Spontaneous Intracranial Hypotension Research Articles

Skull Base CSF Leaks: Potential Underlying Pathophysiology and Evaluation of Brain MR Imaging Findings Associated with Spontaneous Intracranial Hypotension.

Cerebrospinal fluid (CSF) leaks of the skull base and spine share a common process of CSF volume loss, and yet only the latter has been associated with spontaneous intracranial hypotension (SIH). Despite published claims that only spinal leaks cause SIH, no prior studies have evaluated brain MR imaging in patients with skull base leaks for findings associated with SIH such as dural enhancement. The purpose of our study was to use a validated brain MR imaging scoring system to evaluate skull base CSF leak patients for findings associated with SIH. We included patients with confirmed skull base CSF leaks and contrast enhanced pre-operative brain MRI. The pre-operative MR images were reviewed for findings associated with SIH using the Bern score. Patient age, presenting symptoms and their duration, and leak site were also recorded. 31 patients with skull base CSF leaks were included. Mean Bern score was 0.9 (range 0-4, SD 1.1), and only 1 patient (3%) had dural enhancement. Mean age was 53 years (range 18-76). Mean symptom duration was 1.3 years, with 22 patients presenting within one year of symptom onset. 23 patients (74.2%) had intraoperative confirmation of leak from the middle cranial fossa, involving the temporal bone, while 7 (22.6%) had leaks from the anterior skull base. One patient, who had dural enhancement, had an infratentorial CSF leak along the petrous segment of the internal carotid artery. Our study provides further evidence that skull base and spinal CSF leaks represent distinct pathophysiologies and present with different brain MRI findings.ABBREVIATIONS: SIH = Spontaneous Intracranial Hypotension; IIH = Idiopathic Intracranial Hypertension.

Diagnostic Yield of Decubitus CT Myelography for Detection of CSF-Venous Fistulas.

Various imaging techniques have been described to detect CSF-Venous Fistulas (CVFs) in the setting of Spontaneous Intracranial Hypotension (SIH), including decubitus CT myelography (dCTM). The expected diagnostic yield of dCTM for CVF detection is not fully established. The purpose of this study was to assess the yield of dCTM among consecutive patients presenting for evaluation of possible SIH, and to examine what impact brain MRI findings of SIH had on diagnostic yield. Single-center, retrospective cohort of consecutive patients presenting over a one-year period who underwent CTM and had no CSF identified in the epidural space. Patients with epidural CSF leaks were included in a secondary cohort. Subjects were grouped according to positioning for the myelogram, either decubitus or prone, and the presence of imaging findings of SIH on pre-procedure brain MRI. Diagnostic yields for each subgroup were calculated, and the yield of dCTM was compared to prone CTM. The study cohort included 302 subjects, including 247 patients with no epidural fluid. Diagnostic yield of dCTM for CVF detection among subjects with positive brain MRI and no epidural fluid was 73%. No CVFs were identified among subjects with negative brain imaging. Among subjects with epidural leak, brain MRI was negative for signs of SIH in 22%. Prone CTM identified a CVF less commonly than dCTM (43% vs. 73%, p=0.19), although the difference was not statistically significant in this small subgroup. We found a diagnostic yield of dCTM to be similar to the yield previously reported for digital subtraction myelography among patients with positive brain imaging. No CVFs were identified in patients with negative brain imaging; epidural CSF leaks accounted for all cases of patients who had SIH with negative brain imaging. This study provides useful data for counseling patients and helps establish a general benchmark for dCTM yield for CVF detection.ABBREVIATIONS: SIH = spontaneous intracranial hypotension; CVF = CSF-Venous Fistula; CTM = CT Myelography; dCTM = decubitus CT myelography; EBP = epidural blood patch.

