Characteristics of shunt failure in 38,095 adult shunt insertion surgeries: a systematic review and meta-analysis.

OBJECTIVE: The management of excess CSF in patients with hydrocephalus typically requires using a shunt to divert CSF. Unfortunately, there is a high rate of shunt failure despite improvements in device components and insertion techniques. Reoperation is frequently necessary, which contributes to patient harm and increased healthcare costs. While factors affecting shunt failure are well defined in the pediatric population, information regarding adults is lacking. The authors undertook a systematic review and meta-analysis to determine how shunt failure in the adult population is reported and investigated the etiologies of shunt failure. METHODS: This review is reported according to PRIMSA and utilized the MEDLINE, Embase, and Google Scholar databases. Abstracts were screened by two independent reviewers, and data were extracted in duplicate by two independent reviewers. Statistical analyses were performed using SPSS and Stata. RESULTS: The pooled rates of shunt failure were 10% (95% CI 5%-15%) in studies with a mean follow-up time of less than 1 year, 12% (95% CI 8%-14%) with a follow-up time between 1 and 2 years, and 32% in studies with a follow-up time of 2 years or greater (95% CI 19%-43%). The pooled rate of failure was 17% across all studies. The most common cause of shunt failure was obstruction at 3.0% (95% CI 2%-4%), accounting for 23.2% of shunt failures. Infection was the second most common at 2.8% (95% CI 2%-3%), accounting for 22.5% of shunt failures. The most common location of shunt failure was the distal catheter, with a failure rate of 4.0% (95% CI 3%-5%), accounting for 33.4% of shunt failures. The definition of shunt failure was heterogeneous and varied depending on institutional practices. The combination of symptoms with either CT or MRI was the most frequently reported method for assessing shunt failure. CONCLUSIONS: Important variation regarding how to define, investigate, and report shunt failure was identified. The overall shunt failure rate in adults is at least 32% after 2 years, which, while lower than that typically reported in the pediatric population, is significant. The most common causes of shunt failure in adults are infection and obstruction. The most common site of failure occurred at the distal catheter, highlighting the need to develop strategies to both report and mitigate distal shunt failure in adult shunt patients.

How to define CSF overdrainage: a systematic literature review.

PURPOSE: Overdrainage (OD) is one of the most frequent complications related to drainage of the cerebrospinal fluid (CSF). It is mostly associated with valve-bearing shunt systems but should probably be considered as a risk factor in any type of CSF diversion procedure. There is extreme variation in the reported incidence of OD due to the lack of consensus on defining criteria and an unclear perception of the pathophysiology. Hence, OD is probably underreported and underestimated. The objective of this paper was to establish a definition of OD, based on a systematic review of the literature. METHODS: A systematic search was conducted in MEDLNE and EMBASE. Studies providing a definition or a description of diagnostic findings related to OD in ventriculoperitoneal shunt treated hydrocephalus were included. Non-English titles, abstracts and manuscripts were excluded. Extracted descriptions were graded into five groups (class I-V studies) based on how precise the terminology used to describe OD was. Class I studies were included for further analysis and characteristics of OD were extracted. The quality of included descriptions was assessed by a clinical expert panel. RESULTS: A total of 1309 studies were screened, 190 were graded into groups, and 22, which provided specific definitions or descriptions of OD, were graded as class I studies. We extracted 32 different characteristics consistent with OD (e.g., clinical symptoms, radiological signs, and syndromes). CONCLUSION: There was an overall agreement that CSF overdrainage following implantation of a ventriculoperitoneal shunt in a mixed pediatric and adult population is characterized as a persistent condition with clinically manifestations as postural dependent headache, nausea, and vomiting and/or radiological signs of slim ventricles and/or subdural collections.

Extended lumbar drainage in idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis of diagnostic test accuracy.

