A classification system of spontaneous spinal CSF leaks

Schievink, Wouter I.; Maya, M. Marcel; Jean-Pierre, Stacey; Nuño, Miriam A; Prasad, Ravi; Moser, Franklin G.

doi:10.1212/wnl.0000000000002986

Cited by 231 publications

(261 citation statements)

References 40 publications

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“…33 It should be emphasized, however, that meningeal diverticula are only one cause of spontaneous CSF leak (Fig. 36 Another recently identified cause of SIH is the CSF-venous fistula. Ventral dural tears are a major source of spontaneous CSF leaks, and are often associated with calcified thoracic disk herniations or osteophytes.…”

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

“…Some patients may have "high-flow" leaks where CSF rapidly pours out of the thecal sac, while others may have slower "low-flow" leaks, and still others may have leaks that are not apparent on imaging. 36 Although the precise yield of spinal imaging may vary by imaging modality, and depend on the population studied, it is clear that spinal imaging can contribute to the diagnosis of SIH in a substantial proportion of patients. 12 In another series of 102 SIH subjects evaluated with spine MRI, a leak was detected in 26% of patients.…”

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

“…17,35 One series of over 500 patients identified ventral dural tears as the case of SIH in approximately one-quarter of patients. 36 Another recently identified cause of SIH is the CSF-venous fistula. 37,38 In this entity, a direct connection between the CSF and paraspinal veins allows for unregulated loss of spinal fluid into the circulatory system, resulting in CSF hypovolemia.…”

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

“…Some patients may have "high-flow" leaks where CSF rapidly pours out of the thecal sac, while others may have slower "low-flow" leaks, and still others may have leaks that are not apparent on imaging. 17,36 In one investigation of 99 subjects with confirmed SIH, a CT myelogram showed clear CSF leakage 55% of the time. 12 In another series of 102 SIH subjects evaluated with spine MRI, a leak was detected in 26% of patients.…”

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

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Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions

2018

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Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

“…Some patients may have "high-flow" leaks where CSF rapidly pours out of the thecal sac, while others may have slower "low-flow" leaks, and still others may have leaks that are not apparent on imaging. 17,36 In one investigation of 99 subjects with confirmed SIH, a CT myelogram showed clear CSF leakage 55% of the time. 12 In another series of 102 SIH subjects evaluated with spine MRI, a leak was detected in 26% of patients.…”

Section: Summary Of Important Considerations In the Approach To Sihmentioning

confidence: 99%

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Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions

2018

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“…Described etiologies include meningeal diverticula, dural tears, osteophytic spicules, and CSF-venous fistulae 8. Connective tissue matrix disorders that predispose to dural fragility also increase the risk of spontaneous CSF leaks 9 10…”

Section: Introductionmentioning

confidence: 99%

Procedural predictors of epidural blood patch efficacy in spontaneous intracranial hypotension

Pagani-Estévez

Cutsforth‐Gregory

Morris

et al. 2019

Regional Anesthesia &Amp; Pain Medicine

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Background and objectiveEpidural blood patch (EBP) is a safe and effective treatment for spontaneous intracranial hypotension (SIH), but clinical and procedural variables that predict EBP efficacy remain nebulous.MethodsThis study is an institutional review board-approved retrospective case series with dichotomized EBP efficacy defined at 3 months. The study included 202 patients receiving 604 EBPs; iatrogenic cerebrospinal fluid leaks were excluded.ResultsOf the EBPs, 473 (78%) were single-level, 349 (58%) lumbar, 75 (12%) bilevel, and 56 (9%) multilevel (≥3 levels). Higher volume (OR 1.64; p<0.0001), bilevel (3.17, 1.91–5.27; p<0.0001), and multilevel (117.3, 28.04–490.67; p<0.0001) EBP strategies predicted greater efficacy. Only volume (1.64, 1.47–1.87; p<0.0001) remained significant in multivariate analysis. Site-directed patches were more effective than non-targeted patches (8.35, 0.97–72.1; p=0.033). Lower thoracic plus lumbar was the most successful bilevel strategy, lasting for a median of 74 (3–187) days.ConclusionsIn this large cohort of EBP in SIH, volume, number of spinal levels injected, and site-directed strategies significantly correlated with greater likelihood of first EBP efficacy. Volume and leak site coverage likely explain the increased efficacy with bilevel and multilevel patches. In patients with cryptogenic leak site, and either moderate disability, negative prognostic brain MRI findings for successful EBP, or failed previous lumbar EBP, a low thoracic plus lumbar bilevel EBP strategy is recommended. Multilevel EBP incorporating transforaminal administration and fibrin glue should be considered in patients refractory to bilevel EBP. An algorithmic approach to treating SIH is proposed.