Spontaneous Clival Leaks and Their Management

Abstract Introduction Cerebrospinal fluid (CSF) leaks through the nasal cavity occurrence has a rising trend, of which primary spontaneous leak is 6 to 40% of all the CSF leaks. The most common site of CSF leak is ethmoid roof where the bone is thinner in the entire skull base. Clivus being the hard bone is a rare site for spontaneous leak. We present a case series from a single quaternary care center of this rare occurrence and study its reason and management strategy. Materials and Methods A retrospective surgical audit over a period of 10 years of all patients diagnosed with CSF rhinorrhea was done. A PubMed search was conducted with keywords of CSF leak, CSF rhinorrhea, spontaneous CSF rhinorrhea, clival leak, and clivus to identify the literature and these articles were compiled and their management reviewed. Results and Analysis A total of 100 patients underwent surgical management for spontaneous CSF leak, of which there were 5 patients who had spontaneous CSF rhinorrhea from the clivus. There were four female patients; four patients had high body mass index. The most common site of leak was mid-clivus and surgical technique employed was multilayer dural plasty with a nasoseptal flap and measures were taken to reduce the intracranial pressure intra-operatively and postoperatively. Conclusion Spontaneous clival leak is a rare entity with mid and lower clivus being the common site. A combined approach by ENT and neurosurgeons results in best outcome for the patients.

Improved Cerebral Glymphatic Flow after Transvenous Embolization of CSF-Venous Fistula.

Spontaneous intracranial hypotension is characterized by symptoms of low intracranial CSF volume due to various mechanisms of CSF leakage. One such mechanism is a CSF-venous fistula, treatable with transvenous embolization resulting in substantial radiographic and clinical improvement. However, the exact mechanisms underlying these improvements, including the potential involvement of the glymphatic system, remain unclear. To noninvasively assess glymphatic clearance in spontaneous intracranial hypotension, we used an advanced MR imaging technique called the DTI along the perivascular spaces in 3 patients with CSF-venous fistula before and after embolization. All 3 patients with spontaneous intracranial hypotension initially had low glymphatic flow, which improved postembolization. Two patients with symptomatic improvement exhibited a more substantial increase in glymphatic flow compared with a patient with minimal improvement. These findings suggest a possible link between cerebral glymphatics in spontaneous intracranial hypotension pathophysiology and symptomatic improvement, warranting larger studies to explore the role of the glymphatic system in spontaneous intracranial hypotension.

The Spatial Relationship between Spinal Osteoarthritis and CSF Venous Fistulas in Patients with Spontaneous Intracranial Hypotension.

CSF venous fistula leads to spontaneous intracranial hypotension. The exact mechanisms underlying the development of CSF venous fistula remain unclear: Some researchers have postulated that underlying chronic intracranial hypertension may lead to damage to spinal arachnoid granulations, given that many patients with CSF venous fistulas have an elevated body mass index (BMI). However, individuals with higher BMIs are also more prone to spinal degenerative disease, and individuals with CSF venous fistulas also tend to be older. CSF venous fistula tends to occur in the lower thoracic spine, the most frequent location of thoracic degenerative changes. The current study aimed to examine whether CSF venous fistulas are more likely to occur at spinal levels with degenerative changes. Forty-four consecutive patients with CSF venous fistulas localized on dynamic CT myelography were included in analyses. Whole-spine CT was scrutinized for the presence of degenerative changes at each spinal level. The proportion of levels positive for CSF venous fistula containing any degenerative findings was compared to levels without CSF venous fistula using the Fisher exact test. The Pearson correlation coefficient was calculated to explore the association between the burden of degenerative disease and BMI and age and between BMI and opening pressure. Forty-four patients with 49 total CSF venous fistulas were analyzed (5 patients had 2 CSF venous fistulas). Mean patient age was 62.3 (SD, 9.5) years. Forty-seven CSF venous fistulas were located in the thoracic spine; 1, in the cervical spine; and 1, in the lumbar spine. Within the thoracic spine, 39/49 (79.6%) fistulas were located between levels T7-8 and T12-L1. Forty-four of 49 (89.8%) CSF venous fistulas had degenerative changes at the same level. The levels without CSF venous fistulas demonstrated degenerative changes at 694/1007 (68.9%) total levels. CSF venous fistulas were significantly more likely to be present at spinal levels with associated degenerative changes (OR = 4.03; 95% CI, 1.58-10.27; P = .001). Age demonstrated a positive correlation with the overall burden of degenerative disease (correlation coefficient: 0.573, P < .001), whereas BMI did not (correlation coefficient: 0.076, P = .625). There was a statistically significant positive correlation between BMI and opening pressure (correlation coefficient: 0.321, P = .03). Results suggest a potential association between spinal degenerative disease and development of CSF venous fistula.

