Spontaneous intracranial hypotension in a patient without corpus callosum: A case report.

RATIONALE: Spontaneous intracranial hypotension (SIH) is a well-established condition typically presenting with disabling orthostatic headache. Corpus callosum agenesis (CCA) is one of the most common human brain malformations with a wide spectrum of associated malformations, chromosomal abnormalities, and clinical syndromes. PATIENT CONCERNS: A 53-year-old woman presented with recurrent orthostatic headache for about 1 month. The head computed tomography examination of the patient showed CCA and the initial pressure of subsequent lumbar puncture was only 5 centimeters cerebrospinal fluid. Magnetic resonance imaging examination confirmed CCA with gray matter heterotopia. DIAGNOSIS: The final diagnose was SIH related headache with CCA. INTERVENTION: The patient's symptom improved after oral hydration, intravenous fluids, and bed rest. OUTCOME: Favorable outcome was observed. LESSONS: Although this co-occurrence of SIH and CCA is probably coincidental, this finding suggests that the developmental malformations of the brain may lead to structural changes in brain tissue or disturbances in cerebrospinal fluid production and reflux, resulting in pathological changes over time.

The Spatial Relationship between Spinal Osteoarthritis and CSF Venous Fistulas in Patients with Spontaneous Intracranial Hypotension.

BACKGROUND AND PURPOSE: CSF venous fistula leads to spontaneous intracranial hypotension. The exact mechanisms underlying the development of CSF venous fistula remain unclear: Some researchers have postulated that underlying chronic intracranial hypertension may lead to damage to spinal arachnoid granulations, given that many patients with CSF venous fistulas have an elevated body mass index (BMI). However, individuals with higher BMIs are also more prone to spinal degenerative disease, and individuals with CSF venous fistulas also tend to be older. CSF venous fistula tends to occur in the lower thoracic spine, the most frequent location of thoracic degenerative changes. The current study aimed to examine whether CSF venous fistulas are more likely to occur at spinal levels with degenerative changes. MATERIALS AND METHODS: Forty-four consecutive patients with CSF venous fistulas localized on dynamic CT myelography were included in analyses. Whole-spine CT was scrutinized for the presence of degenerative changes at each spinal level. The proportion of levels positive for CSF venous fistula containing any degenerative findings was compared to levels without CSF venous fistula using the Fisher exact test. The Pearson correlation coefficient was calculated to explore the association between the burden of degenerative disease and BMI and age and between BMI and opening pressure. RESULTS: Forty-four patients with 49 total CSF venous fistulas were analyzed (5 patients had 2 CSF venous fistulas). Mean patient age was 62.3 (SD, 9.5) years. Forty-seven CSF venous fistulas were located in the thoracic spine; 1, in the cervical spine; and 1, in the lumbar spine. Within the thoracic spine, 39/49 (79.6%) fistulas were located between levels T7-8 and T12-L1. Forty-four of 49 (89.8%) CSF venous fistulas had degenerative changes at the same level. The levels without CSF venous fistulas demonstrated degenerative changes at 694/1007 (68.9%) total levels. CSF venous fistulas were significantly more likely to be present at spinal levels with associated degenerative changes (OR = 4.03; 95% CI, 1.58-10.27; P = .001). Age demonstrated a positive correlation with the overall burden of degenerative disease (correlation coefficient: 0.573, P < .001), whereas BMI did not (correlation coefficient: 0.076, P = .625). There was a statistically significant positive correlation between BMI and opening pressure (correlation coefficient: 0.321, P = .03). CONCLUSIONS: Results suggest a potential association between spinal degenerative disease and development of CSF venous fistula.

Non-targeted serial epidural blood patches for the treatment of intracranial hypotension headache during late pregnancy: a case report.

Intracranial hypotension can cause persistent and severe orthostatic headaches. It is often the result of cerebrospinal fluid leakage secondary to iatrogenic causes. It can also happen spontaneously. Diagnosing and managing severe orthostatic headaches associated with intracranial hypotension can be challenging, particularly during late pregnancy. This case describes a parturient at 30 weeks' gestation who presented with severe headaches due to intracranial hypotension caused by dural defects and cerebrospinal fluid venous fistula. She was managed with serial non-targeted epidural blood patching as a bridging remedy. This allowed further fetal maturity before delivery and definitive management of the dural leakage after delivery.

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.

"Empty Cyst Sign" Appearance of CSF-Venous Fistula on Digital Spinal Myelography.

Cerebrospinal fluid venous fistulas (CSF-VFs) are an uncommon, yet increasingly recognized, cause of spontaneous intracranial hypotension.1-5 The workup involves magnetic resonance imaging (MRI) of the brain with and without contrast and MRI of the neuroaxis without contrast before dynamic myelography, either computed tomography or digital subtraction.6 The present case of an older woman with symptomatic intracranial hypotension is notable for the specific appearance of CSF-VFs on digital spinal myelography (Video 1). Among her numerous perineural cysts, it was the "disappearing" or "empty" cyst from which the fistula originated. The diagnosis was made using a second lateral fluoroscopy view, not typically used in digital spinal myelography, which demonstrated emptying of contrast from the T6 perineural cyst into the segmental vein at this level, or the "empty cyst sign." The patient then underwent transvenous onyx embolization with resolution of her orthostatic headaches and improvement of contrast-enhanced MRI of the brain with the Bern score decreasing from 7 to 0 at 3 months of follow-up.7 Because transvenous embolization of CSF-VFs is a relatively new procedure, the long-term outcomes of the procedure are not yet known.

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.

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.

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.

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.

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.

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.

Spontaneous absorption of osteophytic calcification associated with chronic dural tear and ventral spinal CSF leak.

Spontaneous intracranial hypotension (SIH) is associated with cerebrospinal fluid (CSF) hypovolemia, often from a traumatic dural tear from a calcified spinal osteophyte. Visualizing osteophytes on CT imaging can guide decision making on candidate leak sites. We report the atypical case of a 41-year-old woman whose ventral CSF leak was associated with an osteophyte that resorbed over an 18-month period. Full workup and treatment were delayed due to unexpected pregnancy and completion of gestational cycle with delivery of a healthy term infant. The patient initially presented with persistent orthostatic headaches with nausea and blurred vision. Initial MRI suggested brain sagging among other findings consistent with SIH. CT myelogram showed an extensive thoracic CSF leak with a prominent ventral T11-T12 osteophyte and multiple small disc herniations. The patient did not respond to epidural blood patches and deferred additional imaging due to her pregnancy. CT myelography performed 5 months post-partum showed an absence of the osteophyte; a follow-up digital subtraction myelogram performed 10 months post-partum showed evidence of source leak at T11-T12 level. T11-T12 laminectomy visualized and repaired a 5 mm ventral dural defect with symptom resolution. This report highlights the potential for a resorbed osteophyte to be the causative agent for long-standing dural tears that do not show visible calcifications on myelography.

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.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

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.

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.