Clinical and Imaging Outcomes of Surgically Repaired Cerebrospinal Fluid-Venous Fistulas Identified by Lateral Decubitus Digital Subtraction Myelography.

OBJECTIVE: To describe clinical and radiographic outcomes of surgical repair of cerebrospinal fluid-venous fistula (CVF), an increasingly recognized cause of spontaneous intracranial hypotension that is poorly responsive to epidural blood patch (EBP). METHODS: Retrospective review identified adult patients who had lateral decubitus digital subtraction myelography indicative of cerebrospinal fluid leak at Mayo Clinic between November 2018 and February 2020, with clearly localized CVF, followed by surgical treatment. Patients without available imaging before or after surgery were excluded. History of EBP and clinical response to EBP were evaluated along with surgical outcomes. RESULTS: Of 25 patients with CVF who met protocol criteria and were included in the data analysis, 22 (88%) received EBP, but clinical benefit lasting ≥4 weeks occurred in only 2 of 22 (9%). Headache was the most prominent preoperative feature among patients (24/25; 96%). Following surgery, 18 of 24 (75%) patients had complete headache improvement, 4 (17%) had partial improvement, and 2 (8%) had no improvement. Ten of 25 (40%) patients reported cognitive disturbance at baseline; at follow-up, 5 of 10 (50%) had complete improvement, 3 (30%) had partial improvement, and 2 (20%) had no improvement. On postoperative brain magnetic resonance imaging, 6 of 25 (24%) patients had complete resolution of findings by Bern score criteria, 18 (72%) showed partial improvement, and 1 (4%) patient showed no improvement. Adverse events were minor and included surgical site pain and paresthesias. CONCLUSIONS: Surgical repair of CVF resulted in improvements in headache and other symptoms, with few side effects.

Cerebrospinal Venous Fistula Presenting with Cognitive Decline: Systematic Literature Review and Report of Two Cases.

OBJECTIVE: A cerebrospinal fluid (CSF) venous fistula (CVF) is an aberrant connection between the subarachnoid space and a vein resulting in CSF loss. The presentation and management of CVF with cognitive decline is incompletely understood. METHODS: A systematic review was completed following the PRISMA guidelines. Articles that included at least 1 case of imaging-confirmed CVF with details on patient treatment were included. A separate review of cases of patients with spontaneous intracranial hypotension (SIH) with frontotemporal dementia (FTD) or dementia symptoms was also completed. RESULTS: Ten CVF articles (69 patients; average age, 51.5 years) and 5 SIH with FTD or dementia articles (n = 41; average age, 55.9 years) were identified. Only 1 patients with CVF with cognitive abnormalities was identified. The most common symptom was headache in both reviews. Brain sag was identified in all patients, whereas CSF leak was identified in only 2 patients with SIH with FTD or dementia (4.9%). An epidural blood or fibrin glue patch was used in all patients with CVF and in 33 patients with SIH with FTD or dementia. Fifty-five patients with CVF (79.7%) and 27 patients with SIH with FTD or dementia (65.9%) had surgery. CONCLUSIONS: The 2 cases and literature reviews show the difficulty in diagnosis and treatment of CVF with cognitive decline. Novel imaging techniques should be used in patients with cognitive decline in whom a CSF leak is suspected. Transvenous embolization or surgery should be considered before patching for treatment of CVF-induced brain sag and resulting dementia.

Bilateral Subdural Hygromas After Deep Brain Stimulation Implantation in the Setting of Unrecognized Intracranial Hypotension.

Cerebrospinal fluid (CSF)-venous fistulas are a recently recognized cause of spontaneous spinal CSF leak and present most commonly with Valsalva ("cough")-exacerbated or orthostatic headaches. By inducing CSF hypotension, they cause diffuse pachymeningeal enhancement and brain sag on MRI. This unusual case demonstrates the potential for bilateral subdural hygroma development in a patient with an undiagnosed CSF-venous fistula after ventral intermediate nucleus (VIM) deep brain stimulation (DBS) implantation. A 68-year-old gentleman with medically-refractory essential tremor underwent extensive preoperative evaluation by the Mayo Clinic-Rochester DBS Committee. Initial MRI during preoperative evaluation had no evidence of CSF hypotension, but MRI performed the day before surgery demonstrated diffuse pachymeningeal enhancement. He underwent bilateral VIM DBS implantation and presented in the subacute postoperative period with bilateral subdural hygromas. Further testing identified a prominent hyperdense paraspinal vein arising from the T10/T11 nerve root, consistent with CSF-venous fistula. Even when patients undergo rigorous preoperative evaluations for surgical procedures, insidious pathologies can develop and cause unexpected postoperative complications.

Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula.

BACKGROUND: The frontotemporal brain sagging syndrome (FTBSS) is defined as an insidious/progressive decline in behavior and executive functions, hypersomnolence, and orthostatic headaches attributed to cerebrospinal fluid (CSF) hypovolemia. Here, a T6 CSF-venous fistula (e.g., between the subarachnoid CSF and a paraspinal vein) resulted in a CSF leak responsible for craniospinal hypovolemia. CASE DESCRIPTION: A 56-year-old male started with orthostatic headaches and fatigue after scuba diving. His symptoms included progressive, vertigo, tinnitus, nausea, lack of judgment, inappropriate behavior, memory dysfunction, apathy, tremor, orofacial dyskinesia, dysarthria, dysphagia, and hypersomnolence. The lumbar puncture revealed an opening pressure of 0 cm H2O. Magnetic resonance imaging (MRI) findings included brain sagging, bilateral temporal lobe herniation, and pachymeningeal enhancement. The computed tomography (CT) myelogram showed a thoracic diverticulum and a CSF-venous leak at the T6-T7 level. Surgery, which comprised a T6-T7 laminotomy, allowed for dissecting, clipping, and ligating the diverticulum/fistula. The patient improved postoperatively (e.g., cognitive, behavioral, and brainstem symptoms). The follow-up MRI's showed the reversion of the sagging index/uncal herniation. CONCLUSION: The FTBSS should be considered in the differential diagnosis of an early onset frontotemporal dementia. Establishing the diagnosis and localizing the site of a spinal CSF/venous leak warrant both MRI and myelogram CT studies, to pinpoint the CSF leak site for proper surgical clipping/ligation of these thoracic diverticulum/CSF-venous leaks.

Clinical Outcomes Following Surgical Ligation of Cerebrospinal Fluid-Venous Fistula in Patients With Spontaneous Intracranial Hypotension: A Prospective Case Series.

BACKGROUND: Cerebrospinal fluid-venous fistula (CVF) is a recently described cause of spontaneous intracranial hypotension (SIH). Surgical ligation of CVF has been reported, but clinical outcomes are not well described. OBJECTIVE: To determine the clinical efficacy of surgical ligation for treatment of CVF. METHODS: Outcomes metrics were collected in this prospective, single-arm, cross-sectional investigation. Inclusion criteria were as follows: diagnosis of SIH, demonstration of CVF on myelography, and surgical treatment of CVF. Pre- and postoperative headache severity was assessed with the Headache Impact Test (HIT-6), a validated headache scale ranging from 36 (asymptomatic) to 78 (most severe). Patient satisfaction with treatment was measured with Patient Global Impression of Change (PGIC). RESULTS: Twenty subjects were enrolled, with mean postoperative follow-up at 16.0 ± 9.7 mo. All CVFs were located in the thoracic region (between T4 and T12). Pretreatment headache severity was high (mean HIT-6 scores 65 ± 6). Surgical treatment resulted in marked improvement in headache severity (mean HIT-6 change of -21 ± -9, mean postoperative HIT-6 of 44 ± 8). Of subjects with baseline headache scores in the most severe category, 83% showed a major improvement in severity (transition to the lowest 2 severity categories) after surgery. All subjects (100%) reported clinically significant levels of satisfaction with treatment (PGIC score 6 or 7); 90% reported the highest level of satisfaction. There were no short- or long-term complications or 30-d readmissions. CONCLUSION: Surgical ligation is highly effective for the treatment of SIH due to CVF. Larger controlled trials with longer follow-up period are indicated to better assess its long-term efficacy and safety profile.

False localizing signs of spinal CSF-venous fistulas in spontaneous intracranial hypotension: report of 2 cases.

A spinal CSF-venous fistula is one of three specific types of spinal CSF leak that can be seen in patients with spontaneous intracranial hypotension (SIH). They are best demonstrated on specialized imaging, such as digital subtraction myelography (DSM) or dynamic myelography, but often they are diagnosed on the basis of increased contrast density in the draining veins (the so-called hyperdense paraspinal vein sign) on early postmyelography CT scans. The authors report on 2 patients who underwent directed treatment (surgery in one patient and glue injection in the other) based on the hyperdense paraspinal vein sign, in whom the actual site of the fistula did not correspond to the level or laterality of the hyperdense paraspinal vein sign. The authors suggest consideration of DSM or dynamic myelography prior to undertaking treatment directed at these fistulas.