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.

Management of post blood patch severe rebound intracranial hypertension by the usage of an external ventricular drain.

Spontaneous intracranial hypotension (SIH) is a condition characterised by postural headaches due to low cerebrospinal fluid (CSF) pressure, often stemming from CSF leakage. Diagnosis poses a significant challenge, and the therapeutic approach encompasses both conservative measures and operative interventions, such as the epidural blood patch (EBP). However, EBP carries the potential risk of inducing rebound intracranial hypertension (RIH), subsequently leading to high-pressure headaches. We present a case wherein RIH following EBP was effectively managed through the implementation of an external ventricular drain (EVD) aimed at reducing CSF pressure. The patient improved significantly, underscoring the potential utility, if not necessity, of EVD in carefully selected cases, highlighting the imperative for further research to enhance the management of SIH and optimise EBP-related complications.

The cervical blood patch: A therapeutic "miraculous" for cerebrospinal fluid leaks: A case report.

RATIONALE: Cerebrospinal fluid (CSF) leaks, arising from abnormal openings in the protective layers surrounding the spinal cord and brain, are a significant medical concern. These leaks, triggered by various factors including trauma, medical interventions, or spontaneous rupture, lead to the draining of CSF-an essential fluid safeguarding the nervous system. A classic symptom of CSF leaks is an incapacitating headache exacerbated by sitting or standing but relieved by lying down. Spontaneous intracranial hypotension (SIH) denotes the clinical condition marked by postural headaches due to spontaneous CSF leakage and hypotension, often misdiagnosed or underdiagnosed. While orthostatic headaches are the hallmark, SIH may manifest with an array of symptoms including nausea, tinnitus, hearing loss, visual disturbances, and dizziness. Treatment options encompass conservative measures, epidural blood patches (EBP), and surgery, with EBP being the primary intervention. PATIENT CONCERN: The patient did not express any specific concerns regarding their medical diagnosis. However, they did harbor apprehensions that their condition might necessitate surgical intervention in the future. DIAGNOSIS: The patient had been treated with antibiotics with a pre-diagnosis of sinusitis and was admitted to the neurology department of our hospital when his symptoms failed to improve. Cranial magnetic resonance imaging was interpreted as thickening of the dural surfaces and increased contrast uptake, thought to be due to intracranial hypotension. Cranial MR angiography was normal. Full-spine magnetic resonance imaging revealed a micro-spur at the C2 to 3 level and the T1 to 2 level in the posterior part of the corpus. INTERVENTIONS: The cervical EBP was performed in the prone position under fluoroscopic guidance. There were no complications. OUTCOMES: The patient was invited for follow-up 1 week after the procedure, and control examination was normal. LESSONS: SIH poses a diagnostic challenge due to its diverse clinical presentation and necessitates precise imaging for effective intervention. Cervical EBP emerges as a promising treatment modality, offering relief and improved quality of life for individuals grappling with this condition. However, clinicians must carefully assess patients and discuss potential risks and benefits before opting for cervical blood patches.

Identifying Patients with CSF-Venous Fistula Using Brain MRI: A Deep Learning Approach.

BACKGROUND AND PURPOSE: Spontaneous intracranial hypotension is an increasingly recognized condition. Spontaneous intracranial hypotension is caused by a CSF leak, which is commonly related to a CSF-venous fistula. In patients with spontaneous intracranial hypotension, multiple intracranial abnormalities can be observed on brain MR imaging, including dural enhancement, "brain sag," and pituitary engorgement. This study seeks to create a deep learning model for the accurate diagnosis of CSF-venous fistulas via brain MR imaging. MATERIALS AND METHODS: A review of patients with clinically suspected spontaneous intracranial hypotension who underwent digital subtraction myelogram imaging preceded by brain MR imaging was performed. The patients were categorized as having a definite CSF-venous fistula, no fistula, or indeterminate findings on a digital subtraction myelogram. The data set was split into 5 folds at the patient level and stratified by label. A 5-fold cross-validation was then used to evaluate the reliability of the model. The predictive value of the model to identify patients with a CSF leak was assessed by using the area under the receiver operating characteristic curve for each validation fold. RESULTS: There were 129 patients were included in this study. The median age was 54 years, and 66 (51.2%) had a CSF-venous fistula. In discriminating between positive and negative cases for CSF-venous fistulas, the classifier demonstrated an average area under the receiver operating characteristic curve of 0.8668 with a standard deviation of 0.0254 across the folds. CONCLUSIONS: This study developed a deep learning model that can predict the presence of a spinal CSF-venous fistula based on brain MR imaging in patients with suspected spontaneous intracranial hypotension. However, further model refinement and external validation are necessary before clinical adoption. This research highlights the substantial potential of deep learning in diagnosing CSF-venous fistulas by using brain MR imaging.

