Pediatric Hydrocephalus Research Articles

Comparative analysis of 333 proGAV® and proGAV 2.0® adjustable valves in pediatric hydrocephalus treatment: survival and complication rate assessment

ObjectiveProGAV and ProGAV2.0 adjustable valves are extensively used for treating hydrocephalus in pediatric patients. This study aims to conduct a comprehensive comparison between these two valves concerning their survival and complication rates.MethodsThis retrospective study included all pediatric patients who underwent ProGAV or ProGAV2.0 valve implantation at our neurosurgical clinic from August 2008 to August 2020. A comparative analysis was performed considering age, gender, follow-up duration, complication and survival rates, adjustments, spontaneous adjustments, and adjustment difficulty rates. All valves were followed up for a maximum of 3 years.ResultsInclusion involved 333 cases (mean age of 5.4 ± 5.1 years; 54.1% males), comprising 173 cases (52.0%) with ProGAV valve implantation and 160 cases (48.0%) with ProGAV 2.0. Early complications within the first month post-implantation were observed in 51 cases (15.3%). No significant differences were noted in valve implantation indications, age distribution, or survival duration (27.1 vs. 27.8 months, p = 0.629) between the two groups. Predominant indications for implantation were post-hemorrhagic hydrocephalus and aqueduct stenosis for both valve types. Notably, both valves showed non-significantly different explantation rates during the first three years after implantation (34.7% vs. 29.7%, p < 0.289). However, there was a significantly higher early post-implantation complication rate (22% vs. 9.4%, p = 0.002), and a significantly increased association with difficulties in valve adjustments and spontaneous adjustments (p = 0.041 and 0.011, respectively). ProGAV2.0 cases displayed notably enhanced clinical and radiological improvement within the initial 6 months after implantation (p = 0.001 and p = 0.038). Younger children (< 2 years of age) also experienced significantly more valve adjustment difficulties (p = 0.049) and had higher rates of valve explantation (p < 0.001).ConclusionThe findings of this study highlight the superior performance of the ProGAV2.0 valve in terms of complication rate and maladjustment rate when employed in the treatment of pediatric hydrocephalus. Both valves demonstrated an acceptable survival rate with 65.3% for ProGAV and 71.3% for ProGAV2.0 within three years of implantation.

Current Trends in the Treatment of Pediatric Hydrocephalus: A Narrative Review Centered on the Indications, Safety, Efficacy, and Long-Term Outcomes of Available Treatment Modalities

The pathophysiologic substrate of pediatric hydrocephalus has not been thoroughly elucidated. Valve-based shunt systems have constituted the main therapeutic option since the late 1950s. The initially used systems were concerning the ventricular system and the atrium. In the 1970s, VA shunts were not the main stay of treatment as the preferred option for the terminal end of the drainage system was the peritoneum. Our review analyzes these valve types and attempts a comparison, based on their functional characteristics. Nowadays, the only available surgical alternative for the treatment of hydrocephalus is ETV. This technique is associated with lower infection rates as well as, on average, a lower re-operation rate. Another term that deserves special mention is related to the outcome of ETV in children who had a medical history of previously incorporated shunts and who were subsequently suffering from shunt malfunction. Well-recognized predictive factors associated with secondary ETV failure include age, early onset of hydrocephalus, and prematurity. Although several attempts have been made in order to establish the optimum surgical treatment management in the different subgroups of patientswho are suffering from shunt dysfunction, there is no universal agreement. Therefore, this review attempts to identify the specific subpopulations of patients in whom the insertion of a drainage system as the preferred treatment modality is associated with an optimum long-term prognosis, compared to ETV, and vice versa. The objective of our study is to analyze the safety, efficacy, and outcomes of drainage devices and ETV in pediatric hydrocephalus patients.

Pediatric CSF diversion procedures for treatment of hydrocephalus during the COVID-19 pandemic.

