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Objective: Hydrocephalus is a lifelong condition punctuated in most cases by unpredictable hospital admissions for surgical maintenance. It occupies more of the attention of the pediatric neurosurgeon than any other condition. Benchmarks for the measurement of outcomes are of interest to patients, their families, and the healthcare system. Compared to other metrics, 30-day outcomes require modest resources to collect, are conceptually transparent, and are responsive to process improvement., Methods: The National Surgical Quality Improvement Program-Pediatric of the American College of Surgeons was queried for operations for hydrocephalus in the years 2013 through 2020. Demographic data and data regarding comorbidities were collected. Thirty-day rates of return to the operating room, of shunt infection, and of readmission to hospital were analyzed on a univariate basis and in multivariate models., Results: There were 29,098 surgical procedures in the sample, including 10,135 shunt insertions, 16,420 shunt revisions, and 2543 endoscopic third ventriculostomies. The overall 30-day reoperation rate was 10.3%. The most powerful associations were with the nature of the index procedure and with a history of extreme prematurity. The 30-day shunt infection rate was 1.80%. The major associations were with young age, major cardiac risk factors, nutritional support, and ventilator dependence. The 30-day readmission rate was 17.2%. The nature of the index procedure, current malignancy, nutritional support, and recent steroid administration were major associations. Comorbidities negatively associated with these outcomes were highly prevalent., Conclusions: Precise benchmarks for important 30-day outcomes have been calculated from a very large sample of operations for hydrocephalus in childhood.

Objective: The prevalence, management, and outcomes of hydrocephalus remain underexplored in Africa. This study aimed to analyze demographic and clinical features, evaluate treatment strategies, and assess neurological outcomes of pediatric hydrocephalus in Africa., Methods: A systematic review of the literature using the PubMed, Google Scholar, and Web of Science electronic databases was completed according to the PRISMA guidelines to identify articles describing pediatric patients in Africa with hydrocephalus., Results: Seventy-four retrospective and prospective studies and 33 case reports involving 12,355 patients were included. In 54 retrospective articles reporting patient demographics, 53.8% (3926/7297) were male with a mean age of 12.3 months. Nineteen studies reported macrocephaly (80.2%, 1639/2043) as the most common presentation. The etiology of hydrocephalus was reported as postinfectious (41.0%, 2303/5614) across 27 articles and congenital (48.6%, 1246/2563) in 10 articles. Eleven articles reported 46.7% (609/1305) of patients had communicating hydrocephalus while 53.3% (696/1305) had obstructive hydrocephalus. Diagnostic imaging included CT (76.1%, 2435/3202; n = 29 articles), ultrasonography (72.9%, 2043/2801; n = 15 articles), and MRI (44.8%, 549/1225; n = 11 articles). In 51 articles, 83.1% (7365/8865) of patients had ventriculoperitoneal shunting (VPS) while 33 articles described 54.1% (2795/5169) receiving endoscopic third ventriculostomy (ETV) for hydrocephalus surgical management. Postoperative complications included sepsis (6.9%, 29/421; n = 4 articles), surgical site infections (5.1%, 11/218; n = 4 articles), and CSF leaks (2.0%, 15/748; n = 8 articles). Shunt-related complications included infections (4.3%, 117/2717; n = 21 articles) and blockages (4.1%, 34/829; n = 6 studies). In 15 articles, 9.0% (301/3358) of patients with shunts had revisions. The mean follow-up duration was 18.9 ± 16.7 months with an overall mortality rate of 7.4% (397/5383; n = 29 articles). In the analysis of comparative studies, the 160 patients undergoing ETV demonstrated significantly higher odds of a successful operation (OR 1.54, 95% CI 0.51-4.69; p = 0.03) and neurological improvement at last follow-up (OR 3.36, 95% CI 0.46-24.79; p < 0.01) compared with the 158 who received VPS, but no significant differences were observed for complications and mortality between the two groups (p > 0.05)., Conclusions: This review offers a comprehensive summary of pediatric hydrocephalus in Africa, highlighting shunting as the primary treatment. However, the observed variations across studies highlight the need to establish standardized guidelines for reporting patient characteristics, management strategies, and outcomes to ensure consistency and comparability in articles.

