Publication abstracts about spinal CSF leak from 2025

A collection of selected publication abstracts about spinal CSF leak / intracranial hypotension from 2025.
- Abstract links are included.
- Note that links to full-text are provided for open access papers.
Revisiting a Rare Anomaly Described 25 Years Ago in the AJNR: A Journey from Pediatric Hemifacial Microsomia and Middle Cranial Fossa Aplasia to CSF-Lymphatic Fistula and Spontaneous Intracranial Hypotension as an Adult
AUTHORS: Andrew L Callen, Ashoke R Khanwalkar, Michael L Cunningham, Samantha L Pisani Petrucci, Debayan Bhaumik, Danielle Wilhour, Premal Trivedi, Peter Lennarson, David A Zander
CITATION: AJNR Am J Neuroradiol. Published online March 27, 2025. doi:10.3174/ajnr.A8760
ABSTRACT: This report presents a unique case of a 39-year-old female with a congenital unilateral aplasia of the middle cranial fossa floor associated with atypical hemifacial microsomia, initially described in a case report at age 14, who later developed a transosseous cerebrospinal fluid (CSF)-lymphatic fistula through the C1 vertebral body. Dynamic CT cisternography confirmed the diagnosis, and a transnasal percutaneous fibrin glue occlusion successfully reversed the patient’s brain sag and improved her symptoms. This case highlights the evolving understanding of CSF dynamics and the diagnostic and therapeutic challenges posed by rare CSF leak variants.ABBREVIATIONS: CSF = cerebrospinal fluid; SIH = spontaneous intracranial hypotension.
PMID: 40147836
DOI:10.3174/ajnr.A8760
Reversal of Coma With Trendelenburg Position in Spontaneous Intracranial Hypotension
AUTHORS: Tony Zhang, Sara J Hooshmand, Nathaniel P Rogers Jr, David O Sohutskay, Michel Toledano, Ajay A Madhavan, John L Atkinson, Jeremy L Fogelson, Alejandro A Rabinstein, Jeremy K Cutsforth-Gregory, Rafid Mustafa
CITATION: Mayo Clin Proc. Published online April 7, 2025. doi:10.1016/j.mayocp.2025.02.006
ABSTRACT: Spontaneous intracranial hypotension (SIH) is a clinical and radiologic syndrome caused by spinal leakage of cerebrospinal fluid due to a dural tear, leaking meningeal diverticulum, or cerebrospinal fluid-venous fistula. Whereas the hallmark clinical feature of SIH is orthostatic headache, in rare instances, life-threatening complications may include altered consciousness and even coma as a result of extreme downward displacement of the midbrain and brainstem. We describe the clinical features, neuroimaging findings, management strategies, and short-term outcomes of 2 unique cases of severe SIH and the role of Trendelenburg position to reverse coma. Both patients demonstrated remarkable recovery to normal mentation (Glasgow Coma Scale score of 15) within 1 hour of placement in Trendelenburg position and recurrence of coma if allowed to be upright. These cases serve to highlight the importance of this simple maneuver for acute management of severe SIH while awaiting definitive leak localization and treatment.
PMID: 40196971
DOI: 10.1016/j.mayocp.2025.02.006
Enhancing Clarity in Dynamic Myelography Reporting: Results of a Survey of Patients and Referring Providers Evaluating a Standardized Reporting System in the Myelographic Workup of Patients with Suspected Spontaneous Intracranial Hypotension
AUTHORS: Andrew L Callen, Samantha L Pisani Petrucci, Debayan Bhaumik, Peter Lennarson, Marius Birlea, Jennifer MacKenzie, Jodi Ettenberg, Lalani Carlton Jones
CITATION: AJNR Am J Neuroradiol. Published online March 26, 2025. doi:10.3174/ajnr.A8751
BACKGROUND AND PURPOSE: Dynamic myelography is a critical diagnostic tool for identifying cerebrospinal fluid (CSF) leaks, yet the current lack of standardized reporting can lead to variability in both clinical decision-making and patient understanding. To address these issues, we developed the Spontaneous Intracranial Hypotension Reporting and Data System (SIH-RADS), a standardized scoring system designed to categorize findings on dynamic myelography based on the degree of diagnostic certainty. We then administered a survey to patients and referring providers in order to evaluate the perceived value, clarity, and impact of SIH-RADS on patient and provider experiences as an adjunct to traditional reporting methods for dynamic myelography.
MATERIALS AND METHODS: The SIH-RADS scoring system was developed as a collaborative effort between patients and physicians, with six categories ranging from “Definite Positive with Precise Localization” (SIH-RADS 5) to “Technical Failure” (SIH-RADS 0). Surveys were distributed to three groups: (1) patients who had undergone myelography at our institution for suspected SIH, (2) anonymous patients via private spinal CSF leak groups on social media who had previously undergone myelography, and (3) referring providers who order myelograms for SIH evaluation. Survey questions assessed understanding of traditional reports, clarity of the SIH-RADS system, its impact on decision-making, and preferences for future reporting. Statistical comparisons between local and anonymous patient responses were performed using chi-square tests for categorical variables and t-tests for continuous variables. The observational study STROBE Checklist was utilized, with the proposed methodology followed.
