Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

OBJECTIVE: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT. METHODS: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF). RESULTS: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort. CONCLUSIONS: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion.

Insights into the epidemiology of infant hydrocephalus.

INTRODUCTION: Infant hydrocephalus represents an important public health issue. Recent analysis of registry-based data has improved our understanding of the variable epidemiology of infant hydrocephalus around the world and the consequent burden of disease that this vulnerable population must carry throughout their lifetimes. The purpose of this article is to review the epidemiology of infant hydrocephalus, highlighting the ways in which analysis of prospectively collected registry data has contributed to our current knowledge and how similar methods may lead to new discovery. DISCUSSION: Congenital abnormalities and spina bifida-associated hydrocephalus, along with acquired postnatal hydrocephalus secondary to intraventricular hemorrhage of prematurity and infection, represent the most common etiologies of infant hydrocephalus, with their relative prevalence dependent on geographic region and socioeconomic status. Best current estimates suggest that the incidence of congenital and acquired infant hydrocephalus may be between 80 and 125 cases/100,000 births depending on world region. These incidence figures and their forecasts, together with improved survival associated with promptly diagnosed and treated hydrocephalus, suggest that the burden of hydrocephalus, as measured by prevalence, is primed to increase. Counterbalancing these statistics is evidence that perhaps, in some regions, improvements in neurosurgical and general perinatal care, as well as shifting indications for initial surgical intervention in these infants (and therefore in the very definition of hydrocephalus itself), the number of infants who require first time surgical treatment for hydrocephalus, may be decreasing. Further longitudinal data collection will undoubtedly assist in determining whether these trends are robust. CONCLUSION: When one takes a global perspective, complexities related to the underlying epidemiology of infant hydrocephalus become abundantly clear. The causes of infant hydrocephalus vary from one world region to another, largely related to the underlying income characteristics of the population. Likewise, increased birth rates in low-income areas of the world are likely to result in an increased incidence and prevalence of infant hydrocephalus in those regions, whereas sophisticated and resource-intensive advancements in perinatal care available in other regions may result in decreased epidemiological estimates of disease burden in others. Further analysis of high-quality registry-based data may help clarify these issues.

Long-term upper extremity performance in children with cerebral palsy following selective dorsal rhizotomy.

PURPOSE: In children with spastic cerebral palsy, selective dorsal rhizotomy (SDR) is conducted to improve lower limb spasticity. Improvements in upper extremity function have also been noted in early follow-up. The purpose of this study was to determine if upper extremity improvements are sustained in the long term. METHODS: A retrospective review of prospectively collected data on children who underwent SDR was conducted. Quality of Upper Extremities Skill Test (QUEST) scores for dissociated movement, grasp and total scores were compared using repeated measures ANOVA for individual patients at three time points: preoperatively, early post-operatively (≤ 2 years) and late post-operatively (9+ years). RESULTS: Out of 200+ patients having SDR, 32 had QUEST assessment at all three time points. Significant improvements in QUEST dissociated movement (F = 3.665, p = 0.045), grasp (F = 7.995, p = 0.001) and total scores (F = 9.471, p = 0.001) were found. Pairwise comparisons were significant from pre-operative to early post-operative times for all QUEST scores (p = 0.001, 0.003, 0.001), and this was maintained at late post-operative assessment for grasp and total scores (p = 0.02, p = 0.02). There was no significant change in scores between early and late post-operative assessment time points. CONCLUSION: Early improvements in upper extremity QUEST total scores are sustained in the long term following SDR.

Hydrocephalus treatment in patients with craniosynostosis: an analysis from the Hydrocephalus Clinical Research Network prospective registry.

OBJECTIVE: Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis. METHODS: Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated. RESULTS: In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC). CONCLUSIONS: This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Tremor and Quality of Life in Patients With Advanced Essential Tremor Before and After Replacing Their Standard Deep Brain Stimulation With a Directional System.