6274683 Spontaneous intracranial hypotension and the obstetric anaesthetist

6274683 Spontaneous intracranial hypotension and the obstetric anaesthetist

Audiometric outcomes following surgery for spontaneous temporal bone cerebrospinal fluid leaks – A single institution study

PurposeTo assess the audiometric outcomes following surgical repair of spontaneous temporal bone cerebrospinal fluid otorrhea and compare different surgical approaches. Materials and methodsRetrospective review of adults (≥18 years old) who underwent repair of spontaneous CSF leak between 2011 and 2022. Audiometric outcomes were compared across the three surgical groups: transmastoid, middle cranial fossa and combined. ResultsThirty-nine patients (40 ears) met the inclusion criteria (71.8 % females; mean age 59.77 +/− 12.4). Forty-two percent underwent transmastoid, 12.5 % middle cranial fossa and 45 % transmastoid-middle cranial fossa. Four patients (10 %) had recurrence, 3 in the transmastoid group and 1 in the combined approach. The mean change in air-bone gap (ABG) for all patients (postoperative-preoperative) was −7.4 (paired t-test, p-value = 0.0003). The postoperative ABG was closed in 28 (70 %) ears (postoperative ABG ≤ 15). The mean change in pure tone average (PTA) for all patients (postoperative-preoperative) was −4.1 (paired t-test, p-value = 0.13). The mean change in word recognition scores (WRS) for all patients (postoperative-preoperative) was −3 (paired t-test, p-value = 0.35). On multivariable analysis (controlling for site and reconstruction material), there was no significant difference in ABG, PTA and WRS change between surgical groups. ConclusionsTransmastoid, middle cranial fossa and combined approaches are all effective in treatment of spontaneous CSF leaks and all showed mean decrease in post-operative ABG. Transmastoid approach showed the greatest decrease in ABG and PTA (although middle cranial fossa approach shows the greatest decrease, when excluding profound hearing loss in a patient with superior canal dehiscence). Further studies comparing audiometric outcomes are needed.

Spontaneous intracranial hypotension: Exploring the viability of non-contrast FLAIR as a substitute for contrast-enhanced T1WI in assessing pachymeningeal thickening.

To avoid contrast administration in spontaneous intracranial hypotension (SIH), some studies suggest accepting diffuse pachymeningeal hyperintensity (DPMH) on non-contrast fluid-attenuated inversion recovery (FLAIR) as an equivalent sign to diffuse pachymeningeal enhancement (DPME) on contrast-enhanced T1WI (T1ce), despite lacking thoroughperformance metrics. This study aimed to comprehensively explore its feasibility. In this single-center retrospective study, between April 2021 and November 2023, brain MRI examinations of 43 patients clinically diagnosed with SIH were assessed using 1.5 and 3.0 Tesla MRI scanners. Two radiologists independentlyassessed the presence or absence of DPMH on FLAIR and DPME on T1ce, with T1ce serving as a gold-standard for pachymeningeal thickening. The contribution of the subdural fluid collections to DPMH was investigated with quantitative measurements. Using Cohen's kappa statistics, interobserver agreement was assessed. In 39 out of 43 patients (90.7%), pachymeningeal thickening was observed on T1ce. FLAIR sequence produced an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 72.1%, 71.8%, 75.0%, 96.6%, and 21.4% respectively, for determining pachymeningeal thickening. FLAIR identified pachymeningeal thickening in 28 cases; however, among these, 21 cases (75%) revealed that the pachymeningeal hyperintense signal was influenced by subdural fluid collections. False-negative rate for FLAIR was 28.2% (11/39). The lack of complete correlation between FLAIR and T1ce in identifying pachymeningeal thickening highlights the need for caution in removing contrast agent administration from the MRI protocol of SIH patients, as it reveals a major criterion (i.e., pachymeningeal enhancement) of Bern score.

