Spontaneous Spinal Cerebrospinal Fluid Leaks Research Articles

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.

Posterior Transdural Repair of a Spontaneous Ventral Thoracic Cerebrospinal Fluid Leak: 2-Dimensional Operative Video.

Spontaneous intracranial hypotension is a rare but serious condition characterized by orthostatic headaches and a variety of neurological symptoms. 1,2 Spontaneous intracranial hypotension should be considered in all patients with new onset, daily, persistent headaches, and orthostatic symptoms. It is typically caused by spontaneous spinal cerebrospinal fluid (CSF) leaks. 1,2 Traditional first-line treatments include hydration, bedrest, epidural blood patches, and fibrin glue injections. However, refractory cases often require surgical intervention, especially those caused by a small ventral osteophyte, which is classified as a type 1 leak. 3-5 The small osteophyte causes a tear in the dura of the ventral canal, usually near the cervicothoracic junction. Diagnosis of these leaks is challenging because these small osteophytes can also occur asymptomatically, or patients may have several of them at multiple levels. Typically, dynamic myelography is needed for accurate localization due to the inadequacy of standard imaging. 6 This video details a young patient with refractory spontaneous intracranial hypotension from a type 1 spontaneous CSF leak, treated successfully using a posterior transdural surgical approach with spinal cord mobilization. Our video presentation outlines the surgical technique and provides an overview of this underdiagnosed condition. Our described approach offers direct visualization, suturing of the leak site, and a multilayer repair without the need for spinal fusion. It also avoids the morbidity to the neck, chest, and mediastinal structures that is at risk with lateral or anterior approaches. A combined intradural and extradural repair may enhance the durability of repair for ventral CSF leaks. The patient consented to the procedure. This operative video did not require Institutional Review Board approval as all patient information has been anonymized, ensuring no identifiable information is disclosed. The video is a single case that does not involve interventions or pose risks beyond standard care, adhering to ethical guidelines and institutional policies.

Management of Spontaneous Spinal CSF Leaks Using Noninvasive Dynamic MR Neurography: A Case Series

AbstractSpontaneous spinal cerebrospinal fluid (CSF) leak presents with orthostatic headache and may lead to formation of subdural collection. Invasive magnetic resonance imaging or computed tomography myelography is routinely used to detect these leaks. Noninvasive dynamic magnetic resonance neurography can also detect these leaks and confirm its resolution post treatment. It includes three-dimensional NerveView, a high-resolution short-tau inversion recovery sequence for better visualization of spinal nerves and leak sites. This article showcases the diagnostic and therapeutic journey of three patients with spontaneous spinal CSF leak all of whom were diagnosed with dynamic magnetic resonance neurography and treated successfully with autologous targeted epidural blood patch. In one case, epidural blood patch was repeated after 2 months for recurrence of the leak.

Acquired tonsillar herniation related to spontaneous intracranial hypotension: case reports.

Acquired prolapse of the cerebellar tonsils in spontaneous intracranial hypotension (SIH) patients is rare. This study aims to evaluate neuroimaging changes of acquired prolapse of the cerebellar tonsils below the foramen magnum in SIH patients due to spontaneous spinal cerebrospinal fluid leakage, which was treated by targeted epidural blood patches (EBP). We retrospectively reviewed clinical and neuroimaging characteristics of 5 cases of SIH with acquired prolapse of the cerebellar tonsils that received targeted EBP in our institution from January 2013 to December 2016. Of these SIH patients, all of them suffered from an orthostatic headache. Initial cranial MRI demonstrated descent of the cerebellar tonsils ≥5 mm. Intrathecal gadolinium-enhanced spinal MR myelography and/or spinal MR hydrography were performed to evaluate the level of spinal cerebrospinal fluid leakage. Symptoms were alleviated in all 5 patients after two (n = 4), or three (n = 1) targeted EBP during hospitalization. Follow-up cranial MRI revealed that the descent of cerebellar tonsils was reversed after EBP treatment. Acquired tonsillar herniation can occur in patients with SIH and spinal cerebrospinal fluid leakage. Symptoms of these patients may be resolved and radiologic findings may be reversed after EBP treatment.

Tandem cranial and spinal cerebrospinal fluid leaks presenting with otogenic tension pneumocephalus: illustrative case.

