Neuro-ophthalmic features of patients with spontaneous cerebrospinal fluid leaks.

Increased Intracranial Pressure in Spontaneous CSF Leak Patients Is Not Associated with Papilledema

Increased intracranial pressure in spontaneous CSF leak patients is not associated with papilledema.

The association between cerebrospinal fluid (CSF) leaks at the skull base and raised intracranial pressure (ICP) has been reported since the 1960s. It has been suggested that spontaneous CSF leaks might represent a variant of idiopathic intracranial hypertension (IIH). We review the evidence regarding the association between spontaneous CSF leaks and IIH, and the role of ICP in the pathophysiology of nontraumatic skull base defects. We also discuss the management of ICP in the setting of CSF leaks and IIH. References were identified by searches of PubMed from 1955 to September 2018 with the terms "idiopathic intracranial hypertension" and "cerebrospinal fluid leak." Additional references were identified using the terms "pseudotumor cerebri," "intracranial hypertension," "benign intracranial hypertension," and by hand search of relevant articles. A CSF leak entails the egress of CSF from the subarachnoid spaces of the skull base into the surrounding cavitary structures. Striking overlaps exist regarding demographic, clinical, and radiological characteristics between IIH patients and those with spontaneous CSF leaks, suggesting that some (if not most) of these patients have IIH. However, determining whether a patient with spontaneous CSF leak may have IIH may be difficult, as signs and symptoms of raised ICP may be obviated by the leak. The pathophysiology is unknown but might stem from progressive erosion of the thin bone of the skull base by persistent pulsatile high CSF pressure. Currently, there is no consensus regarding the management of ICP after spontaneous CSF leak repair when IIH is suspected. IIH is becoming more widely recognized as a cause of spontaneous CSF leaks, but the causal relationship remains poorly characterized. Systematic evaluation and follow-up of patients with spontaneous CSF leaks by neuro-ophthalmologists will help clarify the relation between IIH and spontaneous CSF leaks.

Elevated intracranial pressure can cause skull base defects and a spontaneous cerebrospinal fluid (CSF) leak. Venous sinus stenting (VSS) has emerged as a promising treatment option for patients with a CSF leak in the setting of idiopathic intracranial hypertension (IIH). There is a lack of literature on symptomatology and quality of life (QOL) after VSS for IIH patients with a CSF leak. This study explores the effects of VSS on symptoms and QOL in IIH patients with a CSF leak. This is a retrospective study on patients who have IIH complicated by a CSF leak and underwent VSS. A QOL questionnaire was developed from the migraine disability assessment test and the PROMIS-PI was given to patients included in this study. A total of 10 patients were included in this study. Nine patients underwent endoscopic closure of CSF prior to stent placement and one patient was treated with VSS only. There was no evidence of CSF leak recurrence in this population following VSS. Headaches improved in 5/8, tinnitus in 5/6, and visual disturbance in 4/5 patients. Diamox was discontinued in seven out of eight patients after VSS. There was an improvement in headache-specific questions ( p = 0.0140) and overall QOL ( p = 0.0061) on the QOL questionnaire. This preliminary study demonstrates that VSS is effective in alleviating many symptoms in IIH patients with a CSF leak, especially headaches. Diamox may be able to be discontinued in many patients following VSS. No CSF leak recurrence was noted in this patient population.

