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.

Diagnostic and therapeutic insights in individuals with persistent post-dural puncture headache: A cross-sectional study.

BACKGROUND: Post-dural puncture headache (PDPH) is a frequent complication following lumbar puncture, epidural analgesia, or neuraxial anesthesia. The International Classification of Headache Disorders, third edition categorizes PDPH as a self-limiting condition; however, emerging evidence, including our findings, suggests that PDPH can have a prolonged course, challenging this traditional view. OBJECTIVES: To elucidate the diagnostic characteristics and treatment outcomes of persistent PDPH (pPDPH), offering insights into its demographic profiles and diagnostic features. METHODS: We executed an anonymous, web-based survey targeting individuals aged ≥18 years diagnosed with or suspected of having pPDPH. Recruitment occurred through self-help groups on Facebook. The survey comprised questions regarding diagnostic procedures, treatment regimens, outcomes, and healthcare consultation. RESULTS: The survey achieved a response rate of 179/347 (51.6%) individuals completing the questionnaire. Cerebrospinal fluid (CSF) leaks were confirmed in nine of 179 (5.0%) cases. Signs of intracranial hypotension without a CSF leak were observed in 70/179 (39.1%) individuals. All participants underwent magnetic resonance imaging scans of the brain and spine, with computed tomography myelography performed in 113/179 (63.1%) cases. Medications, including analgesics, theophylline, and gabapentin, provided minimal short-term relief. Epidural blood patch treatments resulted in slight-to-moderate short-term improvement in 136/179 (76.0%), significant improvement in 22/179 (12.3%), and complete effectiveness in eight of 179 (4.5%) individuals. For long-term outcomes, slight-to-moderate improvement was reported by 118/179 (66.0%) individuals. Surgical interventions were carried out in 42/179 (23.5%) patients, revealing pseudomeningoceles intraoperatively in 20/42 (47.6%) individuals. After surgery, 21/42 (50.0%) of the participants experienced slight-to-moderate improvement, 12/42 (28.6%) showed more pronounced improvement, and five of the 42 (11.9%) achieved complete effectiveness. CONCLUSION: This study underscores the complexities of managing pPDPH. The delay in diagnosis can impact the effectiveness of treatments, including epidural blood patch and surgical interventions, resulting in ongoing symptoms. This underscores the importance of tailored and adaptable treatment strategies. The findings advocate for additional research to deepen the understanding of pPDPH and improve long-term patient outcomes.

Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension.

Cerebrospinal fluid (CSF)-venous fistula can cause spontaneous intracranial hypotension (SIH) and poses a significant diagnostic and management challenge. This study aims to provide a comprehensive overview of the clinical and radiological outcomes of endovascular embolization as a novel treatment approach for CSF-venous fistula in patients with SIH. This systematic review adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The primary outcome was the efficacy of the embolization procedure in occlusion of the CSF-venous fistula, and secondary outcomes included procedural complications and improvement of clinical symptoms and radiological findings. A total of nine studies consisting of 77 patients met the inclusion criteria. Orthostatic and/or Valsalva headache was the most common symptom. The mean age of the patients was 57 ± 8.9 years, and females accounted for 59.7% (46/77) of the cases. Sixty-five (84.4%) patients reported complete resolution or significant improvement in symptoms. The Bern score, Headache Impact Test-6, and the Patient Global Impression of Change scales demonstrated significant improvements in radiological findings and patients' quality of life. Following the procedure, 22 patients (28.6%) experienced rebound intracranial hypertension and 27 patients (35.1%) had transient local pain at the site of the embolization. Our study showed that endovascular embolization is a safe and effective treatment for CSF-venous fistula in patients with SIH, providing complete resolution or significant improvement of clinical symptoms and radiological findings, and positive impacts on patients' quality of life.

Spontaneous Intracranial Hypotension: Role of Computed Tomography-guided Cervical Epidural Blood Patch.