RESULTS: A total of 125 patients (78 local patients, 47 anonymous patients) and 13 providers participated in the survey. Among patients, 77% expressed a preference for SIH-RADS over traditional reporting methods, and 58% believed it would improve their understanding of myelography results. Among providers, 92% favored adopting SIH-RADS for future reports, with 85% rating it as very or extremely useful for guiding clinical decisions. 92% of providers reported that the standardized system would enhance communication with patients. Qualitative feedback emphasized the benefits of clearer categorization and actionable recommendations, while also highlighting opportunities to refine patient-facing language and address ambiguities in intermediate scores.
CONCLUSIONS: A structured reporting system improves the perceived clarity, utility, and communication of dynamic myelography findings among both patients and providers.
PMID: 40139902
DOI: 10.3174/ajnr.A8751
Efficacy of Traditional Epidural Patching versus Patching within Spinal Longitudinal Extradural Collections for Ventral Dural Cerebrospinal Fluid Leaks
AUTHORS: Andrew L. Callen, Samantha L. Pisani Petrucci, Peter Lennarson, Mark F. Sedrak, Adriana Gutierrez, Mark D. Mamlouk
CITATION: Radiology. 2025;314(3):e242194. doi:10.1148/radiol.242194
ABSTRACT: Background Epidural blood patching is frequently used to treat spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leaks. However, its effectiveness in sealing ventral dural tears, particularly in chronic cases with organized spinal longitudinal extradural collections (SLECs), is not well documented. Purpose To assess the efficacy of intra-SLEC patching compared with traditional patching for treatment of ventral dural tears. Materials and Methods This two-site retrospective cross-sectional study conducted between January 2019 and July 2024 included patients with SIH due to a ventral dural tear who underwent epidural patching. Organized SLECs, characterized by sharply demarcated, convex edges and confined to the ventral epidural space, were distinguished from unorganized SLECs, which show fluid distribution in both ventral and dorsal spaces. The Fisher exact test was used to compare the complication rate between treatment groups, and the χ2 test was used to compare the proportion of patients with SLEC resolution between treatment groups. Results Fifty-two patients (mean age, 44.9 years ± 9.5 [SD]; 30 male patients) were included; before treatment, 39 had organized SLECs and 13 had unorganized SLECs. Overall, 25% (13 of 52) of patients had SLEC resolution after treatment. Organized SLECs were less likely to resolve than unorganized SLECs (six of 39 [15%] vs seven of 13 [54%]; P = .02). In patients with organized SLECs, intra-SLEC patching had a higher success rate (33%; five of 15) than traditional patching (4%; one of 24; P = .046). Multivariable analysis showed that intra-SLEC patching (odds ratio, 13.24 [95% CI: 1, 149]; P = .04) and unorganized SLECs (odds ratio, 21.47 [95% CI: 2, 216]; P = .009) were associated with higher odds of SLEC resolution. Conclusion In 25% of patients with SIH, MRI performed after epidural blood patching showed resolution of the SLEC. Intra-SLEC patching was more effective than traditional patching for treating organized SLECs. © RSNA, 2025 See also the editorial by Urbach in this issue.
PMID: 40131109
DOI: 2010.1148/radiol.242194
Hypersensitivity Reactions to Fibrin Glue During Epidural Blood Patching
AUTHORS: Jennifer L Smith, Myoung J Kim, Linda Gray, Michael D Malinzak, Samantha Morrison, Amy P Stallings, Alaattin Erkanli, Peter G Kranz, Timothy J Amrhein
CITATION: AJNR Am J Neuroradiol. Published online March 20, 2025. doi:10.3174/ajnr.A8568
BACKGROUND AND PURPOSE: Fibrin glue is increasingly incorporated as a component in epidural blood patching (EBP) for the treatment of spinal CSF leaks. Hypersensitivity reactions are a potential complication of its use but are not well studied in the setting of EBP. The purpose of this study was to determine the incidence of hypersensitivity reactions to fibrin glue during EBP and to identify any predisposing factors associated with increased patient risk.
MATERIALS AND METHODS: A single-center retrospective cohort study with nested case-control design included patients who received fibrin glue EBP for the treatment of iatrogenic CSF leaks or spontaneous intracranial hypotension over 13 years. Patient demographics and multiple procedure-specific variables were collected. Cases were identified from the total cohort as those with hypersensitive reactions and matched with controls in a 1:3 ratio. The incidence of hypersensitivity reactions in the total cohort was calculated. Logistic regression models were fit to test for associations between variables and the development of a hypersensitivity reaction.
