neurological injury. Acute exacerbation of symptoms is uncommon and has been reported by Hurst et al. and Jellema et al.[2],[4] It is suspected to be due to acute thrombosis of the drainage vein. However, in our case, there was no evidence of venous thrombosis on MRI image or angiogram. The diminishing perfusion pressure will finally lead to spinal cord infraction if left untreated. Single drainage vein without collaterals may contribute to higher risk of progression and early decompensation.

Subarachnoid hemorrhage and progressive myelopathy are the two major manifestations of craniocervical junction SDAVF due to the upward or downward drainage, respectively.[1],[2],[6],[7],[8] Other rare presentations such as 6th nerve palsy and brain stem dysfunction have also been reported.[3],[8]

MRI will disclose perimedullary serpentine signal voids on both T1- and T2-weighted image, indicative of the engorged arterialized drainage veins. Spinal cord edema often presents as increased intramedullary signal intensity. The spinal cord enhancement in our case was the evidence of chronic spinal cord congestion with disruption of blood-spinal cord barrier. Catheter angiography is the gold standard for diagnosis. It can demonstrate the real architecture of fistula and provide information about the feeding artery and the drainage pattern, which is important for further treatment, both surgical or endovascular. The feeding arteries of craniocervical junction SDAVF include cervical radicular or meningeal branches of vertebral artery and branches of external carotid artery.[1],[2],[6]

The goal of treatment is to eliminate venous congestion and preserve normal venous drainage as far as possible.[1] Two major strategies are surgical excision or endovascular embolization of the fistula. In general, both treatments provide similar long-term improvement rates, but surgery has a lower recurrence rate.[9] However, due to the rare cases, there is still no outcome comparing the two modalities for SDAVF at craniocervical junction, an anatomical unique region. For this patient, we selected transarterial embolization first because it is less invasive and the fistula is supplied by a single feeder on diagnostic angiography. Under local anesthesia, total obliteration of the fistula was performed successfully with injection of nBCA.

Factors associated with outcome are duration of symptoms, pretreatment neurologic condition, age, and success of the procedure to close the fistula.[4],[9] Muscle power and gait improvement are variable after treatment and has been reported from 50%–100%.[4],[10] Our patient had recovery from total paralysis to paraparesis (muscle power improved from 0 to 4; Aminoff-Logue disability scales = 5-4) after treatment. Early referral and timely treatment remain the most important factor of good outcome. We should be vigilant for the disease because it can present with fluctuate clinical course and acute deterioration, which leads to permanent disability if untreated.

There are still no guidelines for follow-up after endovascular treatment. We think MRI should be followed up for 3 months after complete obliteration of the fistula or when there is any clinical evidence of recurrence.


  Conclusion Top


Craniocervical SDAVFs are rare but are an important vascular malformation that has variable manifestation and fluctuating clinical course. Early treatment will avoid permanent neurological deficit and provide better outcome. As the advance of endovascular techniques and devices continue, more study is needed to evaluate the efficacy of surgical treatment and endovascular embolization.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Oishi H, Okuda O, Arai H, Maehara T, Iizuka Y. Successful surgical treatment of a dural arteriovenous fistula at the craniocervical junction with reference to pre- and postoperative MRI. Neuroradiology 1999;41:463-7.  Back to cited text no. 1
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2.
Hurst RW, Bagley LJ, Scanlon M, Flamm ES. Dural arteriovenous fistulas of the craniocervical junction. Skull Base Surg 1999;9:1.  Back to cited text no. 2
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3.
Wu Q, Wang HD, Shin YS, Zhang X. Brainstem congestion due to dural ateriovenous fistula at the craniocervical junction. J Korean Neurosurg Soc 2014;55:152-5.  Back to cited text no. 3
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4.
Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: A congestive myelopathy that initially mimics a peripheral nerve disorder. Brain 2006;129(Pt 12):3150-64.  Back to cited text no. 4
    
5.
Hacein-Bey L, Konstas AA, Pile-Spellman J. Natural history, current concepts, classification, factors impacting endovascular therapy, and pathophysiology of cerebral and spinal dural arteriovenous fistulas. Clin Neurol Neurosurg 2014;121:64-75.  Back to cited text no. 5
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6.
Igase K, Kumon Y, Shoda D, Fukumoto S, Ohue S, Ohnishi T. Dural arteriovenous fistula at the craniocervical junction has a close relationship with the vertebral artery. J Neurol Sci 2011;28:288-93.  Back to cited text no. 6
    
7.
Cao W, Huang L, Ge L, Lu G, Zhang X, Dong Q. A simple score (AVFS) to identify spinal dural arteriovenous fistula before spinal digital subtraction angiography. J Stroke Cerebrovasc Dis 2014;23:1995-2000.  Back to cited text no. 7
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8.
Kulwin C, Bohnstedt BN, Scott JA, Cohen-Gadol A. Dural arteriovenous fistulas presenting with brainstem dysfunction: Diagnosis and surgical treatment. Neurosurg Focus 2012;32:E10.  Back to cited text no. 8
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9.
Ruiz-Juretschke F, Perez-Calvo JM, Castro E, García-Leal R, Mateo-Sierra O, Fortea F, et al. Asingle-center, long-term study of spinal dural arteriovenous fistulas with multidisciplinary treatment. J Clin Neurosci 2011;18:1662-6.  Back to cited text no. 9
    
10.
Dekeyzer S, Lemmerling M, Fransen H, Dewilde D, Jans L, Verstraete K. A dural craniocervical fistula in a patient with progressive cervical myelopathy. JBR-BTR 2007;90:278-80.  Back to cited text no. 10
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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