OBJECTIVES: Patients with essential tremor treated with thalamic deep brain stimulation may experience increased tremor with the progression of their disease. Initially, this can be counteracted with increased stimulation. Eventually, this may cause unwanted side-effects as the circumferential stimulation from a standard ring contact spreads into adjacent regions. Directional leads may offer a solution to this clinical problem. We aimed to compare the ability of a standard and a directional system to reduce tremor without side-effects and to improve the quality of life for patients with advanced essential tremor. MATERIALS AND METHODS: Six advanced essential tremor patients with bilateral thalamic deep brain stimulation had their standard system replaced with a directional system. Tremor rating scale scores were prospectively evaluated before and after the replacement surgery. Secondary analyses of quality of life related to tremor, voice, and general health were assessed. RESULTS: There was a significantly greater reduction in tremor without side-effects (p = 0.017) when using the directional system. There were improvements in tremor (p = 0.031) and voice (p = 0.037) related quality of life but not in general health for patients using optimized stimulation settings with the directional system compared to the standard system. CONCLUSIONS: In this cohort of advanced essential tremor patients who no longer had ideal tremor reduction with a standard system, replacing their deep brain stimulation with a directional system significantly improved their tremor and quality of life. Up-front implantation of directional deep brain stimulation leads may provide better tremor control in those patients who progress at a later time point.

Contrast-enhanced 3-dimensional Fluid-attenuated Inversion Recovery Sequences Have Greater Sensitivity for Detection of Leptomeningeal Metastases in Pediatric Brain Tumors Compared With Conventional Spoiled Gradient Echo Sequences.

Postcontrast 3-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequences have reduced vascular and flow-related artifacts and high sensitivity to low gadolinium concentrations. We compared the performance of postcontrast spoiled gradient echo images to 3D-FLAIR in the detection of leptomeningeal metastases in 47 pediatric patients with brain tumors. We found 10 cases with more leptomeningeal signal abnormalities on 3D-FLAIR. Overall there were significantly more lesions on 3D-FLAIR than spoiled gradient echo sequences. We believe the increased detection of leptomeningeal signal abnormality is due to increased sensitivity for low concentrations of gadolinium in regions of early blood brain barrier breakdown. Our study was limited by the lack of leptomeningeal metastases in cerebrospinal fluid sampling. We plan to conduct future studies which will determine whether the time-based concentration of gadolinium affects the performance and results. Future studies will also require more cases of pathology-proven leptomeningeal disease.

Canadian Assessment of Deep Brain Stimulation Access: The Canada Study.

BACKGROUND: The Canada Health Act requires reasonable access to all medically necessary therapies. No information is available to assess the current access to neuromodulation across Canada. This study quantifies the current rate of deep brain stimulation (DBS) for the entire country of Canada. Analyses were performed to determine whether there were differences in access based on provincial or territorial location, rural or non-rural region, or socioeconomic status. METHODS: All implanted DBS devices in Canada over a 2-year epoch (January 2015 to December 2016) were supplied by either Boston Scientific or Medtronic. Investigators received anonymized data from these companies, including patient age and home residence region. The 2016 Statistics Canada census data were used to determine the rate of DBS surgery and whether access was related to provincial location, rural versus non-rural region or socioeconomic status. RESULTS: A total of 722 patients were studied. The rate of DBS surgery for the entire country was ten per million population per year. Saskatchewan was significantly above (374%) the national average, whereas Quebec (40%) and Newfoundland & Labrador (32%) were significantly below the national average. No patients from the three territories received DBS. There were no significant differences in access from rural versus non-rural areas or in regions within provinces with different socioeconomic status. CONCLUSIONS: This is the first study to quantify all patients receiving DBS within an entire country. The current rate of DBS surgery within Canada is ten cases per million per year. Statistically significant regional differences were discovered and discussed.

Shunt infection prevention practices in Hydrocephalus Clinical Research Network-Quality: a new quality improvement network for hydrocephalus management.

