Postoperative management of patients with spontaneous cerebrospinal fluid leak.

Headache arising from idiopathic changes in CSF pressure

19 - Radiologic findings in idiopathic intracranial hypertension

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.

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.

Clinical and imaging manifestations of idiopathic intracranial hypertension should prompt early diagnosis and treatment to avoid complications. Multiple diagnostic imaging criteria are reported to suggest the diagnosis of idiopathic intracranial hypertension with questionable sensitivity and/or specificity. Increased intracranial pressure results in dilation of the perineural cisternal spaces such as the optic nerve sheaths and the Meckel cave. It may also cause protrusion of cisternal structures of the Meckel cave through the skull base foramina, which could result in indentation or a bilobed appearance of the Meckel cave. We investigated the changes in the Meckel cave in patients with proved idiopathic intracranial hypertension versus healthy controls. We studied 75 patients with a diagnosis of idiopathic intracranial hypertension and 75 age-and sex-matched healthy controls. The transverse diameter of Meckel cave was measured in the axial and coronal planes of T2-weighted MR imaging sequences, and comparison was made between the 2 groups. The mean diameters of the Meckel cave on the coronal T2 plane in patients with idiopathic intracranial hypertension were 5.21 ± 1.22 mm on the right side and 5.16 ± 0.90 mm on the left side, while in the control group, they measured 3.89 ± 0.62 mm and 4.09 ± 0.68 mm, respectively (P value < .001). Of 75 patients with an approved diagnosis of idiopathic intracranial hypertension, 57 (76%) showed an indented Meckel cave as opposed to 21 (28%) in the control group. Our results confirm for the first time that the shape and size of the Meckel cave can be used as sensitive and specific diagnostic imaging markers for the diagnosis of idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) most typically occurs in women of childbearing age with increased weight as a key risk factor for development or exacerbation of the disease. Pregnancy is common in this group of patients. The longer-term effect of pregnancy on IIH has not been established and was the aim of this study. A prospective cohort study (IIH Life) recruited consecutive patients with IIH between 2012 and 2021 and evaluated outcomes including vision (logarithm of the minimum angle of resolution visual acuity, Humphrey visual field perimetric mean deviation, and optical coherence tomography [OCT] imaging) and headache. Four cohorts were evaluated: those with IIH diagnosed for the first time while pregnant, those with established IIH who became pregnant, those with a pregnancy prior to their diagnosis of IIH, and those with IIH who never became pregnant. Three hundred seventy-seven people with IIH agreed to participate in the IIH Life maternal health study. Mean follow-up was 17.5 months (SD 20.5). IIH diagnosed in pregnancy was rare. Patients diagnosed with IIH while pregnant had greater papilledema (mean OCT total retinal thickness +11.59 µm/mo [95% CI 1.25-21.93]), although they had comparable visual field and acuity measures compared with those with established IIH who became pregnant during their disease course (-1.2 µm/mo [95% CI -2.6 to 0.21]). In those with established IIH, pregnancy did not adversely affect visual or headache outcomes over time, and the trajectory was akin to those with IIH who never had a pregnancy. Headache outcomes showed variability reflecting the IIH cohort as a whole. A diagnosis of IIH while pregnant was rare but associated with more severe papilledema. Long-term visual outcomes in IIH were analogous irrespective of the timing of the pregnancy. These data are reassuring; however, close vigilance of IIH clinical features during pregnancy is recommended.

Predictive role of presenting symptoms and clinical findings in idiopathic intracranial hypertension

