Open Repair of Hemangioma-Associated Cerebrospinal Fluid-Venous Fistula.

Abstract

A 56-year-old woman was diagnosed with insidious onset new daily persistent headache associated with bilateral tinnitus. Her headache had orthostatic features improved with the recumbent position or caffeine intake. Medical work up consistent with spontaneous intracranial hypotension (SIH) included magnetic resonance imaging (MRI) of the brain with sagging and pachymeningeal enhancement, as well as lumbar puncture with opening pressure of 0 cm H2O. Additional imaging noted a right-sided chest wall pleural-based mass, and she underwent video-assisted thoracoscopic surgery where the biopsies were nondiagnostic, but a benign hemangioma was suspected (Fig A, B). Over the next 5 years, her headaches persisted. Conventional computed tomography (CT) myelogram did not identify a cerebrospinal fluid (CSF) leak; however, she had a positive response to epidural blood patching (EBP). Unfortunately, the EBP improved her symptoms only temporarily, and she progressed to photophobia, phonophobia, and decreased daily cognitive function. She was subsequently referred to our hospital where a pre-operative digital subtraction myelogram demonstrated a CSF-venous fistula communicating with the thoracic wall vascular malformation (Fig C). She then underwent a right T3-4 fibrin glue injection (FGI) directed at the venous fistula with some relief of her symptoms, however, her orthostatic headaches, photophobia, phonophobia, and decreased cognitive function persisted. She then underwent a T3-4 hemilaminotomy and foraminotomy and ligation of the CSF-venous fistula with 3 titanium aneurysm clips. Postoperatively, her orthostatic headache improved on postoperative day (POD)1. On POD2, she converted to rebound high-pressure headaches consistent with improvement in brain sag and meningeal enhancement on a postoperative brain MRI completed that day (Fig E) compared to a pre-operative MRI 1 month before (Fig D). Four weeks after surgery she had mild high-pressure headaches, which are being managed medically. SIH is increasingly recognized as a cause of new daily persistent headaches. If a CSF leak cannot be identified on imaging, a diagnosis of CSF leak and SIH consists of meeting radiographic criteria, such as enhancement of the pachymeninges or sagging of the brain, and/or a constellation of clinical signs, including low opening pressure on lumbar puncture. Known causes of SIH include dural tears, meningeal diverticula, and CSF-venous fistulas, however, the etiology of many cases of SIH remain indeterminate or unknown.1 Recently, reports suggest that venous and venolymphatic vascular malformations may underly the pathological development of CSF-venous fistulas and likely represent a previously unidentified class of lesions associated with CSF leak.2 There have been reports of at least 2 patients with Klippel-Trenaunay syndrome and SIH due to epidural venolymphatic malformations, suggesting multiple etiologies of vascular lesion-associated intracranial hypotension.3 Although many patients with SIH due to a CSF leak experience symptomatic relief from bedrest or EBP, the effects may be temporary. Additional treatments include FGI, an effective option for cases refractory to conservative measures.4 Despite temporary symptomatic relief after nonoperative interventions in patients with SIH, many may require surgical intervention, such as ligation of the CSF diverticulum or venous fistula. This case illustrates effective management of a CSF-venous fistula associated with a vascular malformation and demonstrates the importance of open operative intervention for definitive treatment of CSF leaks associated with vascular lesions refractory to less invasive FGI. None. W.S. contributed to the conception and design of the study. W.S., M.M., and J.C. contributed to the acquisition and analysis of data. W.S. and J.C. contributed to drafting the text and preparing the figures. The authors declared no conflict of interest. Video S1: Lateral decubitus digital subtraction myelogram showing a robust spinal CSF-venous fistula draining into a chest wall hemangioma. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.