Spontaneous cerebrospinal fluid (CSF) leaks are thought to arise due to elevated intracranial pressure which is distributed across the skull base, potentially predisposing the development of multifocal CSF leaks. The aims of this study are to evaluate the characteristics of this population at presentation and surgical outcomes. Retrospective case series. Tertiary referral center. Patients 18 years or older with spontaneous CSF leak diagnosis were eligible for study inclusion. Multifocal spontaneous CSF leak was defined as leaks originating from more than one skull base defect at anatomically distinct subsites. None. Clinical presentation and surgical outcome. Two hundred ninety-three patients with diagnosis of spontaneous CSF leak were identified. Of these, 11 (3.8%) were characterized as having multifocal spontaneous CSF leaks. Mean body mass index was significantly higher in multifocal CSF leak patients (31.5 ± 8.5 vs 46.2 ± 9.9, p < 0.0001). There was also higher prevalence of women (100% vs 63.8%, p = 0.012) and African-Americans (63.6% vs 23.4%, p = 0.003). The overall success rate of CSF leak repair was 95%. Fifty percent of patients developed headaches or blurry vision after surgical repair, and two patients required ventriculoperitoneal shunting. Multifocal spontaneous CSF leaks are rare and occurred in 3.8% of patients with spontaneous CSF. These patients were morbidly obese and more likely to be female and African American. Surgical repair conferred excellent outcomes. However, these patients have high risk of developing symptoms suggestive of elevated intracranial pressure postoperatively and should be counseled accordingly.

Transverse Venous Sinus Stenosis in Patients With Nasal Cerebrospinal Fluid Leak and Idiopathic Intracranial Hypertension.

19 - Radiologic findings in idiopathic intracranial hypertension

Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22-55 years). The mean duration of follow up was 36 months (range 6-132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.

Spontaneous CSF leaks: A paradigm for definitive repair and management of intracranial hypertension

We undertook a systematic review of the literature with meta-analysis to identify the role of obesity (BMI ≥30) in the patient characteristics presenting with spontaneous cerebrospinal fluid (sCSF) leaks of the lateral skull base and the outcomes of their repair. A Systematic Review of English Articles using MEDLINE, EMBASE, and Cochrane Library. The research algorithm included the following keywords: "spontaneous CSF leak," "lateral skull base," "temporal bone," "meningocele," "encephalocele," and "otorrhea." We also manually searched the references of included studies, to identify possible studies missed during our literature search. More than two-thirds of the patients were female (69.2%) and often were obese (mean BMI 36.5 kg/m2) with a mean age of 57. Most common presenting symptoms were otorrhea and hearing loss. Most authors did not report a routine use of a post-operative lumbar drain. Most patients had a single skull base defect and encephaloceles prolapsing through, across obese and non-obese groups. Median length of stay in hospital was 3.2 days, and the majority of patients did not have any recurrence during their follow-up (89.6%), which was not affected by obesity. Obesity does not affect length of hospital stay or recurrence rate following surgical repair of lateral skull base sCSF leaks. Surgical repair is a safe and viable approach in the management of obese patients with sCSF leaks in the temporal bone. NA Laryngoscope, 134:2012-2018, 2024.

Cases of cerebrospinal fluid (CSF) rhinorrhea without a clear etiology are known as spontaneous CSF leaks. Spontaneous CSF leaks most commonly occur in the ethmoid skull base or lateral sphenoid and rarely stop without surgical intervention. This chapter reviews the clinical presentation, diagnostic workup, and treatment of patients with spontaneous CSF rhinorrhea. Specific attention will be given to endoscopic methods of repair and the importance of idiopathic intracranial hypertension as a possible comorbidity and contributing factor.

In this study, we aimed to identify the effect of oxygen desaturation due to comparing ganglion cell complex (GCC), retinal nerve fiber layer (RNFL) between patients with obstructive sleep apnea syndrome (OSAS), simple snoring and healthy individuals.132 total patients with OSAS and simple snoring who were diagnosed with polysomnography between 2016 -2018 and 88 healthy individuals were included in the prospective-case control study. The patients were grouped according to the Apnea Hypopnea Index (AHI). Healthy volunteers and the patients with simple snoring (the AHI index value lower than 5) were grouped as respectively Group 1(G1) and Group 2 (G2). Groups 3 to 5 were separated according to the AHI index as mild OSAS (Group 3 (G3); AHI=5-15), moderate OSAS (Group 4(G4);AHI=16-30), and severe OSAS (Group 5(G5); AHI&gt;30). All study participants underwent an examination of both eyes including corrected visual acuity, intraocular pressure measurement, dilated-pupil fundus examination, with RNFL and GCC thickness which were measured with optical coherence tomography (OCT). Of the 132 patients included in the study, 29 had simple snoring, 22 mild, 22 moderate, and 57 severe OSAS. When bilateral mean, superior, inferior, temporal, and nasal RNFL thickness and mean, superior and inferior GCC values were compared among the study groups; no statistically significant difference was found (p&gt;0.005). No significant difference was evaluated between mean RNFL thickness, four quadrants RNFL thicknesses, mean GCC thickness, two quadrants GCC thickness with OCT in the study patients. Indeed, because the patients with diagnosed OSAS could more likely to develop glaucoma, it would be appropriate for these patients to have a regular optic examination.