Safety and efficacy of transvenous embolization of cerebrospinal fluid-venous fistula in patients with spontaneous intracranial hypotension.

Transvenous embolization is a recent treatment strategy for cerebrospinal fluid-venous fistulas (CSFVF), which are associated with spontaneous intracranial hypotension (SIH). Participants were selected from a prospective database on patients with CSFVF that received transvenous Onyx embolization. All patients underwent a brain magnetic resonance imaging (MRI) before and after embolization with MRI follow-up performed at least 3 months after treatment. Clinical and MRI results after treatment were described. Twenty-one consecutive patients (median age 63 years, IQR = 58-71; females: 15/21 = 71.5%) with 30 CSFVF were included. Most lesions were situated between T9 and L1 (19/30 = 63%), 70% were right-sided, and 38% of the patients had multiples fistulas. Embolization was successful in all cases. The mean MRI SIH score before and after treatment was 6 (±2.5) and 1.4 (±1.6), respectively (p < 0.0001). Twenty patients (90%) experienced improvement of their initial condition, of which 67% reported complete clinical recovery. The mean HIT-6 score decreased from 67 (±15) to 38 (±9) (p < 0.0001), the mean amount of monthly headache days from 23.5 (±10) and 3.2 (±6.6) (p < 0.0001), the visual assessment scale (VAS) for headache severity from 8 (±1.9) to 1.2 (±2) (p < 0.0001), and the mean VAS for perception quality of life improved from 2.6 (±2.5) to 8.6 (±1.8) (p < 0.0001). There were no major complications. The suspected rebound headache rate after treatment was 33%. Transvenous embolization of CSFVF allowed high rates of clinical improvement with no morbidity related to the treatment.

Non-invasive biomarkers for spontaneous intracranial hypotension (SIH) through phase-contrast MRI.

Spontaneous intracranial hypotension (SIH) is an underdiagnosed disease. To depict the accurate diagnosis can be demanding; especially the detection of CSF-venous fistulas poses many challenges. Potential dynamic biomarkers have been identified through non-invasive phase-contrast MRI in a limited subset of SIH patients with evidence of spinal longitudinal extradural collection. This study aimed to explore these biomarkers related to spinal cord motion and CSF velocities in a broader SIH cohort. A retrospective, monocentric pooled-data analysis was conducted of patients suspected to suffer from SIH who underwent phase-contrast MRI for spinal cord and CSF velocity measurements at segment C2/C3 referred to a tertiary center between February 2022 and June 2023. Velocity ranges (mm/s), total displacement (mm), and further derivatives were assessed and compared to data from the database of 70 healthy controls. In 117 patients, a leak was located (54% ventral leak, 20% lateral leak, 20% CSF-venous fistulas, 6% sacral leaks). SIH patients showed larger spinal cord and CSF velocities than healthy controls: e.g., velocity range 7.6 ± 3mm/s vs. 5.6 ± 1.4mm/s, 56 ± 21mm/s vs. 42 ± 10mm/s, p < 0.001, respectively. Patients with lateral leaks and CSF-venous fistulas exhibited an exceptionally heightened level of spinal cord motion (e.g., velocity range 8.4 ± 3.3mm/s; 8.2 ± 3.1mm/s vs. 5.6 ± 1.4mm/s, p < 0.001, respectively). Phase-contrast MRI might become a valuable tool for SIH diagnosis, especially in patients with CSF-venous fistulas without evidence of spinal extradural fluid collection.