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Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome

et al. 2021

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IMPORTANCE Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain.OBJECTIVE To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment outcomes for SIH.DATA SOURCES Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline-compliant systematic review and meta-analysis of the literature on SIH. Three databases were searched from inception to April 30, 2020: PubMed/MEDLINE, Embase, and Cochrane. The following search terms were used in each database: spontaneous intracranial hypotension, low CSF syndrome, low CSF pressure syndrome, low CSF volume syndrome, intracranial hypotension, low CSF pressure, low CSF volume, CSF hypovolemia, CSF hypovolaemia, spontaneous spinal CSF leak, spinal CSF leak, and CSF leak syndrome. STUDY SELECTION Original studies in English language reporting 10 or more patients with SIH were selected by consensus.DATA EXTRACTION AND SYNTHESIS Data on clinical presentation, investigations findings, and treatment outcomes were collected and summarized by multiple observers. Random-effect meta-analyses were used to calculate pooled estimates of means and proportions. MAIN OUTCOMES AND MEASURESThe predetermined main outcomes were the pooled estimate proportions of symptoms of SIH, imaging findings (brain and spinal imaging), and treatment outcomes (conservative, epidural blood patches, and surgical). RESULTSOf 6878 articles, 144 met the selection criteria and reported on average 53 patients with SIH each (range, 10-568 patients). The most common symptoms were orthostatic headache (92% [95% CI, 87%-96%]), nausea (54% [95% CI, 46%-62%]), and neck pain/stiffness (43% [95% CI, 32%-53%]). Brain magnetic resonance imaging was the most sensitive investigation, with diffuse pachymeningeal enhancement identified in 73% (95% CI, 67%-80%) of patients. Brain magnetic resonance imaging findings were normal in 19% (95% CI, 13%-24%) of patients. Spinal neuroimaging identified extradural cerebrospinal fluid in 48% to 76% of patients. Digital subtraction myelography and magnetic resonance myelography with intrathecal gadolinium had high sensitivity in identifying the exact leak site. Lumbar puncture opening pressures were low, normal (60-200 mm H 2 O), and high in 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%), and 3% (95% CI, 1%-6%), respectively. Conservative treatment was effective in 28% (95% CI, 18%-37%) of patients and a single epidural blood patch was successful in 64% (95% CI, 56%-72%). Large epidural blood patches (>20 mL) had better success rates than small epidural blood patches (77% [95% CI, 63%-91%] and 66% [95% CI, 55%-77%], respectively).CONCLUSIONS AND RELEVANCE Spontaneous intracranial hypotension should not be excluded on the basis of a nonorthostatic headache, normal neuroimaging findings, or normal lumbar puncture opening pressure. Despite the heterogeneous nature of the studies...

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A classification system of spontaneous spinal CSF leaks

Abstract: We identified 3 types of spontaneous spinal CSF leak in this observational study: the dural tear, the meningeal diverticulum, and the CSF-venous fistula. These 3 types and the presence or absence of extradural CSF form the basis of a comprehensive classification system.

Cited by 231 publications

References 40 publications

Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions

Spontaneous Intracranial Hypotension: 10 Myths and Misperceptions

Procedural predictors of epidural blood patch efficacy in spontaneous intracranial hypotension

Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome

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