BACKGROUND: When appropriately selected, a high proportion of patients with suspected idiopathic normal pressure hydrocephalus (iNPH) will respond to cerebrospinal fluid diversion with a shunt. Extended lumbar drainage (ELD) is regarded as the most accurate test for this condition, however, varying estimates of its accuracy are found in the current literature. Here, we review the literature in order to provide summary estimates of sensitivity, specificity, positive- and negative predictive value for this test through meta-analysis of suitably rigorous studies. METHODS: Studies involving a population of NPH patients with predominantly idiopathic aetiology (>80%) in which the intention of the study was to shunt patients regardless of the outcome of ELD were included in the review. Various literature databases were searched to identify diagnostic test accuracy studies addressing ELD in the diagnosis of iNPH. Those studies passing screening and eligibility were assessed using the QUADAS-2 tool and data extracted for bivariate random effects meta-analysis. RESULTS: Four small studies were identified. They showed disparate results concerning diagnostic test accuracy. The summary estimates for sensitivity and specificity were 94% (CI 41-100%) and 85% (CI 33-100%), respectively. The summary estimates of positive and negative predictive value were both 90% (CIs 65-100% and 48-100%, respectively). CONCLUSION: Large, rigorous studies addressing the diagnostic accuracy of ELD are lacking, and little robust evidence exists to support the use of ELD in diagnostic algorithms for iNPH. Therefore, a large cohort study, or ideally an RCT, is needed to determine best practice in selecting patients for shunt surgery.

Rindopepimut with temozolomide for patients with newly diagnosed, EGFRvIII-expressing glioblastoma (ACT IV): a randomised, double-blind, international phase 3 trial.

BACKGROUND: Rindopepimut (also known as CDX-110), a vaccine targeting the EGFR deletion mutation EGFRvIII, consists of an EGFRvIII-specific peptide conjugated to keyhole limpet haemocyanin. In the ACT IV study, we aimed to assess whether or not the addition of rindopepimut to standard chemotherapy is able to improve survival in patients with EGFRvIII-positive glioblastoma. METHODS: In this randomised, double-blind, phase 3 trial, we recruited patients aged 18 years and older with glioblastoma from 165 hospitals in 22 countries. Eligible patients had newly diagnosed glioblastoma confirmed to express EGFRvIII by central analysis, and had undergone maximal surgical resection and completion of standard chemoradiation without progression. Patients were stratified by European Organisation for Research and Treatment of Cancer recursive partitioning analysis class, MGMT promoter methylation, and geographical region, and randomly assigned (1:1) with a prespecified randomisation sequence (block size of four) to receive rindopepimut (500 µg admixed with 150 µg GM-CSF) or control (100 µg keyhole limpet haemocyanin) via monthly intradermal injection until progression or intolerance, concurrent with standard oral temozolomide (150-200 mg/m2 for 5 of 28 days) for 6-12 cycles or longer. Patients, investigators, and the trial funder were masked to treatment allocation. The primary endpoint was overall survival in patients with minimal residual disease (MRD; enhancing tumour <2 cm2 post-chemoradiation by central review), analysed by modified intention to treat. This trial is registered with ClinicalTrials.gov, number NCT01480479. FINDINGS: Between April 12, 2012, and Dec 15, 2014, 745 patients were enrolled (405 with MRD, 338 with significant residual disease [SRD], and two unevaluable) and randomly assigned to rindopepimut and temozolomide (n=371) or control and temozolomide (n=374). The study was terminated for futility after a preplanned interim analysis. At final analysis, there was no significant difference in overall survival for patients with MRD: median overall survival was 20·1 months (95% CI 18·5-22·1) in the rindopepimut group versus 20·0 months (18·1-21·9) in the control group (HR 1·01, 95% CI 0·79-1·30; p=0·93). The most common grade 3-4 adverse events for all 369 treated patients in the rindopepimut group versus 372 treated patients in the control group were: thrombocytopenia (32 [9%] vs 23 [6%]), fatigue (six [2%] vs 19 [5%]), brain oedema (eight [2%] vs 11 [3%]), seizure (nine [2%] vs eight [2%]), and headache (six [2%] vs ten [3%]). Serious adverse events included seizure (18 [5%] vs 22 [6%]) and brain oedema (seven [2%] vs 12 [3%]). 16 deaths in the study were caused by adverse events (nine [4%] in the rindopepimut group and seven [3%] in the control group), of which one-a pulmonary embolism in a 64-year-old male patient after 11 months of treatment-was assessed as potentially related to rindopepimut. INTERPRETATION: Rindopepimut did not increase survival in patients with newly diagnosed glioblastoma. Combination approaches potentially including rindopepimut might be required to show efficacy of immunotherapy in glioblastoma. FUNDING: Celldex Therapeutics, Inc.