Because there is not a link between COVID-19 and pediatric hydrocephalus, the COVID-19 pandemic should not have altered the incidence of pediatric hydrocephalus or the rate of CSF diversion procedures or shunt failure. Therefore, hydrocephalus-related surgical volume should have remained constant. The goal of this study was to evaluate the rates of hydrocephalus surgeries in the COVID-19 era compared with the baseline pre-COVID-19 era. Ten institutions collected information about all hydrocephalus-related surgeries performed between March 2018 and February 2022. The period after March 1, 2020, was considered the COVID-19 era; the period prior to this date was considered the baseline pre-COVID era. Four COVID surge periods were defined using the New York Times COVID-19 database. Total case volumes were compared between the COVID era and baseline, both overall and for each surge period. Sex, race, ethnicity, insurance status, Area Deprivation Index (ADI), and rural-urban commuter area were collected for each surgery. Proportions of patients were then compared overall and for each surge based on these variables. Of 8056 procedures, 54% were in male patients (n = 4375), 65% in White patients (n = 5247), 18% in Hispanic patients (n = 1423), and 54% in patients with public insurance (n = 4371). There was no change in the number of surgeries per site per month in the COVID era compared with baseline (16.7 vs 17.9, p = 0.113). However, there was a significant decrease in the first surge period (April 2020; 11.5 vs 17.7, p = 0.034). Male sex (p < 0.0039) and Black race (p < 0.001) were found to be associated with a significantly higher proportion of hydrocephalus procedures during the COVID-19 era. Some surge periods showed different proportions of privately insured patient and ADI levels. However, these relationships were inconsistent between surges. Overall average monthly case numbers were not significantly different between the pre-COVID and COVID eras. There was a significant decrease in hydrocephalus surgery during the first COVID surge. More hydrocephalus surgeries were performed in children of male sex and Black race proportionally during the COVID period overall, but not during individual surges.

Intraoperative cone-beam computed tomography for catheter placement verification in pediatric hydrocephalus: technical note.

Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement.

Cerebroventricular deformation and vector mapping, a topographic visualizer for surgical interventions in pediatric hydrocephalus.

Hydrocephalus is a challenging neurosurgical condition due to nonspecific symptoms and complex brain-fluid pressure dynamics. Typically, the assessment of hydrocephalus in children requires radiographic or invasive pressure monitoring. There is usually a qualitative focus on the ventricular spaces even though stress and shear forces extend across the brain. Here, the authors present an MRI-based vector approach for voxelwise brain and ventricular deformation visualization and analysis. Twenty pediatric patients (mean age 7.7 years, range 6 months-18 years; 14 males) with acute, newly diagnosed hydrocephalus requiring surgical intervention for symptomatic relief were randomly identified after retrospective chart review. Selection criteria included acquisition of both pre- and posttherapy paired 3D T1-weighted volumetric MRI (3D T1-MRI) performed on 3T MRI systems. Both pre- and posttherapy 3D T1-MRI pairs were aligned using image registration, and subsequently, voxelwise nonlinear transformations were performed to derive two exemplary visualizations of compliance: 1) a whole-brain vector map projecting the resulting deformation field on baseline axial imaging; and 2) a 3D heat map projecting the volumetric changes along ventricular boundaries and the brain periphery. The patients underwent the following interventions for treatment of hydrocephalus: endoscopic third ventriculostomy (n = 6); external ventricular drain placement and/or tumor resection (n = 10); or ventriculoperitoneal shunt placement (n = 4). The mean time between pre- and postoperative imaging was 36.5 days. Following intervention, the ventricular volumes decreased significantly (mean pre- and posttherapy volumes of 151.9 cm3 and 82.0 cm3, respectively; p < 0.001, paired t-test). The largest degree of deformation vector changes occurred along the lateral ventricular spaces, relative to the genu and splenium. There was a significant correlation between change in deformation vector magnitudes within the cortical layer and age (p = 0.011, Pearson), as well as between the ventricle size and age (p = 0.014, Pearson), suggesting higher compliance among infants and younger children. This study highlights an approach for deformation analysis and vector mapping that may serve as a topographic visualizer for therapeutic interventions in patients with hydrocephalus. A future study that correlates the degree of cerebroventricular deformation or compliance with intracranial pressures could clarify the potential role of this technique in noninvasive pressure monitoring or in cases of noncompliant ventricles.