OBJECTIVE: Knowledge-based tools used to standardize perioperative care, such as the shunt infection prevention protocol of the Hydrocephalus Clinical Research Network (HCRN), have demonstrated their ability to reduce surgeon-based and center-based variations in outcomes and improve patient care. The mere presence of high-quality evidence, however, does not necessarily translate into improved patient outcomes owing to the implementation gap. To advance understanding of how knowledge-based tools are being utilized in the routine clinical care of children with hydrocephalus, the HCRN-Quality (HCRNq) network was started in 2019. With a focus on CSF shunt infection, the authors present baseline data regarding CSF shunt infection rates and current shunt infection prevention practices in use at HCRNq sites. METHODS: Baseline shunt surgery practices, infection rate, and risk factor data were prospectively collected within HCRNq. No standard infection protocol was recommended, but site use of a protocol was implied if at least 3 of 6 common shunt infection prevention practices were used in > 80% of shunt surgical procedures. Univariable and multivariable analyses of shunt infection risk factors were performed. RESULTS: Thirty sites accrued data on 2437 procedures between November 2019 and June 2021. The unadjusted infection rate across all sites was 3.9% (range 0%-13%) and did not differ among shunt insertion, shunt revision, or shunt insertion after infection. Protocol use was implied for only 15/30 centers and 60% of shunt operations. On univariable analysis, iodine/DuraPrep (OR 0.57, 95% CI 0.37-0.88, p = 0.02) and the use of an antibiotic-impregnated catheter in any segment of the shunt (or both) decreased infection risk (OR 0.53, 95% CI 0.34-0.82, p = 0.01). Iodine-based prep solutions (OR 0.56, 95% 0.36-0.86, p = 0.02) and the use of antibiotic-impregnated catheters (OR 0.52, 95% CI 0.34-0.81, p = 0.01) retained significance in the multivariable model, but no relationship between protocol use and infection risk was demonstrated in this baseline analysis. CONCLUSIONS: The authors have demonstrated that children undergoing CSF shunt surgery at HCRNq sites share similar demographic characteristics with other large North American multicenter cohorts, with similar observed baseline infection rates and risk factors. Many centers have implemented standardized shunt infection prevention practices, but considerable practice variation remains. As such, there is an opportunity to decrease shunt infection rates in these centers through continued standardization of care.

Allometry of human calvaria bones during development from birth to 8 years of age shows a nonlinear growth pattern.

Pediatric skulls change rapidly in size and shape during development, especially for children up to 8 years of age. This project was developed to address the gap in understanding of the three-dimensional growth parameters of the human skull during this period and the impact these growth patterns have on fontanelle closure and suture formation. This study offers novel data on the dynamic changes in the anatomy of the skull with the intention of providing better guidance for pediatric surgical care. Craniometric landmarks defined on three-dimensional computed tomography reconstructions were used to map skull development in children aged 0 to 8 years old. A total of 364 datasets were analyzed and statistically representative 3D skulls with anatomical craniometric features such as head shape, bone size, suture and fontanelle closure time were generated for 17 age groups spanning birth to 8 years of age to provide a comprehensive neuroanatomical understanding of how the pediatric skull changes over time. This study indicates that the cranial bones follow a non-linear growth pattern, with the occipital and frontal bones driving the directionality of fontanelle closure and delivers a 3D visualization of the developmental characteristics of the skull providing a landmark resource for understanding the growth dynamics of the human skull. While clinical measurements remain valid approaches for the planning of surgical interventions, these 3D models may provide a more accurate planning paradigm.

A re-evaluation of the Endoscopic Third Ventriculostomy Success Score: a Hydrocephalus Clinical Research Network study.

OBJECTIVE: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities. METHODS: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics. RESULTS: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance. CONCLUSIONS: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.

Comparing ventriculoatrial and ventriculopleural shunts in pediatric hydrocephalus: a Hydrocephalus Clinical Research Network study.

OBJECTIVE: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort. METHODS: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months. RESULTS: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013). CONCLUSIONS: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Comparison of outcomes in the management of abdominal pseudocyst in children with shunted hydrocephalus: a Hydrocephalus Clinical Research Network study.

OBJECTIVE: Abdominal pseudocyst (APC) can cause distal site failure in children with ventriculoperitoneal shunts and is specifically designated as an infection in Hydrocephalus Clinical Research Network (HCRN) protocols. Specific management and outcomes of children with APCs have not been reported in a multicenter study. In this study, the authors investigated the management and outcomes of APC in children with shunted hydrocephalus who were treated at centers in the HCRN. METHODS: The HCRN Registry was queried to identify children < 18 years old with shunts who were diagnosed with an APC (i.e., a loculated abdominal fluid collection containing the peritoneal catheter with abdominal distention and/or displacement of peritoneal contents). The primary outcome was shunt failure after APC treatment. The primary variable was reimplantation of the distal catheter after pseudocyst treatment back into the peritoneum versus implantation in a nonperitoneal site. Other risk factors for shunt failure after APC treatment and variability in APC management were investigated. RESULTS: Among 141 children from 14 centers who underwent first-time management of an APC over a 14-year period, the median time from previous shunt surgery to APC diagnosis was 3.8 months. Overall, 17.7% of children had a positive culture: APC cultures were positive in 14.2% and CSF cultures in 15.6%. Six other children underwent shunt revision without removal; all underwent reoperation within 1 month. There was no difference in shunt survival (log-rank test, p = 0.42) or number of subsequent revisions within 6, 12, or 24 months for shunts reimplanted in the abdomen versus those implanted in a nonperitoneal location. Nonperitoneal implantation was associated with more noninfectious revisions (42.3% vs 22.9%, p = 0.019), whereas infection was more common after reimplantation in the abdomen (25.7% vs 7.0%, p = 0.003). Univariable analysis demonstrated that younger age at APC diagnosis (8.3 vs 12.2 years, p = 0.006) and prior shunt procedure within 12 weeks of APC diagnosis (59.5% vs 40.5%, p = 0.012) were associated with shunt failure after APC treatment. Multivariable modeling confirmed that prior shunt surgery within 12 weeks of APC diagnosis was independently associated with failure (HR 1.79 [95% CI 1.04-3.07], p = 0.035). CONCLUSIONS: In the HCRN, APCs in the setting of CSF shunts are usually managed with externalization. Shunt surgery within 12 weeks of APC diagnosis was associated with risk of failure after APC treatment. Although no differences were found in overall shunt failure rate, noninfectious shunt revisions were more common in the nonperitoneal distal catheter sites, and infection was a more common reason for failure after reimplantation of the shunt in the abdomen.