BACKGROUND: Lumbar epidural blood patch (EBP) is not successful in all cases of cerebrospinal fluid (CSF) leak, particularly in the cervicothoracic region. The present study is a retrospective analysis of a cohort of patients who had undergone computed tomography (CT)-guided cervical EBP for spontaneous intracranial hypotension (SIH) due to CSF leak in the cervical region. METHODS: We retrospectively collected data from March 2009 to 2020. Our inclusion criteria were (1) clinical syndrome associated with CSF leak, (2) headache coming on shortly after assuming the erect position and relief achieved by lying down, (3) CT myelography evidence of CSF leak in the cervical region, and (4) patient not responding to conservative management for 7 days. Exclusion criteria were patients with bleeding diathesis and infection. There were six females and four males. Ages ranged from 32 to 57, with an average of 42 years. On presentation, all patients underwent contrast-enhanced magnetic resonance imaging (MRI) and CT myelography. Cervical EBP was done under CT scan guidance. Assessment of headache was done on a 10-point numerical rating scale (NRS) before and after the procedure. Results are categorized into complete relief, partial relief, and no relief categories. RESULTS: Nine out of 10 patients were completely recovered. One patient was partially recovered. The average NRS scale was 9.6 before treatment, which became 0.4 after EBP. No neurological or vascular complications were seen. CONCLUSION: Computed tomography-guided cervical EBP is an optimum and effective way of treating SIH due to a leak in the cervical region. It has a higher success rate than lumbar EBP. However, prospective randomized controlled trials of cervical vs lumbar EBP will further validate our observation.

[Technical specification of epidural blood patch for treatment of spinal cerebrospinal fluid leakage].

Spinal cerebrospinal fluid leakage is a common cause of spontaneous intracranial hypotension. Traditional treatment methods include conservative treatment and surgical treatment, but conservative treatment is ineffective for some patients, while surgical treatment is rarely used in clinical practice due to severe trauma. Minimally invasive surgery at appropriate time is an important method to handlecerebrospinal fluid leakage. Therefore, the Group of Headache and Facial Pain, Pain Branch of Chinese Medical Association formulated this technical specification of epidural blood patch for treatment of normal dural sac tension spinal cerebrospinal fluid leakage. This paper mainly discusses the concept and mechanism, indications and contraindications, operation methods, complications and treatment methods of epidural blood patch in order to improve clinical efficacy, reduce neuralsystem complications and reduce the incidence of adverse events.

Recurrence of cerebrospinal fluid-venous fistulas at different spinal levels following transvenous embolisation or blood/fibrin glue patching.

AIM OF THE STUDY: This study presents cases of recurrent cerebrospinal fluid-venous fistulas (CVFs) de novo at a different spinal level following successful treatment of initial CVFs. The aim was to highlight this rarely described phenomenon and report the clinical and imaging features after initial treatment, providing insights into the dynamics of recurrent CVFs. CLINICAL RATIONALE FOR THE STUDY: Understanding the course of CVFs post-treatment is crucial for optimising patient management, especially when symptoms persist or recur. MATERIAL AND METHODS: We performed a retrospective chart review of all patients with recurrent CVFs at a different level after treatment of their initial CVF at our institution. Clinical and imaging records were reviewed and summarised, including Bern score features on brain magnetic resonance imaging (MRI) before and after treatment. RESULTS: Four patients with five recurrent CVFs were identified. Recurrent or persistent symptoms encouraged subsequent brain MRI scans, which revealed different outcomes: i.e. persistence, or improvement, or complete resolution of abnormal findings. Initial positive responses included improvement of the pachymeningeal enhancement and venous sinus distension. These improvements were reversed when recurrent symptoms arose, which was also correlated with changes in the Bern score. CONCLUSIONS AND CLINICAL IMPLICATIONS: Recognising the factors of CVF recurrence is crucial for comprehensive management. This study underlines the significance of repeated evaluation of persistent or recurring symptoms of CSF leak after treatment for CVFs.

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.

BACKGROUND: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG). METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH. RESULTS: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided. CONCLUSIONS: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

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.

Spontaneous Intracranial Hypotension: Controversies in Treatment.