Objective: The objective of this study was to report the results of a bibliometric analysis on the modern corpus of literature pertaining to endoscopic third ventriculostomy (ETV). Prior bibliometrics studies on ETV have focused on highly cited articles, but an advanced bibliometric analysis has not yet been conducted., Methods: The authors queried the Web of Science (WoS) for (ALL = (endoscopic third ventriculostomy)) OR (ALL = (ETV) AND ALL = (neurosurgery)). Articles or reviews published in English were included. Articles, along with their metadata, were exported. Statistical, bibliometric, and network analyses were performed using the Bibliometrix R package and various Python packages. Reference publication year spectroscopy (RPYS), a method that analyzes the frequency with which references are cited in terms of these references' publication years, was employed to explore the historical roots of the field., Results: Between 1994 and 2023, 1663 documents were identified (1382 articles) from 5457 authors. The mean annual growth rate of publications was 4.9%. International coauthorship increased 4-fold over this time period and was noted for 18.95% of published studies from 2011 to 2023. We observed that Child's Nervous System published the most articles, Journal of Neurosurgery (JNS) articles were cited most frequently, and JNS: Pediatrics articles had the highest impact. Female coauthorship increased from < 1% of published studies before 2000 to 19% by 2022, with an increase in female first authorship from 2% in 2005 to 22% in 2022 and at least 1 female coauthor rising from 3% in 2000 to 68% in 2022. Likewise, minority authorship has increased, as in the early ETV literature > 75% of authors were White while currently only 43% are White. The authors of this study also identified the most prolific authors on the subject. Early in the publication record, etiological and technical terms such as "aqueductal stenosis" and "technical note" predominated. More recently, "complications," "failure," "success," "neuroendoscopy," and "choroid plexus cauterization" were prominent. Utilizing RPYS, the authors identified 32 articles that comprise the foundational articles on ETV, published between 1966 and 2010., Conclusions: Interest in ETV increased in the 1990s with the advent of advanced endoscopic technologies-particularly digital video. The focus of research has shifted from etiology to outcomes, complication management, and technical mastery.

Objective: The outcome of endoscopic third ventriculostomy (ETV) in children who had previously received shunts and who were experiencing shunt dysfunction is still discussed in terms of efficacy (success rate from 40% to 80%) and safety (0%-32.5% of complications). Reported predictive factors of secondary ETV failure are age, early onset of hydrocephalus, and prematurity. The best surgical strategy in the different subgroups of patients with shunt dysfunction is still debated. Therefore, the authors aimed to identify subgroups of patients in whom shunt treatment was associated with favorable outcome of ETV, to define the role of ETV in patients with global rostral midbrain dysfunction syndrome., Methods: This study was a monocentric retrospective case series and a meta-analysis of children who had previously received shunts and who underwent secondary ETV for shunt dysfunction between 2012 and 2022. Clinical and MRI features were examined, along with surgical outcome, etiology of hydrocephalus, and preoperative ETV Success Score. Univariate and multivariate analyses were performed to find predictors of outcome of secondary ETV. Youden's J index was calculated on age distribution to find an optimal age cutoff. Systematic review of the literature and a meta-analysis were performed according to the PRISMA statement., Results: Seventy consecutive patients were included. The overall success rate of secondary ETV was 63%. Primary obstructive hydrocephalus, age ≥ 36 months, and the presence of aqueductal obstruction were predictors of ETV success. Multivariate analysis found that age < 36 months, primary inflammatory hydrocephalus, and presence of fourth ventricular obstruction were associated with ETV failure. All patients with global rostral midbrain dysfunction syndrome experienced clinical and radiological improvement after ETV. The meta-analysis showed that postinflammatory etiology and age < 36 months were predictors of ETV failure., Conclusions: ETV is safe and effective for children with obstructive hydrocephalus experiencing shunt dysfunction, notably in cases of primary obstructive hydrocephalus with aqueductal stenosis, and among children whose age was ≥ 36 months who had postinflammatory hydrocephalus.

