Spontaneous intracranial hypotension due to CSF-venous fistula: Evaluation of renal accumulation of contrast following decubitus myelography and maintained decubitus CT to improve fistula localization.

PURPOSE: Presented here is a strategy of sequential lateral decubitus digital subtraction myelography (LDDSM) followed closely by lateral decubitus CT (LDCT) to facilitate cerebrospinal fluid (CSF)-venous fistula (CVF) localization. MATERIALS AND METHODS: This is a retrospective analysis of patients referred to our institution for evaluation of CSF leak. Patients with Type 1 and Type 2 leaks, and those not displaying MR brain stigmata of intracranial hypotension were excluded. All patients underwent consecutive LDDSM and LDCT. If the CVF was not localized on the first LDDSM-LDCT pair the patient returned for contralateral examinations. Images were reviewed for CVF and for accumulation of contrast within the renal pelvises expressed as a renal pelvis contrast score (RPCS) in Hounsfield units (HU). RESULTS: Twenty-two patients were included in this study. In 21 of 22 patients (95%) a CVF was identified yielding an RPCS for the LDDSM-LDCT pair ipsilateral to the CVF ranging from 71 to 423 with an average of 146 HU. An RPCS of the negative side LDDSM-LDCT pair contralateral to a CVF was available in 8 patients and averaged 51 HU. In 4 patients the initial bilateral LDDSM-LDCT pairs did not reveal the location of the CVF however in 3 of these 4 cases the CVF was revealed on a third LDDSM repeated ipsilateral to the higher RPCS. CONCLUSION: The strategy of sequential LDDSM-LDCT coupled with evaluation of renal accumulation of contrast agent appears to improve the rate of CVF localization and warrants further evaluation.

The presence of pachymeningeal hyperintensity on non-contrast flair imaging in patients with spontaneous intracranial hypotension.

PURPOSE: Traditionally, in the work-up of patients for spontaneous intracranial hypotension, T1 post-contrast imaging is performed in order to assess for pachymeningeal enhancement. The aim of this study is to assess whether pachymeningeal hyperintensity can be identified on a non-contrast FLAIR sequence in these patients as a surrogate sign for pachymeningeal enhancement. METHODS: The patient cohort was identified from a prospectively maintained database of patients with a clinical diagnosis of intracranial hypotension. Patients who had both a post-contrast T1 sequence brain as well as non-contrast FLAR sequence of the brain were reviewed. Imaging was retrospectively reviewed by three independent neuroradiologists. Each study was assessed for the presence or absence of pachymeningeal hyperintensity on the FLAIR sequence. RESULTS: From January 2010 to July 2022, 177 patients were diagnosed with spontaneous intracranial hypotension. In total, 121 were excluded as post-contrast imaging was not performed during their work-up. Twenty-four were excluded as the FLAIR sequence was performed after administration of contrast. Six were excluded as there was no pachymeningeal thickening present on T1 post-contrast imaging, although there were other signs of intracranial hypotension. The study group therefore consisted of 26 patients. Pachymeningeal thickening was correctly identified on the non-contrast FLAIR sequence in all patients (100%). CONCLUSION: Where present, diffuse pachymeningeal hyperintensity can be accurately identified on a non-contrast FLAIR sequence in patients with spontaneous intracranial hypotension. This potentially obviates the need for gadolinium base contrast agents in the work-up of these patients.

Minimally invasive surgery for spinal cerebrospinal fluid-venous fistula ligation: patient series.

