Cause Of Spontaneous Intracranial Hypotension Research Articles

Direct comparison of digital subtraction myelography versus CT myelography in lateral decubitus position: evaluation of diagnostic yield for cerebrospinal fluid-venous fistulas

BackgroundCerebrospinal fluid (CSF)-venous fistulas (CVFs) are increasingly identified as a cause of spontaneous intracranial hypotension (SIH). Lateral decubitus digital subtraction myelography (LD-DSM) and CT myelography (LD-CTM) are mainly used for...

CSF-venous fistulas associated with traumatic spinal pseudomeningoceles.

CSF-venous fistulas (CVFs) are a common cause of spontaneous intracranial hypotension. These fistulas usually occur without any preceding major trauma, surgery, or other iatrogenic cause. Occasionally, patients have a history of minor trauma, though such cases are usually still considered spontaneous. Little is known about predisposing factors that cause patients to develop spontaneous CVFs. Most patients with CVFs have multiple meningeal diverticula on spine imaging, and fistulas usually arise in association with a diverticulum. In the vast majority of cases, the culprit diverticulum from which the CVF arises is atraumatic in origin, presumably on the spectrum of normal variation in spinal anatomy. Here, we present two cases of CVFs that arose in association with posttraumatic pseudomeningoceles. To our knowledge, this phenomenon has not yet been reported, and it potentially represents a novel etiology for CVFs that furthers understanding of their pathogenesis.

Beta Trace Protein as a Potential Biomarker for CSF-Venous Fistulas.

Accurately identifying patients with CSF-venous fistulas (CVF), one cause of spontaneous intracranial hypotension (SIH), is a diagnostic dilemma. This conundrum underscores the need for a CVF biomarker to help select who should undergo an invasive myelogram for further diagnostic workup. Beta trace protein (BTP) is the most abundant CNS derived protein in the CSF and therefore is a potential venous biomarker for CVF detection. The purpose of our study was to measure venous BTP levels as a potential CVF biomarker. We prospectively enrolled 14 patients with CVF and measured BTP in venous blood samples from the paraspinal veins near the CVF and compared those levels to the peripheral blood. Myelograms used initially to identify the CVF were evaluated for modality, CVF laterality, CVF level, and venous drainage pattern. Patient sex, patient age, and symptom duration were also collected. Brain MR images were reviewed for Bern scores. We also measured the peripheral blood BTP levels in 20 normal controls. In patients with CVF, the mean BTP level near the CVF was 54.5% higher (0.760 [SD 0.673] vs 0.492 [SD 0.095] mg/L; p = 0.069) compared to peripheral blood. Nine (64.3%) patients with CVF had a higher paraspinal BTP level than peripheral BTP level. The 20 control patients had a higher the mean peripheral BTP level 0.720 (SD 0.191) mg/L compared to patients with CVF (p<0.001). We found that venous blood at the site of CVF had higher BTP values compared to peripheral blood in the majority, but not all patients with CVF. This may reflect the intermittent leaking nature of CVF. Additionally, we found that patients with CVF had a lower peripheral blood BTP level compared to normal controls. BTP requires further evaluation as a potential CVF biomarker. SIH = Spontaneous Intracranial Hypotension; CVF = CSF-Venous Fistula; CTM = CT myelogram; DSM = Digital Subtraction Myelography; BTP = Beta Trace Protein.

Cerebrospinal fluid venous fistula causing spontaneous intracranial hypotension resulting in venous sinus thrombosis and coma.

Cerebrospinal fluid (CSF) fistulas have recently been recognized as a cause of spontaneous intracranial hypotension (SIH), predominantly presenting with headaches, especially positional headaches. Atypical presentations like tinnitus and cranial nerve symptoms have also been reported. SIH has been linked to venous sinus thrombosis; however, to our knowledge, no prior cases describe a CSF venous fistula causing SIH that leads to cerebral venous thrombosis and coma. We report a patient who developed progressive venous sinus thrombosis, leading to coma, and was found to have low intracranial pressure indicative of SIH. Invasive monitoring and imaging confirmed the low intracranial pressure, prompting a dynamic myelogram that revealed a T2/3 CSF venous fistula. The patient underwent transvenous embolization of the fistula, which resulted in the resolution of symptoms and almost immediate improvement in both venous thrombosis and intracranial hypotension. CSF venous fistulae as a cause of SIH is a recently recognized entity, with ongoing research into its treatment through transvenous embolization. Most documented cases focus on patients with headaches. This case highlights a novel presentation, emphasizing the importance of thorough diagnostic workup in patients with cerebral venous thrombosis. Early detection and treatment of this condition can lead to significant clinical improvement, including the resolution of coma.