RESULTS: A total of 3065 CT-guided EBPs with fibrin glue were identified in 1574 individual patients. The incidence of hypersensitivity reactions was 0.49% per procedure and 0.95% per patient and never occurred during the first EBP with fibrin glue. Case-control analysis found higher odds for hypersensitivity reactions in patients with a lower BMI (OR 0.82 [0.71-0.96], P = .003), younger age (OR 0.95 [0.91-0.99], P = .011), and during procedures with inadvertent intravenous injections (OR 5.44 [1.34-22.01], P = .014).
CONCLUSIONS: We found a 0.49% incidence of hypersensitivity reactions during EBP with fibrin glue, none occurring during the first exposure. Younger age, lower BMI, and inadvertent intravenous injection during the procedure were associated with a higher likelihood of reactions. This study provides data useful for counseling patients on procedural risk and identifies variables for physicians to be aware of to help prevent life-threatening reactions to fibrin glue during EBP.
PMID: 40113253
DOI: 10.3174/ajnr.A8568
A case of progressive and irreversible visual loss as a consequence of delayed diagnosis in cerebrospinal fluid venous fistula
AUTHORS: Lucio Zeppa, Maria Laura Passaro, Amedeo Guida, Fabio Tortora, Carlo Petruzziello, Michele Rinaldi, Ciro Costagliola
CITATION: Eur J Ophthalmol. Published online March 13, 2025. doi:10.1177/11206721251321880
PURPOSE: Cerebrospinal fluid venous fistulas (CVFs) are a frequently underrecognized cause of cerebrospinal fluid (CSF) leaks, leading to intracranial hypotension and a wide range of symptoms, including visual disturbances. This case highlights the critical role of ophthalmologists in identifying CVFs as a cause of unexplained progressive vision loss.
METHODS: We present the case of a 45-year-old woman who developed progressive visual field loss over 15 years, ultimately resulting in blindness in one eye. Early neuroimaging and testing were normal despite symptoms of orthostatic headaches, photophobia, and hearing loss. Advanced imaging eventually identified a CVF at the T8 level. Surgical correction was performed, and the patient’s clinical response was assessed.
RESULTS: Surgical repair of the CVF led to clinical improvement, halting further progression of symptoms.
CONCLUSIONS: CVFs should be considered in patients with unexplained visual disturbances, particularly when accompanied by symptoms of intracranial hypotension. Early suspicion and collaboration with neurologists and neuroradiologists are essential for timely diagnosis and intervention. Ophthalmologists play a pivotal role in guiding appropriate referrals, ensuring multidisciplinary care to prevent irreversible visual and systemic complications.
PMID: 40080842
DOI: 10.1177/11206721251321880
Technical Tips for CT-Guided Fibrin Glue Patching of CSF-Venous Fistulas
AUTHOR: Mark D Mamlouk
CITATION: AJNR Am J Neuroradiol. 2025;46(3):597. Published 2025 Mar 4. doi:10.3174/ajnr.A8679
ABSTRACT: Fibrin glue patching can be an effective treatment for CSF-venous fistulas, along with transvenous embolization and surgical ligation.1,2 The technique differs from routine epidural patching, which is typically targeted in the dorsal or ventral epidural spaces. Instead, fibrin glue patching for CSF-venous fistulas is specifically targeted to the venous drainage pattern.3 There are 3 main locations to target: 1) the cyst-vein junction, 2) paravertebral “wall,” and 3) direct cyst puncture, and the goal is to sever the connection between the cyst and vein. In this video, technical tips for successful patching will be illustrated for these target locations.
PMID: 40037700
DOI: 2010.3174/ajnr.A8679
An arachnoid bleb and weeping dura-a persistent low flow CSF-leak after lumbar puncture
AUTHORS: Amir El Rahal, Katharina Wolf, Florian Volz, Jürgen Beck
CITATION: Oxf Med Case Reports. 2025;2025(2):omae182. Published 2025 Feb 22. doi:10.1093/omcr/omae182
🔓Open access! Full study available here.
BACKGROUND AND IMPORTANCE: Post-dural puncture headache (PDPH) is a well-recognized and frequently encountered complaint of Lumbar puncture. It usually resolves spontaneously over two weeks or with an epidural blood patch. Although known for a long time, PPDH could be linked to an arachnoidal bleb as a cause of cerebrospinal fluid (CSF) leakage. We report on surgical findings and impressive findings with low-flow CSF leakage in the context of an arachnoidal bleb.
CLINICAL PRESENTATION: We report a 42-year-old Neuroscientist with a 23-month history of positional headaches after a lumbar puncture (LP). Multiple Sclerosis was ruled out, but chronic orthostatic headache and tinnitus developed. Bed rest, caffeine tablets, IV hydration, and five high-volume epidural blood patches did not lead to improvement. However, brain fog and reduced ability to work ensued. A dynamic myelography did not reveal a CSF leak or a CSF-venous fistula, but a high-resolution T2Space Fat-saturated MRI detected an arachnoidal bleb at the alleged L3-L4 level. Microsurgical exploration identified the arachnoid bleb as a low-flow CSF leak. The video depicts Neomenbranes overlaying the dura mater appearing as a web and CSF oozing, washing a layer of blood away. CSF was leaking like a tear, and we had the impression of a weeping dura. Treatment involved bipolar shrinking, reinforcement of the dura and fibrin glue. The patient’s symptoms improved slowly post-surgery.
