• History and Examination 
     
    History
    • 66yo right handed female presents with sudden right arm and right thoracic pain with accompanied weakness/paresthesias.
    • Impaired hand dexterity (unable to hold items in right hand).
    • Accompanied shortly after with bilateral leg weakness and falls.
    • Describes a band of numbness around her lower abdomen and lumbar spine.
    • Inability to feel herself urinate, but was not incontinent.
    • PMH: Chronic obstructive pulmonary disease, Hypertension, bipolar
     
    Physical Exam
    • Right upper extremity: 4/5 hand grip. 4/5 interossei
    • Left upper extremity: 5/5
    • Right lower extremity: 4+/5 hip flexor. 4+/5 Knee flexor
    • Left lower extremity: 4+/5 hip flexor
    • Decreased light touch bilaterally over posterior leg
    • Decreased light touch and pin prick T5-T12 distribution
    • 3+ patella reflexes bilaterally
    • Three beats of bilateral clonus
    • Toes equivocal
    • Remainder of exam normal
    • She had evaluation by ophthalmology and was found to have bilateral papilledema
    • Further imaging was obtained

    Figure 1

    Figure 2

    Intraoperative images demonstrating fistula originating from the dural posterior the CN XI

    ICG-VA pre clipping

    ICG-VA post clipping

    Delayed postop angiogram and cervical MRI demonstrating fistula obliteration and resolution of spinal cord edema.

    1. What is the most likely diagnosis?

    2. What is the reasoning behind the sudden onset of symptoms?

    3 .These lesions are typically supplied by what vessel?

    4. What would be the most ideal surgical approach for clipping?

    5. Which of the following describes you?

    6. I practice in one of the following locations.

    7. Comments

    • Excellent Case
    • Good
    • Thank you for this interesting case
    • Check on G. Yasargil articles in the 1970's English and German literature
    • Ischemia is a restriction in blood supply to tissues, causing a shortage of oxygen and glucose needed for cellular metabolism (to keep tissue alive). hemorrhage would cause ischemia and impaired neural function. I would speak to a neuro-radiologist especially regarding figure 2. the origin of the fistula is higher up in the VA. hence retro-sigmoid would be ideal. the intra-operative picture says CN 11, I would have thought jugular foramen is close by hence a far-lateral approach would be reasonable if the origin of the fistula is around this part of VA. the actual point of obliteration is ideally the arterialized medullary vein because at fistula point there can be a medullary branch to the cord.
    • A good case
    • I would like to know, if it is possible to share for authors, how to treat or manage the spinal cord edema after fistula oclussion?
    • Interesting, and appreciate this educational effort about something that harkens back to training, but something I do not practice routinely. 
    • Case Explanation:


      •Most spinal dural AVFs occur in males (31-74yr) at the thoracolumbar region.  Intracranial foramen magnum spinal dural AVFs with perimedullary venous drainage are rare.  They are most commonly supplied by the external carotid, occipital and meningeal branches of the vertebral artery (63% from the right VA).  The clinical presentation ranges from SAH (34-45% cases) to progressive myelopathy (50% cases) secondary to venous congestion and spinal cord ischemia.  The acute development of symptoms, together with imaging, led us to believe that there was a possible hemorrhage with underlying edema/ischemia of the cervical cord.
      • Cervical and Thoracic MRI demonstrated a dilation of the perimedullary venous plexus. Diagnostic angiography revealed an AV fistula at the V4 segment of the right vertebral artery.  Endovascular embolization was unsuccessful, therefore, the patient underwent a far lateral supracondylar approach for clip ligation.  A fistula  was identified intraoperatively and confirmed pre and post ligation with indocyanine green video (ICG) angiography (fig1-3).
      •Treatment options includes endovascular embolization, clip ligation or a combination. Surgical ligation may be preferred secondary to numerous FM meningeal anastomosis, small target vessel caliber, risk of embolic agent reflux and fistula recanalization. Treatment modality and surgical approach should be individualized to patient pathology and surgeon/interventionalist familiarity.
       

      References:

      •Guo LM, Zhou HY, Xu JW, Wang GS, Tian X, Wang Y, Qiu YM, Jiang JY.  Dural arteriovenous fistula at the foramen magnum presenting with subarachnoid hemorrhage: case reports and literature review. Eur J Neurol. 2010 May;17(5):684-91. doi: 10.1111/j.1468-1331.2009.02895.x. Epub 2009 Dec 29.

      •Ryo H, Terumasa K, Kazuhiko N, Toshihiko K.  Foramen Magnum Dural Arteriovenous Fistula Treated by a Microsurgical Technique Combined with a Feeder Occlusion Using Transarterial Coil Embolization.  Turk Neurosurg 2015, Vol: 25, No: 6, 971-975.
      •Asakawa H, Yanaka K, Fujita K, Marushima A, Anno I, Nose T. Intracranial dural arteriovenous fistula showing diffuse MR en- hancement of the spinal cord: Case report and review of the literature. Surg Neurol 2002;58:251–7.
      •Lee J, Cho Y, Kwon B, Han M. Dural Arteriovenous Fistula at the Foramen Magnum with Holocord Myelopathy: Case Report. Neurointervention 2010;5:53-57.

         

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