A qualitative study of transitioning patients with hydrocephalus from pediatric to adult care: fear of uncertainty, communication gaps, independence, and loss of relationships.

OBJECTIVE: Hydrocephalus is one of the most common condition treated by pediatric neurosurgeons. Many neurosurgeons are unable to continue to care for patients after they become adults. Although significant gaps in care are believed to exist for youth transitioning from pediatric to adult care, very little is known about how patients and their caregivers feel about the process. This qualitative study sought to examine the perceptions of adolescents, young adults, and their caregivers regarding transitioning from pediatric to adult care at a single Canadian center. METHODS: The authors explored the perceptions of patients with treated hydrocephalus and their caregivers using semistructured interviews and qualitative research methodologies. A convenience sample was recruited, composed of adolescent patients and their caregivers at the neurosurgery clinic of BC Children's Hospital, and patients and caregivers recently transitioned to adult care from the clinic. Interviews were transcribed verbatim and coded, with common themes identified. RESULTS: Four overarching themes relating to the process of transitioning from pediatric to adult hydrocephalus care for patients and their caregivers were identified from the data: 1) achieving independence, 2) communication gaps, 3) loss of significant relationships and environment, and 4) fear of uncertainty. CONCLUSIONS: Overall, patients with hydrocephalus and their families are dissatisfied with the process of transitioning. This study identified common themes and concerns among this cohort that may form the basis of an improved transition model for youth with hydrocephalus as they become adults.

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.

Endoscopic third ventriculostomy in previously shunt-treated patients.

OBJECTIVE: Endoscopic third ventriculostomy (ETV) is an option for treatment of hydrocephalus, including for patients who have a history of previous treatment with CSF shunt insertion. The purpose of this study was to report the success of postshunt ETV by using data from a multicenter prospective registry. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (i.e., HCRN Registry) were reviewed. Children who underwent ETV between 2008 and 2019 and had a history of previous treatment with a CSF shunt were included. A Kaplan-Meier survival curve was created for the primary outcome: time from postshunt ETV to subsequent CSF shunt placement or revision. Univariable Cox proportional hazards models were created to evaluate for an association between clinical and demographic variables and subsequent shunt surgery. Postshunt ETV complications were also identified and categorized. RESULTS: A total of 203 children were included: 57% male and 43% female; 74% White, 23% Black, and 4% other race. The most common hydrocephalus etiologies were postintraventricular hemorrhage secondary to prematurity (56, 28%) and aqueductal stenosis (42, 21%). The ETV Success Score ranged from 10 to 80. The median patient age was 4.1 years. The overall success of postshunt ETV at 6 months was 41%. Only the surgeon's report of a clear view of the basilar artery was associated with a lower likelihood of postshunt ETV failure (HR 0.43, 95% CI 0.23-0.82, p = 0.009). None of the following variables were associated with postshunt ETV success: age at the time of postshunt ETV, etiology of hydrocephalus, sex, race, ventricle size, number of previous shunt operations, ETV performed at time of shunt infection, and use of external ventricular drainage. Overall, complications were reported in 22% of patients, with CSF leak (8.6%) being the most common complication. CONCLUSIONS: Postshunt ETV was successful in treating hydrocephalus, without subsequent need for a CSF shunt, in 41% of patients, with a clear view of the basilar artery being the only variable significantly associated with success. Complications occurred in 22% of patients. ETV is an option for treatment of hydrocephalus in children who have previously undergone shunt placement, but with a lower than expected likelihood of success.