Posterior Transdural Repair of a Spontaneous Ventral Thoracic Cerebrospinal Fluid Leak: 2-Dimensional Operative Video.

Spontaneous intracranial hypotension is a rare but serious condition characterized by orthostatic headaches and a variety of neurological symptoms. 1,2 Spontaneous intracranial hypotension should be considered in all patients with new onset, daily, persistent headaches, and orthostatic symptoms. It is typically caused by spontaneous spinal cerebrospinal fluid (CSF) leaks. 1,2 Traditional first-line treatments include hydration, bedrest, epidural blood patches, and fibrin glue injections. However, refractory cases often require surgical intervention, especially those caused by a small ventral osteophyte, which is classified as a type 1 leak. 3-5 The small osteophyte causes a tear in the dura of the ventral canal, usually near the cervicothoracic junction. Diagnosis of these leaks is challenging because these small osteophytes can also occur asymptomatically, or patients may have several of them at multiple levels. Typically, dynamic myelography is needed for accurate localization due to the inadequacy of standard imaging. 6 This video details a young patient with refractory spontaneous intracranial hypotension from a type 1 spontaneous CSF leak, treated successfully using a posterior transdural surgical approach with spinal cord mobilization. Our video presentation outlines the surgical technique and provides an overview of this underdiagnosed condition. Our described approach offers direct visualization, suturing of the leak site, and a multilayer repair without the need for spinal fusion. It also avoids the morbidity to the neck, chest, and mediastinal structures that is at risk with lateral or anterior approaches. A combined intradural and extradural repair may enhance the durability of repair for ventral CSF leaks. The patient consented to the procedure. This operative video did not require Institutional Review Board approval as all patient information has been anonymized, ensuring no identifiable information is disclosed. The video is a single case that does not involve interventions or pose risks beyond standard care, adhering to ethical guidelines and institutional policies.

Progressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report.

Spontaneous intracranial hypotension (SIH) commonly results from ventral spinal cerebrospinal fluid (CSF) leaks and epidural patches are advocated as first-line treatment. Complications such as superficial siderosis can arise but have previously been reported only in the context of long-term persistent, ongoing, CSF leak and SIH. We report a case of a patient with SIH from a ventral spinal CSF leak that was treated with epidural patching and experienced complete resolution of SIH. Four years later SIH symptoms recurred, and brain magnetic resonance imaging unexpectedly showed the interval accumulation of hemosiderin pigmentation on the cerebellum and brainstem during the period when the patient was without symptoms of SIH. This case uniquely demonstrates the progression of superficial siderosis despite the apparent resolution of SIH. Our findings suggest two divergent pathophysiological outcomes from spinal ventral dural tear: (1) CSF loss causing SIH; and (2) persistent low-level bleeding arising from the spinal dural tear leading to superficial siderosis. These divergent pathophysiologies had a discordant response to epidural patching. Epidural patching successfully treated the SIH but did not prevent the progression of superficial siderosis, indicating that some patients may require more than epidural patching despite symptom resolution. This case highlights the need for post-treatment monitoring protocols in patients with ventral spinal CSF leaks and SIH and raises important questions about the adequacy of epidural patching in certain SIH cases arising from ventral spinal CSF leak.

Blinded Epidural Blood Patch for Treating a Man with Antiphospholipid Syndrome and Autoimmune Thrombocytopenia Presenting with Spontaneous Intracranial Hypotension (P10-12.010)

Blinded Epidural Blood Patch for Treating a Man with Antiphospholipid Syndrome and Autoimmune Thrombocytopenia Presenting with Spontaneous Intracranial Hypotension (P10-12.010)

Hiccups, Headaches, Hygromas: Severe Presentation of Spontaneous Intracranial Hypotension (P5-4.012)