Endoscopic third ventriculostomy for treatment of adult hydrocephalus: long-term follow-up of 163 patients.

OBJECTIVE The efficacy of endoscopic third ventriculostomy (ETV) for the treatment of pediatric hydrocephalus has been extensively reported in the literature. However, ETV-related long-term outcome data are lacking for the adult hydrocephalus population. The objective of the present study was to assess the role of ETV as a primary or secondary treatment for hydrocephalus in adults. METHODS The authors performed a retrospective chart review of all adult patients (age ≥ 18 years) with symptomatic hydrocephalus treated with ETV in Calgary, Canada, over a span of 20 years (1994-2014). Patients were dichotomized into a primary or secondary ETV cohort based on whether ETV was the initial treatment modality for the hydrocephalus or if other CSF diversion procedures had been previously attempted respectively. Primary outcomes were subjective patient-reported clinical improvement within 12 weeks of surgery and the need for any CSF diversion procedures after the initial ETV during the span of the study. Categorical and actuarial data analysis was done to compare the outcomes of the primary versus secondary ETV cohorts. RESULTS A total of 163 adult patients with symptomatic hydrocephalus treated with ETV were identified and followed over an average of 98.6 months (range 0.1-230.4 months). All patients presented with signs of intracranial hypertension or other neurological symptoms. The primary ETV group consisted of 112 patients, and the secondary ETV consisted of 51 patients who presented with failed ventriculoperitoneal (VP) shunts. After the initial ETV procedure, clinical improvement was reported more frequently by patients in the primary cohort (87%) relative to those in the secondary ETV cohort (65%, p = 0.001). Additionally, patients in the primary ETV group required fewer reoperations (p < 0.001), with cumulative ETV survival time favoring this primary ETV cohort over the course of the follow-up period (p < 0.001). Fifteen patients required repeat ETV, with all but one experiencing successful relief of symptoms. Patients in the secondary ETV cohort also had a higher incidence of complications, with one occurring in 8 patients (16%) compared with 2 in the primary ETV group (2%; p = 0.010), although most complications were minor. CONCLUSIONS ETV is an effective long-term treatment for selected adult patients with hydrocephalus. The overall ETV success rate when it was the primary treatment modality for adult hydrocephalus was approximately 87%, and 99% of patients experience symptomatic improvement after 2 ETVs. Patients in whom VP shunt surgery fails prior to an ETV have a 22% relative risk of ETV failure and an almost eightfold complication rate, although mostly minor, when compared with patients who undergo a primary ETV. Most ETV failures occur within the first 7 months of surgery in patients treated with primary ETV, but the time to failure is more prolonged in patients who present with failed previous shunts.

Population based analysis ependymoma patients in Alberta from 1975 to 2007.

BACKGROUND: Ependymomas are rare tumors of the central nervous system whose management is controversial. This population-based study of adults and children with ependymoma aims to (1) identify clinical and treatment-related factors that impact survival and (2) determine if postoperative radiotherapy (RT) can improve survival of patients with subtotal resection (STR) to levels similar to patients who had gross total resection (GTR). METHODS: This retrospective population-based study evaluated 158 patients with ependymoma diagnosed between 1975-2007 in Alberta, Canada. RESULTS: Younger patients (<7 years of age) were more likely to be diagnosed with grade III tumors compared with adults in whom grade I tumors were more common (p=0.003). Adults were more likely to have spinally located tumors compared to young children whose tumors were typically found in the brain. Overall, young children with ependymoma were more likely to die than older children or adults (p=0.001). An equivalent number of patients underwent GTR as compared with STR (48% vs 45%, respectively). Overall, older age, spinal tumor location, lower grade, and GTR were associated with improved progression free survival but only GTR was associated with significant improvement in overall survival. Median survival after STR and RT was 82 months compared with 122 months in patients who had GTR (p=0.0022). CONCLUSIONS: This is the first Canadian population-based analysis of patients with ependymoma including adults and children. Extent of resection appears to be the most important factor determining overall survival. Importantly, the addition of RT to patients initially treated with STR does not improve survival to levels similar to patients receiving GTR.

A year in review: brain barriers and brain fluids research in 2022.

