Category: CSF disorders

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Coupling of CSF and sagittal sinus pressure in adult patients with pseudotumour cerebri

Acta Neurochirurgica (2020) 162: 1001–1009

Pseudotumour cerebri syndrome (PTCS including idiopathic intracranial hypertension) is characterised by the symptoms and signs of raised cerebrospinal fluid pressure (CSFp) in the absence of ventricular dilatation or an intracranial mass lesion. Its aetiology is unknown in the majority of cases but there is much evidence for impaired CSF absorption. Traditionally, sagittal sinus pressure has been considered to be independent of CSF pressure in adults. However, the discovery of stenoses of intracranial venous sinuses and introduction of venous sinus stenting has highlighted the importance of the venous drainage in PTCS. In this study, we have explored the relationship between CSFp and SSp before and during a CSF infusion test and during CSF drainage.

Materials and methods Ten patients (9 females:1 male) with PTCS underwent infusion studies in parallel with direct retrograde cerebral venography. Both SSp and CSFp were recorded at a baseline and during CSFp elevation in a course of a CSF infusion test. The drainage of CSF after the CSF infusion was performed in 7 patients. In 5 cases, jugular venous pressure was also measured.

Results CSFp and SSp including their amplitudes correlated significantly and strongly both at baseline (R = 0.96; p = 0.001) and during infusion (R = 0.92; p = 0.0026). During drainage, this correlation was maintained until SSp reached a stable value, whereas CSFp continued to decrease.

Conclusions In this series of ten patients with PTCS, CSFp and SSp were coupled, both at baseline and during infusion. The implications of such coupling for the calculation of CSF outflow resistance are discussed.

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Lumboperitoneal and Ventriculoperitoneal Shunting for Idiopathic Intracranial Hypertension Demonstrate Comparable Failure and Complication Rates

Neurosurgery 86:272–280, 2020

Idiopathic intracranial hypertension results in increased intracranial pressure leading to headache and visual loss. This disease frequently requires surgical intervention through lumboperitoneal (LP) or ventriculoperitoneal (VP) shunting.

OBJECTIVE: To compare postoperative outcomes between LP and VP shunts, including failure and complication rates.

METHODS: A retrospective analysis was conducted using a national administrative database (MarketScan) to identify idiopathic intracranial hypertension (IIH) patients who underwent LP or VP shunting from 2007 to 2014. Multivariate logistic and Cox regressions were performed to compare rates of shunt failure and time to shunt failure between LP and VP shunts while controlling for demographics and comorbidities.

RESULTS: The analytic cohort included 1082 IIH patients, 347 of whom underwent LP shunt placement at index hospitalization and 735 of whom underwent VP shunt placement. Rates of shunt failure were similar among patients with LP and VP shunt (34.6% vs 31.7%; P=.382). Among patients who experienced shunt failure, the mean number of shunt failures was 2.1±1.6 and was similar between LP and VP cohorts. Ninety-day readmission rates, complication rates, and costs did not differ significantly between LP and VP shunts. Patients who experienced more than two shunt failures tended to have an earlier time to first shunt failure (hazard ratio 1.41; 95% confidence interval 1.08-1.85; P = .013).

CONCLUSION: These findings suggest that LP and VP shunts may have comparable rates of shunt failure and complication. Regardless of shunt type, earlier time to first shunt failure may be associated with multiple shunt failures.

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Chiari malformation and syringomyelia

J Neurosurg Spine 31:619–628, 2019

Chiari malformation was first described over a century ago, and consists of posterior fossa anomalies that generally share the feature of cerebellar tonsillar descent through the foramen magnum. Our understanding of this disorder was initially based on autopsy studies, and has been greatly enhanced by the advent of MRI.

The surgical management of Chiari anomalies has also evolved in a parallel fashion. Although the exact surgical technique varies among individual surgeons, the goals of surgery remain constant and consist of relieving brainstem compression and cranial nerve distortion, restoring the normal flow of CSF across the foramen magnum, and reducing the size of any associated syrinx cavity. Syrinx cavities are most commonly associated with Chiari anomalies, yet primary spinal syringomyelia (PSS) can be caused by traumatic, infectious, degenerative, and other etiologies that cause at least a partial CSF flow obstruction in the spinal subarachnoid space. As with syringomyelia associated with Chiari anomalies, the main goal of PSS surgery is to reestablish CSF flow across the area of obstruction. In addition to MRI, myelography with CT can be very helpful in the evaluation and management of these patients by identifying focal regions of CSF obstruction that may be amenable to surgical intervention.

Future directions for the treatment of Chiari anomalies and syringomyelia include the application of advanced imaging techniques, more widespread use of genetic evaluation, large-scale outcome studies, and the further refinement of surgical technique.

