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OBJECTIVE Hydrocephalus is common among children with myelomeningocele and is most frequently treated with a ventriculoperitoneal shunt (VPS). Although much is known about factors related to first shunt failure, relatively less data are available about shunt failures after the first one. The purpose of this study was to use a large data set to explore time from initial VPS placement to first shunt failure in children with myelomeningocele and to explore factors related to multiple shunt failures. METHODS Data were obtained from the National Spina Bifida Patient Registry. Children with myelomeningocele who were enrolled within the first 5 years of life and had all lifetime shunt operations recorded in the registry were included. Kaplan-Meier survival curves were constructed to evaluate time from initial shunt placement to first shunt failure. The total number of children who experienced at least 2 shunt failures was calculated. A proportional means model was performed to calculate adjusted hazard ratios (HRs) for shunt failure on the basis of sex, race/ethnicity, lesion level, and insurance status. RESULTS In total, 1691 children met the inclusion criteria. The median length of follow-up was 5.0 years. Fifty-five percent of patients (938 of 1691) experienced at least 1 shunt failure. The estimated median time from initial shunt placement to first failure was 2.34 years (95% confidence interval [CI] 1.91–3.08 years). Twenty-six percent of patients had at least 2 shunt failures, and 14% of patients had at least 3. Male children had higher likelihood of shunt revision (HR 1.25, 95% CI 1.09–1.44). Children of minority race/ethnicity had a lower likelihood of all shunt revisions (non-Hispanic Black children HR 0.74, 95% CI 0.55–0.98; Hispanic children HR 0.74, 95% CI 0.62–0.88; children of other ethnicities HR 0.80, 95% CI 0.62–1.03). CONCLUSIONS Among the children with myelomeningocele, the estimated median time to shunt failure was 2.34 years. Forty-five percent of children never had shunt failure. The observed higher likelihood of shunt revisions among males and lower likelihood among children of minority race/ethnicity illustrate a possible disparity in hydrocephalus care that warrants additional study. Overall, these results provide important information that can be used to counsel parents of children with myelomeningocele about the expected course of shunted hydrocephalus.

OBJECTIVE Severe traumatic brain injury (TBI) is a leading cause of disability and death in the pediatric population. While intracranial pressure (ICP) monitoring is the gold standard in acute neurocritical care following pediatric severe TBI, brain tissue oxygen tension (PbtO2) monitoring may also help limit secondary brain injury and improve outcomes. The authors hypothesized that pediatric patients with severe TBI and ICP + PbtO2 monitoring and treatment would have better outcomes than those who underwent ICP-only monitoring and treatment. METHODS Patients ≤ 18 years of age with severe TBI who received ICP ± PbtO2 monitoring at a quaternary children’s hospital between 1998 and 2021 were retrospectively reviewed. The relationships between conventional measurements of TBI were evaluated, i.e., ICP, cerebral perfusion pressure (CPP), and PbtO2. Differences were analyzed between patients with ICP + PbtO2 versus ICP-only monitoring on hospital and pediatric intensive care unit (PICU) length of stay (LOS), length of intubation, Pediatric Intensity Level of Therapy scale score, and functional outcome using the Glasgow Outcome Score–Extended (GOS-E) scale at 6 months postinjury. RESULTS Forty-nine patients, including 19 with ICP + PbtO2 and 30 with ICP only, were analyzed. There was a weak negative association between ICP and PbtO2 (β = −0.04). Conversely, there was a strong positive correlation between CPP ≥ 40 mm Hg and PbtO2 ≥ 15 and ≥ 20 mm Hg (β = 0.30 and β = 0.29, p < 0.001, respectively). An increased number of events of cerebral PbtO2 < 15 mm Hg or < 20 mm Hg were associated with longer hospital (p = 0.01 and p = 0.022, respectively) and PICU (p = 0.015 and p = 0.007, respectively) LOS, increased duration of mechanical ventilation (p = 0.015 when PbtO2 < 15 mm Hg), and an unfavorable 6-month GOS-E score (p = 0.045 and p = 0.022, respectively). An increased number of intracranial hypertension episodes (ICP ≥ 20 mm Hg) were associated with longer hospital (p = 0.007) and PICU (p < 0.001) LOS and longer duration of mechanical ventilation (p < 0.001). Lower minimum hourly and average daily ICP values predicted favorable GOS-E scores (p < 0.001 for both). Patients with ICP + PbtO2 monitoring experienced longer PICU LOS (p = 0.018) compared to patients with ICP-only monitoring, with no significant GOS-E score difference between groups (p = 0.733). CONCLUSIONS An increased number of cerebral hypoxic episodes and an increased number of intracranial hypertension episodes resulted in longer hospital LOS and longer duration of mechanical ventilator support. An increased number of cerebral hypoxic episodes also correlated with less favorable functional outcomes. In contrast, lower minimum hourly and average daily ICP values, but not the number of intracranial hypertension episodes, were associated with more favorable functional outcomes. There was a weak correlation between ICP and PbtO2, supporting the importance of multimodal invasive neuromonitoring in pediatric severe TBI.

