Identifying Patients with CSF-Venous Fistula Using Brain MRI: A Deep Learning Approach.

BACKGROUND AND PURPOSE: Spontaneous intracranial hypotension is an increasingly recognized condition. Spontaneous intracranial hypotension is caused by a CSF leak, which is commonly related to a CSF-venous fistula. In patients with spontaneous intracranial hypotension, multiple intracranial abnormalities can be observed on brain MR imaging, including dural enhancement, "brain sag," and pituitary engorgement. This study seeks to create a deep learning model for the accurate diagnosis of CSF-venous fistulas via brain MR imaging. MATERIALS AND METHODS: A review of patients with clinically suspected spontaneous intracranial hypotension who underwent digital subtraction myelogram imaging preceded by brain MR imaging was performed. The patients were categorized as having a definite CSF-venous fistula, no fistula, or indeterminate findings on a digital subtraction myelogram. The data set was split into 5 folds at the patient level and stratified by label. A 5-fold cross-validation was then used to evaluate the reliability of the model. The predictive value of the model to identify patients with a CSF leak was assessed by using the area under the receiver operating characteristic curve for each validation fold. RESULTS: There were 129 patients were included in this study. The median age was 54 years, and 66 (51.2%) had a CSF-venous fistula. In discriminating between positive and negative cases for CSF-venous fistulas, the classifier demonstrated an average area under the receiver operating characteristic curve of 0.8668 with a standard deviation of 0.0254 across the folds. CONCLUSIONS: This study developed a deep learning model that can predict the presence of a spinal CSF-venous fistula based on brain MR imaging in patients with suspected spontaneous intracranial hypotension. However, further model refinement and external validation are necessary before clinical adoption. This research highlights the substantial potential of deep learning in diagnosing CSF-venous fistulas by using brain MR imaging.

Cerebrospinal fluid-lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch - a rare case report and review of the literature.

Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

Spinal Cerebrospinal Fluid Leak Localization with Dynamic Computed Tomography Myelography: Tips, Tricks, and Pitfalls.

Locating spinal cerebrospinal fluid (CSF) leaks can be a diagnostic dilemma for clinicians and radiologists, as well as frustrating for patients. Dynamic computed tomography myelography (dCTM) has emerged as a valuable tool in localizing spinal CSF leaks, aiding in accurate diagnosis, and guiding appropriate management. This article aims to provide insights into the technique, tips, tricks, and potential pitfalls associated with dCTM for spinal CSF leak localization. By understanding the nuances of this procedure, clinicians can optimize the diagnostic process and improve patient outcomes.

Spinal Cerebrospinal Fluid Leak Localization with Digital Subtraction Myelography: Tips, Tricks, and Pitfalls.

Cerebrospinal fluid (CSF) leak can cause spontaneous intracranial hypotension (SIH) which can lead to neurologic symptoms, such as orthostatic headache. Over time, imaging techniques for detecting and localizing CSF leaks have improved. These techniques include computed tomography (CT) myelography, dynamic CT myelography, cone-beam CT, MRI, MR myelography, and digital subtraction myelography (DSM). DSM provides the highest sensitivity for identifying leak sites and has comparable radiation exposure to CT myelography. The introduction of the lateral decubitus DSM has proven invaluable in localizing leaks when other imaging tests have been inconclusive.

Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

A new modified technique of dynamic CT myelography to detect dural tears in spontaneous intracranial hypotension.

We propose a modified dynamic CT-myelography technique for patients with fast CSF leaks caused by ventral dural tears in order to reduce radiation exposure and complications. A fluoroscopy-guided lumbar puncture using an epidural anesthesia kit replaces a CT-guided lumbar puncture, and a smaller volume of less concentrated contrast media is used. This approach has advantages, including speeding up the procedure, reduced radiation exposure, and elimination of the risk of contrast injection into the epidural space.

Temporal Characteristics of CSF-Venous Fistulas on Dynamic Decubitus CT Myelography: A Retrospective Multi-Institution Cohort Study.