CONCLUSION: This case emphasises the importance of considering atypical causes of post-lumbar puncture complications, such as arachnoid blebs, and the role of surgery in identifying and treating these rare conditions.
PMID: 39990024
PMCID: PMC11845598
DOI: 10.1093/omcr/omae182
Patterns of Epidural Patch Distribution: The Influence of Spinal Level, Injection Technique, and Patch Volume/Composition on Craniocaudal and Ventral Epidural Dispersion
AUTHORS: Daniel Montes, Samantha L Pisani Petrucci, Debayan Bhaumik, Nadya Andonov, Peter Lennarson, Andrew L Callen
CITATION: AJNR Am J Neuroradiol. Published online February 25, 2025. doi:10.3174/ajnr.A8720
BACKGROUND AND PURPOSE: Epidural patching with autologous blood and/or fibrin sealant is a common treatment for spinal cerebrospinal fluid (CSF) leaks, yet the factors influencing patch distribution remain poorly understood. This study aimed to analyze the craniocaudal (CC) and ventral epidural (VE) extent of epidural patch material and investigate the impact of variables such as patch volume, composition, spinal level of injection, and patient habitus on distribution patterns.
MATERIALS AND METHODS: This retrospective, cross-sectional cohort study included patients who underwent CT-guided epidural patching from January to September 2024. Inclusion criteria were age ≥18 years, dorsal interlaminar (DI) or transforaminal (TFO) epidural patching using blood, fibrin, or both, and immediate post-patch imaging capturing the entire patch extent. Patch distribution was assessed for CC and VE spread. Statistical analyses included linear and logistic regression models, with multivariate analyses adjusting for confounders.
RESULTS: Of 152 patients patched during the study period, 33 met inclusion criteria (mean age 45.4 years; 84.1% female) with 44 spinal levels patched: cervical (6.8%), thoracic (68.2%), and lumbar (25%). Mean patch volume (PV) per needle was 7.2 mL, with a mean CC spread of 4.6 spinal levels. There was a positive relationship between PV and CC spread across all spinal levels (β = 0.29, p = 0.001). Patches in the cervical region demonstrated the highest CC spread efficiency (0.77 levels/mL) compared to thoracic (0.56 levels/mL) and lumbar patches (0.47 levels/mL; p < 0.01). DI injections achieved greater CC spread but less VE dispersion than TFO injections (5.0 vs. 3.2 levels; p = 0.02; 58.8% vs 70.0%, p = 0.52). VE spread occurred in 61.4% of cases and followed a non-linear pattern along the spine, with an inflection point at T3.
CONCLUSIONS: The distribution of epidural patch material is influenced by spinal level, PV, composition, and injection approach. Cervical patches provide the greatest spread efficiency relative to volume, while DI approaches enhance craniocaudal spread but reduce ventral dispersion.
PMID: 40000121
DOI: 10.3174/ajnr.A8720
Evaluation of Spontaneous Intracranial Hypotension Probabilistic Brain MRI Scoring Systems in Normal Patients
AUTHORS: Crystal H Kang, Ajay A Madhavan, John C Benson, Ian T Mark, Benjamin A Johnson-Tesch, Robert J McDonald, Jared T Verdoorn
CITATION: AJNR. American journal of neuroradiology, ajnr.A8713. Advance online publication. https://doi.org/10.3174/ajnr.A8713
BACKGROUND AND PURPOSE: Probabilistic brain MRI scoring systems have been introduced to stratify the likelihood of identifying a CSF leak at myelography in spontaneous intracranial hypotension (SIH). The Bern scoring system by Dobrocky et al. is now well recognized, with a scoring system by Benson et al. introduced more recently (referred to as the “Mayo” score in this study). Neither of these scoring systems have been thoroughly evaluated in patients without SIH. The goal of this study was to evaluate these scoring systems in patients without SIH to understand the specificity of these MRI findings.
MATERIALS AND METHODS: We retrospectively reviewed normal brain MRIs performed in patients without clinically suspected SIH. Each examination was reviewed by one of four board-certified neuroradiologists with extensive experience in SIH, and all criteria of both scoring systems were evaluated and recorded.
RESULTS: 90 patients were included. Bern score was low probability in 78% and intermediate probability in 22%. Mayo score was low probability in 100%. Relatively high rates of positivity were seen in three specific Bern score parameters, including prepontine cistern effacement 5.0 mm or less (53%), decreased mammilopontine distance 6.5 mm or less (40%), and suprasellar cistern effacement 4.0 mm or less (28%). All intermediate probability Bern scores were due to suprasellar cistern effacement plus either or both prepontine cistern effacement and decreased mammilopontine distance. All other parameters of both scoring systems were either never or very rarely positive.