This aim of this editorial is to highlight progress made in brain barrier and brain fluid research in 2022. It covers studies on the blood-brain, blood-retina and blood-CSF barriers (choroid plexus and meninges), signaling within the neurovascular unit and elements of the brain fluid systems. It further discusses how brain barriers and brain fluid systems are impacted in CNS diseases, their role in disease progression and progress being made in treating such diseases.

A standardized infection prevention bundle for reduction of CSF shunt infections in adult ventriculoperitoneal shunt surgery performed without antibiotic-impregnated catheters.

OBJECTIVE: Ventriculoperitoneal (VP) shunt insertion and revision surgeries are some of the most common procedures that are performed by neurosurgeons. Shunt infections within the adult population are associated with significant morbidity and mortality and rates remain high. The objective of the current study was to use quality improvement (QI) methodology to create a standardized infection prevention bundle aimed at reducing the rate of shunt infections. METHODS: A prospective, single-center, single-surgeon QI study was undertaken. Patients were included if they were 18 years of age or older and were undergoing a VP shunt insertion or revision. The primary outcome of the study was the development of a shunt-related surgical site infection, within 1 year of surgery, as defined according to the Canadian Nosocomial Infection Surveillance Program guidelines. There was no standardized protocol prior to July 2013. A bundle coined as the Calgary Adult Shunt Infection Prevention Protocol (CASIPP) was implemented on July 1, 2013, and updated on July 1, 2015, when 2% chlorhexidine gluconate in 70% isopropyl alcohol replaced povidone-iodine for preoperative skin antisepsis. Protocol compliance was regularly monitored using a standardized process. No antibiotic-impregnated catheters were used. RESULTS: A total of 621 consecutive VP shunt insertions and revisions were included in the study. The rate of shunt infection was 5.8% during the period in which there was no standardized shunt protocol. After the implementation of the CASIPP the infection rate decreased to 4.0%, and after introduction of the chlorhexidine/alcohol skin antisepsis, the infection rate was 0% in 379 consecutive procedures (p < 0.0001). Multivariable logistic regression analysis demonstrated that the use of chlorhexidine/alcohol with CASIPP was associated with a significant reduction in the odds of developing a shunt infection (OR 0.032, 95% CI 0-0.19, p = 0.0005). CONCLUSIONS: The implementation of a standardized shunt infection prevention bundle within the adult population, without the use of antibiotic-impregnated catheters, significantly reduced the rate of shunt infections which was sustained over many years. The use of 2% chlorhexidine gluconate in 70% isopropyl alcohol for preoperative antisepsis may have played a significant role. Multicenter studies should be completed to verify the effectiveness of the authors' protocol.

Placebo-Controlled Effectiveness of Idiopathic Normal Pressure Hydrocephalus Shunting: A Randomized Pilot Trial.

BACKGROUND: Multiple prospective nonrandomized studies have shown 60% to 70% of patients with idiopathic normal pressure hydrocephalus (iNPH) improve with shunt surgery, but multicenter placebo-controlled trial data are necessary to determine its effectiveness. OBJECTIVE: To evaluate the effectiveness of cerebrospinal fluid shunting in iNPH through comparison of open vs placebo shunting groups at 4 months using a pilot study. METHODS: Patients were randomized to a Codman Certas Plus valve (Integra LifeSciences) set at 4 (open shunt group) or 8 ("virtual off"; placebo group). Patients and assessors were blinded to treatment group. The primary outcome measure was 10-m gait velocity. Secondary outcome measures included functional scales for bladder control, activities of daily living, depression, and quality of life. Immediately after 4-month evaluation, all shunts were adjusted in a blinded fashion to an active setting and followed to 12 months after shunting. RESULTS: A total of 18 patients were randomized. At the 4-month evaluation, gait velocity increased by 0.28 ± 0.28 m/s in the open shunt group vs 0.04 ± 0.17 m/s in the placebo group. The estimated treatment difference was 0.22 m/s ([ P = .071], 95% CI -0.02 to 0.46). Overactive Bladder Short Form symptom bother questionnaire significantly improved in open shunt vs placebo ( P = .007). The 4-month treatment delay did not reduce the subsequent response to active shunting, nor did it increase the adverse advents rate at 12 months. CONCLUSION: This multicenter, randomized pilot study demonstrates the effectiveness, safety, and feasibility of a placebo-controlled trial in iNPH, and found a trend suggesting gait velocity improves more in the open shunt group than in the placebo group.

