Influence of health insurance on withdrawal of life sustaining treatment for patients with isolated traumatic brain injury: a retrospective multi-center observational cohort study.

BACKGROUND: Healthcare inequities for patients with traumatic brain injury (TBI) represent a major priority area for trauma quality improvement. We hypothesized a relationship between health insurance status and timing of withdrawal of life sustaining treatment (WLST) for adults with severe TBI. METHODS: This multicenter retrospective observational cohort study utilized data collected between 2017 and 2020. We identified adult (age ≥ 16) patients with isolated severe TBI admitted participating Trauma Quality Improvement Program centers. We determined the relationship between insurance status (public, private, and uninsured) and the timing of WLST using a competing risk survival analysis framework adjusting for baseline, clinical, injury and trauma center characteristics. Multivariable cause-specific Cox regressions were used to compute adjusted hazard ratios (HR) reflecting timing of WLST, accounting for mortality events. We also quantified the between-center residual variability in WLST using the median odds ratio (MOR) and measured insurance status association with access to rehabilitation at discharge. RESULTS: We identified 42,111 adults with isolated severe TBI treated across 509 trauma centers across North America. There were 10,771 (25.6%) WLST events in the cohort and a higher unadjusted incidence of WLST events was evident in public insurance patients compared to private or uninsured groups. After adjustment, WLST occurred earlier for publicly insured (HR 1.07, 95% CI 1.02-1.12) and uninsured patients (HR 1.29, 95% CI 1.18-1.41) compared to privately insured patients. Access to rehabilitation was lower for both publicly insured and uninsured patients compared to patients with private insurance. Accounting for case-mix, the MOR was 1.49 (95% CI 1.43-1.55), reflecting significant residual between-center variation in WLST decision-making. CONCLUSIONS: Our findings highlight the presence of disparate WLST practices independently associated with health insurance status. Additionally, these results emphasize between-center variability in WLST, persisting despite adjustments for measurable patient and trauma center characteristics.

Contemporary Outcomes of a National Fetal Spina Bifida Surgery Service.

OBJECTIVE: To assess contemporary outcomes of fetuses who underwent open fetal spina bifida surgery in Canada. METHODS: Our clinical program prospectively collected outcomes of all consecutive fetuses who underwent open fetal spina bifida closure at the Ontario Fetal Center in Toronto and who were at least 1 year of age at the time of postnatal follow-up. We gathered information on the need for hydrocephalus treatment, motor function, bladder function, as well as neurodevelopment (Ages and Stages Questionnaire and Bayley's scales of infant development). Developmental outcomes were categorized as "Typical Development," "Possible Delay," or "Significantly Delayed." RESULTS: Between 2017 and 2022, 41 fetuses underwent open fetal spina bifida closure. Twenty-four patients (58.5%) responded to the questionnaire at a median age of 46.5 months. Eight children (33.3%) required CSF diversion procedures. Bladder management included clean intermittent catheterization (43.5%), spontaneous voiding (34.8%), or both (21.7%), with 43.5% needing medication for overactive bladder. All patients could sit independently, with 50% walking outside and 50% crawling indoors. Among those walking outdoors (50%), 25% did so without orthotics or aid, 58.3% with orthotics, and 16.7% required additional walking aids. Most children demonstrated typical communication and problem-solving skills (79.2%), while gross motor development was significantly delayed in 91.7% of cases. Fine motor skills varied, with 56.5% showing typical development and 34.8% possibly experiencing delays. CONCLUSIONS: This study showed a mixed developmental profile among patients who underwent open fetal spina bifida repair, consistent with the MOMs trial findings.

The Caregiver Burden of Helmet Therapy following Endoscopic Strip Craniectomy: A Phenomenological Qualitative Study.