IntroductionSpontaneous cerebrospinal fluid (CSF) leak is an increasingly recognized symptom of long-standing, untreated idiopathic intracranial hypertension (IIH). It is not uncommon for patients with IIH to have a delay in...

PurposeTo quantify the effects of CSF pressure alterations on intracranial venous morphology and hemodynamics in idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH) and assess reversibility when the underlying cause is resolved.MethodsWe prospectively examined venous volume, intracranial venous blood flow and velocity, including optic nerve sheath diameter (ONSD) as a noninvasive surrogate of CSF pressure changes in 11 patients with IIH, 11 age-matched and sex-matched healthy controls and 9 SIH patients, before and after neurosurgical closure of spinal dural leaks. We applied multiparametric MRI including 4D flow MRI, time-of-flight (TOF) and T2-weighted half-Fourier acquisition single-shot turbo-spin echo (HASTE).ResultsSinus volume overlapped between groups at baseline but decreased after treatment of intracranial hypotension (p = 0.067) along with a significant increase of ONSD (p = 0.003). Blood flow in the middle and dorsal superior sagittal sinus was remarkably lower in patients with higher CSF pressure (i.e., IIH versus controls and SIH after CSF leak closure) but blood flow velocity was comparable cross-sectionally between groups and longitudinally in SIH.ConclusionWe were able to demonstrate the interaction of CSF pressure, venous volumetry, venous hemodynamics and ONSD using multiparametric brain MRI. Closure of CSF leaks in SIH patients resulted in symptoms suggestive of increased intracranial pressure and caused a subsequent decrease of intracranial venous volume and of blood flow within the superior sagittal sinus while ONSD increased. In contrast, blood flow parameters from 4D flow MRI did not discriminate IIH, SIH and controls as hemodynamics at baseline overlapped at most vessel cross-sections.Supplementary InformationThe online version of this article (10.1007/s00062-023-01381-0) contains supplementary material, which is available to authorized users.

To describe the clinical presentation, pathophysiology, and treatment of spontaneous cerebrospinal fluid (CSF) leaks of the sphenoid bone, with an emphasis on a previously undescribed form in this location, in which CSF is trapped under the mucosa of the sinonasal cavity or in the soft tissue of the skull base. Case series and literature review. Analysis of cases through medical records and literature review. Four examples of unusual spontaneous CSF leaks of the skull base are presented. In each case, a CSF collection was contained behind the sinonasal mucosa of the sphenoid sinus, resembling a nasal polyp or mucocele on exam or imaging. In one case, the fluid collection was also associated with significant bone resorption and extravasation into the soft tissue of the infratemporal fossa. In each case, small defects of the ventral skull base (sphenoid bone) were the source of the CSF leaks. Successful treatment was achieved after transnasal endoscopic repair of the skull base defects using a combination of free abdominal fat grafts, free fascial grafts, and pedicled nasoseptal flaps. Postoperatively, a ventriculoperitoneal shunt was placed if the intracranial pressure was elevated. Spontaneous CSF leaks arising in the sphenoid sinus may not always present with overt CSF rhinorrhea but with a submucosal fluid collection (pseudomeningocele) that may mimic a mucocele or nasal polyp. These bona fide pseudomeningoceles of the skull base may be associated with elevated intracranial pressure and can be managed using endoscopic endonasal surgery.