Impact of ventricle size on neuropsychological outcomes in treated pediatric hydrocephalus: an HCRN prospective cohort study.

OBJECTIVE: In pediatric hydrocephalus, shunts tend to result in smaller postoperative ventricles compared with those following an endoscopic third ventriculostomy (ETV). The impact of the final treated ventricle size on neuropsychological and quality-of-life outcomes is currently undetermined. Therefore, the authors sought to ascertain whether treated ventricle size is associated with neurocognitive and academic outcomes postoperatively. METHODS: This prospective cohort study included children aged 5 years and older at the first diagnosis of hydrocephalus at 8 Hydrocephalus Clinical Research Network sites from 2011 to 2015. The treated ventricle size, as measured by the frontal and occipital horn ratio (FOR), was compared with 25 neuropsychological tests 6 months postoperatively after adjusting for age, hydrocephalus etiology, and treatment type (ETV vs shunt). Pre- and posttreatment grade point average (GPA), quality-of-life measures (Hydrocephalus Outcome Questionnaire [HOQ]), and a truncated preoperative neuropsychological battery were also compared with the FOR. RESULTS: Overall, 60 children were included with a mean age of 10.8 years; 17% had ≥ 1 comorbidity. Etiologies for hydrocephalus were midbrain lesions (37%), aqueductal stenosis (22%), posterior fossa tumors (13%), and supratentorial tumors (12%). ETV (78%) was more commonly used than shunting (22%). Of the 25 neuropsychological tests, including full-scale IQ (q = 0.77), 23 tests showed no univariable association with postoperative ventricle size. Verbal learning delayed recall (p = 0.006, q = 0.118) and visual spatial judgment (p = 0.006, q = 0.118) were negatively associated with larger ventricles and remained significant after multivariate adjustment for age, etiology, and procedure type. However, neither delayed verbal learning (p = 0.40) nor visual spatial judgment (p = 0.22) was associated with ventricle size change with surgery. No associations were found between postoperative ventricle size and either GPA or the HOQ. CONCLUSIONS: Minimal associations were found between the treated ventricle size and neuropsychological, academic, or quality-of-life outcomes for pediatric patients in this comprehensive, multicenter study that encompassed heterogeneous hydrocephalus etiologies.

Anterior versus posterior entry site for ventriculoperitoneal shunt insertion: a randomized controlled trial by the Hydrocephalus Clinical Research Network.

OBJECTIVE: The primary objective of this trial was to determine if shunt entry site affects the risk of shunt failure. METHODS: The authors performed a parallel-design randomized controlled trial with an equal allocation of patients who received shunt placement via the anterior entry site and patients who received shunt placement via the posterior entry site. All patients were children with symptoms or signs of hydrocephalus and ventriculomegaly. Patients were ineligible if they had a prior history of shunt insertion. Patients received a ventriculoperitoneal shunt after randomization; randomization was stratified by surgeon. The primary outcome was shunt failure. The planned minimum follow-up was 18 months. The trial was designed to achieve high power to detect a 10% or greater absolute difference in the shunt failure rate at 1 year. An independent, blinded adjudication committee determined eligibility and the primary outcome. The study was conducted by the Hydrocephalus Clinical Research Network. RESULTS: The study randomized 467 pediatric patients at 14 tertiary care pediatric hospitals in North America from April 2015 to January 2019. The adjudication committee, blinded to intervention, excluded 7 patients in each group for not meeting the study inclusion criteria. For the primary analysis, there were 229 patients in the posterior group and 224 patients in the anterior group. The median patient age was 1.3 months, and the most common etiologies of hydrocephalus were postintraventricular hemorrhage secondary to prematurity (32.7%), myelomeningocele (16.8%), and aqueductal stenosis (10.8%). There was no significant difference in the time to shunt failure between the entry sites (log-rank test, stratified by age < 6 months and ≥ 6 months; p = 0.061). The hazard ratio (HR) of a posterior shunt relative to an anterior shunt was calculated using a univariable Cox regression model and was nonsignificant (HR 1.35, 95% CI, 0.98-1.85; p = 0.062). No significant difference was found between entry sites for the surgery duration, number of ventricular catheter passes, ventricular catheter location, and hospital length of stay. There were no significant differences between entry sites for intraoperative complications, postoperative CSF leaks, pseudomeningoceles, shunt infections, skull fractures, postoperative seizures, new-onset epilepsy, or intracranial hemorrhages. CONCLUSIONS: This randomized controlled trial comparing the anterior and posterior shunt entry sites has demonstrated no significant difference in the time to shunt failure. Anterior and posterior entry site surgeries were found to have similar outcomes and similar complication rates.