INTRODUCTION: This cohort study aimed to elucidate the caregiver burden of helmet therapy (HT), following endoscopic strip craniectomy (ESC) to treat craniosynostosis, in an effort to inform clinicians and future caregivers navigating this therapeutic option. METHODS: Fourteen caregivers of children with positional plagiocephaly (6) and craniosynostosis treated by ESC (8) undergoing HT at a single center were recruited via convenience sampling. Using a phenomenological qualitative approach, semi-structured interviews were conducted to understand the experience of HT for caregivers. Data collection and analysis were iterative and conducted until thematic saturation was reached. RESULTS: Emerging themes revealed five domains of caregiver burden: emotional, cognitive, physical, psychosocial, and financial. No caregiver felt the therapy was too burdensome to complete. Caregivers of both groups also expressed positive aspects of HT related to support from the team, the noninvasive nature of treatment, and the outcomes of therapy. Furthermore, caregivers report overall satisfaction with the process, stating willingness to repeat the treatment with subsequent children if required. CONCLUSION: HT is associated with five major domains of caregiver burden; however, none of the caregivers regret choosing this treatment option, nor was the burden high enough to encourage treatment cessation. This study will inform future prospective analyses that will quantify real-time caregiver burden throughout HT.

Neonatal Paenibacilliosis: Paenibacillus Infection as a Novel Cause of Sepsis in Term Neonates With High Risk of Sequelae in Uganda.

BACKGROUND: Paenibacillus thiaminolyticus may be an underdiagnosed cause of neonatal sepsis. METHODS: We prospectively enrolled a cohort of 800 full-term neonates presenting with a clinical diagnosis of sepsis at 2 Ugandan hospitals. Quantitative polymerase chain reaction specific to P. thiaminolyticus and to the Paenibacillus genus were performed on the blood and cerebrospinal fluid (CSF) of 631 neonates who had both specimen types available. Neonates with Paenibacillus genus or species detected in either specimen type were considered to potentially have paenibacilliosis, (37/631, 6%). We described antenatal, perinatal, and neonatal characteristics, presenting signs, and 12-month developmental outcomes for neonates with paenibacilliosis versus clinical sepsis due to other causes. RESULTS: Median age at presentation was 3 days (interquartile range 1, 7). Fever (92%), irritability (84%), and clinical signs of seizures (51%) were common. Eleven (30%) had an adverse outcome: 5 (14%) neonates died during the first year of life; 5 of 32 (16%) survivors developed postinfectious hydrocephalus (PIH) and 1 (3%) additional survivor had neurodevelopmental impairment without hydrocephalus. CONCLUSIONS: Paenibacillus species was identified in 6% of neonates with signs of sepsis who presented to 2 Ugandan referral hospitals; 70% were P. thiaminolyticus. Improved diagnostics for neonatal sepsis are urgently needed. Optimal antibiotic treatment for this infection is unknown but ampicillin and vancomycin will be ineffective in many cases. These results highlight the need to consider local pathogen prevalence and the possibility of unusual pathogens when determining antibiotic choice for neonatal sepsis.

Outcomes following management of relapsed pediatric posterior fossa ependymoma in the molecular era.

PURPOSE: The overall survival and prognostic factors for children with multiply recurrent posterior fossa ependymoma are not well understood. We aimed to assess prognostic factors associated with survival for relapsed pediatric posterior fossa ependymoma. METHODS: An institutional database was queried for children with a primary diagnosis of posterior fossa ependymoma from 2000 to 2019. Kaplan-Meier survival analysis and Cox-proportional hazard regression were used to assess the relationship between treatment factors and overall survival. RESULTS: There were 60 patients identified; molecular subtype was available for 56, of which 49 (87.5%) were PF-A and 7 (12.5%) were PF-B. Relapse occurred in 29 patients (48%) at a mean time of 24 months following primary resection. Median 50% survival was 12.3 years for all patients and 3.3 years following diagnosis of first relapsed disease. GTR was associated with significantly improved survival following primary resection (HR 0.373, 95% CI 0.14-0.96). Presence of recurrent disease was significantly associated with worse survival (p < 0.0001). At recurrent disease diagnosis, disseminated disease was a negative prognostic factor (HR 11.0 95% CI 2.7-44) while GTR at first relapse was associated with improved survival HR 0.215 (95% CI: 0.048-0.96, p = 0.044). Beyond first relapse, the impact of GTR was not significant on survival, though surgery compared to no surgery was favorable with HR 0.155 (95% CI: 0.04-0.59). CONCLUSIONS: Disseminated disease at recurrence and extent of resection for first relapsed disease were important prognostic factors. Surgery compared to no surgery was associated with improved survival for the multiply recurrent ependymoma cohort.