Describe audiometric outcomes following transmastoid and middle cranial fossa (MCF) approaches for repair of cerebrospinal fluid (CSF) otorhinorrhea. Retrospective case series. Tertiary skull base referral center. Adult patients presenting with CSF otorhinorrhea undergoing operative repair between January 2009 and July 2019. Transmastoid repair, MCF repair, or a combined approach. Primary outcome measures included preoperative and postoperative four-frequency pure-tone average (PTA), air-bone gap (ABG) and word recognition score. Secondary outcomes included success of repair, recurrence of CSF leak, and length of stay. Twenty-nine patients underwent 32 operations (mean age 52 yr, 75.9% female). Twenty (62.5%) patients underwent transmastoid repair, while 8 (25%) underwent an MCF approach. Patients had significant postoperative improvement in both PTA (34.8 dB preop vs. 24.5 dB postop, p = 0.003) and ABG (20.2 dB preop vs. 8.6 dB postop, p = 0.0001). CSF leak recurred in 3 patients (9.4%) over 17-month follow-up. Compared to MCF or combined approaches, transmastoid repair was associated with greater improvement in PTA (15.6 vs. 3.0 dB, p = 0.001) and shorter length of stay (0.3 vs. 1.2 days, p = 0.005). On subset analysis, patients with spontaneous CSF leaks, a single skull base defect, or meningoencephaloceles demonstrated significant audiometric improvements. The transmastoid approach for repair of CSF otorhinorrhea is effective, safe, and can be done on an outpatient basis. Patients with spontaneous CSF leaks, a single skull base defect, and associated encephaloceles may have better audiometric outcomes.

The diagnosis of idiopathic intracranial hypertension (IIH) is supported by papilledema, sometimes with headache, typically in an overweight woman aged 20 to 40. Cerebrospinal fluid (CSF) opening pressure ≥25 cm H2O without another primary etiology is confirmatory. In many cases, ophthalmologic and CSF examinations can be difficult to interpret. Researchers at one referral center retrospectively reviewed records on 165 referrals for new IIH consultations during an 8-month period. Of the 165 patients, …

Increased intracranial pressure is a potential cause of spontaneous cerebrospinal fluid (sCSF) leak. Associated neuro-ophthalmic features have not been well studied, particularly relationships with idiopathic intracranial hypertension (IIH). We hypothesized that neuro-ophthalmic features routinely used in evaluations for IIH can be useful in the investigation of a causal relationship between IIH and sCSF leak. We reviewed the neuro-ophthalmic examination and office-based ophthalmic imaging data of all consecutive patients with sCSF leaks and at least one repair to investigate the clinical and neuro-ophthalmic features of increased intracranial pressure. We conducted a retrospective longitudinal study at a single institution by querying the electronic medical record system for CSF leak Current Procedural Terminology (CPT) codes (G96.00 and G96.01) from June 1, 2019, to July 31, 2022. For patients with a confirmed diagnosis of sCSF leak, demographic information, eye examination results, and ophthalmic imaging details for both eyes were collected. A total of 189 patients with CSF leaks were identified through CPT coding; 159 had iatrogenic or traumatic CSF leaks, and 30 individuals (3 male, 27 female) had confirmed sCSF leaks. The mean age of patients with sCSF leaks was 46 years (range: 29 - 81), with a mean body mass index of 35.2 kg/m2 (range: 18.2 - 54.1). Only 11 of 30 underwent eye examinations (8 before surgical repair and 10 after). The mean pre-repair and post-repair best-corrected visual acuity were 20/30 (range: 20/20 - 20/55) and 20/25 (range: 20/20 - 20/40), respectively (P = 0.188). The mean retinal nerve fiber layer thickness was 99 µm (range: 96 - 104) pre-repair and 97 µm (range: 84 - 103) post-repair (P = 0.195). The mean ganglion cell complex thickness was 84 µm (range: 72 - 94) pre-repair and 82 µm (range: 71 - 94) post-repair (P = 0.500). Humphrey visual field average mean deviation was -5.1 (range: -12.4 - -1.8) pre-repair and -1.0 (range: -10.1 - 2.1) post-repair (P = 0.063). Serial neuro-ophthalmic examinations are recommended for patients with sCSF leaks to screen for signs of current or prior increased intracranial pressure. Larger studies are required to clarify the longitudinal changes in neuro-ophthalmic features, to investigate the incidence of IIH in cases of sCSF leak development or recurrence after surgical repair, and to explore potential causal relationships to guide post-repair management and prevent recurrent leaks. A multicenter consortium is also suggested to develop a standard clinical protocol for comprehensive management of sCSF 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.