Spontaneous intracranial hypotension (SIH) is a disorder of low cerebrospinal fluid (CSF) volume secondary to CSF leakage through a dural defect along the neuraxis with an estimated incidence of 5 people per 100,000.Great debate persists on the optimal treatment of this pathology, and clinical results are often contradictory. SIH may be initially approached via conservative measures, such as bed rest that is often supplemented with hydration, caffeine, and theophylline, which overall relieve symptoms in a small subset of patients at 6 months. Epidural blood patching (EBP) is generally the next consideration in symptom management. It is the most commonly performed intervention for spinal CSF leaks, as the first option or following the failure of conservative treatment. Further procedures may be performed in the case of EBP partial or temporary response and if the spinal CSF leak has been definitively localized (Beck et al., Neurology 87:1220-26, 2016). In those cases, if the CSF leak is well localized, the surgical closure of the spinal CSF leak may be considered.SIH is a complex but treatable CSF disorder. Despite recent advances in the field of neuroimaging and the different therapeutic options available, the appropriate management remains controversial and should be tailored to the patient.

Algorithmic Multimodality Approach to Diagnosis and Treatment of Spinal CSF Leak and Venous Fistula in Patients With Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) is a disorder of CSF dynamics that causes a complex clinical syndrome and severe disability. SIH is challenging to diagnose because of the variability of its presenting clinical symptoms, the potential for subtle imaging findings to be easily overlooked, and the need for specialized diagnostic testing. Once SIH is suggested by clinical history and/or supported by initial neuroim-aging, many patients may undergo initial nontargeted epidural blood patching with variable and indefinite benefit. However, data suggest that precise localization of the CSF leak or CSF-venous fistula (CVF) can lead to more effective and durable treatment strategies. Leak localization can be achieved using a variety of advanced diagnostic imaging techniques, although these may not be widely performed at nontertiary medical centers, leaving many patients with the potential for inadequate workup or treatment. This review describes imaging techniques including dynamic fluoroscopic and CT myelography as well as delayed MR myelography and treatment options including percutaneous, endovascular, and surgical approaches for SIH. These are summarized by an algorithmic framework for radiologists to approach the workup and treatment of patients with suspected SIH. The importance of a multidisciplinary approach is emphasized.

Spontaneous intracranial hypotension: the role of radiology in diagnosis and management.

Spontaneous intracranial hypotension (SIH) is a condition that results from leakage of cerebrospinal fluid (CSF) from the spine, and which typically presents with debilitating orthostatic headache, but can be associated with a wide range of other symptoms. The causes of spontaneous CSF leaks that lead to SIH include dural tears, leaking meningeal diverticula, and CSF-venous fistulas. Imaging plays a central role in the initial diagnosis of SIH and in its subsequent investigation and management. This article reviews the typical neuroimaging manifestations of SIH and discusses the utility of different myelographic techniques for localising spinal CSF leaks as well as the role of image-guided treatment.

Risk factors and management strategies for cerebrospinal fluid leakage following lumbar posterior surgery.

OBJECTIVE: To analyze the risk factors of cerebrospinal fluid leakage (CSFL) following lumbar posterior surgery and summarize the related management strategies. METHODS: A retrospective analysis was performed on 3179 patients with CSFL strategies lumbar posterior surgery in our hospital from January 2019 to December 2020. There were 807 cases of lumbar disc hemiation (LDH), 1143 cases of lumbar spinal stenosi (LSS), 1122 cases of lumbar spondylolisthesis(LS), 93 cases of lumbar degenerative scoliosis(LDS),14 cases of lumbar spinal benign tumor (LST). Data of gender, age, body mass index(BMI), duration of disease, diabete, smoking history, preoperative epidural steroid injection, number of surgical levels, surgical methods (total laminar decompression, fenestration decompression), revision surgery, drainage tube removal time, suture removal time, and complications were recorded. RESULTS: The incidence of 115 cases with cerebrospinal fluid leakage, was 3.6% (115/3179).One-way ANOVA showed that gender, body mass index (BMI), smoking history, combined with type 2 diabetes and surgical method had no significant effect on CSFL (P > 0.05). Age, type of disease, duration of disease, preoperative epidural steroid injection, number of surgical levels and revision surgery had effects on CSFL (P < 0.05). Multivariate Logistic regression analysis showed that type of disease, preoperative epidural steroid injection, number of surgical levels and revision surgery were significantly affected CSFL (P < 0.05).Drainage tube removal time of CSFL patients ranged from 7 to 11 days, with an average of 7.1 ± 0.5 days, drainage tube removal time of patients without CSFL was 1-3 days, with an average of 2.0 ± 0.1 days, and there was a statistical difference between the two groups (P < 0.05).The removal time of CSFL patients was 12-14 days, with an average of 13.1 ± 2.7 days, and the removal time of patients without CSFL was 10-14 days, with an average of 12.9 ± 2.2 days, there was no statistically significant difference between the two groups (P > 0.05). CONCLUSION: Type of disease, preoperative epidural steroid injection, number of surgical levels and revision surgery were the risk factors for CSFL. Effective prevention were the key to CSFL in lumbar surgery. Once appear, CSFL can also be effectively dealt without obvious adverse reactions after intraoperative effectively repair dural, head down, adequate drainage after operation, the high position, rehydration treatment, and other treatments.