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.

Simple prenatal imaging predictors for postnatal cerebrospinal fluid diversion surgery in fetuses undergoing in utero surgery for spina bifida.

OBJECTIVES: To determine simple prenatal imaging parameters that can easily be acquired to predict the need for postnatal CSF diversion (PCD) surgery in fetuses undergoing open fetal surgery for open spina bifida (OSB). METHODS: All fetuses with OSB that underwent open fetal surgery between June 2017 and June 2021 with available follow-up outcomes were included. Imaging parameters including clivus-supraocciput angle (CSA) on magnetic resonance imaging, transcerebellar diameter (TCD) and lateral ventricle size (Vp) on ultrasound (US), were collected pre- and postoperatively. The requirement for PCD surgery was determined at 1 year of age. The predictive strength of each parameter was determined by Receiver Operating Characteristic curve analysis. RESULTS: Among 36 babies eligible for the analyses, 41.7% required PCD by one year of age. Pre-operative Vp (AUC 0.71; 95% confidence interval [CI] 0.54-0.88; p = 0.03), TCD (AUC 0.72; 95% CI 0.55-0.89; p = 0.02) and CSA (AUC 0.72; 95% CI 0.51-0.93; p = 0.04) were fair predictors for PCD surgery. After fetal surgery, TCD (AUC 0.93; 95% CI 0.83-1.00; p < 0.0001) and CSA (AUC 0.94; 95% CI 0.83-1.00; p = 0.0005) were outstanding predictors of PCD, whereas post-operative Vp was a fair predictor (AUC 0.71, 95% CI 0.54-0.88, p = 0.03). CONCLUSION: Post-operative CSA and TCD were outstanding predictors for the need for PCD surgery.

Two congenital cases of pigmented epithelioid melanocytoma with unique clinical and genetic features.

Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.

Longitudinal CSF Iron Pathway Proteins in Posthemorrhagic Hydrocephalus: Associations with Ventricle Size and Neurodevelopmental Outcomes.

OBJECTIVE: Iron has been implicated in the pathogenesis of brain injury and hydrocephalus after preterm germinal matrix hemorrhage-intraventricular hemorrhage, however, it is unknown how external or endogenous intraventricular clearance of iron pathway proteins affect the outcome in this group. METHODS: This prospective multicenter cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent (1) temporary and permanent cerebrospinal fluid (CSF) diversion and (2) Bayley Scales of Infant Development-III testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes. RESULTS: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were boys. Ventricular CSF levels of hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF levels of ceruloplasmin, transferrin, haptoglobin, and hepcidin. There was an increase in CSF hemopexin during this interval. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however, higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease), and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035), and motor (p = 0.008) scores, respectively. INTERPRETATION: Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH. ANN NEUROL 2021;90:217-226.

Preservation of endocrine function after Ommaya reservoir insertion in children with cystic craniopharyngioma.