Cranial and spinal cerebrospinal fluid (CSF) leaks are associated with opposite CSF fluid dynamics. The differing pathophysiology between spontaneous cranial and spinal CSF leaks are, therefore, mutually exclusive in theory. A 66-year-old female presented with tension pneumocephalus. The patient underwent computed tomography (CT) scanning, which demonstrated left-sided tension pneumocephalus, with an expanding volume of air directly above a bony defect of the tegmen tympani and mastoideum. The patient underwent a left middle fossa craniotomy for repair of the tegmen CSF leak. In the week after discharge, she developed a recurrence of positional headaches and underwent head CT. Further magnetic resonance imaging of the brain and thoracic spine showed bilateral subdural hematomas and multiple meningeal diverticula. Cranial CSF leaks are caused by intracranial hypertension and are not associated with subdural hematomas. Clinicians should maintain a high index of suspicion for intracranial hypotension due to spinal CSF leak whenever "otogenic" pneumocephalus is found. Close postoperative follow-up and clinical monitoring for symptoms of intracranial hypotension in any patients who undergo repair of a tegmen defect for otogenic pneumocephalus is recommended.

Updated ultrafast dynamic computed tomography myelography technique for cerebrospinal fluid leaks.

We present an updated ultrafast dynamic computed tomography myelography technique that can be used for the localization of spontaneous spinal cerebrospinal fluid leaks in the setting of spontaneous intracranial hypotension. This has over twice the temporal resolution of previously described techniques at the same radiation dose output.

Surgical Resolution of Severe Headache and Ataxia Caused by Spontaneous Spinal Cerebrospinal Fluid Leak Secondary to Cerebrospinal Fluid Venous Fistula—Case Report

Surgical Resolution of Severe Headache and Ataxia Caused by Spontaneous Spinal Cerebrospinal Fluid Leak Secondary to Cerebrospinal Fluid Venous Fistula—Case Report

Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension.

Spontaneous spinal cerebrospinal fluid (CSF) leaks cause intracranial hypotension (SIH) and also may cause infratentorial superficial siderosis (iSS) but the rate of development among different CSF leak types and outcome of treatment are not known. We determined the time interval from SIH onset to iSS and the outcome of treatment. A total of 1,589 patients with SIH underwent neuroimaging and iSS was detected in 57 (23 men and 34 women, mean age=41.3 years [3.6%]). We examined the type of underlying CSF leak by various imaging modalities. Percutaneous and surgical procedures were used to treat the CSF leaks. The iSS was detected in 46 (10.3%) of 447 patients with ventral CSF leaks, in 2 (3.9%) of 51 patients with dural ectasia, in 5 (2.6%) of 194 patients with CSF-venous fistulas, in 4 (0.9%) of 457 patients with simple meningeal diverticula, and in none of the 101 patients with lateral CSF leaks or the 339 patients with leaks of indeterminate origin (p < 0.001). The estimated median latency period from SIH onset to iSS was 126months. Ventral CSF leaks could not be eliminated with percutaneous procedures in any patient and surgical repair was associated with low risk (<5%) and resulted in resolution of the CSF leak in all patients in whom the exact site of the CSF leak could be determined. Other types of CSF leak were treated with percutaneous or surgical procedures. The iSS can develop in most types of spinal CSF leak, including CSF-venous fistulas, but mainly in chronic ventral CSF leaks, which require surgical repair. ANN NEUROL 2023;93:64-75.

Full Endoscopic Repair of Spontaneous Ventral Cerebrospinal Fluid Leaks in the Spine: Systematic Review of Surgical Treatment Options and Illustrative Case

Spontaneous spinal cerebrospinal fluid (CSF) leaks are a rare entity that can lead to intracranial hypotension and associated headaches, meningismus, and patient debility. Surgical treatment may be necessary for patients who do not respond to conservative management. Surgical repair of CSF leaks located in the ventral thoracic spine traditionally require an invasive, open approach. We describe the case of a patient with a ventral thoracic spontaneous spinal CSF leak associated with a ventral bony osteophyte successfully treated with spinal endoscopy. We also provide a systematic review of the literature to better understand outcomes of this approach. A total of 55 patients were included in the systematic review. The study designs found in the literature review included case reports (66.7%), retrospective cohorts (22.2%), and prospective cohorts (11.1%). Of the studies reporting data, 50% of studies stated they used an open posterior approach to the dural defect, while 37.5% reported using an open anterior approach to the pathology. Only 1 (12.5%) study reported using an endoscope. Most studies (62.5%) used primary closure of the dura in their technique, while 37.5% reported using a local tissue graft (fat or muscle) or a dural sealant for their closure technique, and 25% of studies reported using a dural substitute for their closure technique. Overall mean clinical follow-up was 19.8 months. The endoscopic approach described here for treatment of this rare entity allows for removal of bony spicules/osteophytes and dural repair without the morbidity associated with traditional open dorsolateral or ventrolateral approaches.