BACKGROUND: Cerebrospinal fluid-venous fistulas (CVFs) may cause cerebrospinal fluid leaks resulting in spontaneous intracranial hypotension (SIH). Surgical treatment of CVFs aims to eliminate abnormal fistulous connections between the subarachnoid space and the epidural venous plexus at the level of the nerve root sleeve. The authors propose a percutaneous minimally invasive technique for surgical ligation of CVF as an alternative to the traditional open approach using a tubular retractor system. OBSERVATIONS: Minimally invasive surgical (MIS) ligation of spinal CVF was performed in 5 patients for 6 CVFs. The definite disconnection of the CVF was achieved in all patients by clipping and additional silk tie ligation of the fistula. None of the patients experienced surgical complications or required transition to an open procedure. One patient underwent 2 MIS procedures for 2 separate CVFs. Postoperative clinical follow-up and cranial magnetic resonance imaging confirmed resolution of symptoms and radiographic SIH stigmata. LESSONS: MIS ligation of CVFs is safe and efficient. It represents an elegant and less invasive procedure, reducing the risk of wound infections and time to recovery. However, preparedness for open ligation is warranted within the same surgical setting in cases of complications and difficult accessibility.

Spontaneous intracranial hypotension: searching for the CSF leak.

Spontaneous intracranial hypotension is caused by loss of CSF at the level of the spine. The most frequent symptom of this disorder is orthostatic headache, with the headache worsening in the upright position and subsiding after lying down. Neuroimaging has a crucial role in diagnosing and monitoring spontaneous intracranial hypotension, because it provides objective (albeit often subtle) data despite the variable clinical syndromes and often normal lumbar puncture opening pressure associated with this disorder. Spine imaging aims to classify and localise the site of CSF leakage as either (1) a ventral dural leak, (2) a leaking spinal nerve root diverticulum, or (3) a direct CSF-venous fistula. Searching for a CSF leak can be very difficult; the entire spine must be scrutinised for a dural breach often the size of a pin. Precisely locating the site of CSF leakage is fundamental to successful treatment, which includes a targeted epidural patch and surgical closure when conservative measures do not provide long-term relief. Increased awareness of spontaneous intracranial hypotension among clinicians highlights the need for dedicated diagnostic and therapeutic guidelines.

Digital Subtraction Myelography is Associated with Less Radiation Dose than CT-based Techniques.

PURPOSE: Both CT myelogram (CTM) and digital-subtraction myelogram (DSM) can be used to evaluate patients for possible cerebrospinal fluid (CSF) leaks. DSM is a relatively new technique. No data exists on the radiation dose associated with this procedure, and how it compares with CTM. MATERIALS AND METHODS: All patients who underwent DSM for spontaneous intracranial hypotension (SIH) refractory to blood patching from Dec 2016 - Sept 2019 were retrospectively assessed. DSM dose factors were then recorded (cumulative fluoroscopy time, total kerma area product (KAP, mGy.cm2), cumulative air kerma (mGy), as well as CTM dose factors (included CTDIvol (mGy) and dose-length product (DLP, mGy.cm). These indices were then used to calculate the effective dose for both procedures using standardized conversion factors. RESULTS: 61 DSMs were performed in 42 patients, 33 of which also underwent CTM. The median effective dose was 6.6 mSv per DSM study (range: 1.2 - 17.7). On a per-patient basis (i.e. those patients who underwent more than one DSM (as the initial one was negative), the median total effective dose was 13 mSv for their total DSM imaging (range: 2.6 -31.7). For the CTM, the median effective dose was 19.7 mSv (range: 3.2 - 82.4 mSv). CONCLUSION: The radiation dose with DSM appears to be significantly lower than that of CTM (p = 0.0005), when looking at CTM doses both from our institution and in the published literature.

Radial vs. Femoral Artery Access for Procedural Success in Diagnostic Cerebral Angiography : A Randomized Clinical Trial.