OBJECTIVE The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Craniosynostosis may lead to elevated intracranial pressure, which may be implicated with impaired neurocognitive development. However, accurately measuring intracranial pressure is challenging, and patterns in craniosynostosis patients are poorly characterized. Spectral-domain optical coherence tomography may enable noninvasive assessment of intracranial pressure in pediatric patients with craniosynostosis.Pediatric patients with craniosynostosis undergoing surgical intervention between 2014 and 2019 prospectively underwent optical coherence tomographic evaluation. Intracranial pressure was directly measured intraoperatively in a subset of cases. Optical coherence tomographic parameters were compared to directly measured intracranial pressure and used for pattern assessment.Optical coherence tomography was performed in 158 subjects, among which 42 underwent direct intracranial pressure measurement during an initial cranial procedure. Maximal retinal nerve fiber layer thickness, maximal retinal thickness, and maximal anterior projection optical coherence tomographic parameters were positively correlated with intracranial pressure (p ≤ 0.001), with all parameters showing significantly higher values in patients with intracranial pressure thresholds of 15 mmHg (p0.001) and 20 mmHg (p ≤ 0.007). Patients with maximal retinal nerve fiber layer thickness and maximal anterior projection exceeding set thresholds in optical coherence tomography of either eye demonstrated 77.3 percent sensitivity and 95.0 percent specificity for detecting intracranial pressure above 15 mmHg, and 90.0 percent sensitivity and 81.3 percent specificity for detecting intracranial pressure above 20 mmHg. Patients with associated syndromes or multiple suture involvement and patients aged 9 months or older were significantly more likely to have elevated intracranial pressure above 15 mmHg (p ≤ 0.030) and above 20 mmHg (p ≤ 0.035).Spectral-domain optical coherence tomography can noninvasively detect elevated intracranial pressure in patients with craniosynostosis with reliable sensitivity and specificity. This technology may help guide decisions about the appropriate type and timing of surgical treatment.Diagnostic, I.

OBJECTIVE Children with multiple prematurely fused cranial sutures and those undergoing surgical correction later in life appear to experience worse neurocognitive outcomes, but it is unclear whether higher intracranial pressure (ICP) is implicated in this process. The purpose of this study was to elucidate the effect of age at intervention and number of involved cranial sutures on ICP, as well as to assess which cranial suture closure may be more associated with elevated ICP. METHODS The prospective craniofacial database at the authors’ institution was queried for patients undergoing initial corrective surgery for craniosynostosis in whom intraoperative measurement of ICP was obtained prior to craniectomy. Age, involved sutures, and syndromic status were analyzed in the context of measured ICP by using multiple linear regression. RESULTS Fifty patients met the inclusion criteria. Age at procedure (p = 0.028, β = +0.060 mm Hg/month) and multiple-suture involvement (p = 0.010, β = +4.175 mm Hg if multisuture) were both significantly implicated in elevated ICP. The actual number of major sutures involved was significantly correlated to ICP (p = 0.001; β = +1.687 mm Hg/suture). Among patients with single-suture involvement, there was an overall significant difference of median ICP across the suture types (p = 0.008), with metopic having the lowest (12.5 mm Hg) and sagittal having the highest (16.0 mm Hg). Patients with multiple-suture involvement had significantly higher ICP (p = 0.003; 18.5 mm Hg). Patients with craniofacial syndromes were 79.3 times more likely to have multiple-suture involvement (p < 0.001). Corrective surgery for craniosynostosis demonstrated significant intraoperative reduction of elevated ICP (all p < 0.050). CONCLUSIONS Syndromic status, older age at intervention for craniosynostosis, and multiple premature fusion of cranial sutures were associated with significantly higher ICP.