Ventricular Anatomical Differences—A Different Perspective on Endoscopic Treatment of Pediatric Hydrocephalus: Anomalies, Variations, and Deformations

Ventricular Anatomical Differences—A Different Perspective on Endoscopic Treatment of Pediatric Hydrocephalus: Anomalies, Variations, and Deformations

Pediatric shunt failure in a resource limited Lower-Middle Income Country (LMIC) institution in La Paz, Bolivia.

Shunt failure is an undesirable but common occurrence following neurosurgical shunting for pediatric hydrocephalus. Little is known about the occurrence of failure in lower-middle income country (LMIC) settings in South America. The objective of this study was to evaluate shunt failure in the sole publicly funded pediatric hospital in La Paz, Bolivia, with limited resources. A retrospective review of all patients at the Children's Hospital of La Paz, Bolivia (Hospital del Niño "Dr. Ovidio Aliaga Uria"), was conducted to identify all patients whose index surgical shunting for hydrocephalus was performed between 2019 and 2023. Categorical, continuous, and shunt failure data were statistically summarized. A total of 147 unique pediatric patients underwent index ventriculoperitoneal shunting for hydrocephalus in the study period. There were 90 (61%) male and 57 (39%) female patients, with a median age of 2.2months at index shunting procedure. The most common surgical indications were congenital hydrocephalus (n = 95, 65%), followed by hydrocephalus secondary to congenital defect (n = 25, 17%) and tumor (n = 18, 12%). A total of 18 (12%) of patients experienced inpatient failure during index admission requiring surgical revision at a median time of 12.5days after index shunting. Postoperative imaging (OR 2.97, P = 0.037) and postoperative infection (OR 3.26, P = 0.032) during index admission both independently and statistically predicted inpatient failure. Of the 96 patients (65%) with postoperative follow-up, 16 (n = 16/96, 17%) patients experienced outpatient failure requiring readmission to hospital and surgical revision at a median time of 3.7months after discharge. Kaplan-Meier estimations of overall inpatient and outpatient failure in this cohort were 23% (95% CI 14-37) and 28% (95% CI 15-49), respectively. Both inpatient and outpatient shunt failures are significant complications in the management of pediatric hydrocephalus in La Paz, Bolivia. We identify multiple avenues to improve these outcomes which are institution-specific based on the review of these failures. Lessons learnt may be applicable to other similarly resourced institutions across South American LMICs.

Association of Glymphatic and White Matter Impairment With the Postoperative Outcome of Pediatric Hydrocephalus.

Assessment of postoperative outcomes on pediatric hydrocephalus is critical for adjusting treatment strategies. The aim of this work was to investigate the ability of MRI metrics to predict postoperative outcomes. A total of 55 children with hydrocephalus who underwent MRI and ventriculoperitoneal shunt surgery were prospectively enrolled. MRI was also performed at 6 months postoperatively in 33 of the 55 children. A total of 92 controls matched for age and sex were enrolled and divided into preoperative and postoperative control groups. We calculated the diffusion tensor imaging along the perivascular space (DTI-ALPS) index, Evans index, and diffusion tensor imaging metrics. The ability of various metrics to predict postoperative outcomes was assessed using receiver operating characteristic curve analysis. The DTI-ALPS index was significantly lower in patients with hydrocephalus than in controls. The abnormal DTI-ALPS index trended toward the normal range after surgery. Patients with lower preoperative DTI-ALPS index, lower fractional anisotropy (FA), and higher radial diffusivity in association fibers had less favorable short-term outcomes. Patients with worse long-term outcomes had lower postoperative DTI-ALPS index, higher postoperative Evans index, and lower FA and higher radial diffusivity in association fibers. Predictive performance was better when the DTI-ALPS index and FA in association fibers were used in combination than when either of these metrics was used alone. The DTI-ALPS index and FA in association fibers provided complementary information for prognostic assessment after the ventriculoperitoneal shunt surgery on pediatric hydrocephalus. A combination of DTI-ALPS index and FA would improve our ability to predict postoperative outcomes in these patients.