Objective: The effect of congenital cardiac status on endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) failure in hydrocephalic infants is unknown. Because cardiac status in infants can impact central venous pressure (CVP), it is possible that congenital heart disease (CHD) and congenital cardiac anomalies may render these cerebrospinal fluid diversion interventions more susceptible to failure. Correspondingly, the aim of this study was to determine how CHD and congenital cardiac anomalies may impact the failure of these initial interventions., Methods: A retrospective review of the Nationwide Inpatient Sample (NIS) database was conducted. Infants (aged < 1 year) with known congenital cardiac status managed with either ETV or VPS were included. Quantitative data were compared using either parametric or nonparametric methods, and failure rates were modeled using univariable and multivariable regression analyses., Results: A total of 18,763 infants treated with ETV or VPS for hydrocephalus were identified in our search, with ETV used to treat 7657 (41%) patients and VPS used to treat 11,106 (59%). There were 6722 (36%) patients who presented with CHD at admission, and a total of 25 unique congenital cardiac anomalies were detected across the cohort. Overall, the most common anomaly was patent ductus arteriosus (PDA) in 4990 (27%) patients, followed by atrial septal defect (ASD) in 2437 (13%) patients and pulmonary hypertension in 810 (4%) patients. With respect to initial intervention failure, 3869 (21%) patients required repeat surgical intervention during admission. This was significantly more common in the ETV group than the VPS group (36% vs 10%, p < 0.01). In both the ETV and VPS groups, CHD (p < 0.01), including all congenital cardiac anomalies, was an independent and significant predictor of failure. ASD (p < 0.01) and PDA (p < 0.01) both significantly predicted ETV failure, and PDA (p < 0.01) and pulmonary hypertension (p = 0.02) both significantly predicted VPS failure., Conclusions: These results indicate that congenital cardiac status predicts ETV and VPS failure in patients with infantile hydrocephalus. The authors hypothesized that this finding was primarily due to changes in CVP; however, this may not be completely universal across both interventions and all congenital cardiac anomalies. Future studies about optimization of congenital cardiac status with ETV and VPS are required to understand the practical significance of these findings.

Objective: Posthemorrhagic hydrocephalus (PHH) is associated with significant morbidity, smaller hippocampal volumes, and impaired neurodevelopment in preterm infants. The timing of temporary CSF (tCSF) diversion has been studied; however, the optimal time for permanent CSF (pCSF) diversion is unknown. The objective of this study was to determine whether cumulative ventricle size or timing of pCSF diversion is associated with neurodevelopmental outcome and hippocampal size in preterm infants with PHH., Methods: Twenty-five very preterm neonates (born at ≤ 32 weeks' gestational age) with high-grade intraventricular hemorrhage (IVH), subsequent PHH, and pCSF diversion with a ventriculoperitoneal shunt (n = 20) or endoscopic third ventriculostomy (n = 5) were followed until 2 years of age. Infants underwent serial cranial ultrasounds from birth until 1 year after pCSF diversion, brain MRI at term-equivalent age, and assessment based on the Bayley Scales of Infant and Toddler Development, Third Edition, at 2 years of age. Frontooccipital horn ratio (FOHR) measurements were derived from cranial ultrasounds and term-equivalent brain MRI. Hippocampal volumes were segmented and calculated from term-equivalent brain MRI. Cumulative ventricle size until the time of pCSF diversion was estimated using FOHR measurements from each cranial ultrasound performed prior to permanent intervention., Results: The average gestational ages at tCSF and pCSF diversion were 28.9 and 39.0 