OBJECTIVE Endoscopic strip craniectomy (ESC) and spring-mediated cranioplasty (SMC) are two minimally invasive techniques for treating sagittal craniosynostosis in early infancy. Data comparing the perioperative outcomes of these two techniques are sparse. Here, the authors hypothesized that outcomes would be similar between patients undergoing SMC and those undergoing ESC and conducted a study using the multicenter Pediatric Craniofacial Surgery Perioperative Registry (PCSPR). METHODS The PCSPR was queried for infants under the age of 6 months who had undergone SMC or ESC for sagittal synostosis. SMC patients were propensity score matched 1:2 with ESC patients on age and weight. Primary outcomes were transfusion-free hospital course, intensive care unit (ICU) admission, ICU length of stay (LOS), and hospital length of stay (HLOS). The authors also obtained data points regarding spring removal. Comparisons of outcomes between matched groups were performed with multivariable regression models. RESULTS The query returned data from 676 infants who had undergone procedures from June 2012 through September 2019, comprising 580 ESC infants from 32 centers and 96 SMC infants from 5 centers. Ninety-six SMC patients were matched to 192 ESC patients. There was no difference in transfusion-free hospital course between the two groups (adjusted odds ratio [aOR] 0.78, 95% CI 0.45–1.35). SMC patients were more likely to be admitted to the ICU (aOR 7.50, 95% CI 3.75–14.99) and had longer ICU LOSs (incident rate ratio [IRR] 1.42, 95% CI 1.37–1.48) and HLOSs (IRR 1.28, 95% CI 1.17–1.39). CONCLUSIONS In this multicenter study of ESC and SMC, the authors found similar transfusion-free hospital courses; however, SMC infants had longer ICU LOSs and HLOSs. A trial comparing longer-term outcomes in SMC versus ESC would further define the roles of these two approaches in the management of sagittal craniosynostosis.

OBJECTIVE Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury. METHODS The authors retrospectively analyzed the records of pediatric patients who had been admitted to a quaternary children’s hospital with CHD palliated to bidirectional Glenn (BDG) or Fontan circulation and acute CNS injury and who had undergone intubation and mechanical ventilation. Patients who had been admitted from 2005 to 2019 were included in the study. Clinical characteristics, surgical outcomes, cardiovascular and pulmonary data, and intracranial pressure data were collected and analyzed. RESULTS Nine pediatric single-ventricle patients met the study inclusion criteria. All had undergone the BDG procedure, and the majority (78%) were status post Fontan palliation. The mean age was 7.4 years (range 1.3–17.3 years). At the time of acute CNS injury, which included traumatic brain injury, intracranial hemorrhage, and cerebral infarct, the median time interval from the most recent cardiac surgical procedure was 3 years (range 2 weeks–11 years). Maintaining normocarbia to mild hypercarbia for most patients during intubation periods did not cause neurological deterioration, and hemodynamic profiles were more favorable as compared to periods of hypocarbia. Hypocarbia was associated with unfavorable hemodynamics but was necessary to decrease intracranial hypertension. Most patients were managed using low mean airway pressure (MAWP) in order to minimize the impact on preload and cardiac output. CONCLUSIONS The authors highlight the complex neurological and cardiopulmonary interactions with respect to partial pressure of arterial CO2 (PaCO2) and MAWP when pediatric CHD patients with single-ventricle physiology require mechanical ventilation. The study data demonstrated that tight control of PaCO2 and minimizing MAWP with the goal of early extubation may be beneficial in this population. A multidisciplinary team of pediatric critical care intensivists, cardiac intensivists and anesthesiologists, and pediatric neurosurgeons and neurologists are recommended to ensure the best possible outcomes.

Head and neck positioning is a key element of craniofacial reconstructive surgery and can become challenging when intervention necessitates broad exposure of the calvarium. We present a case of craniosynostosis secondary to Apert's syndrome requiring anterior and posterior cranial vault access during surgical correction. A modified sphinx position was used that required significant neck extension. The patient had concurrent Chiari I malformation with brain stem compression so intraoperative neuromonitoring (IONM) was used to ensure that there were no negative effects on the neural elements with positioning. This highlights benefits of IONM in a setting not typically associated with its use.

Purpose: To better understand the clinical course and impact of tethered cord release surgery on patients who have previously undergone open spinal dysraphism closure in utero. Methods: This is a single-center retrospective observational study on patients undergoing tethered cord release after having previously had open fetal myelomeningocele (MMC) closure. All patients underwent tethered cord release surgery with a single neurosurgeon. A detailed analysis of the patients’ preoperative presentation, intraoperative neuromonitoring (IONM) data, and postoperative course was performed. Results: From 2009 to 2021, 51 patients who had previously undergone fetal MMC closure had tethered cord release surgery performed. On both preoperative and postoperative manual motor testing, patients were found to have on average 2 levels better than would be expected from the determined anatomic level from fetal imaging. The electrophysiologic functional level was found on average to be 2.5 levels better than the anatomical fetal level. Postoperative motor levels when tested on average at 4 months were largely unchanged when compared to preoperative levels. Unlike the motor signals, 46 (90%) of patients had unreliable or undetectable lower extremity somatosensory evoked potentials (SSEPs) prior to the tethered cord release. Conclusion: Tethered cord surgery can be safely performed in patients after open fetal MMC closure without clinical decline in manual motor testing. Patients often have functional nerve roots below the anatomic level. Sensory function appears to be more severely affected in patients leading to a consistent motor-sensory imbalance.

