Cranial and Spinal Cerebrospinal Fluid Leaks: Foundations of Identification and Management.

Cerebrospinal fluid (CSF) leaks may occur at the skull base or along the spinal column and can cause a variety of debilitating neurological symptoms for patients. Recognizing the potential presence of a CSF leak and then identifying its source are necessary for accurate diagnosis and definitive treatment. A standardized workflow can be followed for successful leak localization, which often requires several diagnostic studies, and for definitive leak treatment, which can range from minimally invasive, needle-based approaches to a variety of surgical corrections. This review paper provides an overview of epidemiology, pathophysiology, and diagnostic workup for CSF leaks and introduces available treatment options. An illustrative case of a skull base CSF leak demonstrating diagnosis and surgical correction is provided.

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.

Innovations in the Treatment of Spinal Cerebrospinal Fluid Leaks.

Spontaneous spinal cerebrospinal fluid (CSF) leaks are uncommon but can be neurologically debilitating. When initial treatments fail, definitive repair or closure of the leak is indicated. Depending upon the type of leak present, innovative strategies for their treatment have been developed. Among them are open surgical techniques using a transdural approach for the closure of ventral CSF leaks, minimally invasive tubular techniques for the reduction and repair of lateral meningeal diverticula, and endovascular embolization of CSF-venous fistulas. Illustrative cases demonstrating the indications for and implementation of these techniques are provided.

Confronting multidrug-resistant Klebsiella meningitis after mid-clival Cerebrospinal Fluid leak repair: a therapeutic odyssey.

A man in his 40s with type 2 diabetes mellitus had persistent right-sided watery nasal discharge for 6 months following cerebrospinal fluid (CSF) leak repair at another hospital, prompting his visit to us due to recurring symptoms. Imaging revealed a CSF leak from the mid-clivus for which revision endoscopic CSF leak repair was done. Regrettably, he developed postoperative meningitis caused by multidrug-resistant (MDR) Klebsiella pneumoniaeManaging this complex case was a challenging task due to the pathogen's resistance to conventional drugs and the scarcity of scientific evidence. We initiated a culture-guided combination regimen with ceftazidime, avibactam, aztreonam and tigecycline. This decision stemmed from meticulous literature review and observed antibiotic synergy while testing for this organism.After 4 weeks of vigilant treatment, the patient's symptoms improved significantly, and CSF cultures were sterile. We present our approach to effectively confront and manage a challenging instance of postoperative MDR bacterial meningitis.

Perspectives from the Inaugural "Spinal CSF Leak: Bridging the Gap" Conference: A Convergence of Clinical and Patient Expertise.

BACKGROUND AND PURPOSE: The inaugural "Spinal CSF Leak: Bridging the Gap" Conference was organized to address the complexities of diagnosing and treating spinal CSF leaks. This event aimed to converge the perspectives of clinicians, researchers, and patients with a patient-centered focus to explore the intricacies of spinal CSF leaks across 3 main domains: diagnosis, treatment, and aftercare. MATERIALS AND METHODS: Physician and patient speakers were invited to discuss the varied clinical presentations and diagnostic challenges of spinal CSF leaks, which often lead to misdiagnosis or delayed treatment. Patient narratives were interwoven with discussions on advanced radiologic techniques and clinical assessments. Treatment-focused sessions highlighted patient experiences with various therapeutic options, including epidural blood patches, surgical interventions, and percutaneous and endovascular therapies. The intricacies of immediate and long-term postprocedural management were explored. RESULTS: Key outcomes from the conference included the recognition of the need for increased access to specialized CSF leak care for patients and heightened awareness among health care providers, especially for atypical symptoms and presentations. Discussions underscored the variability in individual treatment responses and the necessity for personalized diagnostic and treatment algorithms. Postprocedural challenges such as managing incomplete symptom relief and rebound intracranial hypertension were also addressed, emphasizing the need for effective patient monitoring and follow-up care infrastructures. CONCLUSIONS: The conference highlighted the need for adaptable diagnostic protocols, collaborative multidisciplinary care, and enhanced patient support. These elements are vital for improving the recognition, diagnosis, and management of spinal CSF leaks, thereby optimizing patient outcomes and quality of life. The event established a foundation for future advancements in spinal CSF leak management, advocating for a patient-centered model that harmonizes procedural expertise with an in-depth understanding of patient experiences.

