OBJECTIVE: Idiopathic intracranial hypertension (IIH) is a condition of high cerebrospinal fluid (CSF) pressure that presents with CSF leak. The implications of multiple skull base defects (SBD) and associated synchronous CSF leaks have not been previously explored.
METHODS: A dual institutional case-control study examined multiple SBD\'s and encephaloceles on the risk of CSF leak and postoperative failures post-repair. IIH patients with CSF leaks and IIH controls without leaks were selected retrospectively. Chi square analysis evaluated for statistically significant alterations in probability with CSF leak development.
RESULTS: 192 patients were selected with 108 IIH controls and 84 spontaneous CSF leak cases. Signs and symptoms for IIH controls and CSF leak cases respectively were pulsatile tinnitus (60.2 % and 29.8 %), headaches (96.3 % and 63.1 %), papilledema (74.1 % and 12.5 %), visual field defects (60.8 % and 13 %) (p < 0.001). Encephalocele formation in controls was 3.7 % compared to cases at 91.6 % (p < 0.001). Multiple SBD\'s in controls compared cases was 0.9 % and 46.4 % respectively (p < 0.001). Subgroup analysis of CSF leak cases showed 15 patients with two CSF leak repairs due to a recurrence. 27 (39.1 %) single leak cases had multiple SDB\'s while 12 (80 %) recurrent leaks had multiple SDB\'s (p = 0.004).
CONCLUSIONS: Patients with radiographic evidence of multiple SBD\'s and encephaloceles represent a high-risk population with a propensity for CSF leaks. Secondary SBD\'s are common in patients with spontaneous CSF rhinorrhea and higher in patients with a recurrence.

Spontaneous cerebrospinal fluid (sCSF) leaks develop from pressure erosion due to idiopathic intracranial hypertension, treatment of which is paramount to preventing recurrence. Direct measurements of intracranial pressure (ICP) for monitoring response to treatment via lumbar drain (LD) or ventriculostomy are invasive and have risks. The objectives of this study are to determine whether ultrasonographic measurements of optic nerve sheath diameter (ONSD) correlate with LD ICP in patients with sCSF leaks undergoing treatment, and whether ONSDs are larger in patients with sCSF leaks than controls.
Subjects with sCSF leaks and controls were prospectively recruited. ONSD, sex, and body mass index (BMI) were analyzed. For sCSF leak subjects, ultrasonography was performed at the time of LD opening and each pressure check postoperatively, including the acetazolamide response. In control patients, measurements were obtained at the time of surgery. Pearson\'s correlation between ONSD and ICP was performed.
Subjects with sCSF leaks (n = 9, age 52.4 ± 9.5, all female) and controls (n = 8, age 60.1 ± 14.8, two females) had significantly different BMIs, 38.4 ± 8.1 vs. 29.2 ± 4.8, t(15) = 2.793, p = 0.014. ONSD was strongly correlated with ICP measurements (r = 0.583, p = 0.002). However, percentage change in ONSD and ICP measurements were more strongly correlated (r = 0.733, p < 0.001). Patients with sCSF leaks had significantly higher ONSDs than controls, 0.63 cm ± 0.044 vs. 0.56 cm ± 0.074, t(15) = 2.329, p = 0.034.
ONSD significantly correlated with ICP in sCSF leak patients and was wider in sCSF leak subjects than controls. Ultrasonography has utility in monitoring the ICP response to acetazolamide.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks (SCSFL) occur in the absence of trauma, surgery, or underlying intracranial pathology. They represent a significant healthcare burden on patients with complications such as meningitis. We present our experience of SCSFL repair via the endonasal endoscopic approach.
METHODS: All patients who underwent CSF fistula repair at a tertiary UK hospital, between 1st January 2012 to 31st December 2019, were identified and had their case notes analyzed retrospectively.
RESULTS: There were 33 patients included consisting of 27 (81.8%) females, with age range from 31 to 81 years (mean 55.2). Mean body mass index (BMI) was 35.2 kg/m2 , with 32 (97.0%) patients overweight (BMI >25). All patients presented with intermittent watery rhinorrhoea and had a positive biochemical analysis. Computed tomography (CT) and/or magnetic resonance imaging (MRI) identified leak sites in 29 patients (87.9%). The most common intraoperative defect site was the cribriform plate (42.4%). A variety of closing techniques were used including onlay grafts (72.7%), tissue glue (87.9%), nasoseptal flaps (63.6%), mucosal free grafts (21.2%), fat grafts (21.2%), and additional support materials (87.9%). Nasal packing was used in all patients. The average length of stay was 1.6 days. Postoperative complications occurred in two patients (6.1%) (meningitis, epistaxis). Overall, there was a successful primary repair in 32 (97.0%) patients with most cases employing a multilayered reconstruction method (78.8%).
CONCLUSIONS: Our results demonstrate excellent success rates with the endoscopic endonasal approach to SCSFL. This adds to the literature by demonstrating a detailed analysis of the experience in SCSFL management in one of the largest UK centres.