The Landscape of Pediatric Hydrocephalus in the Province of KwaZulu-Natal: A Comparative Analysis of the Referral Pattern, Etiology, and Management Outcomes in 4 Distinct 5-Year Periods

BackgroundPaediatric hydrocephalus causes significant health burden globally, particularly in low and middle-income countries. There's a dearth of data from specific regions such as KwaZulu-Natal, South Africa. This study aimed to investigate the landscape of paediatric hydrocephalus, comparing four distinct five-year periods. MethodsData were collected retrospectively (2003 to 2007, 2008 to 2012, and 2013 to 2017) and prospectively (2018 to 2022). Children (≤18 years) treated for hydrocephalus were included. Data on demographics, referral patterns, aetiology, treatment modalities, and outcomes were collected and analyzed. ResultsA total of 3325 children were treated. The peak period was 2008 to 2012 (35.3%). Majority (51.4%) were from rural areas (p=0.013) and 47.9% were referred from regional hospitals, p=<0.001. Males (56.4%) and infants (60.2%) were predominant groups (p<0.001). Post-infectious aetiology (32.7%) was predominant (p<0.001), particularly tuberculous meningitis (54.1%). Ventriculoperitoneal shunts (VPSs) were the mainstay treatment (84.2%), with notable complication rates (20.4%), including infections (9.6%). HIV co-infection was diagnosed in 2.5% of cases. Weekend procedures were associated with VPS complications (HR1.3, CI:1.03-1.66, p=0.03). The mortality rate was 7.9%, and age ≥1 year (HR, 2.43 CI: 1.87-3.17, p<0.001), tertiary hospital referral (HR 1.48, CI: 1.06-2.04, p=0.019), VPS infection (HR, 3.63 CI: 2.66-4.95, p<0.001), acute abdomen (HR 2.17, CI: 1.11-4.25, p=0.024) and pneumonia (HR 7.32, OR 4.84 -11.06, p<0.001) were associated with mortality. ConclusionThis study provides comprehensive insights into pediatric hydrocephalus in KZN. Monitoring temporal trends and predictors of outcomes will aid guide future interventions aimed at mitigating the burden of pediatric hydrocephalus in the region.

Ventriculoperitoneal shunt complication in pediatric hydrocephalus: risk factor analysis from a single institution

Introduction: Ventriculoperitoneal (VP) shunt surgery is a commonly performed neurosurgical procedure. Complications due to shunt failure are associated with high morbidity and mortality. There is an absence of research in Indonesia that focuses on demographic factors related to complications from ventriculoperitoneal shunts. We report an analysis of risk factors for shunt failure in pediatric patients from a single institution Method: A retrospective analytical study with prospective data was designed. Pediatric patients with hydrocephalus were performed ventriculoperitoneal shunt in 5 years (January 2018 - December 2023). All of risk factors such as age, comorbidity, etiologies, types of shunts, CSF analysis were followed up. Result: 448 pediatric patients underwent VP shunts, and 175 of them had complications. The age group &lt;12 months, with 98 patients, noticed the highest number of complications of VP shunt. The logistic regression test showed that age, surgery duration, protein, CSF cells, and birth weight all had p-values &lt;0.05. The number of CSF cells has the largest OR value (OR = 136,800) (95% CI 27.185 – 688.391). The Kaplan Meier survival test showed that the age group (p 0.008, OR 0.580 and 95% CI 0.387 – 0.868). Conclusion: Patients under the age of six months had the highest incidence of VP shunt complications. Cell number, CSF protein, surgery duration, age, and birth weight all influence the risk of VP shunt complications in pediatric patients.

Trends in the Treatment of Pediatric Hydrocephalus Since 2014: Understanding the Role of the 2014 Hydrocephalus Guidelines

