Cerebrospinal Fluid Leaks: Challenges in Localizing Spontaneous Spinal Leak Sites and Minimally Invasive Treatment.

The confirmation of cerebrospinal fluid (CSF) leaks in the setting of spontaneous intracranial hypotension (SIH) by imaging involves a growing toolset of multimodal advanced spinal and skull base imaging techniques, for which exists a unique set of challenges for each CSF leak type. Furthermore, the repertoire of minimally invasive CSF leak treatment beyond nontargeted epidural blood patch administration has grown widely, with varied practices across institutions. This review describes current diagnostic imaging and treatment modalities as they apply to the challenges of CSF leak localization and management.

Lateral Spinal CSF Leaks in Patients with Spontaneous Intracranial Hypotension: Radiologic-Anatomic Study of Different Variants.

BACKGROUND AND PURPOSE: Spinal CSF leaks cause spontaneous intracranial hypotension. Several types of leaks have been identified, and one of these types is the lateral dural tear. Performing myelography with the patient in the decubitus position allows precise characterization of these leaks. The purpose of the current study was to describe the different variants of spontaneous lateral CSF leaks. MATERIALS AND METHODS: This retrospective cohort study included a consecutive group of patients with spontaneous intracranial hypotension and lateral CSF leaks who underwent digital subtraction myelography in the decubitus position and underwent surgery to repair the CSF leak between July 2018 and June 2023. RESULTS: The mean age of the 53 patients (37 women and 16 men) was 35.5 years. Three different variants of lateral CSF leak could be identified. Forty-nine patients (92.5%) had a lateral dural tear associated with the nerve root sleeve. The dural tear was at the axilla of the nerve root sleeve in 36 patients (67.9%) and at the shoulder in 13 patients (24.5%). Four patients (7.5%) had a lateral dural tear at the level of the pedicle that was not associated with the nerve root sleeve. Findings on digital subtraction myelography were concordant with intraoperative findings in all patients. An extradural CSF collection was seen in all patients with a lateral dural tear associated with the nerve root sleeve but in only 2 of the 4 patients with the pedicular variant of a lateral dural tear. CONCLUSIONS: We identified 3 variants of spontaneous lateral dural tears. Most lateral dural tears are associated with extradural CSF collections and arise from either the axilla (67.9%) or the shoulder (24.5%) of the nerve root sleeve. Lateral dural tears at the level of the pedicle (7.5%) not associated with the nerve root sleeve are uncommon and may require specialized imaging for their detection.

Improved Cerebral Glymphatic Flow after Transvenous Embolization of CSF-Venous Fistula.

Spontaneous intracranial hypotension is characterized by symptoms of low intracranial CSF volume due to various mechanisms of CSF leakage. One such mechanism is a CSF-venous fistula, treatable with transvenous embolization resulting in substantial radiographic and clinical improvement. However, the exact mechanisms underlying these improvements, including the potential involvement of the glymphatic system, remain unclear. To noninvasively assess glymphatic clearance in spontaneous intracranial hypotension, we used an advanced MR imaging technique called the DTI along the perivascular spaces in 3 patients with CSF-venous fistula before and after embolization. All 3 patients with spontaneous intracranial hypotension initially had low glymphatic flow, which improved postembolization. Two patients with symptomatic improvement exhibited a more substantial increase in glymphatic flow compared with a patient with minimal improvement. These findings suggest a possible link between cerebral glymphatics in spontaneous intracranial hypotension pathophysiology and symptomatic improvement, warranting larger studies to explore the role of the glymphatic system in spontaneous intracranial hypotension.

Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.

[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.

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.

Mechanism of chronic iatrogenic CSF leak following dural puncture-ventral dural leak: case report.

BACKGROUND: Postdural puncture headache has been traditionally viewed as benign, self-limited, and highly responsive to epidural blood patching (EBP) when needed. A growing body of data from patients experiencing unintended dural puncture (UDP) in the setting of attempted labor epidural placement suggests a minority of patients will have more severe and persistent symptoms. However, the mechanisms accounting for the failure of EBP following dural puncture remain obscure. An understanding of these potential mechanisms is critical to guide management decisions in the face of severe and persistent cerebrospinal fluid (CSF) leak. CASE PRESENTATION: We report the case of a peripartum patient who developed a severe and persistent CSF leak unresponsive to multiple EBPs following a UDP during epidural catheter placement for labor analgesia. Lumbar MRI revealed a ventral rather than dorsal epidural fluid collection suggesting that the needle had crossed the thecal sac and punctured the ventral dura, creating a puncture site not readily accessible to blood injected in the dorsal epidural space. The location of this persistent ventral dural defect was confirmed with digital subtraction myelography, permitting a transdural surgical exploration and repair of the ventral dura with resolution of the severe intracranial hypotension. CONCLUSIONS: A ventral rather than dorsal dural puncture is one mechanism that may contribute to both severe and persistent spinal CSF leak with resulting intracranial hypotension following a UDP.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.

Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

BACKGROUND AND PURPOSE: Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS: This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS: Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS: Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.

Bibrachial amyotrophy as a rare manifestation of intraspinal fluid collection: a case report and systematic review.

INTRODUCTION: Intraspinal cerebrospinal fluid (CSF) collection has been reported as a rare cause of lower motor neuron (LMN) disorder. We report a case of bibrachial diplegia associated with intraspinal CSF collection and perform a systematic literature review. PATIENT AND METHODS: A 52-year-old man developed a bibrachial amyotrophy over 6 years, confirmed by the presence of cervical subacute neurogenic changes at electromyography (EMG). Brain magnetic resonance imaging (MRI) revealed cerebral siderosis, while spine MRI showed a ventral longitudinal intraspinal fluid collection (VLISFC) from C2 to L2. No CSF leakage was localized at myelography; a conservative treatment was chosen. We searched for all published cases until 30th April 2023 and extrapolated data of 44 patients reported in 27 publications. RESULTS: We observed a male predominance, a younger disease onset compared to amyotrophic lateral sclerosis, and a quite long disease duration, highlighting a slow disease progression. LMN signs were more frequently bilateral, mostly involving C5-C6 myotomes. Around 61% of patients presented additional symptoms, but only three referred to a history of headache. Accordingly, CSF opening pressure was mostly normal. Spinal MRI revealed the presence of VLISFC and in some cases myelomalacia. EMG patterns displayed both chronic and subacute neurogenic change in the cervical region. The disease course mainly depended on the treatment choice, which was mostly represented by a surgical approach when a specific dural defect was detected by imaging. CONCLUSION: Bibrachial diplegia due to VLISFC can be a treatable cause of focal amyotrophy and presents some clinical and radiological "red flags" which cannot be missed by a clinical neurologist.

Diagnostic Performance of Decubitus Photon-Counting Detector CT Myelography for the Detection of CSF-Venous Fistulas.

BACKGROUND AND PURPOSE: CSF-venous fistulas are a common cause of spontaneous intracranial hypotension. Lateral decubitus digital subtraction myelography and CT myelography are the diagnostic imaging standards to identify these fistulas. Photon-counting CT myelography has technological advantages that might improve CSF-venous fistula detection, though no large studies have yet assessed its diagnostic performance. We sought to determine the diagnostic yield of photon-counting detector CT myelography for detection of CSF-venous fistulas in patients with spontaneous intracranial hypotension. MATERIALS AND METHODS: We retrospectively searched our database for all decubitus photon-counting detector CT myelograms performed at our institution since the introduction of the technique in our practice. Per our institutional workflow, all patients had prior contrast-enhanced brain MR imaging and spine MR imaging showing no extradural CSF. Two neuroradiologists reviewed preprocedural brain MRIs, assessing previously described findings of intracranial hypotension (Bern score). Additionally, 2 different neuroradiologists assessed each myelogram for a definitive or equivocal CSF-venous fistula. The yield of photon-counting detector CT myelography was calculated and stratified by the Bern score using low-, intermediate-, and high-probability tiers. RESULTS: Fifty-seven consecutive photon-counting detector CT myelograms in 57 patients were included. A single CSF-venous fistula was definitively present in 38/57 patients. After we stratified by the Bern score, a definitive fistula was seen in 56.0%, 73.3%, and 76.5% of patients with low-, intermediate-, and high-probability brain MR imaging, respectively. CONCLUSIONS: Decubitus photon-counting detector CT myelography has an excellent diagnostic performance for the detection of CSF-venous fistulas. The yield for patients with intermediate- and high-probability Bern scores is at least as high as previously reported yields of decubitus digital subtraction myelography and CT myelography using energy-integrating detector scanners. The yield for patients with low-probability Bern scores appears to be greater compared with other modalities. Due to the retrospective nature of this study, future prospective work will be needed to compare the sensitivity of photon-counting detector CT myelography with other modalities.

Spontaneous intracranial hypotension: a case series and literature review.