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Noninvasive Thermal Evaluation of Ventriculoperitoneal Shunt Patency and Cerebrospinal Fluid Flow Using a Flow Enhancing Device

Neurosurgery, Volume 85, Issue 2, August 2019, Pages 240–249

While a noninvasive flow determination would be desirable in the diagnosis of cerebrospinal fluid shunt malfunction, existing studies have not yet defined a role for thermal flow detection.

OBJECTIVE: To evaluate a revised test protocol using a micropumper designed to transiently enhance flow during thermal testing to determine whether thermal detection of flow is associated with progression to shunt revision surgery.

METHODS: Eighty-two unique tests were performed in 71 shunts. The primary outcome, need for revision within 7 d of testing, was compared with results of micropumper-augmented thermal flow detection. Statistical analysis was based on blind interpretation of test results and raw temperature data recorded during testing.

RESULTS: The test was sensitive (73%) and specific (68%) in predicting need for revision, with 5.6-fold higher probability of revision when flow was not detected. Negative predictive value in our sample was 94.2%. The probability of not requiring revision increased with increasing total temperature drop. Analysis of various possible thresholds showed that the optimal temperature cutoff may be lower than suggested by the manufacturer (0.125◦C vs 0.2◦C).

CONCLUSION: This is the first study to report a strong association between thermal flow evaluation and a clinical impression that a shunt is not malfunctioning. The current recommended threshold may increase the false positive rate unnecessarily, and as clinicians gain experience with the method, they may find value in examining the temperature curves themselves. Multicenter studies are suggested to further define a role for this diagnostic test.

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Comparative durability and costs analysis of ventricular shunts

J Neurosurg 130:1252–1259, 2019

Ventricular shunt (VS) durability has been well studied in the pediatric population and in patients with normal pressure hydrocephalus; however, further evaluation in a more heterogeneous adult population is needed. This study aims to evaluate the effect of diagnosis and valve type—fixed versus programmable—on shunt durability and cost for placement of shunts in adult patients.

METHODS The authors retrospectively reviewed the medical records of all patients who underwent implantation of a VS for hydrocephalus at their institution over a 3-year period between August 2013 and October 2016 with a minimum postoperative follow-up of 6 months. The primary outcome was shunt revision, which was defined as reoperation for any indication after the initial procedure. Supply costs, shunt durability, and hydrocephalus etiologies were compared between fixed and programmable valves.

RESULTS A total of 417 patients underwent shunt placement during the index time frame, consisting of 62 fixed shunts (15%) and 355 programmable shunts (85%). The mean follow-up was 30 ± 12 (SD) months. The shunt revision rate was 22% for programmable pressure valves and 21% for fixed pressure valves (HR 1.1 [95% CI 0.6–1.8]). Shunt complications, such as valve failure, infection, and overdrainage, occurred with similar frequency across valve types. Kaplan-Meier survival curve analysis showed no difference in durability between fixed (mean 39 months) and programmable (mean 40 months) shunts (p = 0.980, log-rank test). The median shunt supply cost per index case and accounting for subsequent revisions was $3438 (interquartile range $2938–$3876) and $1504 (interquartile range $753–$1584) for programmable and fixed shunts, respectively (p < 0.001, Wilcoxon rank-sum test). Of all hydrocephalus etiologies, pseudotumor cerebri (HR 1.9 [95% CI 1.2–3.1]) and previous shunt malfunction (HR 1.8 [95% CI 1.2–2.7]) were found to significantly increase the risk of shunt revision. Within each diagnosis, there were no significant differences in revision rates between shunts with a fixed valve and shunts with a programmable valve.

CONCLUSIONS Long-term shunt revision rates are similar for fixed and programmable shunt pressure valves in adult patients. Hydrocephalus etiology may play a significant role in predicting shunt revision, although programmable valves incur higher supply costs regardless of initial diagnosis. Utilization of fixed pressure valves versus programmable pressure valves may reduce supply costs while maintaining similar revision rates. Given the importance of developing cost-effective management protocols, this study highlights the critical need for large-scale prospective observational studies and randomized clinical trials of ventricular shunt valve revisions and additional patient-centered outcomes.

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Origin of Syrinx Fluid in Syringomyelia: A Physiological Study

Neurosurgery 84:457–468, 2019

The origin of syrinx fluid is controversial.

OBJECTIVE: To elucidate themechanisms of syringomyelia associated with cerebrospinal fluid pathway obstruction and with intramedullary tumors, contrast transport from the spinal subarachnoid space (SAS) to syrinx was evaluated in syringomyelia patients.

METHODS: We prospectively studied patients with syringomyelia: 22 with Chiari I malformation and 16 with SAS obstruction-related syringomyelia before and 1 wk after surgery, and 9 with tumor-related syringomyelia before surgery only. Computed tomographymyelography quantified dye transport into the syrinx before and 0.5, 2, 4, 6, 8, 10, and 22 h after contrast injection by measuring contrast density in Hounsfield units (HU).