BACKGROUND AND OBJECTIVES: The management of hydrocephalus has significantly changed over the past decade. Endoscopic third ventriculostomy has become more prevalent, and shunt surgery has become more protocolized through research efforts by the Hydrocephalus Clinical Research Network. In 2014, the Pediatric Hydrocephalus Guidelines were first published, providing a cohesive source for clinical guidance. We planned to investigate national trends in the management of pediatric hydrocephalus after the publication of the 2014 Pediatric Hydrocephalus Guidelines as guidelines act as a compilation of historic and recent literature in a scientific field. METHODS: A multipronged approach was used to evaluate changes in the treatment of pediatric hydrocephalus since 2014. First, we queried the Pediatric Health Information System between 2013 and 2018 to identify national trends in shunt procedures for hydrocephalus. To assess the recommendation in the guidelines to use antibiotic-impregnated catheters (AICs) in shunts, national sales records of AICs were obtained from 2 large neurosurgical device companies. RESULTS: A total of 11,179 shunt procedures were performed within the Pediatric Health Information System database in the study period. In the preguideline publication period (2013-2014), there was a shunt revision-to-placement ratio of 1.63 while in the postguideline publication period (2015-2018), there was a ratio of 0.84 (P &lt; .0001). National sales data revealed that antibiotic-impregnated drain sales increased from 2% to 77% since publication. CONCLUSION: Findings from this investigation suggest progress in pediatric hydrocephalus management since 2014. The shunt revision-to-placement ratio improved, and the use of AICs increased over this period. Improved outcomes are likely associative findings rather than causative with the guidelines representing a culmination of widespread changes in hydrocephalus care such as increased use of endoscopic third ventriculostomies, protocolized care, and image-guided shunt placement. Further research into the impact of clinical practice guidelines is needed to better understand the impact of this tool on surgeons and patient care.

Evaluating normalized registration and preprocessing methodologies for the analysis of brain MRI in pediatric patients with shunt-treated hydrocephalus.

Registration to a standardized template (i.e. "normalization") is a critical step when performing neuroimaging studies. We present a comparative study involving the evaluation of general-purpose registration algorithms for pediatric patients with shunt treated hydrocephalus. Our sample dataset presents a number of intersecting challenges for registration, representing the potentially large deformations to both brain structures and overall brain shape, artifacts from shunts, and morphological differences corresponding to age. The current study assesses the normalization accuracy of shunt-treated hydrocephalus patients using freely available neuroimaging registration tools. Anatomical neuroimages from eight pediatric patients with shunt-treated hydrocephalus were normalized. Four non-linear registration algorithms were assessed in addition to the preprocessing steps of skull-stripping and bias-correction. Registration accuracy was assessed using the Dice Coefficient (DC) and Hausdorff Distance (HD) in subcortical and cortical regions. A total of 592 registrations were performed. On average, normalizations performed using the brain extracted and bias-corrected images had a higher DC and lower HD compared to full head/ non-biased corrected images. The most accurate registration was achieved using SyN by ANTs with skull-stripped and bias corrected images. Without preprocessing, the DARTEL Toolbox was able to produce normalized images with comparable accuracy. The use of a pediatric template as an intermediate registration did not improve normalization. Using structural neuroimages from patients with shunt-treated pediatric hydrocephalus, it was demonstrated that there are tools which perform well after specified pre-processing steps were taken. Overall, these results provide insight to the performance of registration programs that can be used for normalization of brains with complex pathologies.

Comparison of endoscopic third ventriculostomy versus cerebrospinal fluid shunt procedures for the treatment of pediatric hydrocephalus in Taiwan.

Pediatric hydrocephalus is the most common cause of surgically treatable neurological disease in children. Controversies exist whether endoscopic third ventriculostomy (ETV) or cerebrospinal fluid (CSF) shunt placement is the most appropriate treatment for pediatric hydrocephalus. This study aimed to compare the risk of re-operation and death between the two procedures. We performed a retrospective population-based cohort study and included patients younger than 20-years-old who underwent CSF shunt or ETV for hydrocephalus from the Taiwan National Health Insurance Research Database. A total of 3,555 pediatric patients from 2004 to 2017 were selected, including 2,340 (65.8%) patients that received CSF shunt placement and 1215 (34.2%) patients that underwent ETV. The incidence of all-cause death was 3.31 per 100 person-year for CSF shunt group and 2.52 per 100 person-year for ETV group, with an adjusted hazard ratio (HR) of 0.79 (95% confidence interval [CI] = 0.66-0.94, p = 0.009). The cumulative incidence competing risk for reoperation was 31.2% forthe CSF shunt group and 26.4% forthe ETV group, with an adjusted subdistribution HR of 0.82 (95% CI = 0.70-0.96, p = 0.015). Subgroup analysis showed that ETV was beneficial for hydrocephalus coexisting with brain or spinal tumor, central nervous system infection, and intracranial hemorrhage. Our data indicates ETV is a betteroperative procedure for pediatric hydrocephalus when advanced surgical techniques and instruments are available.

