Bilateral Vertebral Arteriovenous Fistulas and Atlantoaxial Dislocation associated with Neurofibromatosis

Abstract

The authors report a rare case involving neurofibromatosis associated with bilateral vertebral arteriovenous fistulas and atlantoaxial dislocation. Multiple neurofibromas in the craniocervical junc tion appeared to be the cause of the atlantoaxial dislocation in this patient, and the dislocation might have contributed to the formation of the bilateral vertebral arteriovenous fistulas. The fistulas were suc cessfully treated by a combination of balloon embolization and surgery followed by occipitocervical fu sion.

Endovascular treatment for bilateral vertebral arteriovenous fistulas in neurofibromatosis 1

“…reported a case of NF similar to the subject of the present paper with bilateral vertebral arteriovenous fistulas and atlantoaxial dislocation. However, the treatment in this case was carried out by combined surgical and endovascular means 8 . Our patient was treated by endovascular technique alone.…”

“…The patient was treated successfully with endovascular treatment in the form of embolization of bilateral fistulas with balloons and coils. A review of the published literature reveals only one similar case of bilateral vertebral arteriovenous fistulas in NF1 reported previously 8 .…”

Magnetic resonance imaging findings in an unusual case of atlanto axial dislocation and vertebral artery–vein fistulas in a patient of neurofibromatosis‐1

“…Multiple neurofibromas in the craniocervical junction appeared to be the cause of the atlantoaxial dislocation in this patient, and the dislocation might have contributed to the formation of the bilateral vertebral arteriovenous fistulas. Hasegawa H et al 5 reported a similar case in which there was presence of vertebral artery and vein fistula in the setting of neurofibromatosis and atlantoaxial dislocation. We feel that the present case might be similar to this extremely rare condition.…”

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“…The mechanism of myelopathy in VVAVFs can result from direct spinal cord compression from epidural venous engorgement, and/or venous hypertension from pial venous reflux [18,26]. Undoubtfully, the mechanism of severe cervical cord compression in our with resolution of cervical cord compression.…”

“…case can occur from forceful epidural compression from bilateral dilated epidural venous pouches. The large epidural venous pouch compressing spinal cord from spontaneous VVAVF was found more frequently in the fistula associated with NF1 [21,[26][27][28][29][30][31].…”