BACKGROUND AND PURPOSE: CSF-venous fistula can be diagnosed with dynamic decubitus CT myelography. This study aimed to analyze the temporal characteristics of CSF-venous fistula visualization on multiphase decubitus CT myelography. MATERIALS AND METHODS: A retrospective, multisite study was conducted on patients diagnosed with CSF-venous fistula at 2 institutions between June 2017 and February 2023. Both institutions perform decubitus CT myelography with imaging immediately following injection and usually with at least 1 delayed scan. The conspicuity of CSF-venous fistula was assessed on each phase of imaging. RESULTS: Forty-eight patients with CSF-venous fistula were analyzed. CSF-venous fistulas were better visualized on the early pass in 25/48 cases (52.1%), the delayed pass in 6/48 cases (12.5%) and were seen equally on both passes in 15/48 cases (31.3%). Of 25 cases in which the CSF-venous fistula was better visualized on the early pass, 21/25 (84%) fistulas were still at least partially visible on a delayed pass. Of 6 cases in which the CSF-venous fistula was better visualized on a delayed pass, 4/6 (67%) were partially visible on the earlier pass. Six of 48 (12.5%) CSF-venous fistulas were visible only on a single pass. Of these, 4/6 (66.7%) were seen only on the first pass, and 2/6 (33.3%) were seen only on a delayed pass. One fistula was found with one pass only, and one fistula was discovered upon contralateral decubitus imaging without a dedicated second injection. CONCLUSIONS: A dynamic decubitus CT myelography imaging protocol that includes an early and delayed phase, likely increases the sensitivity for CSF-venous fistula detection. Further studies are needed to ascertain the optimal timing and technique for CSF-venous fistula visualization on dynamic decubitus CT myelography and its impact on patient outcomes.

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

BACKGROUND AND PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Likelihood of Discovering a CSF Leak Based on Intracranial MRI Findings in Patients without a Spinal Longitudinal Extradural Collection: A New Probabilistic Scoring System.

BACKGROUND AND PURPOSE: The likelihood of discovering a CSF leak can be determined by assessing intracranial abnormalities. However, the Dobrocky scoring system, which is used to determine this likelihood, did not incorporate patients with CSF-venous fistulas. This study sought to create a new probabilistic scoring system applicable to patients without a spinal longitudinal extradural collection. MATERIALS AND METHODS: A retrospective review was completed of patients with suspected spontaneous intracranial hypotension who underwent brain MR imaging followed by digital subtraction myelography with same-day CT myelography. Patients with and without leaks found on digital subtraction myelography were included. MRIs were assessed for numerous reported stigmata of spontaneous intracranial hypotension and were compared between cohorts. RESULTS: One hundred seventy-four patients were included; 113 (64.9%) were women (average age, 52.0 [SD, 14.3] years). A CSF leak was found in 98 (56.3%) patients, nearly all of which (93.9%) were CSF-venous fistulas. Diffuse dural enhancement, internal auditory canals dural enhancement, non-Chiari cerebellar descent, pituitary engorgement, brain sag, dural venous sinus engorgement, and decreased suprasellar cistern size were associated with a CSF leak. A probabilistic scoring system was made in which a single point value was assigned to each of those findings: 0-2 considered low probability and ≥3 considered intermediate-to-high probability of a CSF leak. CONCLUSIONS: This study offers a new probabilistic scoring system for evaluating the likelihood of discovering a CSF leak on the basis of intracranial MR imaging findings, though the new system is not superior to that of the Dobrocky method for predicting the presence of CSF leaks.

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

BACKGROUND AND PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Conebeam CT as an Additional Tool in Digital Subtraction Myelography for the Detection of Spinal Lateral Dural Tears.

Lateral dural tears as a cause spontaneous intracranial hypotension occur in ∼20% of patients. Common imaging modalities for their detection are lateral decubitus digital subtraction myelography or dynamic CT myelography. Reports on the use of conebeam CT are scarce. We show 3 patients in whom the targeted use of conebeam CT during digital subtraction myelography was helpful in confirming the site of the leak.

Utility of Photon-Counting Detector CT Myelography for the Detection of CSF-Venous Fistulas.

CSF-venous fistulas are an increasingly recognized type of CSF leak that can be particularly challenging to detect, even with recently improved imaging techniques. Currently, most institutions use decubitus digital subtraction myelography or dynamic CT myelography to localize CSF-venous fistulas. Photon-counting detector CT is a relatively recent advancement that has many theoretical benefits, including excellent spatial resolution, high temporal resolution, and spectral imaging capabilities. We describe 6 cases of CSF-venous fistulas detected on decubitus photon-counting detector CT myelography. In 5 of these cases, the CSF-venous fistula was previously occult on decubitus digital subtraction myelography or decubitus dynamic CT myelography using an energy-integrating detector system. All 6 cases exemplify the potential benefits of photon-counting detector CT myelography in identifying CSF-venous fistulas. We suggest that further implementation of this imaging technique will likely be valuable to improve the detection of fistulas that might otherwise be missed with currently used techniques.