Spontaneous Intracranial Hypotension

(N Engl J Med. 2021;385:2173–2178) Spontaneous intracranial hypotension presents as below-normal cerebrospinal fluid (CSF) due to CSF leakage from an unknown cause. While spontaneous intracranial hypotension is treatable, it is difficult to diagnose. Imaging has identified 3 types of spontaneous spinal CSF leaks: linear tear in the dura posterolateral or ventral to the spinal cord; diffuse dilatations of the dural sac or leakage at the simple meningeal diverticula; or the space between the CSF-venous fistulas and proximal veins. It is estimated that spontaneous intracranial hypotension occurs in 4 to 5 cases per 100,000 population.

Popping the balloon: Abrupt onset of a spinal CSF leak and spontaneous intracranial hypotension in idiopathic intracranial hypertension, a case report.

Spontaneous intracranial hypotension (SIH) is a debilitating neurologic condition that is often thought of as separate from idiopathic intracranial hypertension (IIH). The unique case presented here details a spontaneous spinal cerebrospinal fluid (CSF) leak that developed abruptly following a CSF pressure elevating maneuver in a patient with pre-existing intracranial hypertension, raising the possibility of a causative link between the two conditions. A 40-year-old woman with symptomatic IIH developed a dural tear of a thoracic spinal nerve root sleeve during an episode of Valsalva maneuver leading to a CSF leak and development of symptomatic SIH. This was successfully treated with epidural blood and fibrin glue patch and the patient is now symptom-free. The implication of a possible causative link between elevated CSF pressure and subsequent development of dural rupture and SIH raises important questions regarding the pathophysiology of SIH in some cases. Furthermore, it suggests that there could be a potential prophylactic benefit of CSF pressure lowering medications in preventing the development of SIH in patients with IIH.

The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: Challenges and opportunities

Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM). We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31-70 years]). Nine patients (42.8%) were found to have a CSF-venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near-complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF-venous fistula could be detected (P = 0.0011), and who were treated with non-targeted therapies. Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients.

In Reply to the Letter to the Editor Regarding “Spontaneous Spinal Cerebrospinal Fluid Leak: Review and Management Algorithm”

Expandable vertebrectomy devices are a key technology that has facilitated the adoption of minimally invasive approaches to spine oncology surgery. However, advanced technology still requires proper attention to surgical fundamentals. Here we illustrate a cage of a misplaced expandable vertebrectomy device causing esophageal perforation. Examination of the postoperative radiographs suggests that haptic feedback from the expandable technology may have given the false impression of bony engagement. This case highlights the need for proper mortise work and complete visualization of the segments to be instrumented even during minimally invasive surgery.

Microsurgical anatomy and treatment of dural defects in spontaneous spinal cerebrospinal fluid leaks.

Spontaneous spinal CSF leaks are caused by abnormalities of the spinal dura mater. Although most cases are treated conservatively or with an epidural blood patch, some intractable cases require neurosurgical treatment. However, previous reports are limited to a small number of cases. Preoperative detection and localization of spinal dural defects are difficult, and surgical repair of these defects is technically challenging. The authors present the anatomical characteristics of dural defects and surgical techniques in treating spontaneous CSF leaks. Among the consecutive patients who were diagnosed with spontaneous CSF leaks at the authors' institution between 2010 and 2020, those who required neurosurgical treatment were included in the study. All patients' clinical information, radiological studies, surgical notes, and outcomes were reviewed retrospectively. Outcomes of two different procedures in repairing dural defects were compared. Among 77 patients diagnosed with spontaneous CSF leaks, 21 patients (15 men; mean age 57 years) underwent neurosurgery. Dural defects were detected by FIESTA MRI in 7 patients, by CT myelography in 12, by digital subtraction myelography in 1, and by dynamic CT myelography in 1. The spinal levels of the defects were localized at the cervicothoracic junction in 16 patients (76%) and thoracolumbar junction in 4 (19%). Intraoperative findings revealed that the dural defects were small, circumscribed longitudinal slits located at the ventral aspect of the dura mater. The median dural defect size was 5 × 2 mm. The presence of dural defects at the thoracolumbar junction was associated with manifestation of an altered mental status, which was an unusual manifestation of CSF leaks (p = 0.003). Eight patients were treated via the posterior transdural approach with watertight primary sutures of the ventral defects, and 13 were treated with muscle or fat grafting. Regardless of the two different procedures, postoperative MRI showed either complete disappearance or significant reduction of the extradural CSF collection. No patient experienced postoperative neurological deficits. Clinical symptoms improved or stabilized in 20 patients with a median follow-up of 12 months. Dural defects in spontaneous CSF leaks were small, circumscribed longitudinal slits located ventral to the spinal cord at either the cervicothoracic or thoracolumbar junction. Muscle/fat grafting may be an alternative treatment to watertight primary sutures of ventral dural defects with a good outcome.