BACKGROUND AND PURPOSE: Radial artery access has become the standard of care in percutaneous coronary procedures due to demonstrated patient safety and comfort benefits; however, uptake of radial access for diagnostic cerebral angiography has been limited by practitioner concerns over the ability to achieve procedural success. We aimed to provide randomized clinical trial evidence for the non-inferiority of radial access to achieve procedural success. MATERIAL AND METHODS: Monocentric open label randomized controlled trial with a non-inferiority design and blinded primary outcome assessment. Adult patients referred in-hours for diagnostic cerebral angiography were eligible. Participants underwent permuted block randomization to radial or femoral artery access with an intention-to-treat analysis. The primary outcome was procedural success, defined as selective cannulation and/or diagnostic angiography of predetermined supra-aortic vessels of interest. The non-inferiority limit was 10.0%. Secondary outcomes included postprocedural complications, fluoroscopy and procedural times, radiation dose, contrast volume and rates of vertebral artery cannulation. RESULTS: A total of 80 participants were enrolled (female 42, male 38, mean age 47.0 years, radial access group n = 43, femoral n = 37). One patient in the radial group was excluded after enrollment due to insufficient sonographic radial artery internal diameter. Procedural success was achieved in 41 of 42 participants in the radial group (97.6%) and 36 of 37 in the femoral group (97.3%). The difference between groups was -0.3% (one-sided 95% confidence interval, CI 6.7%) and the null hypothesis was rejected. CONCLUSION: Radial artery access is non-inferior to femoral artery access for procedural success in cerebral angiography. A large multicenter trial is recommended as the next step.

Spontaneous spinal CSF-venous fistulas associated with venous/venolymphatic vascular malformations: report of 3 cases.

Spontaneous CSF-venous fistulas may be present in up to one-fourth of patients with spontaneous intracranial hypotension. This is a recently discovered type of CSF leak, and much remains unknown about these fistulas. Spinal CSF-venous fistulas are usually seen in coexistence with a spinal meningeal diverticulum, suggesting the presence of an underlying structural dural weakness at the proximal portion of the fistula. The authors now report the presence of soft-tissue venous/venolymphatic malformations associated with spontaneous spinal CSF-venous fistulas in 2 patients with spontaneous intracranial hypotension, suggesting a role for distal venous pathology. In a third patient with spontaneous intracranial hypotension and a venolymphatic malformation, such a CSF-venous fistula is strongly suspected.

Lateral decubitus digital subtraction myelography to identify spinal CSF-venous fistulas in spontaneous intracranial hypotension.

OBJECTIVE: Spontaneous spinal CSF-venous fistulas are a distinct type of spinal CSF leak recently described in patients with spontaneous intracranial hypotension (SIH). Using digital subtraction myelography (DSM) with the patient in the prone position, the authors have been able to demonstrate such fistulas in about one-fifth of patients with SIH in whom conventional spinal imaging (MRI or CT myelography) showed no evidence for a CSF leak (i.e., the presence of extradural CSF). The authors compared findings of DSM with patients in the lateral decubitus position versus the prone position and now report a significantly increased yield of identifying spinal CSF-venous fistulas with this modification of their imaging protocol. METHODS: The population consisted of 23 patients with SIH who underwent DSM in the lateral decubitus position and 26 patients with SIH who underwent DSM in the prone position. None of the patients had evidence of a CSF leak on conventional spinal imaging. RESULTS: A CSF-venous fistula was demonstrated in 17 (74%) of the 23 patients who underwent DSM in the lateral decubitus position compared to 4 (15%) of the 26 patients who underwent DSM in the prone position (p < 0.0001). The mean age of these 16 women and 5 men was 52.5 years (range 36-66 years). CONCLUSIONS: Among SIH patients in whom conventional spinal imaging showed no evidence of a CSF leak, DSM in the lateral decubitus position demonstrated a CSF-venous fistula in about three-fourths of patients compared to only 15% of patients when the DSM was performed in the prone position, an approximately five-fold increase in the detection rate. Spinal CSF-venous fistulas are not rare among patients with SIH.

Management of multiple meningiomas.