The Hydrocephalus Clinical Research Network quality improvement initiative: the role of antibiotic-impregnated catheters and vancomycin wound irrigation.

OBJECTIVE: Two previous Hydrocephalus Clinical Research Network (HCRN) studies have demonstrated that compliance with a standardized CSF shunt infection protocol reduces shunt infections. In this third iteration, a simplified protocol consisting of 5 steps was implemented. This analysis provides an updated evaluation of protocol compliance and evaluates modifiable shunt infection risk factors. METHODS: The new simplified protocol was implemented at HCRN centers on November 1, 2016, for all shunt procedures, excluding external ventricular drains, ventricular reservoirs, and subgaleal shunts. Procedures performed through December 31, 2019, were included (38 months). Compliance with the protocol, use of antibiotic-impregnated catheters (AICs), and other variables of interest were collected at the index operation. Outcome events for a minimum of 6 months postoperatively were recorded. The definition of infection was unchanged from the authors' previous report. RESULTS: A total of 4913 procedures were performed at 13 HCRN centers. The overall infection rate was 5.1%. Surgeons were compliant with all 5 steps of the protocol in 79.4% of procedures. The infection rate for the protocol alone was 8.1% and dropped to 4.9% when AICs were added. Multivariate analysis identified having ≥ 2 complex chronic conditions (odds ratio [OR] 1.76, 95% confidence interval [CI] 1.26-2.44, p = 0.01) and a history of prior shunt surgery within 12 weeks (OR 1.84, 95% CI 1.37-2.47, p < 0.01) as independent risk factors for shunt infection. The use of AICs (OR 0.70, 95% CI 0.50-0.97, p = 0.05) and vancomycin irrigation (OR 0.36, 95% CI 0.21-0.62, p < 0.01) were identified as independent factors protective against shunt infection. CONCLUSIONS: The authors report the third iteration of their quality improvement protocol to reduce the risk of shunt infection. Compliance with the protocol was high. These updated data suggest that the incorporation of AICs is an important, modifiable infection prevention measure. Vancomycin irrigation was also identified as a protective factor but requires further study to better understand its role in preventing shunt infection.

Endoscopic third ventriculostomy revision after failure of initial endoscopic third ventriculostomy and choroid plexus cauterization.

OBJECTIVE: Primary treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) and choroid plexus cauterization (CPC) is well described in the neurosurgical literature, with wide reported ranges of success and complication rates. The purpose of this study was to describe the safety and efficacy of ETV revision after initial ETV+CPC failure. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network Core Data Project registry were reviewed. Children who underwent ETV+CPC as the initial treatment for hydrocephalus between 2013 and 2019 and in whom the initial ETV+CPC was completed (i.e., not abandoned) were included. Log-rank survival analysis (the primary analysis) was used to compare time to failure (defined as any other surgical treatment for hydrocephalus or death related to hydrocephalus) of initial ETV+CPC versus that of ETV revision by using random-effects modeling to account for the inclusion of patients in both the initial and revision groups. Secondary analysis compared ETV revision to shunt placement after failure of initial ETV+CPC by using the log-rank test, as well as shunt failure after ETV+CPC to that after ETV revision. Cox regression analysis was used to identify predictors of failure among children treated with ETV revision. RESULTS: The authors identified 521 ETV+CPC procedures that met their inclusion criteria. Ninety-one children underwent ETV revision after ETV+CPC failure. ETV revision had a lower 1-year success rate than initial ETV+CPC (29.5% vs 45%, p < 0.001). ETV revision after initial ETV+CPC failure had a lower success rate than shunting (29.5% vs 77.8%, p < 0.001). Shunt survival after initial ETV+CPC failure was not significantly different from shunt survival after ETV revision failure (p = 0.963). Complication rates were similar for all examined surgical procedures (initial ETV+CPC, ETV revision, ventriculoperitoneal shunt [VPS] placement after ETV+CPC, and VPS placement after ETV revision). Only young age was predictive of ETV revision failure (p = 0.02). CONCLUSIONS: ETV revision had a significantly lower 1-year success rate than initial ETV+CPC and VPS placement after ETV+CPC. Complication rates were similar for all studied procedures. Younger age, but not time since initial ETV+CPC, was a risk factor for ETV revision failure.