weeks, respectively. An earlier chronological age at the time of pCSF diversion was associated with larger right hippocampal volumes on term-equivalent MRI (Pearson's r = -0.403, p = 0.046) and improved cognitive (r = -0.554, p = 0.047), motor (r = -0.487, p = 0.048), and language (r = -0.414, p = 0.021) outcomes at 2 years of age. Additionally, a smaller cumulative ventricle size from birth to pCSF diversion was associated with larger right hippocampal volumes (r = -0.483, p = 0.014) and improved cognitive (r = -0.711, p = 0.001), motor (r = -0.675, p = 0.003), and language (r = -0.618, p = 0.011) outcomes. There was no relationship between time to tCSF diversion or cumulative ventricle size prior to tCSF diversion and neurodevelopmental outcome or hippocampal size. Finally, a smaller cumulative ventricular size prior to either tCSF diversion or pCSF diversion was associated with a smaller ventricular size 1 year after pCSF diversion (r = 0.422, p = 0.040, R2 = 0.178 and r = 0.519, p = 0.009, R2 = 0.269, respectively)., Conclusions: In infants with PHH, a smaller cumulative ventricular size and shorter time to pCSF diversion were associated with larger right hippocampal volumes, improved neurocognitive outcomes, and reduced long-term ventriculomegaly. Future prospective randomized studies are needed to confirm these findings.

Objective: Treating Dandy-Walker syndrome-related hydrocephalus (DWSH) involves either a CSF shunt-based or endoscopic third ventriculostomy (ETV)-based procedure. However, comparative investigations are lacking. This study aimed to compare shunt-based and ETV-based treatment strategies utilizing archival data from the Hydrocephalus Clinical Research Network (HCRN) registry., Methods: A retrospective review of prospectively collected and maintained data on children with DWSH, available from the HCRN registry (14 sites, 2008-2018), was performed. The primary outcome was revision-free survival of the initial surgical intervention. The primary exposure was either shunt-based (i.e., cystoperitoneal shunt [CPS], ventriculoperitoneal shunt [VPS], and/or dual-compartment) or ETV-based (i.e., ETV alone or with choroid plexus cauterization [CPC]) initial surgical treatment. Primary analysis included multivariable Cox proportional hazards models., Results: Of 8400 HCRN patients, 151 (1.8%) had DWSH. Among these, the 102 patients who underwent shunt placement (79 VPSs, 16 CPSs, 3 other, and 4 multiple proximal catheter) were younger (6.6 vs 18.8 months, p < 0.001) and more frequently had 1 or more comorbidities (37.3% vs 14.3%, p = 0.005) than the 49 ETV-treated children (28 ETV-CPC). Fifty percent of the shunt-based and 51% of the ETV-based treatments failed. Notably, 100% (4/4) of the dual-compartment shunts failed. Adjusting for age, baseline ventricular size, and comorbidities, ETV-based treatment was not significantly associated with earlier failure compared with shunt-based treatment (HR for failure 1.32, 95% CI 0.77-2.26; p = 0.321). Complication rates were low: 4.9% and 6.1% (p = 0.715) for shunt- and ETV-based procedures, respectively. There was no difference in survival between ETV-CPC- and ETV-based treatment when adjusting for age (HR for failure 0.86, 95% CI 0.29-2.55, p = 0.783)., Conclusions: In this North American, multicenter, prospective database review, shunt-based and ETV-based primary treatment strategies of DWSH appear similarly durable. Pediatric neurosurgeons can reasonably consider ETV-based initial treatment given the similar durability and the low complication rate. However, given the observational nature of this study, the treating surgeon might need to consider subgroups that were too small for a separate analysis. Very young children with comorbidities were more commonly treated with shunts, and older children with fewer comorbidities were offered ETV-based treatment. Future studies may determine preoperative characteristics associated with ETV treatment success in this population.

