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Introduction Spontaneous intracranial hypotension (SIH) is an important cause of headache that might require invasive treatment. The aim of this study was to systematically investigate (1) clinical presentation, (2) factors associated with incomplete headache resolution, and (3) the long‐term outcomes in patients with persistent headache after invasive treatment for SIH. Methods This is an observational longitudinal study. We used a structured questionnaire to assess details on primary headache, SIH‐headache, and headache after treatment. Persistent headache was defined as headache on more than 15 days per month lasting longer than 3 months. Results Fifty‐six patients invasively treated for SIH were included in the study. The mean age was 49 ± 12 years, and 60% were women. After sealing of the leak, 11/56 (20%) had persistent headache. Compared to subjects without persistent headache, patients with persistent headache had been treated after a longer delay from SIH symptom onset (362 days [IQR 138–714] vs. 111 [68–365]). In 2/11 (18%) patients, a second leak at another level and rebound intracranial hypertension were found, respectively. Medication overuse was reported by 3/11 (27%) patients. After a median follow‐up of 5 years, headache subsided completely in 4/11 (36%) patients and improved in 4/11 (36%). Conclusion In our cohort, one fifth of patients suffered from persistent headache despite successful sealing of the CSF leak. Although the majority of patients showed improvement in the long run, important secondary headaches should be considered, namely medication overuse, rebound hypertension, and a persistent, reopened, de novo or second leak at another level.

Dynamic CT myelography is used to precisely localize fast spinal CSF leaks. The procedure is most commonly performed in the prone position, which successfully localizes most fast ventral leaks. We have recently encountered a small subset of patients in whom prone dynamic CT myelography is unsuccessful in localizing leaks. We sought to determine the added value of lateral decubitus dynamic CT myelography, which is occasionally attempted in our practice, in localizing the leak after failed prone dynamic CT myelography. We retrospectively identified 6 patients who underwent lateral decubitus dynamic CT myelography, which was performed in each case because their prone dynamic CT myelogram was unrevealing. Two neuroradiologists independently reviewed preprocedural spine MRI and all dynamic CT myelograms for each patient. Lateral decubitus positioning allowed for precise leak localization in all 6 patients. Five of six patients were noted to have dorsal and/or lateral epidural fluid collections on spine MRI. One patient had a single prominent diverticulum on spine MRI (larger than 6 mm), whereas the others had no prominent diverticula. Our study suggests that institutions performing dynamic CT myelography to localize fast leaks should consider a lateral decubitus study if performing the study in the prone position is unrevealing. Furthermore, the presence of dorsal and/or lateral epidural fluid collections on spine MRI may suggest that a lateral decubitus study is of higher yield and could be considered initially.

Background This report analyzes traumatic anterior skull base CSF leaks following nasopharyngeal swab testing for detection of SARS-CoV-2 in the largest case series to date, combined with a systematic literature review. Methods Retrospective multi-institutional case-series of traumatic anterior skull base CSF leak with clear antecedent history of COVID-19 swab was completed. A comprehensive search of databases was performed for the systematic literature review. Results Thirty-four patients with traumatic CSF leak after COVID-19 nasopharyngeal swab testing were identified. Women were more than twice as likely to experience a CSF leak, as compared to men. The majority of patients (58.8%) had no reported predisposing factor in their clinical history. Common defect sites included the cribriform plate (52.9%), sphenoid sinus (29.4%), and ethmoid roof (17.6%). Four patients (11.8%) presented with meningitis. The median time between the traumatic COVID swab and the detection of CSF leak was 4 weeks (IQR 1–9). Patients with meningitis had a median leak duration of 12 weeks (IQR 8–18). The average leak duration was significantly longer in patients with meningitis compared to without meningitis (p = 0.029), with a moderate effect size (r = − 0.68). Most cases (92.9%) managed with endoscopic endonasal surgical repair were successful. Conclusions This report clarifies the presentation, risk factors, and management of CSF leaks attributable to diagnostic nasopharynx swabbing procedures in the COVID-19 era. Timely surgical repair is the recommended management option for such leaks.