Spontaneous intracranial hypotension presenting with bilateral subdural hematoma: Decision-making and treatment strategies.

BACKGROUND: The timing and decision to drain subdural hematoma (SDH) in spontaneous intracranial hypotension (SIH) remains a dilemma. We reviewed our experience of bilateral SDH secondary to SIH, focusing on decision making and treatment strategies. METHODS: We retrospectively reviewed bilateral SDH secondary to SIH between March 2010 and September 2021. Baseline characteristics of patients, diagnosis, radiologic findings, treatments, and clinical outcome were investigated. RESULTS: Fifteen patients (7 men, 8 women) with bilateral SDH secondary to SIH were included in this study. Initially, patients were treated conservatively (4 patients, 26.7 %), with an epidural blood patch (EBP, 3 patients, 20.0 %), and SDH drainage followed by the Trendelenburg position (8 patients, 53.3 %). All 3 patients that were initially treated with EBP required SDH drainage. Of the 8 patients initially treated with SDH drainage via burr hole followed by Trendelenburg position, 7 patients showed sustained improvements without EBP; however, 1 patient needed EBP. Deterioration to coma occurred in 6 out of 15 patients (40.0 %). All 6 deteriorated patients immediately recovered after SDH drainage with Trendelenburg position; 5 achieved sustained improvement without EBP and 1 required EBP. During the follow-up period, 14 out of 15 patients (93.3 %) showed good recovery. CONCLUSIONS: Evacuation of SDH is not always necessary in SIH; however, we did not hesitate to perform hematoma drainage, in deteriorated patients or those with thick hematoma that is associated with significant sagging and cistern effacement. This can prevent irreversible neurologic complications. Moreover, the Trendelenburg position may help to achieve sustained improvement without additional treatment.

Cerebrospinal fluid-lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch - a rare case report and review of the literature.

Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

Percutaneous Treatment and Post-treatment Management of CSF Leaks and CSF-Venous Fistulas in Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) is a treatable cause of orthostatic headaches secondary to pathologic loss of cerebrospinal fluid (CSF) from the subarachnoid space. SIH has several known pathologic causes including dural tears from disc osteophytes, leaks emanating from nerve root sleeve diverticula, and CSF-venous fistulas (CVFs). Depending on the type of leak, surgical repair or endovascular techniques may be options for definite treatment. However, epidural blood patching (EBP) remains first-line therapy for many patients due to its long track record, broad availability, and relatively lower risk profile. This review focuses on indications and techniques for the percutaneous treatment of SIH and provides an overview of post-procedural management of these patients.

Endovascular Embolization Techniques for Cerebrospinal Fluid-Venous Fistula in the Treatment of Spontaneous Intracranial Hypotension.

Cerebrospinal fluid-venous fistula (CVF) is an important cause of spontaneous intracranial hypotension (SIH), a condition characterized by low cerebrospinal fluid (CSF) volume and orthostatic headaches. The pathogenesis of CVF is thought to be direct connection of the spinal dura to one or more veins in the epidural space, allowing unregulated flow of CSF into the venous system. Herein, we provide a comprehensive review of the endovascular management of CVF in patients with SIH. We also focus on the various techniques and devices used in endovascular treatment, as well as the pathogenesis, diagnosis, and alternative treatment options of CVF.

A new modified technique of dynamic CT myelography to detect dural tears in spontaneous intracranial hypotension.