OBJECTIVE Multiple meningiomas account for 1%-10% of meningiomas. This study describes epidemiological aspects of the disease and its management, which is more challenging than for single tumors. METHODS A consecutive series of adult patients with ≥ 2 spatially separated meningiomas was reviewed. Patients with neurofibromatosis Type 2 were excluded. The authors collected clinical, imaging, histological, and treatment data to obtain information on epidemiology, management options, and outcomes of active treatment and surveillance. RESULTS A total of 133 consecutive patients were included over 25 years, with a total of 395 synchronous and 53 metachronous meningiomas, and a median of 2 tumors per patient. One hundred six patients had sporadic disease, 26 had radiation-induced disease, and 1 had familial meningiomatosis. At presentation, half of the patients were asymptomatic. In terms of their maximum cross-sectional diameter, the tumors were small (≤ 2 cm) in 67% and large (> 4 cm) in 11% of the meningiomas. Fifty-four patients had upfront treatment, and 31 had delayed treatment after an observation period (mean 4 years). One in 4 patients had ≥ 2 meningiomas treated. Overall, 64% of patients had treatment for 142 tumors-67 with surgery and 18 with radiotherapy alone. The mean follow-up was 7 years, with 13% of treated patients receiving salvage therapy. Approximately 1 in 4 patients who underwent surgery had ≥ 1 WHO Grade II or III meningioma. Meningiomas of different histological subtypes and grades in the same patient were not uncommon. CONCLUSIONS Multiple meningiomas are often asymptomatic, probably because the majority are small and a significant proportion are induced by radiation. Approximately two-thirds of patients with multiple meningiomas require therapy, but only one-third of all meningiomas need active treatment. The authors recommend surveillance for stable and asymptomatic meningiomas and therapy for those that are symptomatic or growing.

Spontaneous Intracranial Hypotension: A Review and Introduction of an Algorithm For Management.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a condition of low cerebrospinal fluid volume and pressure caused by a leak of cerebrospinal fluid through a dural defect. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines for myelography, blood patching, and possible surgical repair. Patients should be monitored closely, because they can deteriorate into a coma or even death. There are no widely accepted guidelines for the management of SIH. METHODS AND CONCLUSIONS: We review the existing SIH literature, illustrate management challenges via a case review, and propose an algorithm developed by neurosurgeons, radiologists, and anesthesiologists intended to simplify and streamline the management of SIH.

Mediators of the Age Effect in the Carotid Revascularization Endarterectomy Versus Stenting Trial (CREST).

BACKGROUND AND PURPOSE: There is higher combined risk of stroke or death (S+D) at older ages with carotid stenting. We assess whether this can be attributed to patient or arterial characteristics that are in the pathway between older age and higher risk. METHODS: Mediation analysis of selected patient (hypertension, diabetes mellitus, and dyslipidemia) and arterial characteristics assessed at the clinical sites and the core laboratory (plaque length, eccentric plaque, ulcerated plaque, percent stenosis, peak systolic velocity, and location) was performed in 1123 carotid artery stenting-treated patients in the Carotid Revascularization Endarterectomy Versus Stenting Trial (CREST). We assessed the association of age with these characteristics, the association of these characteristics with stroke risk, and the amount of mediation of the association of age on the combined risk of periprocedural S+D with adjustment for these factors. RESULTS: Only plaque length as measured at the sites increased with age, was associated with increased S+D risk and significantly mediated the association of age on S+D risk. However, adjustment for plaque length attenuated the increased risk per 10 years of age from 1.72 (95% confidence interval, 1.26-2.37) to 1.66 (95% confidence interval, 1.20-2.29), accounting for only 8% of the increased risk. CONCLUSIONS: Plaque length seems to be in the pathway between older age and higher risk of S+D among carotid artery stenting-treated patients, but it mediated only 8% of the age effect excess risk of carotid artery stenting in CREST. Other factors and mechanisms underlying the age effect need to be identified as plaque length will not identify elderly patients for whom stenting is safe relative to endarterectomy. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00004732.

The accuracy and utility of contrast-enhanced MR angiography for localization of spinal dural arteriovenous fistulas: the Toronto experience.