INTRODUCTION: Children with craniopharyngiomas (CP) can experience significant morbidities caused by extensive surgery and/or radiation. Ommaya reservoir insertion (ORI) into cystic CP represents a minimally invasive approach allowing immediate decompression and aims to avoid additional injuries. The purpose of this study was to determine the surgical outcome and relevance of upfront ORI (± intracystic treatment) for preservation of endocrine function. METHODS: We performed a retrospective chart review of children with CP treated at the Hospital for Sick Children between 01/01/2000 and 15/01/2020. Endocrine function was reviewed at the time of initial surgery and throughout follow-up. New endocrinological deficits related to the index procedure were defined as immediate failure (IF), whereas postoperative duration of endocrinological stability (ES) was analyzed using the Kaplan-Meier method. The rate of IF and ES was compared between the treatment groups. RESULTS: Seventy-nine patients were included and had a median age of 8.3 years (range 2.1-18.0 years); 31 were males. Fifty-three patients with upfront surgical treatment, including 29 ORI and 24 gross total or partial resections had sufficient endocrinological follow-up data. Endocrine dysfunction occurring immediately after the index procedure (IF) was observed in 15 patients (62.5%) in the resection group compared to two patients (6.8%) in the ORI group, odds ratio: 0.05 (CI: 0.01-0.26, p < 0.0001). Excluding those with immediate endocrinological deficits, mean ES after ORI was 19.4 months (CI: 11.6-34.2), compared to 13.4 months (CI:10.6-NA) after surgical resection. CONCLUSIONS: Endocrine function was preserved in patients with upfront ORI (± intracystic treatment), which was confirmed as a minimally invasive procedure with an overall low morbidity profile.

Moderate procedural confidence improvement following hands-on practice using the hemispherectomy simulator.

INTRODUCTION: Pediatric hemispherectomy is a technically demanding procedure with significant risk. There are relatively few opportunities for surgeon trainees to gain confidence in this nuanced surgery solely through traditional apprenticeship prior to independent practice. Surgeon confidence has been linked to better intraoperative performance and surgical simulation has, according to literature, resulted in improved surgeon confidence. This manuscript measures the effect of epilepsy simulation on the procedural confidence of neurosurgical trainees as a marker of future improved intraoperative performance. METHODS: Eleven neurosurgery residents and fellows were allowed to practice on a novel hemispherectomy simulator. Pre- and post-simulation procedural confidence was measured using a 10-item questionnaire, with total scores ranging from 10 (least confident) to 50 (most confident). Matched pair t-testing was performed to determine participant mean difference between the pre- and post-procedural data sets. RESULTS: The assessment tool bore a high reliability coefficient (Cronbach's α = 0.93). The procedural confidence of all the study participants increased following simulation (p value < 0.001). The overall mean increase in confidence was 7.2 ± 4.0 (mean ± standard deviation), 7.5 ± 4.7 among fellows and 6.8 ± 3.6 among residents. Procedural confidence values were higher among the fellows (26.9 ± 8.1) compared to the residents (19.0 ± 7.4). This difference in means was statistically significant (p value 0.03). A positive association was calculated between the perceived confidence and the postgraduate year of training (p value 0.005, r = 0.57). CONCLUSION: The hemispherectomy simulator moderately improves perceived confidence among neurosurgical trainees and may augment pre-operative surgical practice opportunities.

Reliability and feasibility of optic nerve point-of-care ultrasound in pediatric patients with ventricular shunts.

PURPOSE: To determine the interrater reliability of optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) via ocular ultrasound by emergency and neurosurgery providers in children with ventricular shunts, and to explore the feasibility of acquiring and measuring images. METHODS: Two novices who underwent focused training and one expert in ocular ultrasound independently acquired images and measured ONSD and ODE on the same children, 0-18 years with ventricular shunts, blinded to each other's images and measurements. Patient tolerance, image quality, and time-to-complete exams were recorded. Images meeting a priori defined quality metrics were included. Mixed models and bootstrap analysis were used to obtain inter-rater reliability and 95% confidence intervals. RESULTS: Eighty-one children were enrolled from August 2016 to July 2017, with mean age 9.6 years (SD 5.25, range 5 months-17.7 years). High-quality images (≥ 4 on 7-point quality Likert scale) were obtained in 83% of ONSD assessments and 95% of ODE assessments. The ICCONSD was 0.82 (95% CI 0.76-0.91) for right eyes and 0.73 (95% CI 0.69-0.85) for left, while ICCODE was 0.81 (95% CI 0.75-0.89) for right eyes and 0.85 (95% CI 0.79-0.91) for left. Mean study duration (both eyes) was 2:52 min (SD 54 s). CONCLUSION: Clinicians generated high-quality ocular ultrasound images with excellent interrater reliability when acquiring and measuring images of ONSD and ODE in children with ventricular shunts.