We propose a modified dynamic CT-myelography technique for patients with fast CSF leaks caused by ventral dural tears in order to reduce radiation exposure and complications. A fluoroscopy-guided lumbar puncture using an epidural anesthesia kit replaces a CT-guided lumbar puncture, and a smaller volume of less concentrated contrast media is used. This approach has advantages, including speeding up the procedure, reduced radiation exposure, and elimination of the risk of contrast injection into the epidural space.

Distinct Pattern of Membrane Formation With Spinal Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension.

BACKGROUND AND OBJECTIVES: To systematically describe pertinent, intraoperative anatomic findings encountered when approaching spinal cerebrospinal fluid (CSF) leaks and CSF-venous fistulas in spontaneous intracranial hypotension (SIH). METHODS: In a retrospective study, we included surgically treated patients suffering from SIH at our institution from April 2018 to March 2022. Anatomic, intraoperative data were extracted from operative notes and supplemented with data from surgical videos and images. Prominent anatomic features were compared among different types of CSF leaks. RESULTS: The study cohort consists of 120 patients with a mean age of 45.2 years. We found four distinct patterns of spinal membranes specifically associated with different types of CSF leaks: (i) thick, dorsal membranes, which were hypervascular and may mimic the dura (pseudodura); (ii) thin, lateral membranes encapsulating a ventral epidural CSF compartment (confining the spinal longitudinal extradural CSF collection); (iii) ventral membranes constituting a transdural funnel-like CSF channel; and (iv) lateral membranes forming spinal cysts/meningeal diverticulae associated with lateral CSF leaks. The latter three types resemble a layer of arachnoid herniated through the dural defect. CONCLUSION: We describe four distinct spinal (neo-)membranes in association with spinal CSF leaks. Formation of these membranes, or emergence by herniation of arachnoid through a dural defect, constitutes a specific pathoanatomic feature of patients with SIH and CSF leaks. Recognition of these membranes is of paramount importance for diagnosis and treatment of patients with spinal CSF leaks.

Patient-reported symptomatology and its course in spontaneous intracranial hypotension - Beware of a chameleon.

OBJECTIVE: Although orthostatic headache is the hallmark symptom of spontaneous intracranial hypotension (SIH), patients can present with a wide range of different complaints and thereby pose a diagnostic challenge for clinicians. Our aim was to describe and group the different symptoms associated with SIH and their course over time. METHODS: We retrospectively surveyed consecutive patients diagnosed and treated for SIH at our institution from January 2013 to May 2020 with a specifically designed questionnaire to find out about their symptomatology and its course. RESULTS: Of 112 eligible patients, 79 (70.5%) returned the questionnaire and were included in the analysis. Of those, 67 (84.8%) reported initial orthostatic headaches, whereas 12 (15.2%) denied having this initial symptom. All except one (98.7%) patients reported additional symptoms: most frequently cephalic pressure (69.6%), neck pain (68.4%), auditory disturbances (59.5%), nausea (57%), visual disturbances (40.5%), gait disturbance (20.3%), confusion (10.1%) or sensorimotor deficits (21.5%). Fifty-seven (72.2%) patients reported a development of the initial symptoms predominantly in the first three months after symptom onset. Age and sex were not associated with the symptomatology or its course (p > 0.1). CONCLUSION: Although characteristic of SIH, a relevant amount of patients present without orthostatic headaches. In addition, SIH can manifest with non-orthostatic headaches at disease onset or during the course of the disease. Most patients report a wide range of associated complaints. A high degree of suspicion is crucial for an early diagnosis and targeted treatment.

[Spontaneous intracranial hypotension]. / ABC om Spontan intrakraniell hypotension.

Spontaneous intracranial hypotension (SIH) is a disease presenting mostly with orthostatic head and neck pain due to a spontaneous cerebrospinal fluid (CSF) leak or a CSF-venous fistula in the spinal region. It demonstrates typical MRI findings with sagging of the brain causing tension of the meninges and sometimes the cranial nerves. It shares some clinical similarities with post puncture headache but differs in its pathophysiological cause, diagnosis, and treatment. Many patients remain misdiagnosed or wait too long for the correct diagnosis. The diagnostic work-up includes an MRI of the head and spine in search of typical SIH signs. Myelography and CT scans are performed to identify the location of the CSF leak or CSF-venous fistula. Treatment options may involve (1) initial conservative treatment with bed rest, caffein and fluids, (2) interventions such as epidural blood patch, fibrin patch, and embolization, or (3) surgical closure of the leak.