Spontaneous intracranial hypotension (SIH) is a condition of negative intracranial pressure resulting from cerebrospinal fluid (CSF) leakage from the dural sac and is a well-known cause of orthostatic headache. Diagnosis and management can be difficult, often requiring coordination between multiple disciplines. Low CSF pressure and diffuse meningeal enhancement on brain MRI are the major instrumental features of the classic syndrome. Neuroimaging plays a key role in diagnosing SIH, particularly in atypical clinical presentations, by recognizing the specific findings of brain sagging on MRI and detecting the level of CSF leak on spinal imaging, thus guiding therapy accordingly. Since SIH could present with such a heterogeneous clinical picture, careful history taking and increased awareness of atypical presentations are of utmost importance. We review the existing SIH literature, illustrate management, clinical and neuroimaging findings of four consecutive patients with atypical SIH, who were recently referred to our hospital for evaluation to simplify and streamline the management of SIH.

[Complicated course of the postoperative period with the development of epidural hygroma and intracranial hypotension after removal of cranio-orbital meningioma. Clinical case and literature review]. / Oslozhnennoe techenie posleoperatsionnogo perioda s razvitiem epidural'noi gigromy i vnutricherepnoi gipotenzii posle udaleniya kranioorbital'noi meningiomy. Klinicheskii primer i obzor literatury.

Surgical removal of cranio-orbital meningiomas is an effective method of treating this pathology. Modern surgical techniques and technologies make it possible to perform operations with a low risk of complications. Lumbar drainage or repeated lumbar punctures are often used intraoperatively or in the early postoperative period to prevent nasal CSF leak; this rarely leads to the development of significant neurological symptoms. We present a case of the development of severe intracranial hypotension with the formation of a subdural hygroma in the early postoperative period after removal of a cranio-orbital meningioma in a 41-year-old patient. The operation was performed using an individual model and molds for simultaneous reconstruction of the bone defect with an implant made of polymethyl methacrylate. On the 1st and 2nd days after surgery, lumbar punctures were performed. From the 2nd day there was a progressive deterioration with the development of symptoms characteristic of intracranial hypotension. Computed tomography revealed an increasing displacement of the midline structures of the brain and an increasing volume of epidural fluid accumulation in the area of surgical intervention. Magnetic resonance imaging revealed characteristic signs of intracranial hypotension. Conservative treatment (bed rest, active hydration) had no effect. On the 6th day after surgery, an epidural blood patch procedure was performed and closed external drainage of the epidural hygroma was performed, and a rapid regression of neurological symptoms was noted. Our experience and literature data indicate that it is necessary to remember the possibility of developing clinically significant intracranial hypotension even after a single lumbar puncture. The formation of hygromas in the surgical area is characteristic of intracranial hypotension, but in most cases does not require additional surgical intervention and does not have a negative impact on the outcome of treatment. Conservative treatment of intracranial hypotension is the first choice and often sufficient. If there is no effect and the patient's condition worsens, it is necessary to perform an epidural blood patch procedure.

Intracranial Hypotension Syndrome after Lumbar Drainage in Skull Base Surgery: Diagnosis and Correct Management.

Lumbar drainage is commonly used in skull base surgery; however, very few cases of intracranial hypotension syndrome are reported to be caused by this procedure. We present a clinical case of lumbar drainage-assisted orbital and optic canal decompression surgery for a recurrent voluminous spheno-orbital meningioma, together with a literature review. A 49-year-old woman became confused and drowsy on postoperative day 3, after initially experiencing neurologic stability. Computed tomography (CT) scan of the head showed extradural frontotemporal fluid collection with moderate right to left midline shift. Magnetic resonance imaging (MRI) of the brain showed signs of intracranial hypotension, such as brain sagging and diffuse dural contrast enhancement. Conservative treatment with bed rest, aggressive hydration, steroids, and aminophylline led to progressive neurologic improvement. A systematic literature review was also performed, and previous reported cases were analyzed. Overall, neurosurgeons must be aware of the lumbar drainage-induced hypotension syndrome in skull base surgeries, because immediate diagnosis is essential for therapeutic decision-making. In this setting, conservative management is the first-line treatment as surgery may lead to severe complications.

[Guidelines for the Diagnosis and Treatment of Spontaneous Intracranial Hypotension]. / Protocolo de Abordagem Diagnóstica e Terapêutica da Hipotensão Intracraniana Espontânea.

Spontaneous intracranial hypotension (SIH) is a syndrome characterized by disabling orthostatic headache, due to reduced cerebrospinal fluid (CSF) volume probably caused by a CSF fistula. It affects mostly women of working-age, although it is probably underdiagnosed. The aim of this article is to present a practical approach to the diagnosis and treatment of SIH. After a description of its symptoms and signs, we present a step-by-step approach to the confirmation of the diagnosis and treatment, considering different clinical scenarios. This is intended to guide clinical decision making, through a systematized and individualized management, aimed at the best interest of the patient.