ObjectiveCerebrospinal fluid rhinorrhoea takes place when there is a breakdown of the barriers separating the nasal cavity from the subarachnoid space. The aim of this study was to assess the surgical outcomes of endoscopic transnasal cerebrospinal fluid leak repair with and without fibrin glue.MethodThere were 43 patients with cerebrospinal fluid rhinorrhoea who underwent surgery for cerebrospinal fluid leak repair between 2014 and 2018. Patients were divided into group A, where fibrin glue was used, and group B, where fibrin glue was not used.ResultsIt was found that 74.4 per cent of cases were due to spontaneous cerebrospinal fluid leak. The most common site of a leak was the cribriform plate (65 per cent). There was a success rate of 96.1 per cent (25 of 26) in group A and 83 per cent (15 of 17) in group B. There was no statistically significant difference between the results of the two groups (chi-square test: p = 0.31).ConclusionThere was no statistically significant difference in the results of cerebrospinal fluid leak repair with and without fibrin glue.

Background Spontaneous intracranial hypotension (SIH) is a debilitating condition requiring effective treatment; however, objective data on treatment response are scarce. Purpose To assess the suitability of the brain MRI-based SIH score (bSIH) for monitoring treatment success in SIH patients with a proven spinal cerebrospinal fluid (CSF) leak after microsurgical closure of the underlying dural breach. Methods This retrospective cohort study included consecutive SIH patients with a proven spinal CSF leak, investigated at dedicated referral centre January 2012 to March 2020. The bSIH score integrates 6 imaging findings; 3 major (2 points) and 3 minor (1 point), and ranges from 0 to 9, with 0 indicating low and 9 high probability of spinal CSF loss. The score was calculated using brain magnetic resonance imaging (MRI) before and after surgical treatment of the underlying CSF leak. Headache intensity was registered on a numeric rating scale (NRS) (range 0–10). Results In this study 52 SIH patients, 35 (67%) female, mean age 45.3 years, with a proven spinal CSF leak were included. The mean bSIH score decreased significantly from baseline to after surgical closure of the underlying dural breach (6.9 vs. 1.3, P  < 0.001). A decrease in the NRS score was reported (8.6 vs. 1.2, P  < 0.001). Conclusion The bSIH score is a simple tool which may serve to monitor treatment success in SIH patients after surgical closure of the underlying spinal dural leak. Its decrease after surgical closure of the underlying spinal dural breach indicates restoration of an equilibrium within the CSF compartment.

Intracranial hypotension due to multiple spinal epidural cerebrospinal fluid (CSF) leak is rare and has multiple etiologies. These patients require epidural blood patches (EBP). We report a patient with recurrent paraplegia due to intracranial hypotension following recurrent epidural CSF leak. Cerebrospinal fluid leak was noted at D1, D10, D11, and L2 levels. Autologous epidural blood patch (EBP) at lower thoracic (3 ml) and lumbar region (4 ml) was performed. The patient developed paraplegia with sensory level at D2 for which 3 ml of EBP was done at D1 level after two months. Following EBP, the patient developed quadriplegia and root pain, which recovered in one month. The patient was free of symptoms for 18 months. Spinal epidural CSF leak should be suspected in patients with features of low pressure headache and recurrent paraplegia at multiple levels. Nuclear scintigraphy and CT myelography help in localizing the site of leak.