Myelographic Techniques for the Localization of CSF-Venous Fistulas: Updates in 2024.

CSF-venous fistulas (CVFs) are a common cause of spontaneous intracranial hypotension. Despite their relatively frequent occurrence, they can be exceedingly difficult to detect on imaging. Since the initial description of CVFs in 2014, the recognition and diagnosis of this type of CSF leak has continually increased. As a result of multi-institutional efforts, a wide spectrum of imaging modalities and specialized techniques for CVF detection is now available. It is important for radiologists to be familiar with the multitude of available techniques, because each has unique advantages and drawbacks. In this article, we review the spectrum of imaging modalities available for the detection of CVFs, explain the advantages and disadvantages of each, provide typical imaging examples, and discuss provocative maneuvers that may improve the conspicuity of CVFs. Discussed modalities include conventional CT myelography, dynamic myelography, digital subtraction myelography, conebeam CT myelography, decubitus CT myelography by using conventional energy-integrating detector scanners, decubitus photon counting CT myelography, and intrathecal gadolinium MR myelography. Additional topics to be discussed include optimal patient positioning, respiratory techniques, and intrathecal pressure augmentation.

Cerebrospinal fluid-venous fistula visualisation by intrathecal pressurization: A technical note and illustrative case.

CSF-venous fistulas (CVFs) are an important cause of spontaneous intracranial hypotension and challenging to diagnose. Lateral decubitus positioning during myelography and a technique called "resisted inspiration" has shown to improve CVF detection. However, the impact of intrathecal pressurization to improve visualization of CVF has mostly been speculated on. In this brief report, we demonstrate how a CVF became progressively more visible only after stepwise intrathecal pressurization: An indication of the importance of pressurization for CVF detection.

Myelography Using Energy-Integrating Detector CT Versus Photon-Counting Detector CT for Detection of CSF-Venous Fistulas in Patients With Spontaneous Intracranial Hypotension.

BACKGROUND. CSF-venous fistulas (CVFs), which are an increasingly recognized cause of spontaneous intracranial hypotension (SIH), are often diminutive in size and exceedingly difficult to detect by conventional imaging. OBJECTIVE. This purpose of this study was to compare energy-integrating detector (EID) CT myelography and photon-counting detector (PCD) CT myelography in terms of image quality and diagnostic performance for detecting CVFs in patients with SIH. METHODS. This retrospective study included 38 patients (15 men and 23 women; mean age, 55 ± 10 [SD] years) with SIH who underwent both clinically indicated EID CT myelography (slice thickness, 0.625 mm) and PCD CT myelography (slice thickness, 0.2 mm; performed in ultrahigh-resolution mode) to assess for CSF leak. Three blinded radiologists reviewed examinations in random order, assessing image noise, discernibility of spinal nerve root sleeves, and overall image quality (each assessed using a scale of 0-100, with 100 denoting highest quality) and recording locations of the CVFs. Definite CVFs were defined as CVFs described in CT myelography reports using unequivocal language and having an attenuation value greater than 70 HU. RESULTS. For all readers, PCD CT myelography, in comparison with EID CT myelography, showed higher mean image noise (reader 1: 69.9 ± 18.5 [SD] vs 37.6 ± 15.2; reader 2: 59.5 ± 8.7 vs 49.3 ± 12.7; and reader 3: 57.6 ± 13.2 vs 42.1 ± 16.6), higher mean nerve root sleeve discernibility (reader 1: 81.6 ± 21.7 [SD] vs 30.4 ± 13.6; reader 2: 83.6 ± 10 vs 70.1 ± 18.9; and reader 3: 59.6 ± 13.5 vs 50.5 ± 14.4), and higher mean overall image quality (reader 1: 83.2 ± 20.0 [SD] vs 38.1 ± 13.5; reader 2: 80.1 ± 10.1 vs 72.4 ± 19.8; and reader 3: 57.8 ± 11.2 vs 51.9 ± 13.6) (all p < .05). Eleven patients had a definite CVF. Sensitivity and specificity of EID CT myelography and PCD CT myelography for the detection of definite CVF were 45% and 96% versus 64% and 85%, respectively, for reader 1; 36% and 100% versus 55% and 96%, respectively, for reader 2; and 57% and 100% versus 55% and 93%, respectively, for reader 3. The sensitivity was significantly higher for PCD CT myelography than for EID CT myelography for reader 1 and reader 2 (both p < .05) and was not significantly different between the two techniques for reader 3 (p = .45); for all three readers, specificity was not significantly different between the two modalities (all p > .05). CONCLUSION. In comparison with EID CT myelography, PCD CT myelography yielded significantly improved image quality with significantly higher sensitivity for CVFs (for two of three readers), without significant loss of specificity. CLINICAL IMPACT. The findings support a potential role for PCD CT myelography in facilitating earlier diagnosis and targeted treatment of SIH, avoiding high morbidity during potentially prolonged diagnostic workups.