A hipotensão intracraniana espontânea (HIE) é uma síndrome caracterizada por cefaleia ortostática incapacitante, fruto de uma redução do volume de líquido cefalorraquidiano (LCR) provavelmente causada por uma fístula de LCR. Afeta sobretudo mulheres em idade ativa, estando provavelmente subdiagnosticada. Este protocolo visa apresentar uma proposta de abordagem prática ao diagnóstico e tratamento da HIE. Após uma secção descritiva das manifestações clínicas da HIE, apresentamos um modelo de atuação passo-a-passo para a confirmação do seu diagnóstico e tratamento, considerando diferentes cenários clínicos. Pretende-se, assim, facilitar a decisão clínica através de uma conduta sistematizada e individualizada, visando o melhor interesse do doente.

Conebeam CT as an Additional Tool in Digital Subtraction Myelography for the Detection of Spinal Lateral Dural Tears.

Lateral dural tears as a cause spontaneous intracranial hypotension occur in ∼20% of patients. Common imaging modalities for their detection are lateral decubitus digital subtraction myelography or dynamic CT myelography. Reports on the use of conebeam CT are scarce. We show 3 patients in whom the targeted use of conebeam CT during digital subtraction myelography was helpful in confirming the site of the leak.

Intraoperative Visualization of Flow in Direct Cerebrospinal Fluid-Venous Fistulas Using Intrathecal Fluorescein.

BACKGROUND: Cerebrospinal fluid-venous fistulas (CVFs) are a rare cause of spontaneous intracranial hypotension. Intraoperatively, CVFs are not readily identifiable and difficult to differentiate from normal veins. OBJECTIVE: To assess the utility of intrathecal fluorescein injection intraoperatively to visualize and identify the CVF. METHODS: We report a case series of patients treated surgically for a CVF. After surgical exposure, we injected intrathecal fluorescein (5-10 mg) through a lumbar catheter, which we placed immediately before surgery. RESULTS: Four patients with spontaneous intracranial hypotension with a suspected CVF underwent surgical ligation with adjunctive intrathecal fluorescein application. Intraoperative fluorescein injection confirmed the presence of a CVF in 3 cases. In 2 cases, we observed rapid fluorescein filling of a single epidural vein constituting the CVF. Other epidural vessels did not fill with fluorescein. In 1 case, fluorescein helped to identify a residual CVF after previous incomplete embolization. In the fourth case, no CVF was found intraoperatively. By contrast, a meningeal nerve root diverticulum was visualized, wrapped, and clipped. CONCLUSION: We demonstrate for the first time the direct intraoperative visualization of CVF using intrathecal fluorescein. CVF can be identified intraoperatively using fluorescein dye, which can be a valuable adjunct for the surgeon confronted with this disease.

Postoperative Spinal Cerebrospinal Fluid-Venous Fistulas Associated With Dural Tears in Patients With Intracranial Hypotension or Superficial Siderosis-A Digital Subtraction Myelography Study.

BACKGROUND: Postoperative spinal cerebrospinal fluid (CSF) leaks are common but rarely cause extensive CSF collections that require specialized imaging to detect the site of the dural breach. OBJECTIVE: To investigate the use of digital subtraction myelography (DSM) for patients with extensive extradural CSF collections after spine surgery. METHODS: A retrospective review was performed to identify a consecutive group of patients with extensive postoperative spinal CSF leaks who underwent DSM. RESULTS: Twenty-one patients (9 men and 12 women) were identified. The mean age was 46.7 years (range, 17-75 years). The mean duration of the postoperative CSF leak was 3.3 years (range, 3 months to 21 years). MRI showed superficial siderosis in 6 patients. DSM showed the exact location of the CSF leak in 19 (90%) of the 21 patients. These 19 patients all underwent surgery to repair the CSF leak, and the location of the CSF leak could be confirmed intraoperatively in all 19 patients. In 4 (19%) of the 21 patients, DSM also showed a CSF-venous fistula at the same location as the postoperative dural tear. CONCLUSION: In this study, DSM had a 90% detection rate of visualizing the exact site of the dural breach in patients with extensive postoperative spinal CSF leaks. The coexistence of a CSF-venous fistula in addition to the primary dural tear was present in about one-fifth of patients. The presence of a CSF-venous fistula should be considered if CSF leak symptoms persist in spite of successful repair of a durotomy.