Timing of Temporizing Neurosurgical Treatment in Relation to Shunting and Neurodevelopmental Outcomes in Posthemorrhagic Ventricular Dilatation of Prematurity: A Meta-analysis.

OBJECTIVE: To determine the relationship between timing of initiation of temporizing neurosurgical treatment and rates of ventriculoperitoneal shunt (VPS) and neurodevelopmental impairment in premature infants with post-hemorrhagic ventricular dilatation (PHVD). STUDY DESIGN: We searched MEDLINE, EMBASE, CINAHL, Web of Science, the Cochrane Database of Systematic Reviews, and the Cochrane Center Register of Controlled Trials for studies that reported on premature infants with PHVD who underwent a temporizing neurosurgical procedure. The timing of the temporizing neurosurgical procedure, gestational age, birth weight, outcomes of conversion to VPS, moderate-to-severe neurodevelopmental impairment, infection, temporizing neurosurgical procedure revision, and death at discharge were extracted. RESULTS: Sixty-two full-length articles and 6 conference abstracts (n = 2533 patients) published through November 2020 were included. Pooled rate for conversion to VPS was 60.5% (95% CI, 54.9-65.8), moderate-severe neurodevelopmental impairment 34.8% (95% CI, 27.4-42.9), infection 8.2% (95% CI, 6.7-10.1), revision 14.6% (95% CI, 10.4-20.1), and death 12.9% (95% CI, 10.2-16.4). The average age at temporizing neurosurgical procedure was 24.2 ± 11.3 days. On meta-regression, older age at temporizing neurosurgical procedure was a predictor of conversion to VPS (P < .001) and neurodevelopmental impairment (P < .01). Later year of publication predicted increased survival (P < .01) and external ventricular drains were associated with more revisions (P = .001). Tests for heterogeneity reached significance for all outcomes and a qualitative review showed heterogeneity in the study inclusion and diagnosis criteria for PHVD and initiation of temporizing neurosurgical procedure. CONCLUSIONS: Later timing of temporizing neurosurgical procedure predicted higher rates of conversion to VPS and moderate-severe neurodevelopmental impairment. Outcomes were often reported relative to the number of patients who underwent a temporizing neurosurgical procedure and the criteria for study inclusion and the initiation of temporizing neurosurgical procedure varied across institutions. There is need for more comprehensive outcome reporting that includes all infants with PHVD regardless of treatment.

Endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) for hydrocephalus of infancy: a technical review.

In the twenty-first century, choroid plexus cauterization (CPC) in combination with endoscopic third ventriculostomy (ETV) has emerged as an effective treatment for some infants with hydrocephalus, leading to the favourable condition of 'shunt independence'. Herein we provide a narrative technical review considering the indications, procedural aspects, morbidity and its avoidance, postoperative care and follow-up. The CP has been the target of hydrocephalus treatment for more than a century. Early eminent neurosurgeons including Dandy, Putnam and Scarff performed CPC achieving generally poor results, and so the procedure fell out of favour. In recent years, the addition of CPC to ETV was one of the reasons greater ETV success rates were observed in Africa, compared to developed nations, and its popularity worldwide has since increased. Initial results indicate that when ETV/CPC is performed successfully, shunt independence is more likely than when ETV is undertaken alone. CPC is commonly performed using a flexible endoscope via septostomy and aims to maximally cauterize the CP. Success is more likely in infants aged >1 month, those with hydrocephalus secondary to myelomeningocele and aqueductal obstruction and those with >90% cauterized CP. Failure is more likely in those with post-haemorrhagic hydrocephalus of prematurity (PHHP), particularly those <1 month of corrected age and those with prepontine scarring. High-quality evidence comparing the efficacy of ETV/CPC with shunting is emerging, with data from ongoing and future trials offering additional promise to enhance our understanding of the true utility of ETV/CPC.