Objectives To review the safety and efficacy of surgical management for spontaneous cerebrospinal fluid (CSF) leaks of the anterior and lateral skull base. Data Sources A systematic review of English articles using MEDLINE. Review Methods Search terms included spontaneous, CSF, cerebrospinal fluid, endoscopic, middle fossa, transmastoid, leak, rhinorrhea. Independent extraction of articles by 3 authors. Results Patients with spontaneous CSF leaks are often obese (average BMI of 38 kg/m2) and female (72%). Many patients also have obstructive sleep apnea (∼45%) and many have elevated intracranial pressure when measured by lumbar puncture. In addition to thinning of the skull base, radiographic studies also demonstrate cortical bone thinning. Endoscopic surgical repair of anterior skull base leaks and middle cranial fossa (MCF) approach for repair of lateral skull base leaks are safe and effective with an average short‐term failure rate of 9% and 6.5%, respectively. Long‐term failure rates are low. One randomized trial failed to show improved success of anterior leak repairs with the use of a lumbar drain (LD) (95% with vs. 92% without; P = 0.2). In a large retrospective cohort of MCF lateral skull base repairs, perioperative LD use was not necessary in >94% of patients. Conclusions Spontaneous CSF leaks are associated with female gender, obesity, increased intracranial hypertension, and obstructive sleep apnea. Endoscopic repair of anterior skull base leaks and MCF or transmastoid approaches for lateral skull base leaks have a high success rate of repair. In most cases, intraoperative placement of lumbar drain did not appear to result in improved success rates for either anterior or lateral skull base leaks. Level of Evidence 2a, Systematic Review.

Cerebrospinal fluid (CSF) rhinorrhoea is the leakage of CSF through the communication between the subarachnoid space and the nasal cavity. Surgical repair is indicated in all cases of spontaneous leaks, recurrent leaks, leaks that do not stop after conservative management and cases with history of meningitis. We present a rare case of spontaneous (delayed onset post traumatic) CSF rhinorrhoea with multiple defect sites, which was treated with an endonasal endoscopic repair. The patient was asymptomatic for 9 years after surgery, and then presented with a spontaneous left frontal recess CSF leak, which was closed using endonasal approach.

Abstract BACKGROUND Spontaneous intracranial hypotension (SIH) is usually caused by a spinal cerebrospinal fluid (CSF) leak. CSF-venous fistula is an underdiagnosed cause of spinal CSF leak, as it is challenging to identify on myelography. OBJECTIVE To review existing literature to summarize common presentations, diagnostic imaging modalities, and current treatment strategies for CSF-venous fistulas. METHODS We conducted a systematic review using PubMed, Embase, Scopus, and Web of Science databases to identify studies discussing CSF-venous fistulas. Titles and abstracts were screened. Studies meeting prespecified inclusion criteria were reviewed in full. RESULTS Of 180 articles identified, 16 articles met inclusion criteria. Individual patient data was acquired from 7 studies reporting on 18 patients. CSF-venous fistula most frequently presented as positional headache. Digital subtraction myelography provided greatest detection of CSF-venous fistula in the lateral decubitus position and detected CSF-venous fistula in all individual patient cases. Dynamic computed tomography (CT) myelogram enabled detection and differentiation of CSF-venous fistulas from low-flow epidural leaks. The majority of fistulas were in the thoracic spine and slightly more common on the right. Epidural blood patch (EBP) provided temporary or no relief in all individual patients. Resolution or improvement of clinical symptoms and radiologic normalization were observed in all surgically treated patients. CONCLUSION Although rare, CSF-venous fistula is an important cause of spinal CSF leak contributing to SIH. Dynamic CT myelogram and digital subtraction myelography, particularly in the lateral decubitus position, are the most accurate and effective diagnostic imaging modalities. EBPs often provide only transient relief, while surgical management is preferred.

Purpose of Review The purpose of this study is to provide an update on recent developments in the understanding, diagnosis, and treatment of spontaneous intracranial hypotension (SIH). Recent Findings SIH is an important cause of headaches caused by spinal cerebrospinal fluid (CSF) leaks, with an increasingly broad spectrum of clinical presentations and diagnostic findings. A simple conception of the condition as being defined by the presence of low CSF pressure is no longer sufficient or accurate. A number of etiologies for spinal CSF leaks have been identified, including the recent discovery of CSF-venous fistulas, and these various etiologies may require different diagnostic and therapeutic pathways in order to affect a cure. Summary Familiarity with the spectrum of presentations and causes of SIH is critical to accurate and timely diagnosis and management. Challenges exist in both diagnosis and treatment, and require understanding of the underlying pathogenesis of the condition in order to appropriately select testing and treatment. Prospective studies are needed going forward in order to inform workup and guide treatment decisions.