CONCLUSIONS: All intermediate probability Bern scores were due to decreased CSF cistern measurements, which had relatively high positivity rates in our non-SIH patient cohort. Due to substantial overlap with normals, these measurements are not specific indicators of “brain sag”, a hallmark imaging finding for SIH, and are not specific for SIH when the only “positive” brain MRI finding(s). The Mayo score is likely more specific for SIH with low probability scores in all patients in our cohort.
PMID: 39979026
DOI: 10.3174/ajnr.A8713
Volumetric Changes of the Choroid Plexus Before and After Spinal CSF Leak Repair
AUTHORS: Karen Buch, Aaron Paul, Neo Poyiadji, William A Mehan
CITATION: American journal of neuroradiology, 10.3174/ajnr.A8514. Advance online publication. https://doi.org/10.3174/ajnr.A8514
BACKGROUND AND PURPOSE: Patients with intracranial hypotension from spinal CSF leaks have increased choroid plexus volumes in response to CSF leakage. The purpose of this study was to assess changes in choroid plexus volumes in patients before and after spinal CSF leak repair.
MATERIALS AND METHODS: This was a retrospective, institutional review board-approved study on patients with spinal CSF leak who had pre- and post-CSF leak repair MRI examinations. Brain MRIs with contrast were performed on a 1.5/3T scanner with acquisition of 3D T1 postcontrast (eg, Bravo, MPRAGE, and so forth). Choroid plexus volumes at the level of the trigonum ventriculi were calculated for the left and right sides on all pre- and posttreatment MRIs using Visage-7 segmentation tools. Basic demographic data, type of CSF leak, and choroid plexus volumes were recorded for all patients. Basic 2-tailed t tests were used to compare choroid plexus volumes between the pre- and posttreatment groups.
RESULTS: Twenty patients with spontaneous intracranial hypotension from spinal CSF leaks were included. Eleven patients (55%) had a type 1a (ventral tear) spinal CSF leak, 5 patients (25%) had type 1b (lateral tear), and 4 patients (20%) had a type 3 spinal CSF leak. The mean age was 47.6 years (SD, 13.8 years). The mean choroid plexus volumes pretreatment were 0.82 cm3 (SD, 0.29 cm3) compared with 0.38 cm3 (SD, 0.19 cm3) posttreatment (P value 0.01).
CONCLUSIONS: Significantly decreased choroid plexus volumes were seen in patients with spontaneous intracranial hypotension following spinal CSF leak repair. This finding highlights the modulation and dynamic role of the choroid plexus in states of low CSF volumes.
PMID: 39979028
PMCID: PMC11979801 DOI: 10.3174/ajnr.A8514
Early brain MRI changes following transvenous embolization of cerebrospinal fluid-venous fistulas in spontaneous intracranial hypotension
AUTHORS: Federico Cagnazzo, Emmanuelle Le Bars, Gaetano Ris, Nicolas Lonjon, Liesjet E H van Dokkum, Lucas Corti, Vincent Costalat, Anne Ducros
CITATION: Journal of neurointerventional surgery, jnis-2024-022957. Advance online publication. https://doi.org/10.1136/jnis-2024-022957
OBJECTIVE: To evaluate early and mid-term imaging and clinical outcomes following transvenous embolization of cerebrospinal fluid-venous fistulas (CSFVFs) in patients with spontaneous intracranial hypotension (SIH).
METHODS: From November 2022 to November 2024, 60 consecutive patients with SIH and confirmed CSFVF underwent transvenous embolization using Onyx. Of these, 40 patients underwent brain MRI pre-treatment, 24 hours post-treatment, and at a 3-month follow-up. The primary outcome was regression of brain MRI abnormalities at 24 hours and 3 months. Secondary outcomes included rates of symptom improvement, predictors of clinical improvement, and complication rates.
RESULTS: The mean patient age was 61 years, and 65% were female. All procedures were technically successful. The median SIH score significantly decreased from 6 pre-treatment to 3.5 at 24 hours (P=0.01) and to 2 at 3 months (P=0.004). Early improvement in SIH score correlated with clinical improvement at 24 hours (P=0.002), which was observed in 77.5% of patients. Pachymeningeal enhancement (87.5%) and venous sinus engorgement (75%) were the most common MRI abnormalities. Both findings regressed in approximately 50% of patients at 24 hours and in 80% of patients at 3 months. At 3 months, 82.5% of patients achieved complete clinical recovery. Rebound post-treatment headaches occurred in 32.5% of patients but resolved within 7 days. The morbidity rate was 0%.
CONCLUSIONS: Transvenous embolization of CSFVFs results in early and sustained clinical and imaging improvements in patients with SIH. These findings support the efficacy of this intervention as a primary treatment for CSFVFs.