Use of cortical volume to predict response to temporary CSF drainage in patients with idiopathic normal pressure hydrocephalus.

OBJECTIVE: Temporary drainage of CSF with lumbar puncture or lumbar drainage has a high predictive value for identifying patients with suspected idiopathic normal pressure hydrocephalus (iNPH) who may benefit from ventriculoperitoneal shunt insertion. However, it is unclear what differentiates responders from nonresponders. The authors hypothesized that nonresponders to temporary CSF drainage would have patterns of reduced regional gray matter volume (GMV) as compared with those of responders. The objective of the current investigation was to compare regional GMV between temporary CSF drainage responders and nonresponders. Machine learning using extracted GMV was then used to predict outcomes. METHODS: This retrospective cohort study included 132 patients with iNPH who underwent temporary CSF drainage and structural MRI. Demographic and clinical variables were examined between groups. Voxel-based morphometry was used to calculate GMV across the brain. Group differences in regional GMV were assessed and correlated with change in results on the Montreal Cognitive Assessment (MoCA) and gait velocity. A support vector machine (SVM) model that used extracted GMV values and was validated with leave-one-out cross-validation was used to predict clinical outcome. RESULTS: There were 87 responders and 45 nonresponders. There were no group differences in terms of age, sex, baseline MoCA score, Evans index, presence of disproportionately enlarged subarachnoid space hydrocephalus, baseline total CSF volume, or baseline white matter T2-weighted hyperintensity volume (p > 0.05). Nonresponders demonstrated decreased GMV in the right supplementary motor area (SMA) and right posterior parietal cortex as compared with responders (p < 0.001, p < 0.05 with false discovery rate cluster correction). GMV in the posterior parietal cortex was associated with change in MoCA (r2 = 0.075, p < 0.05) and gait velocity (r2 = 0.076, p < 0.05). Response status was classified by the SVM with 75.8% accuracy. CONCLUSIONS: Decreased GMV in the SMA and posterior parietal cortex may help identify patients with iNPH who are unlikely to benefit from temporary CSF drainage. These patients may have limited capacity for recovery due to atrophy in these regions that are known to be important for motor and cognitive integration. This study represents an important step toward improving patient selection and predicting clinical outcomes in the treatment of iNPH.

Syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH).

INTRODUCTION: Most patients with acute hydrocephalus have ventriculomegaly and high intracranial pressure (ICP). However, there is a subset of patients who are symptomatic with acute ventriculomegaly and inappropriately low ICP. METHODS: Two patient groups were defined. Each patient presented with clinical deterioration that included a significant decrease in level of consciousness with new and significant ventriculomegaly. Patients in group 1 (n = 10) were managed without endoscopic third ventriculostomy (ETV). Group 2 was a series of patients (n = 10) managed with ETV. RESULTS: Treatment for both groups involved insertion of an external ventricular drain (EVD) with ICP <5 cmH(2)O. Further treatment consisted of either neck wrapping with a tensor bandage and/or lowering the EVD to negative levels to facilitate drainage of cerebrospinal fluid (CSF), which resulted in clinical improvement and resolution of ventriculomegaly. All 20 patients had anatomical obstruction to CSF flow into the subarachnoid space (SAS) confirmed by magnetic resonance imaging (MRI) with cine MRI studies. Group 1 patients were treated until shunt revision/insertion was possible (n = 7), ICP normalized, and the EVD could be removed (n = 2), or death (n = 1) occurred. Patients in group 2 all underwent ETV, and ICP patterns normalized in all. Group 2 patients were managed with an EVD until shunt revision/insertion was required (n = 2), ICP normalized and the EVD could be removed (n = 7), or death (n = 1) occurred. DISCUSSION/CONCLUSIONS: The syndrome of inappropriately low-pressure acute hydrocephalus (SILPAH) is an important entity in both children and adults. A possible hypothesis invokes loss of an effective SAS. ETV reestablishes communication between the SAS and ventricles, producing a rapid return of normal ICP dynamics and a significant decrease in the number of shunt-dependent patients.

Neuronavigation and Laparoscopy Guided Ventriculoperitoneal Shunt Insertion for the Treatment of Hydrocephalus.