Objective: The objectives of this study were to determine the quality of life of a pediatric cohort with hydrocephalus treated by endoscopic third ventriculostomy (ETV), using the Hydrocephalus Outcome Questionnaire-Spanish version (HOQ-Sv), and study the clinical and radiological factors associated with a better or worse functional status., Methods: This cross-sectional study was undertaken between September 2018 and December 2019. It comprised a series of 40 patients ranging from 5 to 18 years old with hydrocephalus treated by ETV. ETV was considered to be successful if there was no need for surgery for the treatment of hydrocephalus after a minimum follow-up of 6 months. The clinical variables included gender, age at hydrocephalus diagnosis, age at the time of ETV, age at completion of the questionnaire, etiology and type of hydrocephalus (communicating or not), prior shunt, repeat ETV, number of neurosurgical procedures, number of epileptic seizures, presenting signs, and follow-up duration until last office revision. The radiological variables were the Evans Index and the pre- and posttreatment frontooccipital horn ratio. An analysis was conducted of the association between all these variables and the various dimensions on the HOQ-Sv, completed by the parents of the patients via telephone or in the outpatient offices., Results: The mean age of the children at ETV was 7 years (range 7-194 months), and on completing the questionnaire was 12 years (range 60-216 months). The mean HOQ scores were as follows: overall 0.82, physical domain 0.86, social-emotional (SE) domain 0.84, cognitive domain 0.75, and utility score 0.90. A history of epileptic crises was a predictive factor for a worse score overall and in the SE and cognitive domains. Factors related to a worse score in the physical domain were a previous shunt, the number of procedures, and the etiology and type of hydrocephalus. The mean follow-up duration from ETV to the last office visit was 5 years (64.5 months). No association was found between the degree of ventricular reduction and the quality of life., Conclusions: The factors related to a worse score in the different dimensions of the HOQ were a history of epileptic seizures, the number of procedures, communicating hydrocephalus, and having had a previous valve. No association was found between the reduction in ventricular size and the quality of life as measured on the HOQ-Sv.

Objective: Children with posterior fossa tumors (PFTs) may present with hydrocephalus. Persistent (or new) hydrocephalus is common after PFT resection. Endoscopic third ventriculostomy (ETV) is sometimes performed prior to resection to 1) temporize hydrocephalus prior to resection and 2) prophylactically treat post-resection hydrocephalus. The objective of this study was to establish, in a historical cohort study of pediatric patients who underwent primary craniotomy for PFT resection, whether or not pre-resection ETV prevents the need for post-resection CSF diversion to manage hydrocephalus., Methods: The authors interrogated their prospectively maintained surgical neuro-oncology database to find all primary PFT resections from a single tertiary pediatric neurosurgery unit. These data were reviewed and supplemented with data from case notes and radiological review. The modified Canadian Preoperative Prediction Rule for Hydrocephalus (mCPPRH) score was retrospectively calculated for all patients. The primary outcome was the need for any form of postoperative CSF diversion within 6 months of PFT resection (including ventriculoperitoneal shunting, ETV, external ventricular drainage [EVD], and lumbar drainage [LD]). This was considered an ETV failure in the ETV group. The secondary outcomes were time to CSF diversion, shunt dependence at 6 months, and complications of ETV. Statistical analysis was done in RStudio, with significance defined as p < 0.05., Results: A total of 95 patients were included in the study. There were 28 patients in the ETV group and 67 in the non-ETV group. Patients in the ETV group were younger (median age 5 vs 7 years, p = 0.04) and had more severe preoperative hydrocephalus (mean frontal-occipital horn ratio 0.45 vs 0.41 in the non-ETV group, p = 0.003) and higher mCPPRH scores (mean 4.42 vs 2.66, p < 0.001). The groups were similar in terms of sex and tumor histology. The overall rate of post-resection CSF diversion of any kind (shunt, repeat ETV, LD, or EVD) in the entire cohort was 25.26%. Post-resection CSF diversion was needed in 32% of patients in the ETV group and in 22% of the patients in the non-ETV group (p > 0.05). Shunt dependence at 6 months was seen in 21% of the ETV group and 16% of the non-ETV group (p > 0.05). The median time to ETV failure was 9 days. ETV failure correlated with patients with ependymoma (p = 0.02). Children who had ETV failure had higher mCPPRH scores than the ETV success group (5.67 vs 3.84, p = 0.04)., Conclusions: Pre-resection ETV did not reliably prevent the need for post-resection CSF diversion. ETV was more likely to fail in children with ependymoma and those with higher mCPPRH scores. Based on the findings of this study, the authors will change the practice at their institution; pre-resection ETV will now be performed based on a newly defined protocol.