Quality of Life in Patients With Confirmed and Suspected Spinal CSF Leaks.

BACKGROUND AND OBJECTIVES: Spontaneous intracranial hypotension (SIH) is a debilitating condition typically producing orthostatic headache limiting upright time. SIH is often difficult to diagnose and treat, negatively affecting quality of life (QoL) in patients with the disorder. We studied QoL in patients with confirmed and suspected SIH using standardized instruments, including suicidality. METHODS: We performed a cross-sectional survey of adult patients with confirmed and clinically suspected SIH evaluated in our Headache and Facial Pain Program from 2016 to 2022. Using an online data collection tool (REDCap V 11.2.2), participants completed validated questionnaires assessing general well-being (SF-36), depression (PHQ-9), generalized anxiety disorder-7 (GAD-7), spiritual well-being during chronic illness therapy (FACIT-Sp-12), and headache impact (HIT-6). Subsequently, we interviewed willing participants to administer the Columbia-Suicide Severity Rating Scale (C-SSRS) assessing suicidal behavior and ideation. RESULTS: A total of 234 patients met inclusion criteria and were invited to participate in the study, and 95 patients (59 confirmed and 36 clinically suspected) completed the questionnaires. The average age of the cohort was 51.1 years (SD: 15.5), predominantly female (69.5%), White (91.6%), and married (69.5%). Three-quarters (74.5%) scored within the most severe headache category (HIT-6). SF-36 scores were significantly inferior (p < 0.0001) to the general population and lower than reported values for patients with multiple sclerosis and idiopathic intracranial hypertension. Almost half (49.1%) of respondents scored in the moderate depression range or worse (>10), and 25.4% scored with moderate anxiety or worse (>10). FACIT-Sp-12 scores were significantly worse (p < 0.0001) in symptomatic participants than in the validation cohorts of patients with AIDS and cancer. Of the 67 respondents who completed the C-SSRS, more than half (64.2%) endorsed a wish to be dead, and 22.4% had demonstrated suicidal behavior. Patients with symptom-free SIH (n = 22) scored significantly better than symptomatic patients, comparable with the general population. DISCUSSION: Based on our single-center cohort, SIH is associated with severe headache pain and high rates of depression, anxiety, and disability, affecting basic activities of daily living. Individuals with confirmed and suspected spinal CSF leaks scored similarly on these measures including suicidality. Outcomes were comparable with the general population after successful treatment or spontaneous remission. Improved identification and treatment of SIH are imperative to improve patients' QoL.

Bed Rest as a Protective Factor for Subdural Hematoma in Spontaneous Intracranial Hypotension: A Retrospective Study.