PMID: 39947894
DOI: 10.1136/jnis-2024-022957
Intracranial pressure monitoring in patients with spontaneous onset of orthostatic headache
AUTHORS: Linda D’Antona, Sanjay Cheema, Dwij Mehta, Fion Bremner, Laurence Dale Watkins, Ahmed Kassem Toma, Manjit Singh Matharu
CITATION: The journal of headache and pain, 26(1), 27. https://doi.org/10.1186/s10194-024-01928-7
🔓 Open access! Full study available here.
BACKGROUND: Spontaneous intracranial hypotension (SIH) is a debilitating disorder, with an estimated annual incidence of 3.7 per 100,000. Diagnosing SIH can be challenging for clinicians, as patients frequently present with normal investigation findings. Intracranial pressure (ICP) monitoring has been proposed as a valuable tool for patients with orthostatic headaches that are highly suggestive of SIH but have inconclusive investigation results. The primary objective of this study was to determine the proportion of patients with spontaneous orthostatic headaches and normal diagnostic work-up who exhibited abnormal ICP monitoring results.
METHODS: This single-centre, retrospective observational study was conducted at a tertiary referral centre specialising in SIH and CSF dynamics disorders. Consecutive patients with spontaneous orthostatic headaches and inconclusive diagnostic work-up who underwent 24-hour ICP monitoring were considered eligible. The 24-hour ICP monitoring followed a standardised protocol, measuring median ICP and pulse amplitude (a marker of brain compliance) during the daytime, nighttime, and over the entire 24-hour period. Specific cut-offs for low and high ICP states were predetermined based on the best available current evidence.
RESULTS: Thirty-eight patients (23 females, mean age 41 years ± 14SD) were identified. All patients had orthostatic headaches with a spontaneous onset. The mean duration of symptoms was 46 months ± 36SD. ICP monitoring identified 3 patients (7.9%) with low ICP (mean of the median 24-hour ICP – 2 mmHg ± 2SD) and 6 patients (15.8%) with high ICP (mean of the median 24-hour ICP 9 mmHg ± 3SD). Obvious CSF dynamics disturbances were excluded in the remaining 29 patients (76.3%, mean of the median 24-hour ICP 3 mmHg ± 3SD). The only clinical feature that was more common in patients with abnormal ICP compared to patients with normal ICP results was audiovestibular disturbance, namely aural fullness or muffled hearing (67% versus 17%, p = 0.015). There were no complications from the ICP monitoring procedure for any patient.
CONCLUSIONS: When appropriately selected, patients with a clinical picture highly suggestive of SIH, who have a negative diagnostic work-up, may benefit from consideration of invasive ICP monitoring. Moreover, a significant minority of patients with orthostatic headache may paradoxically have a high CSF pressure state, which can be detected using ICP monitoring.
MEETING PRESENTATIONS: Portions of this work were presented in abstract and oral presentation form at the Twenty-eighth Anglo-Dutch Migraine Association meeting (08/06/2018), the Tenth Meeting of the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders (20/10/2018; Bologna, Italy), the Society of British Neurological Surgeons 2018 Autumn Meeting (19/09/2018; London, United Kingdom), and the European Association of Neurosurgical Societies 2023 congress (27/09/2023; Barcelona, Spain). This work is also part of the doctoral thesis of one of the authors (LD).
PMID: 39905291
PMCID: PMC11792413
DOI: 10.1186/s10194-024-01928-7
CT-Guided Epidural Contrast Injection for the Identification of Dural Defects
AUTHORS: Ian T Mark, Michael Oien, John C Benson, Jared Verdoorn, Ben Johnson-Tesch, D K Kim, Jeremy Cutsforth-Gregory, Ajay A Madhavan
CITATION: AJNR. American journal of neuroradiology, 46(1), 207–210. https://doi.org/10.3174/ajnr.A8437
ABSTRACT: Post-dural puncture headache is an increasingly recognized cause of chronic headache. Outside of clinical history and myelography that requires an additional dural puncture, there is no reliable diagnostic test to evaluate for persistent dural defects. We describe the injection of iodinated contrast into the dorsal epidural space under CT guidance in 5 patients as a potential tool to visualize persistent dural defects.