The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

A larger volume cranial vault expansion is likely facilitated by a low posterior cranial osteotomy beneath the torcula; however, this may impart an increased risk of venous bleeding. The authors compared the safety of infratorcular versus supratorcular osteotomy in patients undergoing posterior vault reconstruction or posterior vault distraction osteogenesis and analyzed volumetric changes.Patients undergoing initial posterior vault reconstruction or distraction osteogenesis between 2009 and 2021 at the authors' institution were grouped by occipital osteotomy location and analyzed retrospectively. Craniometric analysis was performed if patients had high-resolution computed tomography scans available within 180 days preoperatively and postoperatively.A total of 187 patients were included: 106 (57 percent) who underwent posterior vault distraction osteogenesis and 81 (43 percent) who underwent posterior vault reconstruction. Infratorcular osteotomy was more common in reconstruction [ n = 65 (80 percent)] than in distraction osteogenesis [ n = 61 (58 percent); p0.002]. Blood transfused was similar between low and high osteotomy cohorts in the distraction osteogenesis ( p = 0.285) and reconstruction ( p = 0.342) groups. However, median transfused blood volume per kilogram of patient weight was greater in the low versus high osteotomy distraction osteogenesis ( p = 0.010) and reconstruction ( p = 0.041) cohorts. Intraoperative venous sinus injury was rare. In the distraction osteogenesis cohort, there was increased median intracranial volumetric gain in the low (263 ml) compared with the high osteotomy cohort (127 ml; p = 0.043); however, when controlled for distraction distance, only a trend was observed ( p = 0.221). Patients undergoing distraction osteogenesis showed a larger median intracranial volume increase (168 ml) compared with those undergoing reconstruction (73 ml; p0.001).Infratorcular osteotomy can be performed safely in most patients undergoing cranial vault remodeling and does not appear to be associated with greater hemodynamic instability or sinus injury.Therapeutic, III.

OBJECTIVE Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

Background: Brachial plexus region tumors are rare. In this study, we reviewed our experience with resection of tumors involving or adjacent to the brachial plexus to identify patterns in presentation and outcome. Methods: We report a retrospective case series of brachial plexus tumors operated on by a single surgeon at a single institution over 15 years. Outcome data were recorded from the most recent follow-up office visit. Findings were compared to a prior internal series and comparable series in the literature. Results: From 2001 to 2016, 103 consecutive brachial plexus tumors in 98 patients met inclusion criteria. Ninety percent of patients presented with a palpable mass, and 81% had deficits in sensation, motor function, or both. Mean follow-up time was 10 months. Serious complications were infrequent. For patients with a preoperative motor deficit, the rate of postoperative motor decline was 10%. For patients without a preoperative motor deficit, the rate of postoperative motor decline was 35%, which decreased to 27% at 6 months. There were no differences in motor outcome based on extent of resection, tumor pathology, or age. Conclusion: We present one of the largest recent series of tumors of the brachial plexus region. Although the rate of worsened postoperative motor function was higher in those without preoperative weakness, the motor deficit improves over time and is no worse than antigravity strength in the majority of cases. Our findings help guide patient counseling in regard to postoperative motor function.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

As the diagnosis of Spina Bifida (SB) is often made prenatally, SB-specific prenatal counseling is needed. It is essential to provide information about medical care and lifelong impact of this diagnosis, treatment options available to women carrying fetuses affected, and resources that will assist in the care of individuals with SB. This article outlines the SB Prenatal Counseling Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida and acknowledges that further research in SB prenatal counseling is warranted.

Anterior cranial vault fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis. A minimally invasive, endoscope-assisted approach offers the additional potential benefits of less scalp scarring, decreased blood loss, and decreased scalp dissection. In this article, the authors present a novel technique for minimally invasive, endoscope-assisted fronto-orbital distraction osteogenesis.

Background Suppurative intracranial complications of sinusitis are rare events in children and can lead to harmful neurologic sequelae and significant morbidity. We sought to review the presentation and management of patients admitted at our hospital with these conditions. Methods This was a retrospective study of pediatric patients admitted to a quaternary children’s hospital from 2007 to 2019 for operative management of sinusitis with intracranial extension. Clinical characteristics, including surgical and microbiological data, were collected and analyzed. Results Fifty-four patients were included; the median age was 11.0 years, and there was a male predominance. Eighty-nine percent of patients had prior healthcare visits for the current episode of sinusitis; 46% of patients had an abnormal neurologic exam on admission. Epidural abscess and subdural empyema were the most common complications, and subdural empyema was associated with repeat surgical intervention. The dominant pathogens were Streptococcus anginosus group organisms (74%). The majority of patients completed treatment parenterally, with a median duration of therapy of 35 days. Neurological sequelae, including epilepsy or ongoing focal deficits, occurred in 22% of patients. History of seizure or an abnormal neurological exam at admission were associated with neurological sequelae. Conclusions Clinicians should consider intracranial complications of sinusitis in patients with symptoms of sinusitis for >1 week. Patients should undergo urgent neuroimaging, as neurosurgical intervention is essential for these patients. Subdural empyema was associated with repeat neurosurgical intervention. Neurological sequelae occurred in 22% of patients, and new onset seizure or an abnormal neurological exam at admission were associated with neurological sequelae.

