Cerebrospinal fluid venous fistulae (CSF-VF) are an uncommon yet increasingly recognized cause of spontaneous intracranial hypotension (SIH) [1-5]. Workup involves MRI brain with and without contrast and MRI of the neuroaxis without contrast before dynamic myelography, either CT or digital subtraction.[6] The present case of a older female with symptomatic intracranial hypotension (SIH) is notable for the specific appearance of CSF-VF on digital spinal myelography (DSM). Among her numerous perineural cysts, it was the "disappearing" or "empty" cyst from which the fistula originated. The diagnosis was made using a second lateral fluoroscopy view, not typically employed in DSM, which demonstrated emptying of contrast from the T6 perineural cyst into the segmental vein at this level, or the "Empty Cyst Sign." The patient then underwent transvenous onyx embolization[7] with resolution of orthostatic headaches and improvement of contrasted brain MRI from a Bern score of 7 to 0 at 3-months follow-up. As transvenous embolization of CSF-VF is a relatively new procedure, the long term outcomes of the procedure are not yet known.
Keywords: CSF-VF; digital spinal myelography; embolization; spontaneous intracranial hypotension.
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