PMID: 39134368
PMCID: PMC11735425 (available on 2026-01-01)
DOI: 10.3174/ajnr.A8437
MRI and Surgical Findings Refine Concepts of Type 2 Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension
AUTHORS: Niklas Lützen, Jürgen Beck, Lalani Carlton Jones, Christian Fung, Theo Demerath, Alexander Rau, Charlotte Zander, Katharina Wolf, Florian Volz, Amir El Rahal, Horst Urbach
CITATION: Radiology, 314(2), e241653. https://doi.org/10.1148/radiol.241653
ABSTRACT: Background Type 2 lateral spinal cerebrospinal fluid (CSF) leakage occurs in approximately 20% of cases of spontaneous intracranial hypotension (SIH); however, the underlying pathologic mechanism remains ambiguous. Purpose To characterize MRI features of type 2 leaks, correlate them with intraoperative observations, and evaluate their diagnostic value. Materials and Methods Patients with SIH and type 2 leaks diagnosed between January 2021 and February 2023 were retrospectively identified. Characteristic imaging features from heavily T2-weighted MR myelography (T2-MRM) images were reevaluated (independently and blinded) in the type 2 leak sample mixed with a sample of 40 patients with SIH and type 1 (ventral) leaks. Available intraoperative data were reviewed for lateral dural tears, arachnoid outpouching, and ruptured spinal meningeal diverticula. Results Twenty-eight patients with SIH (mean age, 37.3 years ± 8.2 [SD]; 22 [79%] female patients) had 29 type 2 leaks between the T7 and L2 levels without side predominance. Characteristic cystic lesions with a broad dural base on the exiting nerve root sleeve were identified at T2-MRM; this “bud-on-branch” sign reflects an arachnoid outpouching herniating through a lateral dural tear, distinct from a meningeal diverticulum, which yielded a sensitivity of 79% (22 of 28; 95% CI: 59, 92) and a specificity of 100% (40 of 40; 95% CI: 91, 100) for leak location. Arachnoid outpouching was confirmed intraoperatively in 23 of 25 patients (92%; 95% CI: 81, 100), originating from the nerve root sleeve axilla in most patients (19 of 25, 76%; 95% CI: 59, 93); two of 25 patients (8%; 95% CI: 0, 19) had a dural tear only, and none had an underlying ruptured meningeal diverticulum. Conclusion This study showed that type 2 leaks are actually due to a lateral dural nerve root sleeve tear through which the arachnoid herniates, which contrasted the common perception that these leaks result from ruptured meningeal diverticula. These leaks had a characteristic anatomic distribution and MRI appearance with substantially facilitated leak localization in patients with SIH.
PMID: 39932414
DOI: 10.1148/radiol.241653
Defining the typical characteristics of orthostatic headache in patients with spontaneous intracranial hypotension
CITATION: Cephalalgia : an international journal of headache, 45(1), 3331024241308154. https://doi.org/10.1177/03331024241308154
🔓 Open access! Full study available here.
AUTHORS: Dwij Mehta, Sanjay Cheema, Sophie Glover, Ayman M Qureshi, Indran Davagnanam, Salwa Kamourieh, Parag Sayal, Ahmed Toma, Susie Lagrata, Clare Joy, Callum Duncan, Jane Anderson, Brendan Davies, Paul J Dorman, Heather Angus-Leppan, James Walkden, Jonathan Rohrer, Manjit S Matharu
BACKGROUND: Orthostatic headache (OH) is a common feature of various conditions, including spontaneous intracranial hypotension (SIH), but no precise definition currently exists outlining the typical OH characteristics. This ambiguity risks misdiagnosis with unnecessary investigations and delay in institution of treatment. The present study aimed to carry out structured phenotyping of OH in patients with SIH with the aim of outlining its typical characteristics.
METHODS: Eligible patients with clinico-radiological confirmed SIH underwent a structured interview, after which a specialist interest group utilised the modified Delphi process to analyse the data and achieve consensus on defining the typical characteristics of OH in SIH.
RESULTS: In total, 137 patients were recruited. OH was present in 75.9%. Typical OH characteristics in SIH were defined as having a baseline severity (lying flat) on waking up of ≤3 (0-10, verbal response scale), headache onset-time of ≤4.5 h on becoming upright, time to peak severity of ≤7.5 h and an offset to baseline severity within 1.5 h of recumbency. Intra-individual consistency in the onset and offset-time was deemed a necessary characteristic.
CONCLUSIONS: Defining typical OH characteristics has the potential of enhancing SIH diagnostics and management, at the same time as minimising unwarranted invasive procedures.
PMID: 39781568
DOI: 10.1177/03331024241308154
Cerebrospinal Fluid Leaks: Diagnosis, Management, and Outcomes
AUTHORS: Peter J Lennarson, Andrew L Callen
CITATION: Neurosurgery clinics of North America, 36(1), 53–64. https://doi.org/10.1016/j.nec.2024.08.003
ABSTRACT: Cerebrospinal fluid (CSF) leaks are a challenging condition characterized by the loss of CSF, leading to severe orthostatic headaches and other debilitating symptoms. Diagnosis and management require a multifaceted approach involving clinical evaluation, imaging, and various treatment modalities to improve patient outcomes and quality of life.
PMID: 39542549
DOI: 10.1016/j.nec.2024.08.003
Volumetric response after closure of a spinal CSF leak in patients with spontaneous intracranial hypotension: a multicompartmental longitudinal study
AUTHORS: Charlotte Zander, Niklas Lützen, Alexander Rau, Katharina Wolf, Philipp Arnold, Hansjörg Mast, Amir El Rahal, Florian Volz, Petra Cimflova, Jürgen Beck, Horst Urbach, Theo Demerath
CITATION: Journal of neurointerventional surgery, jnis-2024-022712. Advance online publication. https://doi.org/10.1136/jnis-2024-022712
BACKGROUND: Cerebrospinal fluid (CSF) loss in spontaneous intracranial hypotension (SIH) is accompanied by volume shifts between the intracranial compartments. This study investigated tricompartimental and longitudinal volume shifts after closure of a CSF leak.
