Abstract
CSF venous fistula (CVF) is a rare cause of spontaneous intracranial hypotension (SIH) which is estimated to be found in 2.5% of patients with orthostatic headaches.1 In patients with persistent SIH symptoms, advanced imaging techniques such as magnetic resonance (MR) or computer tomography (CT) myelography are recommended to detect the location of the cerebrospinal fluid (CSF) leak. However, up to 10–15% of patients may not have a visible leak on conventional imaging.2 3 This obviates the need for more advanced spinal imaging with intrathecal contrast administration to identify the presence of perineural cysts and draining venous networks. The main imaging modality for the diagnosis of CVFs is digital subtraction myelography (DSM), wherein the CVF is seen as a vessel filling with contrast, usually arising from a nerve root sleeve.
We describe a case of a 76-year old male who presented with chronic orthostatic headaches in which initial brain imaging demonstrated features of SIH but with no further evidence of CSF leak on conventional spinal imaging. Subsequent DSM confirmed the presence of a T9 perineural cyst with an associated CVF, in the setting of multiple thoracic perineural pseudocysts. Surgical correction with coagulation of the draining vessel, has led to improvement of his symptoms.
References
Schievink WI, Maya MM, Jean-Pierre S, Nuño M, Prasad RS, Moser FG. A classification system of spontaneous spinal CSF leaks. Neurology. 2016 Aug 16;87(7):673–9.
Schievink WI, Moser FG, Maya MM. CSF-venous fistula in spontaneous intracranial hypotension. Neurology. 2014 Jul 29;83(5):472–3.
Goldberg J, Häni L, Jesse CM, Zubak I, Piechowiak EI, Gralla J, et al. Spontaneous Intracranial Hypotension Without CSF Leakage—Concept of a Pathological Cranial to Spinal Fluid Shift. Front Neurol. 2021 Nov 1;12:760081.