Hiccups, Headaches, Hygromas: Severe Presentation of Spontaneous Intracranial Hypotension (P5-4.012)

A Rare Case of Orthostatic Tremor Secondary to Spontaneous Intracranial Hypotension (P3-3.009)

A Rare Case of Orthostatic Tremor Secondary to Spontaneous Intracranial Hypotension (P3-3.009)

Characterization of the Prevalence and Treatment Strategies of Migraine in Patients with Refractory Spontaneous Intracranial Hypotension (P6-12.003)

Characterization of the Prevalence and Treatment Strategies of Migraine in Patients with Refractory Spontaneous Intracranial Hypotension (P6-12.003)

Analysis of the Epidemiologic Profile, Prevalence, and Incidence of Migraine out of 15,490 Spontaneous Intracranial Hypotension Patients from a Multicenter Record Database (TriNetX) (P2-12.008)

Analysis of the Epidemiologic Profile, Prevalence, and Incidence of Migraine out of 15,490 Spontaneous Intracranial Hypotension Patients from a Multicenter Record Database (TriNetX) (P2-12.008)

A hidden pathophysiology of endolymphatic hydrops: case report of a patient with spontaneous intracranial hypotension presenting with sudden sensorineural hearing loss with vertigo.

Spontaneous intracranial hypotension (SIH) is characterized by decreased cerebrospinal fluid (CSF) volume due to leakage through the dural membrane. We present the case of a patient with SIH manifested by fluctuating low-frequency hearing loss, tinnitus, and vertigo. In this patient, endolymphatic hydrops in the cochlea and saccule were visualized by means of a special sequence of inner ear magnetic resonance imaging scans, with a gadolinium-based contrast agent administered intravenously. Endolymphatic hydrops is a potential underlying pathophysiology of SIH-associated hearing impairment. We hypothesize that SIH may be a rare cause of endolymphatic hydrops.

Effect of blind versus fluoroscopic guidance epidural blood patch in patients with intracranial hypotension

Epidural blood patch (EBP)—the most effective treatment for intracranial hypotension (IH)—can be performed using the blind technique in the lateral position or under fluoroscopic guidance in the prone position. This study aimed to compare the effectiveness of EBP in patients with spontaneous intracranial hypotension (sIH) and iatrogenic intracranial hypotension (iIH) when performed with the blind technique in the lateral decubitus position or fluoroscopic guidance in the prone position. We reviewed IH patients who underwent EBP between January 2015 and September 2019 in a single hospital and divided them into two groups according to the type of IH. Of the 84 included patients, 36 had sIH and 48 had iIH. We compared the effectiveness of EBP using the two methods in each group. There was no significant difference in the effect of EBP between the patients with iIH (p > 0.05). For patients with sIH, fluoroscopic guidance in the prone position technique showed better improvement than the blind technique in the lateral decubitus position (p < 0.05). We observed similar outcomes after blind EBP versus fluoroscopic guidance EBP in patients with iIH. However, procedure-dependent differences were observed in patients with sIH. For patients with sIH, it would be effective to consider fluoroscopic EBP first. Further study is needed to investigate interactions between method of EBP and other factors that affect the effectiveness of EBP.

Full endoscopic resection of ventral thoracic osteophyte and repair of spontaneous CSF leak.

Spontaneous CSF leaks frequently cause headaches, meningismus, and nausea due to intracranial hypotension. When conservative treatment fails, surgical repair is indicated. Especially ventral leaks necessitate invasive approaches with substantial blood loss and tissue trauma. Full endoscopic spine surgery (FESS) enables circumferential access via the transforaminal approach. Here, the authors show the successful repair of a ventral CSF leak in the thoracic spine after removal of bony osteophytes utilizing FESS with placement of a dural substitute and sealant. Lasting symptom relief was reported. These results suggest that FESS is safe and efficient for the repair of spontaneous and incidental CSF leaks. The video can be found here: https://stream.cadmore.media/r10.3171/2024.1.FOCVID23209.