Spontaneous Intracranial Hypotension and Spinal Epidural CSF Leaks: Diagnosis and Management.

Spontaneous intracranial hypotension from spinal cerebrospinal fluid leak is a condition that often presents as orthostatic headaches. Diagnosis and localisation of spinal CSF leaks remain difficult despite multiple imaging modalities that can be used to aid identification. These include traditional CT myelography and MRI as well as newer techniques such as dynamic and digital subtraction myelography. Leaks can be classified into types and optimal localisation and management techniques vary by type of leak. Localisation of a leak can aid in targeting treatment such as an epidural blood patch if conservative measures fail. Where unsuccessful, repeated blood patches and novel techniques can be used to improve patient symptoms. Much of this condition is not well understood and evidence is lacking, with many avenues for potential research.

Prone Dynamic CT Myelography in Spontaneous Intracranial Hypotension : Diagnostic Need and Radiation Doses.

BACKGROUND AND PURPOSE: The diagnostic work-up in patients with spontaneous intracranial hypotension (SIH) and spinal longitudinal extradural CSF collection (SLEC) on magnetic resonance imaging (MRI) comprises dynamic digital subtraction myelography (dDSM) in prone position for leak detection. Dynamic computed tomography (CT) myelography (dCT-M) in prone position follows if the leak is not unequivocally located. A drawback of dCT­M is a high radiation dose. This study evaluates the diagnostic needs of dCT-M examinations and measures to reduce radiation doses. METHODS: Frequency, leak sites, length and number of spiral acquisitions, DLP and effective doses of dCT­M were retrospectively recorded in patients with ventral dural tears. RESULTS: Of 42 patients with ventral dural tears, 8 underwent 11 dCT­M when the leak was not unequivocally shown on digital subtraction myelography. The median number of spiral acquisitions was 4 (range 3-7) and the mean effective radiation dose 30.6 mSv (range 13.1-62.16 mSv) mSv. Five of eight leaks were located in the upper thoracic spine (range C7/Th1-Th2/3). Bolus tracking of intrathecal contrast agent in dCT­M was used to limit the number and length of spiral acquisitions. DISCUSSION: A dCT­M in prone position to localize a ventral dural tear is needed in every fifth patient with a SLEC on MRI. It is typically needed when the leak is located in the upper thoracic spine and when patients have broad shoulders. Measures to reduce the radiation dose include bolus tracking or to repeat the DSM with adjusted positioning of patient.

Postoperative Spinal Cerebrospinal Fluid-Venous Fistulas Associated With Dural Tears in Patients With Intracranial Hypotension or Superficial Siderosis-A Digital Subtraction Myelography Study.

BACKGROUND: Postoperative spinal cerebrospinal fluid (CSF) leaks are common but rarely cause extensive CSF collections that require specialized imaging to detect the site of the dural breach. OBJECTIVE: To investigate the use of digital subtraction myelography (DSM) for patients with extensive extradural CSF collections after spine surgery. METHODS: A retrospective review was performed to identify a consecutive group of patients with extensive postoperative spinal CSF leaks who underwent DSM. RESULTS: Twenty-one patients (9 men and 12 women) were identified. The mean age was 46.7 years (range, 17-75 years). The mean duration of the postoperative CSF leak was 3.3 years (range, 3 months to 21 years). MRI showed superficial siderosis in 6 patients. DSM showed the exact location of the CSF leak in 19 (90%) of the 21 patients. These 19 patients all underwent surgery to repair the CSF leak, and the location of the CSF leak could be confirmed intraoperatively in all 19 patients. In 4 (19%) of the 21 patients, DSM also showed a CSF-venous fistula at the same location as the postoperative dural tear. CONCLUSION: In this study, DSM had a 90% detection rate of visualizing the exact site of the dural breach in patients with extensive postoperative spinal CSF leaks. The coexistence of a CSF-venous fistula in addition to the primary dural tear was present in about one-fifth of patients. The presence of a CSF-venous fistula should be considered if CSF leak symptoms persist in spite of successful repair of a durotomy.

Temporal Characteristics of CSF-Venous Fistulas on Digital Subtraction Myelography.