The effects of negative pressure wound therapy on wounds with cerebrospinal fluid leakages.

OBJECTIVE: Negative pressure wound therapy (NPWT) has been widely used for a variety of wounds with good outcomes. However, it is contraindicated in the management of wounds with cerebrospinal fluid (CSF) leakages. The reason is the fear of a sudden shift of CSF fluid as a result of suction leading to brain herniation or coning. There is a paucity of data to either support or negate such claims. We aimed to determine the effect of NPWT in the management of patients with wounds with CSF leakages. METHOD: This was a longitudinal prospective study of patients having wounds with CSF leakages managed with NPWT in our institute. Outcome measures evaluated were changes in the level of consciousness, Glasgow Coma Scale (GCS) score, length of hospital stay, mean effluent collected, time taken for the wounds to heal/close and complications related to NPWT. RESULTS: A total of 12 patients with wounds that had CSF leakage were managed with NPWT over two years. Of the patients, nine had wounds in communication with the brain, with three patients having spinal cord wounds. The average effluent collected on the first day of applying NPWT was 510ml, and by the third day this had fallen to approximately 200ml. The mean time taken for wounds to close was 12.3 days. There was no evidence of herniation nor any other morbidities or mortalities reported in any of our patients. CONCLUSION: NPWT, as indicated in this series, had a beneficial effect in the management of wounds with CSF leakage. NPWT, once applied to the wound, caused a tamponade effect that resulted in stoppage of the leakage of the CSF and did not lead to suction of the fluid, as previously thought. The dressings ensured an airtight system, preventing communication between the wound and the external environment, thus reducing sepsis while awaiting definitive wound closure.

[Bilateral subdural hematomas due to idiopathic cerebral spinal fluid leak treated by radiologically guided blood patch]. / Hygromes sous-duraux bilatéraux sur fuite de liquide céphalo-rachidien idiopathique traitée par «blood patch¼ radio-guidé.

We report a case of cerebral hypotension due to an idiopathic cerebral spinal fluid leak associated with bilateral sub-dural hygromas. The symptoms were not relieved despite of multiple lumbar, thoracic and cervical «blind¼ blood patches. The check-up led to a scopic controlled cervical blood patch directly on the leak by a paramedian approach with a complete clinical and radiological response to treatment.

Nous rapportons un cas d'hypotension cérébrale sur une fuite de liquide céphalo-rachidien d'origine idiopathique associée à la présence d'hygromes sous-duraux bilatéraux. La symptomatologie n'est pas soulagée malgré plusieurs «blood patchs¼ lombaires, thoraciques et cervicaux dits à l'aveugle. Le bilan a conduit à la réalisation d'un «blood patch¼ sous contrôle radiologique, ciblée sur le niveau de fuite par un abord paramédian avec réponse complète clinique et radiologique après cette procédure.

Efficacy and safety of cervicothoracic epidural blood patch for patients with spontaneous intracranial hypotension.