BACKGROUND: Subdural hematoma (SDH) is a potentially life-threatening complication in patients with spontaneous intracranial hypotension (SIH). Though bed rest is the basis of conservative treatment, no clear evidence exists regarding the association between bed rest and the later complication of SDH in these patients. OBJECTIVES: This study aimed to evaluate the association between bed rest and SDH development in patients with SIH. STUDY DESIGN: A retrospective study was conducted from March 2013 through December 2019. Four hundred twenty adult patients diagnosed with SIH were enrolled. Clinical presentations and radiographic findings were recorded. The cumulative duration of bed rest in hours was used to measure the bed rest length. The clinical outcomes during follow-up were assessed. METHODS: Categorical data were compared using chi-square tests; continuous data were compared using the Mann-Whitney U test or Kruskal-Wallis test. A backwards stepwise Cox proportional hazard regression model adjusted with confounders which differed between SDH and non-SDH in univariate analysis was used to estimate the risk of cumulative duration of bed rest for SDH. A stratified Cox regression was performed to exclude the effect of the treatment algorithm. RESULTS: Of the 420 patients with SIH, 88 (21%) were in the SDH Group and 332 (79%) were in the non-SDH (NSDH) Group. The cumulative duration of bed rest in hours was a protective factor for SDH in SIH (Hazard Ratio [HR] = 0.997; P < 0.001). A stratified Cox regression analysis showed that the cumulative duration of bed rest remained a protective factor for SDH both in patients who received conservative treatment before admission (HR = 0.997; P < 0.001) and in those who did not (HR = 0.996; P = 0.061). Age (HR = 1.029, 95% CI, 1.009-1.050; P = 0.004) and orthostatic headache (HR = 4.770, 95% 95% CI, 2.177-10.450; P < 0.001) were risk factors for SDH in SIH. The clinical outcomes, including length of hospital stay, epidural blood patch (EBP) therapy, and repeated EBP therapy, were higher in the SDH Group. The revisit rate was similar between the 2 groups. LIMITATIONS: Retrospective studies are susceptible to different radiological procedures and therapeutic strategies. A bed rest score based on a patient's memory is susceptible to recognition and reporting bias. This is a single-center study and the sample size is not large. The validity of the bed rest scale has not been previously evaluated in any other study. CONCLUSIONS: Bed rest was a protective factor for SDH in patients with SIH. With more time and proper treatment, patients with SIH who have an SDH can achieve good prognosis in the long term.

Recovery and long-term outcome after neurosurgical closure of spinal CSF leaks in patients with spontaneous intracranial hypotension.

INTRODUCTION: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid leak causes orthostatic headaches and impacts quality of life. Successful closure rates are often reported, whereas data on long-term outcome are still scarce. METHODS: Between April 2020 and December 2022 surgically treated patients completed the Headache Impact Test-6 prior to surgery and at 14 days, three months, six months, and 12 months postoperatively. In addition to the Headache Impact Test-6 score, we extracted data related to orthostatic symptoms. RESULTS: Eighty patients were included. Median Headache Impact Test-6 score preoperatively was 65 (IQR 61-69), indicating severe and disabling impact of headaches. At three months headache impact significantly improved to 49 (IQR 44-58) (p < 0.001) and remained stable up to 12 months (48, IQR 40-56), indicating little to no impact of headaches on quality of life. The need to lie down "always" or "very often" was reduced from 79% to 23% three months postoperatively (p < 0.001). CONCLUSIONS: Surgical closure of spinal CSF leaks significantly improves the impact of headaches in the long term. At least three months should be expected for recovery. Despite permanent closure of the CSF-leak, a quarter of patients still have relevant long-term impairment, indicating the need for further research on its cause and possible treatment.

Case Report: Unilateral Cranial Nerve VI Palsy Secondary to Intracranial Hypotension.

SIGNIFICANCE: Intracranial hypotension is a condition that occurs from a cerebrospinal fluid leak. Various visual symptoms have been associated with this condition. Cranial nerve VI (CN VI) palsies are the most common ocular manifestation, as the abducens nerve is prone to injury because of its intracranial anatomical course. PURPOSE: This case report presents a CN VI palsy secondary to intracranial hypotension from ventriculoperitoneal shunt overfiltration. Diagnosis, treatment, and management considerations are discussed. No identifiable health information was included in this case report. CASE REPORT: A 70-year-old White man was referred to the eye clinic for evaluation of binocular horizontal diplopia. The patient had a recent history of a left ventriculoperitoneal shunt for a persistent cerebrospinal fluid leak after complex mastoid surgery. The patient was also symptomatic for positional headaches, which improved in a recumbent position. He was diagnosed with a left CN VI palsy secondary to intracranial hypotension from a ventriculoperitoneal shunt overfiltration. The patient was followed up by neurosurgery for shunt adjustments to resolve the overfiltration. Binocular horizontal diplopia was managed conservatively with Fresnel prism. CONCLUSIONS: Intracranial hypotension should be considered in patients presenting with cranial nerve palsies and positional headaches. Obtaining neuroimaging and comanaging with neurology or neurosurgery are advised to make prompt diagnosis and treatment. Careful clinical monitoring and conservative diplopia therapy are recommended as visual symptoms improve upon resolution of the cerebrospinal fluid leak.