BACKGROUND AND PURPOSE: Fetal imaging is crucial in the evaluation of open neural tube defects. The identification of intraventricular hemorrhage prenatally has unclear clinical implications. We aimed to explore fetal imaging findings in open neural tube defects and evaluate associations between intraventricular hemorrhage with prenatal and postnatal hindbrain herniation, postnatal intraventricular hemorrhage, and ventricular shunt placement. MATERIALS AND METHODS: After institutional review board approval, open neural tube defect cases evaluated by prenatal sonography between January 1, 2013 and April 24, 2018 were enrolled (n = 504). The presence of intraventricular hemorrhage and gray matter heterotopia by both prenatal sonography and MR imaging studies was used for classification. Cases of intraventricular hemorrhage had intraventricular hemorrhage without gray matter heterotopia (n = 33) and controls had neither intraventricular hemorrhage nor gray matter heterotopia (n = 229). A total of 135 subjects with findings of gray matter heterotopia were excluded. Outcomes were compared with regression analyses. RESULTS: Prenatal and postnatal hindbrain herniation and postnatal intraventricular hemorrhage were more frequent in cases of prenatal intraventricular hemorrhage compared with controls (97% versus 79%, 50% versus 25%, and 63% versus 12%, respectively). Increased third ventricular diameter, specifically >1 mm, predicted hindbrain herniation (OR = 3.7 [95% CI, 1.5–11]) independent of lateral ventricular size and prenatal intraventricular hemorrhage. Fetal closure (n = 86) was independently protective against postnatal hindbrain herniation (OR = 0.04 [95% CI, 0.01–0.15]) and postnatal intraventricular hemorrhage (OR = 0.2 [95% CI, 0.02–0.98]). Prenatal intraventricular hemorrhage was not associated with ventricular shunt placement. CONCLUSIONS: Intraventricular hemorrhage is relatively common in the prenatal evaluation of open neural tube defects. Hindbrain herniation is more common in cases of intraventricular hemorrhage, but in association with increased third ventricular size. Fetal closure reverses hindbrain herniation and decreases the rate of intraventricular hemorrhage postnatally, regardless of the presence of prenatal intraventricular hemorrhage.

OBJECTIVEFactors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.METHODSUsing the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.RESULTSThe authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16–0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34–1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12–0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02–2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31–3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67–5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.CONCLUSIONSThese data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

Corpus callosotomy is a palliative surgical option for patients with refractory epilepsy and frequent drop attacks, decreasing seizure frequency and severity by disconnecting the cerebral hemispheres. Though often successful, open surgery is not without risk. Corpus callosotomy by MRI-guided laser interstitial thermal therapy (MRgLITT) is a minimally invasive alternative to the standard open procedure. This report aims to present an illustrative case and systematically review the current literature on the surgical technique. A systematic review of the English literature through January 2019 was performed. Articles were searched by title utilizing the following key word combinations: “laser” and “callosotomy”, “callosotomy” and “ablation”, “laser” and “corpus”, “callosotomy” and “thermal”, and “stereotactic” and callosotomy”. The articles recovered were then classified by level of evidence and summarized. Fifteen papers were reviewed, of which 6 met inclusion and exclusion criteria. All included studies were classified as level IV evidence. There was a total of 13 patients ranging from 13 months to 44 years old (mean 23.5 years old). The number of laser fibers utilized ranged from 1 to 3. Engel class I was achieved in 5 patients, II in 4, III in 2, IV in 1. Reported patient follow up was 4–39.7 months (mean 15.43). Corpus callosotomy utilizing MRI-guided laser interstitial thermal therapy results in improvement in seizure frequency and severity with minimal complications. Prospective trials are needed to compare its seizure control and long-term outcomes to that of standard open callosotomy.