Benefits of Photon-Counting CT Myelography for Localization of Dural Tears in Spontaneous Intracranial Hypotension.

Photon-counting CT is an increasingly used technology with numerous advantages over conventional energy-integrating detector CT. These include superior spatial resolution, high temporal resolution, and inherent spectral imaging capabilities. Recently, photon-counting CT myelography was described as an effective technique for the detection of CSF-venous fistulas, a common cause of spontaneous intracranial hypotension. It is likely that photon-counting CT myelography will also have advantages for the localization of dural tears, a separate type of spontaneous spinal CSF leak that requires different myelographic techniques for accurate localization. To our knowledge, prior studies on photon-counting CT myelography have been limited to techniques for detecting CSF-venous fistulas. In this technical report, we describe our technique and early experience with photon-counting CT myelography for the localization of dural tears.

Diagnostic Performance of Decubitus Photon-Counting Detector CT Myelography for the Detection of CSF-Venous Fistulas.

CSF-venous fistulas are a common cause of spontaneous intracranial hypotension. Lateral decubitus digital subtraction myelography and CT myelography are the diagnostic imaging standards to identify these fistulas. Photon-counting CT myelography has technological advantages that might improve CSF-venous fistula detection, though no large studies have yet assessed its diagnostic performance. We sought to determine the diagnostic yield of photon-counting detector CT myelography for detection of CSF-venous fistulas in patients with spontaneous intracranial hypotension. We retrospectively searched our database for all decubitus photon-counting detector CT myelograms performed at our institution since the introduction of the technique in our practice. Per our institutional workflow, all patients had prior contrast-enhanced brain MR imaging and spine MR imaging showing no extradural CSF. Two neuroradiologists reviewed preprocedural brain MRIs, assessing previously described findings of intracranial hypotension (Bern score). Additionally, 2 different neuroradiologists assessed each myelogram for a definitive or equivocal CSF-venous fistula. The yield of photon-counting detector CT myelography was calculated and stratified by the Bern score using low-, intermediate-, and high-probability tiers. Fifty-seven consecutive photon-counting detector CT myelograms in 57 patients were included. A single CSF-venous fistula was definitively present in 38/57 patients. After we stratified by the Bern score, a definitive fistula was seen in 56.0%, 73.3%, and 76.5% of patients with low-, intermediate-, and high-probability brain MR imaging, respectively. Decubitus photon-counting detector CT myelography has an excellent diagnostic performance for the detection of CSF-venous fistulas. The yield for patients with intermediate- and high-probability Bern scores is at least as high as previously reported yields of decubitus digital subtraction myelography and CT myelography using energy-integrating detector scanners. The yield for patients with low-probability Bern scores appears to be greater compared with other modalities. Due to the retrospective nature of this study, future prospective work will be needed to compare the sensitivity of photon-counting detector CT myelography with other modalities.

Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

Cerebrospinal fluid-venous fistulas (CSFVFs) are increasingly recognized as a cause of spontaneous intracranial hypotension (SIH), a debilitating neurologic condition that may present with postural headaches, hearing loss, and/or cognitive impairment. Curative treatment is available for patients whose fistulas are localized with myelography. Transvenous endovascular embolization has emerged in recent years as a safe, effective, and durable treatment option for patients with CSFVF. Here, we briefly review the clinical presentation of SIH, present diagnostic algorithm by which CSFVFs are identified, and discuss a stepwise treatment paradigm. We then detail endovascular treatment strategies of CSFVF with particular emphasis on direct versus indirect approaches to the fistula, embolization principles, and techniques to minimize complications. With increasing awareness of SIH and enhanced detection of CSFVF, it is important for endovascular neurosurgeons and neurointerventionalists to be familiar with transvenous CSFVF embolization strategies to meet the needs of this patient population.