Radiological society of North America: 106th annual meeting, November 29 to December 5, 2020

Radiological society of North America: 106th annual meeting, November 29 to December 5, 2020

Headache due to spontaneous spinal cerebrospinal fluid leak secondary to cerebrospinal fluid-venous fistula: Case series.

Cerebrospinal fluid-venous fistula is an uncommon cause of spontaneous spinal cerebrospinal fluid leak (SSCSFL). We aim to describe the clinical presentation, imaging evaluation, treatment and outcome of SSCSFL secondary to cerebrospinal fluid-venous fistula. A retrospective review was undertaken of SSCSFL cases secondary to cerebrospinal fluid-venous fistula confirmed radiologically or intraoperatively, seen at our institution from January 1994 to March 2019. Cases with undetermined SSCSFL etiology, alternative etiology or unconfirmed fistula were excluded. Forty-four of 156 patients met the inclusion criteria (31 women, 13 men). Mean age of symptom onset was 52.6 years (SD 8.7, range 33-71 years). Headache was the presenting symptom in almost all, typically daily (69%), and most often in occipital/suboccipital regions. Headache character was most commonly pressure (38%), followed by throbbing/pulsing (21.4%). Orthostatic headache worsening occurred in 69% and an even greater percentage of patients (88%) reported Valsalva-induced headache exacerbation or precipitation. Headache occurred in isolation to Valsalva maneuvers in 12%. Of 37 patients with documented cerebrospinal fluid opening pressure, 13% were <6 cmH2O; 84%, 7-20 cmH2O; and one, 25 cmH2O. Fistulas were almost exclusively thoracic (95.5%). Only one patient responded definitively to epidural blood patch (EBP). Forty-two patients underwent surgery. Most improved following surgery; 48.7% were completely headache free and 26.8% had at least 50% improvement. In our series, cerebrospinal fluid-venous fistula was associated with a greater occurrence of Valsalva-induced headache exacerbation or precipitation than orthostatic headache and did not respond to EBP. Surgery provided significant improvement. Cerebrospinal fluid-venous fistula should be considered early in the differential diagnosis of Valsalva-induced ("cough") headache.

Spontaneous intracranial hypotension: review and expert opinion.

Spontaneous intracranial hypotension (SIH) results from spinal cerebrospinal fluid (CSF) leaking. An underlying connective tissue disorder that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. During the last decades, a much larger number of spontaneous cases are identified and a far broader clinical SIH spectrum is recognized. Orthostatic headache is the main presentation symptom of SIH; some patients also have other manifestations, mainly cochlear-vestibular signs and symptoms. Differential diagnosis with other syndromes presenting with orthostatic headache is crucial. Brain CT, brain MR, spine MRI, and MRI myelography are the imaging modalities of first choice for SIH diagnosis. Invasive imaging techniques, such as myelography, CT myelography, and radioisotopic cisternography, are progressively being abandoned. No randomized clinical trials have assessed the treatment of SIH. In a minority of cases, SIH resolved spontaneously or with only conservative treatment. If orthostatic headache persists after conservative treatment, a lumbar epidural blood patch (EBP) without previous leak identification (so-called "blind" EBP) is a widely used initial intervention and may be repeated several times. If EBPs fail, after the CSF leak sites identification using invasive imaging techniques, other therapeutic approaches include: a targeted epidural patch, surgical reduction of dural sac volume, or direct surgical closure. The prognosis is generally good after intervention, but serious complications may occur. More research is needed to better understand SIH pathophysiology to refine imaging modalities and treatment approaches and to evaluate clinical outcomes.