OBJECTIVEAlthough it is known that intersurgeon variability in offering elective surgery can have major consequences for patient morbidity and healthcare spending, data addressing variability within neurosurgery are scarce. The authors performed a prospective peer review study of randomly selected neurosurgery cases in order to assess the extent of consensus regarding the decision to offer elective surgery among attending neurosurgeons across one large academic institution.METHODSAll consecutive patients who had undergone standard inpatient surgical interventions of 1 of 4 types (craniotomy for tumor [CFT], nonacute redo CFT, first-time spine surgery with/without instrumentation, and nonacute redo spine surgery with/without instrumentation) during the period 2015–2017 were retrospectively enrolled (n = 9156 patient surgeries, n = 80 randomly selected individual cases, n = 20 index cases of each type randomly selected for review). The selected cases were scored by attending neurosurgeons using a need for surgery (NFS) score based on clinical data (patient demographics, preoperative notes, radiology reports, and operative notes; n = 616 independent case reviews). Attending neurosurgeon reviewers were blinded as to performing provider and surgical outcome. Aggregate NFS scores across various categories were measured. The authors employed a repeated-measures mixed ANOVA model with autoregressive variance structure to compute omnibus statistical tests across the various surgery types. Interrater reliability (IRR) was measured using Cohen’s kappa based on binary NFS scores.RESULTSOverall, the authors found that most of the neurosurgical procedures studied were rated as “indicated” by blinded attending neurosurgeons (mean NFS = 88.3, all p values < 0.001) with greater agreement among neurosurgeon raters than expected by chance (IRR = 81.78%, p = 0.016). Redo surgery had lower NFS scores and IRR scores than first-time surgery, both for craniotomy and spine surgery (ANOVA, all p values < 0.01). Spine surgeries with fusion had lower NFS scores than spine surgeries without fusion procedures (p < 0.01).CONCLUSIONSThere was general agreement among neurosurgeons in terms of indication for surgery; however, revision surgery of all types and spine surgery with fusion procedures had the lowest amount of decision consensus. These results should guide efforts aimed at reducing unnecessary variability in surgical practice with the goal of effective allocation of healthcare resources to advance the value paradigm in neurosurgery.

BACKGROUND Thermal flow evaluation (TFE) is a non-invasive method to assess ventriculoperitoneal shunt function. Flow detected by TFE is a negative predictor of the need for revision surgery. Further optimization of testing protocols, evaluation in multiple centers, and integration with clinical and imaging impressions prompted the current study. OBJECTIVE To compare the diagnostic accuracy of 2 TFE protocols, with micropumper (TFE+MP) or without (TFE-only), to neuro-imaging in patients emergently presenting with symptoms concerning for shunt malfunction. METHODS We performed a prospective multicenter operator-blinded trial of a consecutive series of patients who underwent evaluation for shunt malfunction. TFE was performed, and preimaging clinician impressions and imaging results were recorded. The primary outcome was shunt obstruction requiring neurosurgical revision within 7 d. Non-inferiority of the sensitivity of TFE vs neuro-imaging for detecting shunt obstruction was tested using a prospectively determined a priori margin of −2.5%. RESULTS We enrolled 406 patients at 10 centers. Of these, 68/348 (20%) evaluated with TFE+MP and 30/215 (14%) with TFE-only had shunt obstruction. The sensitivity for detecting obstruction was 100% (95% CI: 88%-100%) for TFE-only, 90% (95% CI: 80%-96%) for TFE+MP, 76% (95% CI: 65%-86%) for imaging in TFE+MP cohort, and 77% (95% CI: 58%-90%) for imaging in the TFE-only cohort. Difference in sensitivities between TFE methods and imaging did not exceed the non-inferiority margin. CONCLUSION TFE is non-inferior to imaging in ruling out shunt malfunction and may help avoid imaging and other steps. For this purpose, TFE only is favored over TFE+MP.

OBJECTIVEThe lack of a continuous, noninvasive modality for monitoring intracranial pressure (ICP) is a major obstacle in the care of pediatric patients with hydrocephalus who are at risk for intracranial hypertension. Intracranial hypertension can lead to cerebral ischemia and brain tissue hypoxia. In this study, the authors evaluated the use of near-infrared spectroscopy (NIRS) to measure regional cerebral oxygen saturation (rSO2) in symptomatic pediatric patients with hydrocephalus concerning for elevated ICP.METHODSThe authors evaluated the NIRS rSO2 trends in pediatric patients presenting with acute hydrocephalus and clinical symptoms of intracranial hypertension. NIRS rSO2 values were recorded hourly before and after neurosurgical intervention. To test for significance between preoperative and postoperative values, the authors constructed a linear regression model with the rSO2 values as the outcome and pre- and postsurgery cohorts as the independent variable, adjusted for age and sex, and used the generalized estimating equation method to account for within-subject correlation.RESULTSTwenty-two pediatric patients underwent NIRS rSO2 monitoring before and after CSF diversion surgery. The mean durations of NIRS rSO2 recording pre- and postoperatively were 13.95 and 26.82 hours, respectively. The mean pre- and postoperative rSO2 values were 73.84% and 80.65%, respectively, and the adjusted mean difference estimated from the regression model was 5.98% (adjusted p < 0.0001), suggestive of improved cerebral oxygenation after definitive neurosurgical CSF diversion treatment. Postoperatively, all patients returned to baseline neurological status with no clinical symptoms of elevated ICP.CONCLUSIONSCerebral oxygenation trends measured by NIRS in symptomatic pediatric hydrocephalus patients with intracranial hypertension generally improve after CSF diversion surgery.