Paediatric Neurosurgical emergency operations:Clinical spectrum, Presentation-Intervention Interval and Early operative outcome in Regional Neurosurgical center, Nigeria

BACKGROUND: Pediatric neurosurgical emergencies are prevalent worldwide, often triggered by acute, life-threatening conditions that necessitate immediate surgery to prevent disability and death. Managing these conditions perioperatively poses significant challenges for neurosurgeons and anesthetists due to potential complications like anemia, seizures, electrolyte imbalances, intracranial hypertension, and vision threats, alongside the primary neurosurgical issues. Proper correction and optimization of these physiological derangements before surgery are crucial for favorable postoperative outcomes. PATIENTS AND METHODS: This retrospective study analyzes emergency neurosurgical operations performed on children aged sixteen and under at a neurosurgical center in Sokoto, Nigeria, from January 2018 to December 2021. The center caters to a population of approximately twenty million people from three north-western and one north-central Nigerian states, as well as neighboring Niger and Benin republics. The area features a mostly arid climate with limited seasonal rainfall and experiences the Sahara’s harmattan winds. RESULTS A total of 131 children underwent emergency neurosurgical procedures during the study period. The majority were males (71%), with an average age of 5.6 years. Hydrocephalus was the most frequent emergency condition, leading to 44.3% of surgeries, primarily through ventriculoperitoneal shunt procedures, which accounted for 42.7% of interventions. Anemia was the most common comorbid condition. The mean time from presentation to intervention was 10 days. Postoperatively, 17.9% of patients suffered from surgical site infections, yet 67.9% had favorable outcomes with a mortality rate of 7.1%. CONCLUSION: This study underscores the importance of timely surgical interventions and highlights the predominance of ventriculoperitoneal shunts in treating pediatric hydrocephalus in the region, with generally positive outcomes despite delays

Ventriculoperitoneal shunt placement following endoscopic third ventriculostomy failure in the treatment of pediatric hydrocephalus.

The goal of this study was to evaluate the impact of endoscopic third ventriculostomy (ETV) failure on subsequent risk of ventriculoperitoneal shunt (VPS) placement. A retrospective chart review was performed to identify pediatric patients receiving ETV followed by a VPS at Oklahoma Children's Hospital between January 1, 2016, and December 31, 2021. A control group of patients receiving a VPS alone was also gathered. Complication and shunt failure rates were compared between the 2 groups at 12 months postoperatively. A total of 222 patients were included in this study. The VPS placement after ETV failure (VPSEF) group included 21 patients; 53% were male and 47% were female, with a mean age of 2.2 years and standard deviation of 4.3 years. The etiology of hydrocephalus was chiefly intraventricular hemorrhage (43%) and neural tube defects (19%). At 12 months after VPS placement, the complication rate was 24%, predominantly including infection (19%) or CSF leakage (10%). The VPS-only (VPSO) group included 201 patients; 51% were male and 49% were female, with a mean age of 4.2 years and standard deviation of 6.5 years. The etiology of hydrocephalus was chiefly intraventricular hemorrhage (26%) and neural tube defects (30%). At 12 months postoperatively, the complication rate was 10%, predominantly including infection (6%) or catheter-associated hemorrhage (3%). The difference in complication rates between the VPSEF and VPSO groups was not significant at 12 months postoperatively (p = 0.07); however, on subgroup analysis there was a significantly higher rate of CSF leakage at 12 months in the VPSEF group compared to the VPSO group (p = 0.0371). There was no difference in overall complication rates for the treatment of pediatric hydrocephalus by VPS following failed ETV compared to VPS placement alone, yet prior ETV may predispose patients to a higher rate of CSF leaks within 12 months of VPS placement. Further study is indicated to determine whether a prior ETV procedure predisposes patients to a higher complication rate on VPS placement.

Temporal trends in paediatric hydrocephalus - Rising prematurity and persistent ophthalmological challenges.