Hydrocephalus is a common adult neurosurgical condition typically requiring treatment with a cerebrospinal fluid (CSF) shunt, of which the ventriculoperitoneal (VP) shunt is the most common type. Unfortunately, the failure rates of VP shunts are alarmingly high, with up to 50% of patients requiring revision surgery within 2 years. VP shunt failure may occur due to infection, or catheter mispositioning, migration, and occlusion. We undertook a joint neurosurgery and general surgery collaboration in a 7-year prospective non-randomized consecutive quality improvement cohort study to reduce the rates of ventriculoperitoneal (VP) shunt failures in 224 adult patients at a tertiary care institution. The initiative combined the use of electromagnetic stereotactic neuronavigation to guide the placement of the proximal catheter and laparoscopy to place the distal catheter under direct visualization. With laparoscopic assistance, the distal catheter was anchored through a small hole created in the falciform ligament and placed into the right retrohepatic space, free from the omentum, adhesions, or bowel that might obstruct the catheter tip. The surgeries were performed using a shunt infection prevention protocol to reduce the risk of shunt infections. Here, we present an intraoperative video of the surgical procedure. Compliance with shunt infection reduction strategies and the combined utilization of neuronavigation and laparoscopy techniques in adult VP shunt surgery resulted in a 44% reduction in the risk of overall shunt failure. The significant positive impact with regard to shunt-failure-free patient outcomes among patients who underwent VP shunt surgery using this strategy underscores the value associated with the use of these modern intraoperative techniques and cross-specialty collaboration during VP shunt surgery.

Reducing the risks of proximal and distal shunt failure in adult hydrocephalus: a shunt outcomes quality improvement study.

OBJECTIVE: Patient outcomes of ventriculoperitoneal (VP) shunt surgery, the mainstay treatment for hydrocephalus in adults, are poor because of high shunt failure rates. The use of neuronavigation or laparoscopy can reduce the risks of proximal or distal shunt catheter failure, respectively, but has less independent effect on overall shunt failures. No adult studies to date have combined both approaches in the setting of a shunt infection prevention protocol to reduce shunt failure. The goal of this study was to determine whether combining neuronavigation and laparoscopy with a shunt infection prevention strategy would reduce the incidence of shunt failures in adult hydrocephalic patients. METHODS: Adult patients (age ≥ 18 years) undergoing VP shunt surgery at a tertiary care institution prior to (pre-Shunt Outcomes [ShOut]) and after (post-ShOut) the start of a prospective continuous quality improvement (QI) study were compared. Pre-ShOut patients had their proximal and distal catheters placed under conventional freehand approaches. Post-ShOut patients had their shunts inserted with neuronavigational and laparoscopy assistance in placing the distal catheter in the perihepatic space (falciform technique). A shunt infection reduction protocol had been instituted 1.5 years prior to the start of the QI initiative. The primary outcome of interest was the incidence of shunt failure (including infection) confirmed by standardized criteria indicating shunt revision surgery. RESULTS: There were 244 (115 pre-ShOut and 129 post-ShOut) patients observed over 7 years. With a background of shunt infection prophylaxis, combined neuronavigation and laparoscopy was associated with a reduction in overall shunt failure rates from 37% to 14%, 45% to 22%, and 51% to 29% at 1, 2, and 3 years, respectively (HR 0.44, p < 0.001). Shunt infection rates decreased from 8% in the pre-ShOut group to 0% in the post-ShOut group. There were no proximal catheter failures in the post-ShOut group. The 2-year rates of distal catheter failure were 42% versus 20% in the pre- and post-ShOut groups, respectively (p < 0.001). CONCLUSIONS: Introducing a shunt infection prevention protocol, placing the proximal catheter under neuronavigation, and placing the peritoneal catheter in the perihepatic space by using the falciform technique led to decreased rates of infection, distal shunt failure, and overall shunt failure.

Cognitive and gait outcomes after primary endoscopic third ventriculostomy in adults with chronic obstructive hydrocephalus.