Placement of ventriculoperitoneal shunts (VPS) can be challenging in children with previous VPS, previous abdominal surgery, or complex abdominal pathology. Laparoscopy can help identify intra-abdominal adhesions and determine the optimal site for insertion of the distal VPS catheter. We analyzed the feasibility and safety of laparoscopy assisted VPS placement (lapVPS) in children.A retrospective review from January/2015 to December/2018 was performed. Laparoscopy was performed via a 5 mm trans-umbilical port. Once the optimal entry site was identified, the distal end of the VPS was inserted by Seldinger technique under direct laparoscopic guidance.One hundred ten lapVPS procedures were reviewed. Median age was 1 (IQR 0.3-9.37) year. Fifty-five (50%) patients were1 year, and 15 (13.6%) were ≤28 days old. The mean age of the neonates was 14.7 (SD 7.6, range 4-28) days, and the mean weight was 3 (SD 0.39, range 2.4-3.7) kg. Thirty-one (28.2%) lapVPS were initial VPS placements, whereas 79 (71.8%) were subsequent VPS placements. Thirty-nine (35.5%) patients had a history of abdominal surgery or complex abdominal pathology. Median operative time was 36 (IQR 28-56) minutes. One hundred seven (97.3%) patients underwent successful lapVPS. Two (1.8%) patients underwent diagnostic laparoscopy, and lapVPS was deemed infeasible due to intraabdominal adhesions. One (0.9%) patient had an intestinal perforation from trocar placement. Three patients developed 5 postoperative complications that required a reoperation.Laparoscopy is a valuable tool to assess the suitability of the peritoneal cavity to accommodate a VPS. LapVPS is safe and can be performed in children of all ages.Level IV.

Background: Open maternal-fetal surgery for in utero closure of myelomeningocele (MMC) has become an accepted treatment option for prenatally diagnosed open neural tube defects. Historically, this option has been limited to women with BMI < 35 due to concern for increasing complications in patients with obesity. Objective: The aim of this study was to evaluate maternal, obstetric, and fetal/neonatal outcomes stratified by maternal BMI classification in women who undergo open maternal-fetal surgery for fetal myelomeningocele (fMMC) closure. Methods: A single-center fMMC closure registry was queried for maternal demographics, preoperative factors, fetal surgery outcomes, delivery outcomes, and neonatal outcomes. Data were stratified based on maternal BMI: Results: A total of 264 patients were analyzed, including 196 (74.2%) with BMI Conclusion: Increasing maternal BMI did not result in a negative impact on maternal, obstetric, and fetal/neonatal outcomes in a large cohort of patients undergoing open maternal-fetal surgery for fMMC closure. Further study is warranted to determine the generalizability of these results.

OBJECTIVE Complex tethered spinal cord (cTSC) release in children is often complicated by surgical site infection (SSI). Children undergoing this surgery share many similarities with patients undergoing correction for neuromuscular scoliosis, where high rates of gram-negative and polymicrobial infections have been reported. Similar organisms isolated from SSIs after cTSC release were recently demonstrated in a single-center pilot study. The purpose of this investigation was to determine if these findings are reproducible across a larger, multicenter study. METHODS A multicenter, retrospective chart review including 7 centers was conducted to identify all cases of SSI following cTSC release during a 10-year study period from 2007 to 2017. Demographic information along with specific microbial culture data and antibiotic sensitivities for each cultured organism were collected. RESULTS A total of 44 SSIs were identified from a total of 655 cases, with 78 individual organisms isolated. There was an overall SSI rate of 6.7%, with 43% polymicrobial and 66% containing at least one gram-negative organism. Half of SSIs included an organism that was resistant to cefazolin, whereas only 32% of SSIs were completely susceptible to cefazolin. CONCLUSIONS In this study, gram-negative and polymicrobial infections were responsible for the majority of SSIs following cTSC surgery, with approximately half resistant to cefazolin. Broader gram-negative antibiotic prophylaxis should be considered for this patient population.

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.