UNASSIGNED: Spontaneous cerebrospinal fluid (CSF) rhinorrhea is a diagnostic challenge due to its overlapping symptomatology with other sinonasal diseases. The objective of this study was to investigate whether items on the sinonasal outcome test (SNOT)-22 could suggest a diagnosis of spontaneous CSF rhinorrhea versus chronic rhinosinusitis without nasal polyps (CRSsNP).
UNASSIGNED: A multi-institutional retrospective chart review of patients with spontaneous CSF rhinorrhea and a control group of CRSsNP patients was performed. Individual SNOT-22 scores and domain scores were compared.
UNASSIGNED: One hundred fifteen patients were included in both cohorts. Of the patients in the CSF rhinorrhea group, 48% were misdiagnosed as chronic rhinosinusitis (CRS) prior to the correct identification of a CSF leak. On bivariate analysis, the CSF rhinorrhea group scored significantly higher on the SNOT-22 for runny nose (P < .001) and was more likely to designate this symptom as most important (P < .001). The CRSsNP group scored significantly higher in nasal blockage (P < .001), thick nasal discharge (P < .001), facial pain/pressure (P < .001), and in the ear/facial (P < .001) and rhinologic (P = .003) domains. Multivariable logistic regression revealed that runny nose (P < .001) was most predictive of spontaneous CSF rhinorrhea while nasal blockage (P < .001), thick nasal discharge (P < .001), and facial pain/pressure (P = .001) were predictive of CRSsNP after adjusting for relevant confounders. No significant difference was observed in total SNOT-22 scores between groups (P = .676).
UNASSIGNED: Spontaneous CSF rhinorrhea is commonly misdiagnosed as other sinonasal pathologies. However, individual SNOT-22 items can help aid in suggesting a CSF leak. Spontaneous CSF rhinorrhea should be suspected in patients who have high SNOT-22 scores for runny nose and report this symptom as most important, but have lower scores related to the other cardinal symptoms of CRS.

Cerebrospinal Fluid (CSF) leakage results from a defect in the skull base, which communicates the subarachnoid space with the nasal cavity. The most common cause of CSF leakage is traumatic, and non-traumatic causes are less common. This case report illustrates a case of a woman who presented to the emergency department with clear fluid pouring from her nose for three weeks with a fever. The patient had pneumococcal meningitis and Idiopathic Intracranial Hypertension (ICH) seven years ago. Computed Tomography (CT) sinuses showed the defect seen on the right side of the cribriform plate, and the Magnetic Resonant Imaging (MRI) confirmed the CSF leakage. The CSF leakage was diagnosed by positive B transferrin.  This case highlights a rare condition that needs early detection and treatment to prevent complications such as ascending meningitis.