Fetal Surgery for Open Spina Bifida in Canada: Initial Results.

OBJECTIVE: Fetal myelomeningocele closure results in better infant outcomes than postnatal closure at the cost of potential prematurity and maternal morbidity. Our aim is to describe the setup of a fetal myelomeningocele closure program in Canada and document its outcomes. METHODS: We conducted a retrospective review of all open fetal myelomeningocele closure surgeries performed at the Ontario Fetal Centre in its first 3 years of operation (2017-2020). Maternal and fetal baseline characteristics, surgical details, pregnancy outcomes, and infant follow-up until 1 year of age were recorded. RESULTS: Twenty-seven women underwent fetal myelomeningocele closure surgery, 10 of whom (37%) resided outside of Ontario. Mean gestational age at surgery was 25.0 ± 0.7 weeks. All surgeries were technically uncomplicated and no fetal deaths occurred. There was a significant negative correlation between increasing experience and skin-to-skin surgical time (R²â€¯= 0.36; P = 0.001). Of the 26 patients who have delivered, 4 (15.4%) experienced preterm prelabour rupture of membranes. Mean gestational age at delivery was 34.9±3.0 weeks. All but 1 patient delivered by cesarean. Maternal complications occurred in 9 women (34.6%). There were no maternal deaths, but 3 (11.5%) infant deaths. Of the 14 surviving infants who have reached at least 1 year of age, 5 (35.7%) underwent ventriculo-peritoneal shunting. Of the 9 infants who have not yet reached 1 year of age, 3 (33.3%) underwent endoscopic third ventriculostomy and none underwent shunting. CONCLUSION: Fetal open spina bifida closure can be performed in Canada, with results similar to those reported by other international expert centres. Long-term follow-up is ongoing.

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.

Endoscopic Treatment versus Shunting for Infant Hydrocephalus in Uganda.

BACKGROUND: Postinfectious hydrocephalus in infants is a major health problem in sub-Saharan Africa. The conventional treatment is ventriculoperitoneal shunting, but surgeons are usually not immediately available to revise shunts when they fail. Endoscopic third ventriculostomy with choroid plexus cauterization (ETV-CPC) is an alternative treatment that is less subject to late failure but is also less likely than shunting to result in a reduction in ventricular size that might facilitate better brain growth and cognitive outcomes. METHODS: We conducted a randomized trial to evaluate cognitive outcomes after ETV-CPC versus ventriculoperitoneal shunting in Ugandan infants with postinfectious hydrocephalus. The primary outcome was the Bayley Scales of Infant Development, Third Edition (BSID-3), cognitive scaled score 12 months after surgery (scores range from 1 to 19, with higher scores indicating better performance). The secondary outcomes were BSID-3 motor and language scores, treatment failure (defined as treatment-related death or the need for repeat surgery), and brain volume measured on computed tomography. RESULTS: A total of 100 infants were enrolled; 51 were randomly assigned to undergo ETV-CPC, and 49 were assigned to undergo ventriculoperitoneal shunting. The median BSID-3 cognitive scores at 12 months did not differ significantly between the treatment groups (a score of 4 for ETV-CPC and 2 for ventriculoperitoneal shunting; Hodges-Lehmann estimated difference, 0; 95% confidence interval [CI], -2 to 0; P=0.35). There was no significant difference between the ETV-CPC group and the ventriculoperitoneal-shunt group in BSID-3 motor or language scores, rates of treatment failure (35% and 24%, respectively; hazard ratio, 0.7; 95% CI, 0.3 to 1.5; P=0.24), or brain volume (z score, -2.4 and -2.1, respectively; estimated difference, 0.3; 95% CI, -0.3 to 1.0; P=0.12). CONCLUSIONS: This single-center study involving Ugandan infants with postinfectious hydrocephalus showed no significant difference between endoscopic ETV-CPC and ventriculoperitoneal shunting with regard to cognitive outcomes at 12 months. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT01936272 .).