Clinical and imaging outcomes of 100 patients with cerebrospinal fluid-venous fistulas treated by transvenous embolization

BackgroundCerebrospinal fluid-venous fistulas (CSFVF) are a common cause of spontaneous intracranial hypotension (SIH). Transvenous embolization has emerged as a reliable treatment option. We review the clinical presentation, imaging, and clinical...

Resisted Inspiration Improves Visualization of CSF-Venous Fistulas in Spontaneous Intracranial Hypotension.

CSF-venous fistulas are an important cause of spontaneous intracranial hypotension but are challenging to detect. A newly described technique known as resisted inspiration has been found to augment the CSF-venous pressure gradient and was hypothesized to be of potential use in CSF-venous fistula detection but has not yet been investigated in patients with spontaneous intracranial hypotension. The purpose of this investigation was to determine whether resisted inspiration results in improved visibility of CSF-venous fistulas on CT myelography in patients with spontaneous intracranial hypotension. A retrospective cohort of patients underwent CT myelography from November 2022 to January 2023. Patients with an observed or suspected CSF-venous fistula identified during CT myelography using standard maximum suspended inspiration were immediately rescanned using resisted inspiration and the Valsalva maneuver. The visibility of the CSF-venous fistula among these 3 respiratory phases was compared, and changes in venous drainage patterns between phases were assessed. Eight patients with confirmed CSF-venous fistulas who underwent CT myelography using the 3-phase respiratory protocol were included. Visibility of the CSF-venous fistula was greatest during resisted inspiration in 5/8 (63%) of cases. Visibility was optimal with the Valsalva maneuver and maximum suspended inspiration in 1 case each, and it was equivalent in all respiratory phases in 1 case. In 2/8 (25%) cases, the pattern of venous drainage shifted between respiratory phases. In patients with spontaneous intracranial hypotension, resisted inspiration improved visualization of CSF-venous fistulas in most, but not all, cases. Further investigation is needed to determine the impact of this technique on the overall diagnostic yield of myelography in this condition.

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

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). 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. 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. Surgical repair of CVF resulted in improvements in headache and other symptoms, with few side effects.

Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation.

Spontaneous intracranial hypotension (SIH) occurs due to a leakage of the cerebrospinal fluid (CSF) lowering the pressure of subarachnoid space, mostly caused by a dural breach or discogenic microspur. As a result of less support provided by CSF pressure, intracranial structures are stretched downward, leading to a constellation of more or less typical MRI findings, including venous congestion, subdural effusions, brainstem sagging and low-lying cerebellar tonsils. Clinic examination and an MRI are usually enough to allow for the diagnosis; however, finding the location of the dural tear is challenging. SIH shares some MRI features with Chiari malformation type I (CM1), especially low-lying cerebellar tonsils. Since SIH is likely underdiagnosed, these findings could be interpreted as signs of CM1, leading to a misdiagnosis and an incorrect treatment pathway. Medical treatment, including steroids, bed rest, hydration caffeine, and a blind epidural blood patch, have been used in this condition with variable success rates. For some years, CSF venous fistulas have been described as the cause of SIH, and a specific diagnostic and therapeutic pathway have been proposed. The current literature on SIH with a focus on diagnosis, treatment, and differential diagnosis with CM1, is reviewed and discussed.

Intraoperative Visualization of Flow in Direct Cerebrospinal Fluid-Venous Fistulas Using Intrathecal Fluorescein.

Cerebrospinal fluid-venous fistulas (CVFs) are a rare cause of spontaneous intracranial hypotension. Intraoperatively, CVFs are not readily identifiable and difficult to differentiate from normal veins. To assess the utility of intrathecal fluorescein injection intraoperatively to visualize and identify the CVF. We report a case series of patients treated surgically for a CVF. After surgical exposure, we injected intrathecal fluorescein (5-10 mg) through a lumbar catheter, which we placed immediately before surgery. Four patients with spontaneous intracranial hypotension with a suspected CVF underwent surgical ligation with adjunctive intrathecal fluorescein application. Intraoperative fluorescein injection confirmed the presence of a CVF in 3 cases. In 2 cases, we observed rapid fluorescein filling of a single epidural vein constituting the CVF. Other epidural vessels did not fill with fluorescein. In 1 case, fluorescein helped to identify a residual CVF after previous incomplete embolization. In the fourth case, no CVF was found intraoperatively. By contrast, a meningeal nerve root diverticulum was visualized, wrapped, and clipped. We demonstrate for the first time the direct intraoperative visualization of CVF using intrathecal fluorescein. CVF can be identified intraoperatively using fluorescein dye, which can be a valuable adjunct for the surgeon confronted with this disease.