OBJECTIVE: The object of this study was to determine the short- and long-term efficacy of primary endoscopic third ventriculostomy (ETV) on cognition and gait in adults with chronic obstructive hydrocephalus. METHODS: Patients were prospectively accrued through the Adult Hydrocephalus Clinical Research Network patient registry. Patients with previously untreated congenital or acquired obstructive hydrocephalus were included in this study. Gait velocity was assessed using a 10-m walk test. Global cognition was assessed with the Montreal Cognitive Assessment (MoCA). Only patients with documented pre- and post-ETV gait analysis and/or pre- and post-ETV MoCA were included. RESULTS: A total of 74 patients had undergone primary ETV, 42 of whom were analyzed. The remaining 32 patients were excluded, as they could not complete both pre- and post-ETV assessments. The mean age of the 42 patients, 19 (45.2%) of whom were female, was 51.9 ± 17.1 years (range 19-79 years). Most patients were White (37 [88.1%]), and the remainder were Asian. Surgical complications were minor. Congenital etiologies occurred in 31 patients (73.8%), with aqueductal stenosis in 23 of those patients (54.8%). The remaining 11 patients (26.2%) had acquired cases. The gait short-term follow-up cohort (mean 4.7 ± 4.1 months, 35 patients) had a baseline median gait velocity of 0.9 m/sec (IQR 0.7-1.3 m/sec) and a post-ETV median velocity of 1.3 m/sec (IQR 1.1-1.4 m/sec). Gait velocity significantly improved post-ETV with a median within-patient change of 0.3 m/sec (IQR 0.0-0.6 m/sec, p < 0.001). Gait velocity improvements were sustained in the long term (mean 14 ± 2.8 months, 12 patients) with a baseline median velocity of 0.7 m/sec (IQR 0.6-1.3 m/sec), post-ETV median of 1.3 m/sec (IQR 1.1-1.7 m/sec), and median within-patient change of 0.4 m/sec (IQR 0.2-0.6 m/sec, p < 0.001). The cognitive short-term follow-up cohort (mean 4.6 ± 4.0 months, 38 patients) had a baseline median MoCA total score (MoCA TS) of 24/30 (IQR 23-27) that improved to 26/30 (IQR 24-28) post-ETV. The median within-patient change was +1 point (IQR 0-2 points, p < 0.001). However, this change is not clinically significant. The cognitive long-term follow-up cohort (mean 14 ± 3.1 months, 15 patients) had a baseline median MoCA TS of 23/30 (IQR 22-27), which improved to 26/30 (IQR 25-28) post-ETV. The median within-patient change was +2 points (IQR 1-3 points, p = 0.007), which is both statistically and clinically significant. CONCLUSIONS: Primary ETV can safely improve symptoms of gait and cognitive dysfunction in adults with chronic obstructive hydrocephalus. Gait velocity and global cognition were significantly improved, and the worsening of either was rare following ETV.

Establishing ranked priorities for future hydrocephalus research.

OBJECTIVE: The aim of this initiative was to develop a ranked list of hydrocephalus research priorities as determined by the hydrocephalus patient community in conjunction with the healthcare and scientific community. METHODS: Using the validated methodology published by the James Lind Alliance (JLA), the Hydrocephalus Association (HA) administered two surveys and hosted a final prioritization workshop. Survey One solicited open-ended responses from the community. From these responses, a long list of priority statements was developed. This list was then consolidated into a short list of research priority statements, which, after a nonsystematic literature review, were verified as being research uncertainties. Survey Two asked the community members to select their top 10 priorities from the short list. The final prioritization leading to a final ranked top 20 list of hydrocephalus research priorities took place at a virtual workshop led by a team of trained facilitators, by means of an iterative process of consensus building. RESULTS: From Survey One, 3703 responses from 890 respondents were collected, leading to a long list of 146 priority statements. The consolidated short list contained 49 research priority statements, all of which were verified as uncertainties in hydrocephalus research. From an analysis of Survey Two responses, the top 21 research priority statements were determined. A consensus on these statements was reached at the virtual workshop, leading to a final ranked top 20 list of hydrocephalus research priorities, within which needs were apparent in several areas: development of noninvasive and/or one-time therapies, reduction of the burden of current treatments, improvement of the screening and diagnosis of hydrocephalus, improved quality of life, and improved access to care. CONCLUSIONS: By gathering extensive input from the hydrocephalus community and using an iterative process of consensus building, a ranked list of the top 20 hydrocephalus research priorities was developed. The HA will use this ranked list to guide future research programs and encourages the healthcare and scientific community to do the same.