OBJECTIVES: The purpose of this study was to determine the accuracy and utility of contrast-enhanced MR angiography (CE-MRA) in spinal dural arteriovenous fistulas (SDAVF). METHODS: A retrospective analysis from 1999-2012 identified 70 patients clinically suspected of harboring a SDAVF. Each patient underwent consecutive conventional MR-imaging, CE-MRA, and digital subtraction angiography (DSA). The presence or absence of serpentine flow voids, T2-weighted hyperintensity, and cord enhancement were evaluated, as well as location of the fistula as predicted by CE-MRA. DSA was used as the reference standard. RESULTS: Of the 70 cases, 53 were determined to be a SDAVF, 10 cases were shown to be other forms of vascular malformation, and 7 were DSA-negative. On MRI, all reported cases of SDAVF showed serpentine flow voids (100 %). T2-weighted hyperintensity was seen in 48 of 50 cases (96 %), extending to the conus in 41 of 48 cases (85 %). Cord enhancement was seen in 38 of 41 cases (93 %). CE-MRA correctly localized the SDAVF in 43 of the 53 cases (81 %). CONCLUSIONS: CE-MRA is a useful non-invasive examination for the detection and localization of SDAVF. CE-MRA facilitates but does not replace DSA as confirmation of location, fistula type, and arterial detail, which are required before treatment. KEY POINTS: • CE-MRA correctly localized the site of the SDAVF in over 80 % of cases. • CE-MRA facilitates diagnostic DSA and expedites the diagnostic process. • CE-MRA does not replace diagnostic DSA in SDAVF cases as confirmative test. • CE-MRA provides better understanding of missed or mislocalized SDAVF cases.

Optic chiasm involvement on MRI with ethambutol-induced bitemporal hemianopia.

While ethambutol optic neuropathy usually causes central or cecocentral scotomas, bitemporal visual field defects also have been reported. The pathogenesis of the bitemporal hemianopia has not been established. This article describes magnetic resonance imaging abnormalities involving the optic chiasm in a patient with bitemporal visual field loss. To our knowledge, these neuroimaging findings have not been previously described in association with ethambutol therapy.

Captured Macro-embolus of Fractured Atheromatous Plaque by the Embolic Protection Device during Carotid Stent Assisted Angioplasty.

The authors present a case in which macro-embolus from the ruptured atheromatous plaque developed during carotid artery stenting (CAS). A 63-year-old man who had suffered a left middle cerebral artery territory infarction had significant proximal left internal carotid artery stenosis required CAS procedure. Immediate after stent deployment, the patient showed abrupt neurological deterioration with 12 × 3 mm sized macro-embolus which was caught by the embolus protection device (EPD). Retrieval of the macro-embolus was performed safely and the patient recovered to pre-procedure status. Macro-embolus can be resulted during the CAS. The EPD can capture the macro-embolus and safe removal is technically feasible.

Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results.

BACKGROUND AND PURPOSE: Prospective differentiation between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis can be challenging. We hypothesized that high-resolution vessel wall MRI would demonstrate arterial wall enhancement in central nervous system vasculitis but not in reversible cerebral vasoconstriction syndrome. METHODS: We identified all patients with multifocal segmental narrowing of large intracranial arteries who had high-resolution vessel wall MRI and follow-up angiography at our institute over a 4-year period and performed a detailed chart review. RESULTS: Three patients lacked arterial wall enhancement, and these all had reversal of arterial narrowing within 3 months. Four patients demonstrated arterial wall enhancement, and these had persistent or progressive arterial narrowing at a median follow-up of 17 months (range, 6-36 months) with final diagnoses of central nervous system vasculitis (3) and cocaine vasculopathy (1). CONCLUSIONS: Preliminary results suggest that high-resolution contrast-enhanced vessel wall MRI may enable differentiation between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis.

Magnetic resonance imaging demonstration of a single lesion causing Wallerian degeneration in ascending and descending tracts in the spinal cord.

A magnetic resonance image of a 50-year-old man with a remote history of cervical spine injury showed focal myelomalacia at C5 and hyperintense areas on T2-weighted images laterally and posteriorly in the cord above and below C5. We believe these lesions to be due to Wallerian degeneration, with the cephalocaudal level of the Wallerian degeneration lesions dependant on the direction of the tracts relative to the C5 lesion.