Conebeam CT as an Adjunct to Digital Subtraction Myelography for Detection of CSF-Venous Fistulas.

Lateral decubitus digital subtraction myelography is an effective technique for precisely localizing CSF-venous fistulas, a common cause of spontaneous intracranial hypotension. However, despite an optimal imaging technique, digital subtraction myelography fails to identify some CSF-venous fistulas for a variety of reasons. Here, we describe a technique involving conebeam CT performed during intrathecal contrast injection as an adjunct to digital subtraction myelography, allowing identification of some otherwise-missed CSF-venous fistulas.

Cervical epidural blood patch in spontaneous intracranial hypotension: A case report

Background: Spontaneous Intracranial Hypotension (SIH) results from a decrease in cerebrospinal fluid volume without any iatrogenic cause and presents most commonly with orthostatic headache. Cervical dural tears are an uncommon cause of SIH. Cervical epidural blood patch has been successfully used in many developed countries for the treatment of spontaneous intracranial hypotension after failure of conservative management

The \u201chyperdense basivertebral vein\u201d sign: another marker of a CSF-venous fistula

CSF-venous fistulas — initially described in 2014 — are a significant cause of spontaneous intracranial hypotension. Dynamic lateral decubitus digital subtraction and/or CT myelography typically show a hyperdense paraspinal vein. In case of a central drainage toward the internal vertebral venous plexus, it is the “hyperdense basivertebral vein” which should be searched for carefully.

CSF-Venous Fistulas: Anatomy and Diagnostic Imaging.

CSF-venous fistulas (CVFs), first described in 2014, are an important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional anatomic imaging because, unlike other types of spinal CSF leak, they do not typically result in pooling of fluid in the epidural space, and imaging signs of CVF may be subtle. Specialized myelographic techniques have been developed to help with CVF identification, but these techniques are not yet widely disseminated. This article reviews the current understanding of CVFs, emphasizing correlations between venous anatomy and imaging findings as well as potential mechanisms for pathogenesis, and describes current imaging techniques used for CVF diagnosis and localization. These techniques are broadly classified into fluoroscopy-based methods, including digital subtraction myelography and dynamic myelography, and cross-sectional methods, including decubitus CT myelography and MR myelography with intrathecal injection of gadolinium. Knowledge of these various options, including their relative advantages and disadvantages, is critical in the care of patients with spontaneous intracranial hypotension. Investigation is ongoing, and continued advances in knowledge about CVFs as well as in optimal imaging detection are anticipated.

Spinal CSF-Venous Fistulas in Morbidly and Super Obese Patients with Spontaneous Intracranial Hypotension.

Spinal CSF-venous fistulas are increasingly recognized as the cause of spontaneous intracranial hypotension. Here, we describe the challenges in the care of patients with CSF-venous fistulas who are morbidly or super obese. A review was undertaken of all patients with spontaneous intracranial hypotension and a body mass index of >40 who underwent digital subtraction myelography in the lateral decubitus position to look for CSF-venous fistulas. Eight patients with spontaneous intracranial hypotension with a body mass index of >40 underwent lateral decubitus digital subtraction myelography. The mean age of these 5 women and 3 men was 53 years (range, 45 to 68 years). Six patients were morbidly obese (body mass indexes = 40.2, 40.6, 41, 41.8, 45.4, and 46.9), and 2 were super obese (body mass indexes = 53.7 and 56.3). Lumbar puncture showed an elevated opening pressure in 5 patients (26.5-47 cm H2O). The combination of an elevated opening pressure and normal conventional spine imaging findings resulted in a misdiagnosis (midbrain glioma and demyelinating disease, respectively) in 2 patients. Prior treatment included surgical nerve root ligation for suspected CSF-venous fistula in 3 patients. Digital subtraction myelography demonstrated a CSF-venous fistula in 6 patients (75%). Rebound high-pressure headache occurred in all 6 patients following surgical ligation of the fistula, and papilledema developed in 3. In our series, opening pressure was generally elevated in patients with morbid or super obesity. The yield of identifying CSF-venous fistulas with digital subtraction myelography in this patient population can approach that of the nonobese patient population. These patients may be at higher risk of developing rebound high-pressure headaches and papilledema.