A complication of an epidural blood patch

Epidural blood patch (EBP) is generally accepted as an effective and relatively low risk therapeutic option for post dural puncture headache, as well spontaneous spinal cerebrospinal fluid (CSF) leak in patients not responding to conservative management [1]. Subdural hematoma (SDH) after EBP is a very rare complication. As in the case described by Verduzco et al., our patients also demonstrated an eight segment spread of the hematoma [2]. There are generally three predominant theories for development of SDH after EBP [3,4]. One is that the initial dural puncture with the Tuohy needle created a means of communication between the epidural and subdural spaces. The other is that the Tuohy needle

A complication of an epidural blood patch

C. Subdural hematoma. Epidural blood patch (EBP) is generally accepted as an effective and relatively low risk therapeutic option for post dural puncture headache, as well spontaneous spinal cerebrospinal fluid (CSF) leak in patients not responding to conservative management [[1]Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks.Headache. 2013; 53: 1034-1053Crossref PubMed Scopus (196) Google Scholar]. Subdural hematoma (SDH) after EBP is a very rare complication. As in the case described by Verduzco et al., our patients also demonstrated an eight segment spread of the hematoma [[2]Verduzco L.A. Atlas S.W. Riley E.T. Subdural hematoma after an epidural blood patch.Int J Obstet Anesth. 2012; 21: 189-192Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar]. There are generally three predominant theories for development of SDH after EBP [3Riley C.A. Spiegel J.E. Complications following large–volume epidural blood patches for postdural puncture headache.J Clin Anesth. 2009; 21: 355-359Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar, 4Devroe S. Van de Velde M. Demaerel P. et al.Spinal subdural hematoma after an epidural blood patch.Int J Obstet Anesth. 2015; 24: 288-289Abstract Full Text Full Text PDF Scopus (10) Google Scholar]. One is that the initial dural puncture with the Tuohy needle created a means of communication between the epidural and subdural spaces. The other is that the Tuohy needle was in the subdural space during the EBP with the blood injected into the subdural space. A third possibility is that both events occurred simultaneously. Intrathecal injection of an EBP has also been reported following accidental dural puncture with MRI demonstrating the intrathecal hematoma [[5]Kalina P. Craigo P. Weingarten T. Intrathecal injection of epidural blood patch.Emerg Radiol. 2004; 11: 56-59Crossref PubMed Scopus (38) Google Scholar]. A complication of an epidural blood patchJournal of Clinical NeuroscienceVol. 31PreviewA 58-year-old woman presented 1 week following an epidural blood patch (EBP) performed due to spontaneous cerebrospinal fluid (CSF) leak (diagnosed by intracranial MRI findings of CSF hypotension obtained for headaches). A spine MRI was performed as part of the continuing work-up to identify the source of the CSF leak (Fig. 1). She was otherwise asymptomatic. Full-Text PDF

Evaluation of Spontaneous Spinal Cerebrospinal Fluid Leaks Disease by Computerized Image Processing.

Spontaneous Spinal Cerebrospinal Fluid Leaks (SSCFL) is a disease based on tears on the dura mater. Due to widespread symptoms and low frequency of the disease, diagnosis is problematic. Diagnostic lumbar puncture is commonly used for diagnosing SSCFL, though it is invasive and may cause pain, inflammation or new leakages. T2-weighted MR imaging is also used for diagnosis; however, the literature on T2-weighted MRI states that findings for diagnosis of SSCFL could be erroneous when differentiating the diseased and control. One another technique for diagnosis is CT-myelography, but this has been suggested to be less successful than T2-weighted MRI and it needs an initial lumbar puncture. This study aimed to develop an objective, computerized numerical analysis method using noninvasive routine Magnetic Resonance Images that can be used in the evaluation and diagnosis of SSCFL disease. Brain boundaries were automatically detected using methods of mathematical morphology, and a distance transform was employed. According to normalized distances, average densities of certain sites were proportioned and a numerical criterion related to cerebrospinal fluid distribution was calculated. The developed method was able to differentiate between 14 patients and 14 control subjects significantly with p = 0.0088 and d = 0.958. Also, the pre and post-treatment MRI of four patients was obtained and analyzed. The results were differentiated statistically (p = 0.0320, d = 0.853). An original, noninvasive and objective diagnostic test based on computerized image processing has been developed for evaluation of SSCFL. To our knowledge, this is the first computerized image processing method for evaluation of the disease. Discrimination between patients and controls shows the validity of the method. Also, post-treatment changes observed in four patients support this verdict.