BACKGROUND: Epidural blood patch (EBP) is a generally effective treatment for spontaneous intracranial hypotension (SIH) caused by cerebrospinal fluid (CSF) leakage through the spinal dura mater. It is still unclear; however, whether application near the leakage site (targeted EBP) is more effective than distal application (untargeted EBP). Further, EBP targeted to high thoracic or cervical spine levels is infrequent due to greater technical requirements and potential complications. Here, we examined the safety and efficacy of EBP applied to high thoracic or cervical spine levels. METHODS: We retrospectively reviewed the clinical and outcome data of 13 patients receiving cervical or high thoracic EBP for SIH. All patients were referred by neurologists following poor response to conservative treatment and presented with persistent headache aggravated by orthostatic changes. RESULTS: Neuroimaging confirmed CSF leakage and targeted EBP resulted in immediate pain improvement. Repeated injections provided additional improvement for patients with recurrent headache. No serious adverse events were documented during follow-up. CONCLUSION: Based on recent studies and our clinical experience, we conclude that EBP targeted to the high thoracic and cervical spine is safe and effective for early-stage SIH.

[Spinal CSF-venous fistula: case report and literature review]. / Spinal'naya likvoro-venoznaya fistula kak prichina sindroma spontannoi vnutricherepnoi gipotenzii: sluchai iz praktiki i obzor literatury.

Syndrome of spontaneous intracranial hypotension is increasingly described in the literature as a multifactorial disease with impairment of the quality of life and risk of mortality. CSF-venous fistula as a cause of intracranial hypotension syndrome is extremely rare and requires complex diagnosis and timely surgical treatment. OBJECTIVE: We present a 55-year-old patient with acute spontaneous intracranial hypotension and spinal CSF-venous fistula. Literature data are also analyzed. RESULTS: Algorithm for diagnosis and efficacy of microsurgical resection of CSF-venous fistula is demonstrated. CONCLUSION: Intracranial hypotension following spinal fistula requires careful examination. Accurate understanding of pathophysiological and anatomical features of this disease is necessary to select an effective treatment method.

CT-guided Fibrin Glue Occlusion of Cerebrospinal Fluid-Venous Fistulas.

Background Cerebrospinal fluid-venous fistulas (CVFs) are one of the less common etiologic causes of spontaneous intracranial hypotension. CVFs are most commonly treated with open surgical ligation and have reportedly not responded well to percutaneous treatments. Purpose To study treatment outcomes of CT-guided fibrin glue occlusion for CVFs. Materials and Methods Retrospective review of medical records from two institutions was performed for all patients with CVFs who underwent CT-guided percutaneous fibrin glue occlusion from March to October 2020. CVFs were assessed for resolution or persistence at posttreatment decubitus CT myelography (CTM). Pre- and posttreatment brain MRI scans were reviewed for principal signs of spontaneous intracranial hypotension. Clinical symptoms were documented before and immediately after therapy, and the current symptoms to date after fibrin glue occlusion were documented. Results CT-guided fibrin glue occlusion was performed in 13 patients (mean age, 62 years ± 14 [standard deviation]; eight women) with CVFs. Ten of 10 patients who underwent final posttreatment decubitus CTM examinations showed CVF resolution. All 13 patients showed improvement on posttreatment brain MRI scans. All 13 patients are currently asymptomatic, although three patients were asymptomatic before fibrin glue occlusion. Conclusion CT-guided fibrin glue occlusion is an effective treatment for patients with cerebrospinal fluid-venous fistulas (CVFs). Direct fibrin glue administration within the CVF may be one of the key factors for success. Further studies are needed to determine the long-term efficacy of this treatment. © RSNA, 2021.

Risk factors for nonresponsive hydration in patients with spinal cerebrospinal fluid leakage.