Long-Term Epidural Patching Outcomes and Predictors of Benefit in Patients With Suspected CSF Leak Nonconforming to ICHD-3 Criteria.

BACKGROUND AND OBJECTIVES: Spinal CSF leaks lead to spontaneous intracranial hypotension (SIH). While International Classification of Headache Disorders, Third Edition (ICHD-3) criteria necessitate imaging confirmation or low opening pressure (OP) for SIH diagnosis, their sensitivity may be limited. We offered epidural blood patches (EBPs) to patients with symptoms suggestive of SIH, with and without a documented low OP or confirmed leak on imaging. This study evaluates the efficacy of this strategy. METHODS: We conducted a prospective cohort study with a nested case-control design including all patients who presented to a tertiary headache clinic with clinical symptoms of SIH who completed study measures both before and after receiving an EBP between August 2016 and November 2018. RESULTS: The mean duration of symptoms was 8.7 ± 8.1 years. Of 85 patients assessed, 69 did not meet ICHD-3 criteria for SIH. At an average of 521 days after the initial EBP, this ICHD-3-negative subgroup experienced significant improvements in Patient-Reported Outcomes Measurement Information System (PROMIS) Global Physical Health score of +3.3 (95% CI 1.5-5.1), PROMIS Global Mental Health score of +1.8 (95% CI 0.0-3.5), Headache Impact Test (HIT)-6 head pain score of -3.8 (95% CI -5.7 to -1.8), Neck Disability Index of -4.8 (95% CI -9.0 to -0.6) and PROMIS Fatigue of -2.3 (95% CI -4.1 to -0.6). Fifty-four percent of ICHD-3-negative patients achieved clinically meaningful improvements in PROMIS Global Physical Health and 45% in HIT-6 scores. Pain relief following lying flat prior to treatment was strongly associated with sustained clinically meaningful improvement in global physical health at an average of 521 days (odds ratio 1.39, 95% CI 1.1-1.79; p < 0.003). ICHD-3-positive patients showed high rates of response and previously unreported, treatable levels of fatigue and cognitive deficits. DISCUSSION: Patients who did not conform to the ICHD-3 criteria for SIH showed moderate rates of sustained, clinically meaningful improvements in global physical health, global mental health, neck pain, fatigue, and head pain after EBP therapy. Pre-treatment improvement in head pain when flat was associated with later, sustained improvement after EBP therapy among patients who did not meet the ICHD-3 criteria. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that epidural blood patch is an effective treatment of suspected CSF leak not conforming to ICHD-3 criteria for SIH.

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.

[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.

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.

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.

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.

Cerebrospinal Fluid Refill Test as a Novel Diagnostic Tool for Cerebrospinal Fluid Hypovolemia: Preliminary Result.

OBJECTIVE: Cerebrospinal fluid (CSF) hypovolemia presents with orthostatic headaches due to CSF leakage. However, a direct association between the lack of CSF and clinical symptoms has not been found. CSF hypovolemia can be improved by refilling CSF. Therefore, we assessed the validity of a CSF refill test. METHODS: From November 2019 to August 2021, we included 10 patients (≥18 years old) with potential CSF hypovolemia, clear orthostatic headaches, and a CSF opening pressure <10 cmH2O. In the CSF refill test, 10 mL of artificial CSF was injected intrathecally. The primary outcome was improvement in orthostatic headache assessed using a visual analog scale (VAS), while the secondary outcomes were the 10-m walk time and adverse events. When the symptoms temporarily improved after intrathecal injection, the patients underwent radiologic imaging to identify the CSF leak, and an epidural blood patch was proposed accordingly. RESULTS: All patients showed post-test improvements in the VAS score (median [interquartile range], pretest 63.0 [50.3-74.3] vs. post-test 1.5 [0.0-26.0]). The 10-m walk time also significantly improved (9.5 [8.5-10.2] s vs. 8.2 [7.9-8.7] s). One patient experienced temporary right leg numbness associated with a lumbar puncture. After radiologic investigation, 9 patients underwent epidural blood patches, of which 6 were completely cured, and 3 revealed partial improvement. CONCLUSIONS: The cerebrospinal fluid (CSF) refill test was safe and effective in demonstrating the direct association between the lack of CSF and clinical symptoms and may help predict the outcome of an epidural blood patch.

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

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

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.

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

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

CT-guided percutaneous cyanoacrylate injection targeting the spinal cerebrospinal fluid leak: a potential therapeutic option for spontaneous intracranial hypotension.