BACKGROUND AND PURPOSE: CSF-venous fistula can be diagnosed with multiple myelographic techniques; however, no prior work has characterized the time to contrast opacification and the duration of visualization. The purpose of our study was to evaluate the temporal characteristics of CSF-venous fistula on digital subtraction myelography. MATERIALS AND METHODS: We reviewed the digital subtraction myelography images of 26 patients with CSF-venous fistulas. We evaluated how long the CSF-venous fistula took to opacify after contrast reached the spinal level of interest and how long it remained opacified. Patient demographics, CSF-venous fistula treatment, brain MR imaging findings, CSF-venous fistula spinal level, and CSF-venous fistula laterality were recorded. RESULTS: Eight of the 26 CSF-venous fistulas were seen on both the upper- and lower-FOV digital subtraction myelography, for a total of 34 CSF-venous fistula views evaluated on digital subtraction myelography. The mean time to appearance was 9.1 seconds (range, 0-30 seconds). Twenty-two (84.6%) of the CSF-venous fistulas were on the right. The highest fistula level was C7, while the lowest was T13 (13 rib-bearing vertebral bodies). The most common CSF-venous fistula levels were T6 (4 patients) followed by T8, T10, and T11 (3 patients each). The mean age was 58.3 years (range, 31.7-87.6 years). Sixteen patients were women (61.5%). CONCLUSIONS: This is the first study to report the temporal characteristics of CSF-venous fistulas using digital subtraction myelography. We found that on average, the CSF-venous fistula appeared 9.1 seconds (range, 0-30 seconds) after intrathecal contrast reached the spinal level.

Modified Dynamic CT Myelography for Type 1 and 2 CSF Leaks: A Procedural Approach.

BACKGROUND AND PURPOSE: Dynamic CT myelography can identify spinal CSF leaks secondary to dural tears (type 1) and ruptured meningeal diverticula (type 2), but the radiation can be high secondary to multiple successive acquisitions. The purpose of this article is to discuss the procedural approach of a modified dynamic CT myelography technique with single scan acquisitions, reduced contrast volume, and condensed scan coverage and compare its radiation dose with that in traditional dynamic CT myelography. MATERIALS AND METHODS: Retrospective review was performed for patients with spontaneous CSF leaks showing extradural collections on spine MR imaging who underwent traditional and modified dynamic CT myelography. The radiation doses between the 2 cohorts were compared. RESULTS: Thirty-seven patients (25 women, 12 men) had a type 1 or 2 CSF leak on dynamic CT myelography. Thirty-one patients had a type 1 CSF leak, and 6 patients had type 2 leaks. The traditional dynamic CT myelography was performed in 25 patients, and the average number of acquisitions per dynamic CT myelography was 3.6. The mean total effective dose per dynamic CT myelography was 31.3 mSv (range, 11.3-68.4 mSv). The modified dynamic CT myelography was performed in 12 patients, and the average number of acquisitions was 2.8. The mean total effective dose per dynamic CT myelography was 15.1 mSv (range, 4.8-24.6 mSv). The effective dose and dose-length product between the cohorts were statistically significant (P < .0001 and .01, respectively). CONCLUSIONS: Modified dynamic CT myelography performed with single scan acquisitions, smaller volume of contrast, and reduced scan coverage can reduce the radiation dose for type 1 and 2 CSF leak detection.

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.

"The Up and Down Approach" to Localize and Exclude Sites of Intraspinal CSF Leaks.

Myelography is a commonly performed procedure to locate cerebrospinal fluid (CSF) leaks in patients with spontaneous intracranial hypotension. Often, the site of leak within the spinal canal cannot be located creating a diagnostic dilemma for clinicians. This technical report describes a novel method to locate and exclude intraspinal CSF leaks in patients with multiple potential sites of CSF leak using a lumbar and cervical approach to inject intrathecal contrast and subsequently performing CT myelography.

Spontaneous intracranial hypotension: updates from diagnosis to treatment.

Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leaks, which result in continued loss of CSF volume and multiple debilitating clinical manifestations. The estimated annual incidence of SIH is 5/100,000. Diagnostic methods have evolved in recent years due to improved understanding of pathophysiology and implementation of advanced myelographic techniques. Here, we synthesize recent updates and contextualize them in an algorithm for diagnosis and treatment of SIH, highlighting basic principles and points of practice variability or continued debate. This discussion includes finer points of SIH diagnosis, CSF leak classification systems, less common types and variants of CSF leaks, brain MRI Bern scoring, potential SIH complications, key technical considerations, and positioning strategies for different types of dynamic myelography. The roles of conservative measures, non-targeted or targeted blood patches, surgery, and recently developed endovascular techniques are presented.