OBJECTIVE: Spontaneous CSF leaks into the anterior skull base nasal sinuses are often associated with meningoencephaloceles and occur in patients with idiopathic intracranial hypertension (IIH). Endonasal endoscopic repair has become the primary method of choice for repair. The authors sought to evaluate the success rate of endoscopic closure and to identify predictive factors for CSF leak recurrence.
METHODS: A consecutive series of endonasally repaired anterior skull base meningoencephaloceles was drawn from a prospectively acquired database. Lumbar punctures were not performed as part of a treatment algorithm. All patients had at least 5 months of follow-up. Chart review and phone calls were used to determine the timing and predictors of recurrence. Demographic information and details of operative technique were correlated with recurrence. Two independent radiologists reviewed all preoperative imaging to identify radiographic markers of IIH, as well as the location and size of the meningoencephalocele.
UNASSIGNED: From a total of 54 patients there were 5 with recurrences (9.3%), but of the 39 patients in whom a vascularized nasoseptal (n = 31) or turbinate (n = 8) flap was used there were no recurrences (p = 0.0009). The mean time to recurrence was 24.8 months (range 9-38 months). There was a trend to higher BMI in patients whose leak recurred (mean [± SD] 36.6 ± 8.6) compared with those whose leak did not recur (31.8 ± 7.4; p = 0.182). Although the lateral recess of the sphenoid sinus was the most common site of meningoencephalocele, the fovea ethmoidalis was the most common site in recurrent cases (80%; p = 0.013). However, a vascularized flap was used in significantly more patients with sphenoid (78.3%) defects than in patients with fovea ethmoidalis (28.6%) defects (Fisher\'s exact test, p = 0.005). Radiographic signs of IIH were equally present in all patients whose leak recurred (75%) compared with patients whose leak did not recur (63.3%); however, an enlarged Meckel cave was present in 100% (2/2) of patients whose leaks recurred compared with 13.3% (4/30) of patients whose leaks did not recur (p = 0.03). The average meningoencephalocele diameter tended to be larger (1.73 ± 1.3 cm) in patients with recurrence compared to those without recurrence (1.2 ± 0.66 cm; p = 0.22). A ventriculoperitoneal shunt was already in place in 3 patients, placed perioperatively in 5, and placed at recurrence in 2, none of whose leaks recurred.
CONCLUSIONS: Recurrence after endonasal repair of spontaneous CSF leaks from meningoencephaloceles can be dramatically reduced with the use of a vascularized flap. Although failures of endonasal repair tend to occur in patients who have higher BMI, larger brain herniations, and no CSF diversion, the lack of vascularized flap was the single most important risk factor predictive of failure.

Primary, spontaneous cerebrospinal fluid (CSF) leaks secondary to defects in the clivus are exceedingly rare. Additionally, primary, spontaneous CSF leaks are typically present in obese women with idiopathic intracranial hypertension (IIH). In the present study, we report the first case of a primary, spontaneous CSF leak in the inferior-posterior wall of the clivus in an atypical patient with a BMI of 18.9 kg/m2 without IIH. Accurate diagnoses of CSF leaks are imperative in the context of preventing meningitis, and delays in diagnosis and treatment are associated with worse outcomes. Improved characterization of rare, spontaneous CSF leaks may prove beneficial in correctly diagnosing affected patients.