BACKGROUND: Spinal cerebrospinal fluid (CSF) leakage is frequently encountered clinically after lumbar puncture or spontaneous events. Although some patients recover without treatment or after intensive hydration, some require an epidural blood patch (EBP). The risks of nonresponsive hydration remain unknown. Therefore, we identified the risk factors for patients with spinal CSF leakage nonresponsive to hydration. METHODS: We retrospectively reviewed patients diagnosed with spinal CSF leakage between January 2010 and March 2021. Clinical data, including patient age, sex, etiology, and radiological indications in magnetic resonance imaging, were compared between patients who were responsive and non-responsive to hydration. RESULTS: Of the 74 patients with spinal CSF leakage, 25 were responsive to hydration and 49 required EBP. Patients who were nonresponsive to hydration were older (39.27 vs. 34.32 years, P = 0.01), had a higher percentage of spontaneous intracranial hypotension (93.88% vs. 68.00%, P = 0.005), had more spinal CSF leakage (12.04 vs. 8.04, P = 0.01), and had a higher percentage of dural sinus engorgement (81.63% vs. 60.00%, P = 0.044). Spontaneous intracranial hypotension (odds ratio [OR]: 4.63; 95% confidence interval [CI]: 1.00-21.38) and having ≥9 spinal CSF leakages (OR: 3.29; 95% CI: 1.08-10.01), as indicated by magnetic resonance myelography, are considered risk factors for noneffective hydration. CONCLUSIONS: Patients with spinal CSF leakage who have spontaneous intracranial hypotension and those with ≥9 spinal CSF leakages are considered at risk for noneffective hydration. EBP should be considered early in these patients.

Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging.

OBJECTIVE: To determine the occurrence of cerebrospinal fluid (CSF)-venous fistulas, a type of spinal CSF leak that cannot be detected with routine computerized tomography myelography, among patients with orthostatic headaches but normal brain and spine magnetic resonance imaging. BACKGROUND: Spontaneous spinal CSF leaks cause orthostatic headaches but their detection may require sophisticated spinal imaging techniques. METHODS: A prospective cohort study of patients with orthostatic headaches and normal brain and conventional spine imaging who underwent digital subtraction myelography (DSM) to look for CSF-venous fistulas, between May 2018 and May 2020, at a quaternary referral center for spontaneous intracranial hypotension. RESULTS: The mean age of the 60 consecutive patients (46 women and 14 men) was 46 years (range, 13-83 years), who had been suffering from orthostatic headaches between 1 and 180 months (mean, 43 months). DSM demonstrated a spinal CSF-venous fistula in 6 (10.0%; 95% confidence interval [CI]: 3.8-20.5%) of the 60 patients. The mean age of these five women and one man was 50 years (range, 41-59 years). Spinal CSF-venous fistulas were identified in 6 (19.4%; 95% CI: 7.5-37.5%) of 31 patients with spinal meningeal diverticula but in none (0%; 95% CI: 0-11.9%) of the 29 patients without spinal meningeal diverticula (p = 0.024). All CSF-venous fistulas were located in the thoracic spine. All patients underwent uneventful surgical ligation of the fistula. Complete and sustained resolution of symptoms was obtained in five patients, while in one patient, partial recurrence of symptoms was noted 3 months postoperatively. CONCLUSION: Concerns about a spinal CSF leak should not be dismissed in patients suffering from orthostatic headaches when conventional imaging turns out to be normal, even though the yield of identifying a CSF-venous fistula is low.

Spontaneous intracranial hypotension: diagnostic and therapeutic workup.

Spontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal enhancement, and effacement of the suprasellar cistern have the highest diagnostic sensitivity. SIH is in almost all cases caused by spinal CSF leaks. Spinal MRI scans showing so-called spinal longitudinal extradural fluid (SLEC) are suggestive of ventral dural tears (type 1 leak) which are located with prone dynamic (digital subtraction) myelography. As around half of the ventral dural tears are located in the upper thoracic spine, additional prone dynamic CT myelography is often needed. Leaking nerve root sleeves typically associated with meningeal diverticulae (type 2 leaks) and CSF-venous fistulas (type 3 leaks) are proven via lateral decubitus dynamic digital subtraction or CT myelography: type 2 leaks are SLEC-positive if the tear is proximal and SLEC-negative if it is distal, and type 3 leaks are always SLEC-negative. Although 30-70% of SIH patients show marked improvement following epidural blood patches applied via various techniques definite cure mostly requires surgical closure of ventral dural tears and surgical ligations of leaking nerve root sleeves associated with meningeal diverticulae or CSF-venous fistulas. For the latter, transvenous embolization with liquid embolic agents via the azygos vein system is a novel and valuable therapeutic alternative.