Treatment Strategies and Related Outcomes for Brain Arteriovenous Malformations in Children: A Systematic Review and Meta-Analysis.

OBJECTIVE. The objective of this study was to assess the available evidence in the literature regarding treatment outcomes for pediatric patients with brain arteriovenous malformation (bAVM) with the aim of providing practice guidelines for treatment decisions and highlighting research areas that need attention. MATERIALS AND METHODS. Keyword searches for studies published from January 1, 1981, to April 16, 2018, were performed in MEDLINE, Embase, and Web of Science. Predefined inclusion criteria were used to identify studies. Poisson regression analysis for associations between patient and bAVM characteristics and treatment outcomes. RESULTS. We identified 34 articles comprising 2158 children with bAVM who underwent treatment or observation. The mean age of the study cohort was 12.0 ± 1.6 (SD) years, and 48.1% of the patients were female; 64.3% of bAVMs were hemorrhagic at presentation. The mean follow-up was 50.6 ± 32.3 months. Overall, the meta-analysis of pooled data showed an obliteration rate of 69.8% (95% CI, 62.9-75.9%), recurrence rate of 2.2% (95% CI, 1.1-4.3%), and mortality rate of 2.4%. The pooled complication rate was 22.5% (95% CI, 15.7-31.1%) after surgery, 26.4% (95% CI, 15.2-41.9%) after embolization, and 27.1% (95% CI, 18.1-38.4%) after radiosurgery. Mortality was not associated with age, sex, or hemorrhage; however, recurrence after treatment was inversely associated with age. Complication and mortality rates were reduced for multimodal treatments. For patients with bAVM treated with observation only, complication and mortality rates were 35.9% and 23.5%, respectively. CONCLUSION. Multimodality treatments for pediatric bAVM had lower mortality and complication rates than individual treatments. However, there is a lack of evidence for long-term outcomes. The mortality rate was highest in conservatively managed patients (i.e., observation only). Further research directly comparing different treatment modalities for recurrence and complications is warranted. Gathering data prospectively through multiinstitutional registries will be key to provide strong evidence.

Fetal spina bifida: What we tell the parents.

Worldwide, about 150 000 infants are born with spina bifida yearly, making this condition one of the most common fetal central nervous system anomalies compatible with life. Over the last decade, major changes have been introduced in the prenatal diagnosis and management of spina bifida. In this review, we provide a brief summary of the current management of fetal spina bifida and present essential information that should be provided to expecting parents when their fetus has been diagnosed with spina bifida. This information is focused around common parental questions, as encountered in our typical clinical practice, to facilitate knowledge translation.

Stopping CSF leaks at external ventricular drain exit sites: a technical note.

PURPOSE: Cerebrospinal fluid (CSF) leak is a major risk factor for external ventricular drain infection. Here, we present a surgical technique to reduce the possibility of CSF leak at the external ventricular drain (EVD) exit site in high-risk patients. METHODS: Vertical mattress sutures are placed circumferentially around the EVD catheter tract as it passes under the skin, so to close off the tract along which CSF may travel toward the exit site. RESULTS: Specific case examples are discussed where the introduction of this technique was found to be helpful in stopping exit-site CSF leak. CONCLUSION: In our experience, these surgical steps are useful adjuncts that should be considered in all high-risk patients undergoing EVD placement.