METHODS: Patients with SIH and suitable pre-therapeutic and post-therapeutic imaging for volumetric analysis were identified from our tertiary care center between 2020 and 2023. The Bern SIH score was calculated. Pre-interventional and post-interventional volumetry encompassed the CSF, parenchymal and venous compartments (ie, venous sinus and choroid plexus volumes).
RESULTS: In total, 32 patients with SIH (49.7±16.0 years, 22 women) met inclusion criteria. The mean SIH score decreased between baseline (4.5±2.7) and early (2.7±2.3, <7 days after intervention), and also late follow-up (1.4±1.7, follow-up ≥7 days) after leak closure. This was accompanied by a significant increase in ventricular volume from 22.1 to 25.0 mL (P=0.01) at early follow-up, and 23.9 mL at later follow-up (P=0.080). In contrast, venous sinus volumes decreased from 13.8 to 9.6 mL (P=0.016) at early follow-up, and 10.0 mL (P=0.007) at late follow-up. No significant change in mean choroid plexus, total gray or total white matter volume was observed.
CONCLUSIONS: Closure of a spinal CSF leak leads to an early increase in ventricular CSF volume and a decrease in venous sinus volume. The results reflect the long-term convergence of the SIH score to normal values and indicate that permanent closure of a CSF leak induces a stable recompensation of the intracranial compartments without involving significant volume shifts within the cerebral parenchyma.
PMID: 39870517
DOI: 10.1136/jnis-2024-022712
CSF-Venous Fistulas Arising Intraosseously within Bone Remodeled by Meningeal Diverticula
AUTHORS: Ajay A Madhavan, Vinil Shah, J Levi Chazen, Waleed Brinjikji, Jeremy K Cutsforth-Gregory, Thien Huynh, Ben A Johnson-Tesch, Ian T Mark, Darya P Shlapak, Mark D Mamlouk
CITATION: AJNR. American journal of neuroradiology, 46(2), 421–425. https://doi.org/10.3174/ajnr.A8507
ABSTRACT: CSF-venous fistulas (CVFs) are a common and increasingly recognized type of spinal CSF leak. Most of these fistulas occur in the setting of spontaneous intracranial hypotension, though nonspontaneous cases have been described as well. In most instances, CVFs arise from the dome or neck of nerve root sleeve diverticula (also called meningeal diverticula). Venous drainage typically involves some combination of the internal epidural venous plexus and external vertebral venous plexus. Not uncommonly, venous drainage into the basivertebral venous plexus or other intraosseous veins can be seen. However, the drainage is usually a secondary finding related to normal communication between intraosseous and extraosseous veins. We have recently observed unique cases in which CVFs arise directly within the vertebral elements, resulting in primarily intraosseous drainage. It is possible that this phenomenon is secondary to prominent meningeal diverticula remodeling the adjacent vertebral elements. In this clinical report, we reviewed a multi-institutional series of CVFs exhibiting primary intraosseous drainage, with the goal of illustrating the imaging findings, treatment strategies, and outcomes of the patients. Nine cases were identified demonstrating this phenomenon. We show that intraosseous CVFs can arise in virtually any part of the vertebrae and describe how this feature affects
PMID: 39884834
DOI: 10.3174/ajnr.A8507
"Flow Void Sign": Flow Artifact on T2-Weighted MRI Can Be an Indicator of Dural Defect Location in Ventral Type 1 Spinal CSF Leaks
AUTHORS: Lalani Carlton Jones, Diogo G.L. Edelmuth, David Butteriss and Daniel J. Scoffings
CITATION: AJNR. American journal of neuroradiology, 46(1), 211–218. https://doi.org/10.3174/ajnr.A8445
ABSTRACT: Patients with spontaneous intracranial hypotension caused by type 1 dural defects typically have an epidural fluid collection on MRI. Still, the location of the defect is not usually readily identifiable on standard MRI sequences and can be at any point along the length of the collection. The most common location for type 1 leaks is ventral and, as such, these are most commonly associated with ventral predominant epidural fluid. Dynamic myelography (either digital subtraction myelography or dynamic CT myelography) is currently the standard of care for localizing the defect. We describe an imaging sign on T2-weighted images caused by CSF-flow egress at the site of the defect that may permit accurate prediction of the site of the CSF leak noninvasively. Importantly, this sign was only observed on 2D T2-weighted and STIR images and not on 3D acquisitions, which notably suppress artifacts. This has implications for optimal MRI spine protocol construction. This sign can be used to limit myelographic range, reduce radiation dose, and increase diagnostic confidence in dural defect location.
PMID: 39134372
PMCID: PMC11735451 (available on 2026-01-01)
DOI: 10.3174/ajnr.A8445