RESULTS: Before surgery, more contrast passed into the syrinx in Chiari I malformation related syringomyelia and spinal obstruction-related syringomyelia than in tumor-related syringomyelia, as measured by (1) maximum syrinx HU, (2) area under the syrinx concentration-time curve (HU AUC), (3) ratio of syrinx HU to subarachnoid cerebrospinal fluid (CSF; SAS) HU, and (4) AUC syrinx/AUC SAS. More contrast (AUC) accumulated in the syrinx and subarachnoid space before than after surgery.

CONCLUSION: Transparenchymal bulk flow of CSF from the subarachnoid space to syrinx occurs in Chiari I malformation-related syringomyelia and spinal obstruction-related syringomyelia. Before surgery, more subarachnoid contrast entered syringes associated with CSF pathway obstruction than with tumor, consistent with syrinx fluid originating from the subarachnoid space in Chiari I malformation and spinal obstruction-related syringomyelia and not from the subarachnoid space in tumor-related syringomyelia. Decompressive surgery opened subarachnoid CSF pathways and reduced contrast entry into syringes associated with CSF pathway obstruction.

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Shunt age-related complications in adult patients with suspected shunt dysfunction

Acta Neurochir (2017) 159:1421–1428

Patients admitted for suspicion of shunt dysfunction (SD) often show unspecific symptoms and require timeconsuming, expensive and even invasive diagnostics involving significant radiation exposure. The purpose of this retrospective study was to analyse the current diagnostic procedures and to propose a process optimisation.

Method: As all patients admitted for suspicion of SD receive imaging studies, we searched for adult patients receiving neuroimaging in the period from January 2010 to July 2013, analysing referring diagnosis, clinical signs, products, diagnostic process and final diagnosis. Recursive partitioning was used to define time intervals for differentiating types of SD.

Results A total of 148 patients, aged 18–89 (mean, 54) years, were studied. Forty-two percent were referred by a hospital or rehabilitation centre, 30% by general practitioners and 24% were self-referrals. The admission diagnosis was in the majority “shunt dysfunction” only. Further differentiations were rarely made. An SD was confirmed in 46% of the patients. In 17%, the symptoms were based on another cause and in 37% they could not be clearly attributed to any specific disorder. Abdominal dislocations (2%) and shunt infections (5%) were found within the first 6 months. Over- (3%) and underdrainage (14%) were the most frequent complications during the first 4 years. Disconnections (13%) occurred generally 4 years or more after implantation. Only shunt obstruction (9%) showed no temporal pattern.

Conclusions: Symptoms of SD remain mostly unspecific. This study showed that the type of SD depends on the time interval from implantation.We propose a workup strategy for patients with SD based on the temporal profile.

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Imaging of Intracranial Pressure Disorders

Neurosurgery 80:341–354, 2017

Intracranial pressure (ICP) is the pressure inside the bony calvarium and can be affected by a variety of processes, such as intracranial masses and edema, obstruction or leakage of cerebrospinal uid, and obstruction of venous out flow. This review focuses on the imaging of 2 important but less well understood ICP disorders: idiopathic intracranial hypertension and spontaneous intracranial hypotension. Both of these ICP disorders have salient imaging findings that are important to recognize to help prevent their misdiagnosis from other common neurological disorders.

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An update on idiopathic intracranial hypertension

Acta Neurochir (2017) 159:491–499

Idiopathic intracranial hypertension (IIH) is still a grey area in the knowledge of the aetiology, diagnosis and management of neurosurgical diseases. The definition of IIH has been reviewed over time and many hypotheses have been expressed as cause of the disease. The literature supplies very little evidence-based information to guide our decision-making process when it comes to treat the disease. In this review we sum up the latest information regarding the aetiology and therapy of IIH. Additionally, we make an attempt to unify the diagnostic criteria of Dandy, Friedman and Wall established from 1937 to date.

Method

In this narrative review, we attempt to update the current standpoint to IIH, evaluate the input until now and consider future directions for research. The vast majority of the literature consisted of cohort studies, case control studies, systematic reviews and other narrative reviews.

Results

Pathophysiology: The incidence of IIH is steadily in- creasing. Several pathophysiological theories have been pro- posed in the literature based on the flow of cerebrospinal fluid.

Diagnosis: We attempt to fuse all the three diagnostic ap- proaches published in the literature that detect IIH, while pre- serving the individual characteristics of each approach.

Treatment:

Based on evidence-based trials, the current use of acetazolamide in comparison with placebo or with topiramate has been evaluated. In the interventions’ field, there seems to be a consonance about the alarming symptoms and what the most suitable operation is in each case. There is some disagreement about the indications for venous sinus transversus stenting and its risk/benefit ratio.

Conclusions

Until now there is no class I guideline to which our decision-making can be based on for the management of IIH. A lack of systematic reviews and randomised control trials has been noted. If we focused our research on that, we could develop a standardised treatment protocol.

 

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