Introduction Cerebrospinal fluid (CSF) rhinorrhea is the result of a bony defect at the skull base with disruption of the arachnoid, dura mater, and sinonasal mucosa that leads to an active CSF leak and flow of clear fluid from the nose. The endoscopic repair of CSF leaks and skull defects have been used by an increasing number of surgeons and is the standard of care for repairing CSF leaks. Materials and methods We conducted a retrospective study of all cases of CSF leaks managed via the endonasal endoscopic approach from 2010 to 2020 at a tertiary referral hospital of King Fahad Specialist Hospital, Dammam (KFSH-D). Results Over 10 years, 61 procedures were performed on a total of 56 patients (average age, 39.9 years) with 26 spontaneous CSF leaks and 30 traumatic CSF leaks. The leak sites were frontal bone in 14% of the cases, the roof of the ethmoid in 25%, the cribriform plate of ethmoid in 39%, and the walls of sphenoid sinus in 21%; multiple site defects were found in eight patients. The defect was localized by high-resolution computed tomography (CT) of the paranasal sinuses and skull base and magnetic resonance imaging (MRI) in all patients. CT cisternography, intrathecal fluorescein injection, and topical application of fluorescein dye were used in patients as required. A combination of free grafts and flaps materials were used in most patients. A middle and inferior turbinate graft was used in 12 patients, a septal cartilage graft in 18 patients, and a pedicled nasoseptal flap in 12 patients. The success rate was 92% after the first closure attempt. A recurrence of CSF leaks was observed in four patients. The mean hospitalization time was 6.5 days. The postoperative follow-up period ranged from one year to 10 years with a mean postoperative follow-up time of three years. Conclusions The endonasal endoscopic approach is the current standard of care for repairing most CSF leaks and skull base defects. We have had an excellent experience with endonasal endoscopic CSF leak repair, with high success rates and low morbidity. Our results support the effectiveness and safety of this technique and should encourage otolaryngologists to apply the procedure in cases of CSF leak.

The association between spontaneous cerebrospinal fluid (CSF) leak/rhinorrhea and idiopathic intracranial hypertension (IIH) has been increasingly recognized over the last years. However, considerable variability of opinion regarding the assessment, investigations, and management of patients with spontaneous CSF rhinorrhea remains.
A consensus group was formed from experts from Europe, Asia, Australia, South and North America. Following literature review and open discussions with members of the panel, a set of 61 statements was produced. A modified Delphi method was used to refine expert opinion with 3 rounds of questionnaires and a consensus group meeting in Santo-Rhino meeting in September 2019.
Fifty statements (82% of total) on spontaneous CSF leak and IIH reached consensus. In 38 of 50 statements, the median response was 7 (strongly agree) and in the 12 remaining statements the median response was 6 (agree). Eleven statements were excluded because they did not reach consensus and one new statement was added during SantoRhino meeting. The final statements refer to patient history and clinical examination (\"History taking should include presence of headache, tinnitus and visual defects\"), investigations (role of Thin Slice Computed Tomography and CISS/FLAIR sequences in Magnetic Resonance Imaging), principles of management (watchful waiting or measures to reduce ICP are supplementary but cannot subsitute surgical closure), surgical technique, intraoperative, early postoperative and long term management.
We present fifty consensus statements on the diagnosis, investigation, and management of spontaneous CSF rhinorrhea based on the currently available evidence and expert opinion. Although by no means comprehensive and final, we believe they can contribute to the standardization of clinical practice. Early diagnosis, prompt surgical closure of the defect, assesment for and treatment of potentially co-existing idiopathic intracranial hypertension in a comprehensive multidisciplinary approach are essential in order to successfully manage spontaneous CSF rhinorrhea, reduce associated morbidity and prevent recurrence.

Most spontaneous CSF leaks (SCSFL) are associated with an underlying pseudotumor cerebri syndrome (PTCS). Treatment generally includes surgical leak repair and PTCS correction, as untreated PTCS carries a risk of recurrence. We describe a 72-year-old woman with rhinorrhea, aural fullness, and posterior nasal drip. CT and MRI showed signs of CSF hypovolemia and PTCS, as well as bilateral transverse sinus stenoses. CT and MRI cisternography documented CSF leaks through the right cribriform plate and the posterior aspect of the petrous bone. Opening CSF pressure was 6 cm H2O. Dural venous sinus stenting (DVSS) was performed after failed conservative treatment. Rhinorrhea resolved 3 days after stenting, aural fullness 1 month later. After 6 months, signs of CSF hypovolemia had disappeared on MRI and the stents were patent. After 9 months, the patient had a transient, spontaneously resolving episode of rhinorrhea. She has been symptom-free for the remaining 39 months of follow-up.