OBJECTIVEThe goal of this study was to clarify the long-term outcome of endoscopic third ventriculostomy (ETV) in pediatric hydrocephalus in light of the ETV Success Score (ETVSS), shunt dependency, and intellectual development.METHODSThe authors retrospectively analyzed pediatric patients with hydrocephalus who underwent ETV between 2002 and 2012 and who were followed for longer than 5 years as a single-center cohort. The data of the patients' pre- and postoperative status were collected. The relationships between ETVSS and the full-scale IQ as well as shunt dependency were analyzed. The usefulness of ETVSS for repeat ETV and the change of radiological parameters of ventricle size before and after ETV were also analyzed. The success of ETV was defined as no requirement for further CSF diversion procedures.RESULTSFifty ETVs were performed in 40 patients. The average ETVSS was 61 and the success rate at 6 months was 64%. The mean follow-up was 9.9 years (5.2-15.3 years), and the long-term success rate of ETV was 50%. The Kaplan-Meier survival curve continued to show a statistically significant difference among patients with a low, moderate, and high ETVSS, even after 6 months (p = 0.002). After 15 months from the initial ETV, no patients required additional CSF diversion surgery. There was no statistical significance between ETVSS and the long-term full-scale IQ or shunt dependency (p = 0.34 and 0.12, respectively). The radiological improvement in ventricle size was not associated with better future educational outcome.CONCLUSIONSThe ETVSS was correlated with the long-term success rate. After 15 months from the initial ETV, no patients required an additional CSF diversion procedure. The ETVSS was not considered to be correlated with long-term intellectual status.

OBJECTIVE Endoscopic third ventriculostomy (ETV) is an effective treatment for obstructive hydrocephalus and avoids the risk for foreign-body infection associated with ventriculoperitoneal (VP) shunts. The short-term failure rate of ETV strongly depends on the indications for its use but is generally thought to be lower in the long term than that of VP shunts. However, few studies are available with long-term follow-up data of ETV for hydrocephalus in children. The authors reviewed the long-term success of ETV at their institution to investigate the rate of any late failures of this procedure. METHODS Between April 1998 and June 2006, 113 children (including neonates and children up to 16 years old) had primary or secondary ETV for different causes of hydrocephalus. The patients' medical records and the authors' electronic operation database were reviewed for evidence of additional surgery (i.e., repeat ETV or VP shunt insertion). These records were checked at both the pediatric and adult neurosurgical hospitals for those patients who had their care transferred to adult services. RESULTS The median length of follow-up was 8.25 years (range 1 month to 16 years). Long-term follow-up data for 96 patients were available, 47 (49%) of whom had additional ETV or VP shunt insertion for ETV failure. Twenty patients (21%) had a second procedure within 1 month, 17 patients (18%) between 1 and 12 months, 7 patients (7%) between 1 and 5 years, and 3 patients (3%) between 5 and 8 years. CONCLUSIONS In the authors' series, ETV had an initial early failure rate for the treatment of pediatric hydrocephalus as reported previously, and this rate significantly depended on patient age and hydrocephalus etiology. Once stabilized and effective, ETV appeared to be durable but not guaranteed, and some late decline in effectiveness was observed, with some ETV failures occurring many years later. Thus, successful ETV in children cannot be guaranteed for life, and some form of follow-up is recommended long term into adulthood.