We reviewed the medical case report literature to determine the proportion of cases of idiopathic intracranial hypertension (IIH) that were either inappropriately labelled as IIH or prematurely given this diagnosis. We searched OVID MEDLINE from 2012 to 2022 to identify case reports that diagnosed patients with IIH. Case reports were assessed for diagnostic accuracy using Friedman et al.'s revised diagnostic criteria for primary pseudotumor cerebri syndrome. Our primary outcome was the crude prevalence of inappropriate or premature IIH diagnoses. Our secondary outcome was determining if inaccurate IIH diagnoses were associated with variables such as journal subscription model and impact factor, author affiliation, country of origin, and year of publication. A total of 33/185 case reports (17.8%) either incorrectly labelled a patient as having IIH or did not perform all of the investigations necessary to make a diagnosis of IIH. Some of these studies (4.8%) were believed to still represent 'probable' IIH given the clinical presentation, whereas 13.0% of studies were determined to have mislabelled their patients as having IIH. The most common reason that case reports did not meet diagnostic criteria included: a lack of MRV in atypical patient cases (42.4%, n = 14), no papilledema in addition to a lack of characteristic neuroimaging features (33.3%, n = 11), intracranial hypertension being secondary to another documented cause (12.1%, n = 4), normal LP opening pressure in addition to other factors (12.1%,n = 4), no description of neuroimaging (6.1%, n = 2), and abnormal CSF composition (6.1%, n = 2). Case reports that used the term 'IIH' incorrectly had a significantly lower journal impact factor (2.0 vs. 2.6, p = 0.01). There is a high prevalence of premature or inappropriate diagnoses of IIH in the peer-reviewed case report literature. Adherence to published diagnostic criteria is needed when publishing IIH case reports, and authors are expected to report all relevant data in their report to ensure that an accurate diagnosis is made.

Objective Idiopathic Intracranial Hypertension (IIH) was first reported in 1897 as “Pseudo tumor Cerebri” when lumbar puncture was initiated in medicine. However, it is still a challenging diagnosis for many neurologists and ophthalmologists. Likewise, the sequela of IIH can be debilitating overall health and quality of life in most patients. This report presents the evolving diagnostic modalities that are different from mainstream IIH reports on gender, obesity and headaches ratios. We believe, using standard optical coherence tomography (OCT) can allow for a much earlier and more accurate diagnosis of IIH, with marked reduction in visual loss morbidity.Methods Retrospective, observational method was used to review 171 patients’ charts with suspected IIH over the past 12 years. The OCT of optic nerve and Macula was conducted by considering a list of signs and symptoms. Brain MRI, lumbar puncture, and our standard laboratory workups for optic neuropathies and visual fields were done. Visual Evoked Potentials were performed by neurologists. For purpose of this paper all IIH diagnosis fit Dandy diagnosis criteria of pseudo tumor cerebri as having a normal MRI.Results We evaluated 164 cases that had LPs done due to our proposed OCT Patterns to rule out IIH with no bio-microscopic evidence of overt papilledema. The majority of LP positive patients had some degree of visual field damage. Twenty out of 164 cases had false positive OCTs (with negative LPs) 88% accuracy in OCT diagnosis of IIH. No false positive OCT in Pattern-I.Conclusion Four different OCT diagnostic Patterns presented for IIH cases. The demographics of the patients considered having IIH by OCT Patterns I-IV and proven by positive LP; do not fit the classic demographic patterns. Classically, IIH has been reported to be observed in obese females with headaches and visible papilledema. Our findings put this classic set of presenting signs into question.

The purpose of this review is to shed light into the diagnostic challenges regarding idiopathic intracranial hypertension (IIH), giving more emphasis to the role of optical coherence tomography (OCT) in IIH, while it also presents recent advances in the management of IIH. A literature search in PubMed was performed for studies about the diagnosis and management of IIH published before June 30, 2017. Then, a comprehensive review of the eligible studies and the relevant references was performed. Apart from the updated modified criteria, OCT has been found to be important for the diagnosis of IIH, along with assessment of clinical signs and symptoms, brain imaging, and lumbar puncture. On the other hand, there is no current consensus for the management of IIH, but various interventions have been used, such as dietary therapy (weight loss, bariatric surgery, lifestyle modification, low-salt diet), medications, and surgical treatment. IIH is a challenging and serious disorder, which may lead to permanent visual impairment if there is a delay in its diagnosis. Advances in retinal imaging have led to better understanding of the pathogenesis of the disease and prompt diagnosis even in subtle cases can be done using OCT. Surgical interventions should be minimized and only used in cases refractory to medical treatment and in those with rapid progression.