To study changes in aetiology, prematurity, comorbidity and ophthalmological outcomes in children with surgically treated hydrocephalus to provide information needed to maintain the best possible healthcare for a fragile and changing population. Two population-based cohorts, born two decades apart in Region Västra Götaland Sweden, surgically treated for hydrocephalus at Sahlgrenska University Hospital in Gothenburg were recruited at approximately 10 years of age. The participants were examined according to an ophthalmological protocol, including history taking regarding perceptual visual dysfunction (PVD). Gestational age, aetiology and comorbidities were registered. The 1989-1993 group, comprised 52 children (48% girls; mean age 10, range 7.7-12.8 years), was compared with 24 children, born in 2007-2012 (29% girls; mean age 10, range 7.0-13.8 years). Extreme prematurity (gestational age ≤ 28 weeks) increased over time (p = 0.001). The vast majority of the children showed ophthalmological abnormalities, although motility defects and nystagmus decreased in the latter population. Subnormal visual acuity was associated with extreme prematurity (RR = 4.69; p = 0.030), and PVD with learning disability (RR = 2.44; p = 0.032). Paediatric hydrocephalus populations may change with improved healthcare. Since a high percentage shows ophthalmological abnormalities and more children are born extremely preterm, the entirety needs consideration both neurologically and ophthalmologically.

Low surgical weight associated with ETV failure in pediatric hydrocephalus patients.

The use of endoscopic third ventriculostomy (ETV) for treatment of pediatric hydrocephalus has higher failure rates in younger patients. Here we investigate the impact of select perioperative variables, specifically gestational age, chronological age, birth weight, and surgical weight, on ETV failure rates. A retrospective review was performed on patients treated with ETV - with or without choroid plexus cauterization (CPC) - from 2010 to 2021 at a large academic center. Analyses included Cox regression for independent predictors and Kaplan-Meier survival curves for time to-event outcomes. In total, 47 patients were treated with ETV; of these, 31 received adjunctive CPC. Overall, 66% of the cohort experienced ETV failure with a median failure of 36 days postoperatively. Patients aged < 6 months at time of surgery experienced 80% failure rate, and those > 6 months at time of surgery experienced a 41% failure rate. Univariate Cox regression analysis showed weight at the time of ETV surgery was significantly inversely associated with ETV failure with a hazard ratio of 0.92 (95% CI 0.82, 0.99). Multivariate analysis redemonstrated the inverse association of weight at time of surgery with ETV failure with hazard ratio of 0.76 (95% CI 0.60, 0.92), and sensitivity analysis showed < 4.9kg as the optimal cutoff predicting ETV/CPC failure. Neither chronologic age nor gestational age were found to be significantly associated with ETV failure.In this study, younger patients experienced higher ETV failure rates, but multivariate regression found that weight was a more robust predictor of ETV failure than chronologic age or gestational age, with an optimal cutoff of 4.9kg in our small cohort. Given the limited sample size, further study is needed to elucidate the independent role of weight as a peri-operative variable in determining ETV candidacy in young infants. Previous presentations: Poster Presentation, Congress of Neurological Surgeons.

268 Integrative Genomics Identifies Evolutionary, Temporal, and Cell-lineage Origin of Hydrocephalus Risk Gene

INTRODUCTION: Our previous work identified MAEL, a gene involved in regulation of DNA transposon activity and histone methylation, as a transcriptome-wide predictor of pediatric hydrocephalus (Hale et al., Cell Reports, 2021). Here we aim to characterize the function of MAEL across timescales and cell-lineages in the developing human brain to understand the pathophysiological basis of hydrocephalus. METHODS: We performed taxonomic analysis of MAEL across species using Ensemble. Analysis of single-cell RNA sequencing (scRNA-seq) of 49 brain regions across the prenatal period from the Developing Human Brain Atlas (Allen Institute) and an atlas of late-prenatal cortical tissue identified temporospatial MAEL expression patterns. Finally, to estimate the degree to which in vitro models recapitulate expression patterns of MAEL in the developing brain, we analyzed scRNA-seq from human neural and choroid-plexus organoids. RESULTS: We performed taxonomic evolutionary gene-mapping to define the evolutionary origin of MAEL to assess suitability for mechanistic characterization in model systems. MAEL is among the top 0.01% human-specific genes and shares &lt; 50% sequence homology with mammalian model organisms. scRNA-seq identified MAEL first detected in the telencephalon at 9 weeks post-conception, highest expressed in the sub-ventricular zone at 16 weeks, and lowest expressed in the hippocampus at 21 weeks post conception. Temporospatial expression of MAEL in the late-prenatal cortex identifies MAEL expression largely restricted to the neural progenitor cells before reaching steady-state expression throughout the cortical layers at 39 weeks gestation. Finally, spatial MAEL expression is largely preserved in brain organoids and robustly expressed in choroid plexus organoids highlighting the utility of these models in future mechanistic studies. CONCLUSIONS: We delineate the evolutionary origin and temporospatial patterns of MAEL expression in the developing human brain and organoid models. These data provide the premise for understanding the detailed molecular-genetic underpinnings of hydrocephalus.