BACKGROUND: We report the first case series of spontaneous intracranial hypotension (SIH) patients who underwent CT-guided percutaneous cyanoacrylate injection targeting the cerebrospinal fluid (CSF) leak. METHODS: A retrospective analysis was performed for all consecutive cases of SIH patients with CSF leak confirmed on CT myelography, treated by CT-guided percutaneous cyanoacrylate injection at our institution from 2016 to 2022. On pretreatment brain and spine MRIs, we analyzed signs of SIH according to the Bern score, and dichotomized cases into positive/negative for spinal longitudinal extradural CSF collection (SLEC-P or SLEC-N). The leaks detected on CT myelography were classified into three types according to Schievink et al. We collected the Headache Impact Test 6 (HIT-6) scores throughout a 6-month follow-up, with a brain CT scan at each visit. RESULTS: 11 patients were included (mean age 48.4 years, six men). Five SLEC-P type 1, three SLEC-P type 2, and three SLEC-N type 3 leaks were identified. All patients had significant signs of SIH on pretreatment brain MRI (mean Bern score 7.8±1.1). Six patients underwent a foraminal puncture, and five patients had a cervical epidural approach. Two patients experienced mild and transient locoregional pain after cervical epidural injection. Mean HIT-6 score at baseline was 66.8±3.2 and at the 6-month follow-up was 38±3.6 (P<0.001). All patients achieved improvement in their symptoms, with 82% of them (9/11) having complete resolution of headaches and SIH findings on CT scans at 6 months. No clinical worsening or recurrence was observed. CONCLUSIONS: CT-guided percutaneous cyanoacrylate injection may be a potential therapeutic option for the different types of CSF leak causing SIH.

Spontaneous Intracranial Hypotension: Controversies in Treatment.

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

Sacral Dural Tears as a Cause of Spontaneous Intracranial Hypotension.

PURPOSE: Dural tears at the level of the cervical, thoracic, and lumbar spine cause spontaneous intracranial hypotension (SIH) in patients with a spinal longitudinal extradural CSF collection (SLEC); however, sacral dural tears have rarely been reported so far. This study focuses on sacral dural tears as a cause of SIH. METHODS: Retrospective data from SIH patients with confirmed sacral dural tears studied between October 2020 and November 2022 were analyzed with respect to demographic, clinical and imaging features. Digital subtraction myelography (DSM) and lumbar epidural blood patch (EBP) were modified by placing the patient in reversed Trendelenburg position. RESULTS: Of the SIH patients, 9 (all women; mean age, 38.5 years; mean body mass index, BMI, 22.9) out of 149 had a sacral dural leak (6%) that occurred spontaneously in 7/9, while 2/9 were likely associated with minor trauma. None had a sacral fracture. The mean SIH score was 6.8. All patients showed SLEC on heavily T2-weighted MR myelography (T2-MRM), 4/9 exclusively sacral and 5/9 with partial or complete involvement of the remaining spine. 4/9 had none, but 5/9 had meningeal sacral cysts, 2/5 had large cysts/ectasia. Confirmation of the sacral origin of the leak was provided in 4/9 by T2-MRM, in 2/9 by DSM and 3/9 by CT myelography (CTM) whereas 0/9 revealed the exact site of leak within the sacrum. CONCLUSION: Sacral dural tears should be considered as a possible cause for SIH. It is concluded to implement T2-MRM covering the entire sacrum in the standard MRI protocol and propose EBP in the reverse Trendelenburg position as a therapeutic approach.

[Intracranial hypotension syndrome in a pediatric patient with Marfan syndrome].

Intracranial hypotension syndrome (IHS) is attributed to reduced cerebrospinal fluid (CSF) pressure. It may be spontaneous or secondary to a history of trauma or systemic disease. We present the case of an 11-year-old boy, with medical history of Marfan syndrome, with orthostatic headache and persistent vomiting (12 hours) following a fall on the sacrococcygeal region. Magnetic resonance showed extradural fluid collections at dorsal and lumbosacral levels, compatible with CSF leak. The condition was resolved with treatment, but the patient had two new episodes during the follow-up period. Thus, an epidural blood patch was performed two years after the first episode. Although HIS is uncommon in children, it should be suspected in patients with orthostatic headache, particularly if the patient presents a connectivopathy. Few studies have assessed the management of HIS in paediatric age. The case presented here and the reviewed available literature provides further data for these type of cases.

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

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