Comparing motor development in children with hydrocephalus after treatment with ventriculoperitoneal shunt and endoscopic third ventriculostomy: a cross-sectional study.

Ventriculoperitoneal shunt insertion (VPSI) and endoscopic third ventriculostomy (ETV) are the major procedures for treating pediatric hydrocephalus. However, studies comparing motor development following the two treatments are limited. We aimed to determine motor development outcomes in children with hydrocephalus up to 2 years of age after undergoing VPSI or ETV, to identify which surgical approach yields better motor outcomes and may be more effective for Malawian children. This was a cross-sectional study where we recruited two groups of participants: one group consisted of children with hydrocephalus treated with VP shunt whilst the other group were treated with ETV, at least 6 months prior to this study. Participants were identified from the hospital records and were called to come for neurodevelopmental assessment using the Malawi Development Assessment Tool (MDAT). A total 152 children treated for hydrocephalus within an 18-month period met the inclusion criteria. Upon follow up and tracing, we recruited 25 children who had been treated: 12 had VPSI and 13 had ETV. MDAT revealed delays in both assessed motor domains: 19 out of the 25 children had delayed gross motor whilst 16 of 25 had delayed fine motor development. There was no significant difference between the shunted and the ETV groups. Children with hydrocephalus demonstrate delays in motor development six to 18 months after treatment with either VPSI or ETV. This may necessitate early and prolonged intensive rehabilitation to restore motor function after surgery. Long-term follow-up studies with bigger sample sizes are required to detect the effect of the two treatment approaches.

Can diffuse reflectance spectroscopy identify shuntodynia in pediatric hydrocephalus patients?

Shuntodynia is patient reported pain at the site of the implanted ventriculoperitoneal (VP) shunt. Pediatric hydrocephalus requiring shunt placement is a chronic and prevalent standard of care treatment and requires lifetime management. Shuntodynia is a subjective measure of shunt dysfunction. Quantitative, white-light tissue spectroscopy could be used to objectively identify this condition in the clinic. Pediatric subjects were recruited for optical sensing during routine clinical follow-up visits, post-VP shunt implantations. Acquired optical signals were translated into skin-hemodynamic signatures and were compared between subjects that reported shuntodynia versus those that did not. Diffuse reflectance spectroscopy (DRS) measurements were collected between 450 and 700nm using a single-channel fiber-optical probe from () patients. Multiple reflectance spectra were obtained by the attending physician from regions both proximal and distal to the VP shunt sites and from a matched contralateral site for each subject. Acquired reflectance spectra were processed quantitatively into functional tissue optical endpoints. A two-way, repeated measures analysis of variance was used to assess whether and which of the optical variables were statistically separable, across subjects with shuntodynia versus those without. Analyses indicated that intrapatient differences in vascular oxygen saturation measured between shunt sites relative to that obtained at the scar or contralateral sites was significantly lower in the pain group. We also find that the total hemoglobin concentrations at the shunt site were lowest relative to the other sites for subjects reporting pain. These findings suggest that shuntodynia pain arises in the scalp tissue around the implanted shunts and may be caused due to hypoxia and inflammation. Optically derived hemodynamic variables were statistically significantly different in subjects presenting with shuntodynia relative to those without. DRS could provide a viable mode in routine bedside monitoring of subjects with VP shunts for clinical management and assessment of shuntodynia.