OBJECTIVES Spring-mediated cranial vault expansion (SMC) may enable less invasive treatment of sagittal craniosynostosis than conventional methods. The influence of spring characteristics such as force, length, and quantity on cranial vault outcomes are not well understood. Using in vivo and ex vivo models, we evaluate the interaction between spring force, length, and quantity on correction of scaphocephalic deformity in patients undergoing SMC. METHODS The authors retrospectively studied subjects with isolated sagittal craniosynostosis who underwent SMC between 2011 and 2019. The primary outcome measure of in vivo analysis was head shape determined by cephalic index (CI). Ex vivo experimentation analyzed the impact of spring length, bend, and thickness on resultant force. RESULTS Eighty-nine subjects underwent SMC at median 3.4 months with median preoperative CI 69% (interquartile range: 66, 71%). Twenty-six and 63 subjects underwent SMC with 2 and 3 springs, with mean total force 20.1 and 27.6 N, respectively (P < 0.001).Postoperative CI increased from 71% to 74% and 68% to 77% in subjects undergoing 2- and 3-spring cranioplasty at the 6-month timepoint, respectively (P < 0.001). Total spring force correlated to increased change in CI (P < 0.002). Spring length was inversely related to transverse cranial expansion at Postoperative day 1, however, directly related at 1 and 3 months (P < 0.001). Ex vivo modeling of spring length was inversely related to spring force regardless of spring number (P < 0.0001). Ex vivo analysis demonstrated greater resultant force when utilizing wider, thicker springs independent of spring arm length and degree of compression. CONCLUSIONS A dynamic relationship among spring characteristics including length, bend, thickness, and quantity appear to influence SMC outcomes.

Introduction: Ultrasound (US)-based indexes such as fronto-occipital ratio (FOR) can be used to obtain an acceptable estimation of ventricular volume. Patients with colpocephaly present a unique challenge due to the shape of their ventricles. In the present study, we aimed to evaluate the validity and reproducibility of the modified US-FOR index in children with Chiari II-related ventriculomegaly. Methods: In this retrospective study, we evaluated Chiari II patients younger than 1 year who underwent head US and MR or CT scans for ventriculomegaly evaluation. MR/CT-based FOR was measured in the axial plane by identifying the widest diameter of frontal horns, occipital horns, and the interparietal diameter (IPD). US-based FOR (US-FOR) was measured using the largest diameter based on the following landmarks: frontal horn and IPD in the coronal plane at the level of the foramen of Monro, IPD just superior to the Sylvian fissures, and occipital horn posterior to the thalami and inferior to the superior margins of the thalami. Intraclass correlation coefficients (ICCs) were used to evaluate inter-rater reliability, and Pearson correlation coefficients and Bland-Altman plots were applied to assess agreement between US and other two modalities. Results: Sixty-three paired US and MR/CT exams were assessed for agreement between US-FOR and MR/CT-FOR measurements. ICC showed an excellent inter-rater reliability for US-FOR (ICC = 0.99, p < 0.001) and MR/CT-FOR (ICC = 0.99, p < 0.001) measurements. The mean (range) values based on US-FOR showed a slight overestimation in comparison with MR/CT-FOR (0.51 [0.36–0.68] vs. 0.46 [0.34–0.64]). The Pearson correlation coefficient showed high cross-modality agreement for the FOR index (r = 0.83, p < 0.001). The Bland-Altman plot showed excellent concordance between US-FOR and MR/CT-FOR with a bias of 0.05 (95% CI: −0.03 to 0.13). Conclusion: US-FOR in the coronal plane is a comparable tool for evaluating ventriculomegaly in Chiari II patients when compared with MR/CT-FOR, even in the context of colpocephaly.

This study aimed to determine clinical care practices for infants at risk for posthemorrhagic hydrocephalus (PHH) across level IV neonatal intensive care units (NICUs). Cross-sectional survey that addressed center-specific surveillance, neurosurgical intervention, and follow-up practices within the Children's Hospitals Neonatal Consortium. We had a 59% (20/34 sites) response rate, with 10 sites having at least two participants. Respondents included neonatologists (53%) and neurosurgeons (35%). Most participants stated having a standard guideline for PHH (79%). Despite this, 42% of respondents perceive inconsistencies in management. Eight same-center pairs of neonatologists and neurosurgeons were used to determine response agreement. Half of these pairs disagreed on nearly all aspects of care. The greatest agreement pertained to a willingness to adopt a consensus-based protocol. Practice variation in the management of infants at risk of PHH in level IV NICUs exists despite the perception that a common practice is available and used.· Practice variation exists despite the perception that common practices are available/used for PHH.. · Our survey had same-center pairs of neonatologist and neurosurgeons to determine response agreement.. · The greatest agreement pertained to a willingness to adopt a consensus-based protocol..

BACKGROUND The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure. OBJECTIVE To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution. METHODS A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables. RESULTS From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P

Conjoined twins who are classified as craniopagus (joined at the cranium) have a rare congenital anomaly. Despite advances in surgical techniques and critical care, the rate of complications and death is still high among twins with total fusion in which the superior sagittal sinus is shared. Here, we describe total-fusion craniopagus twins who at 10 months of age underwent successful surgical separation performed by a multidisciplinary team. Computer-aided design and modeling with a three-dimensional printer, custom-designed cranial distraction and constriction devices, and intraoperative navigation techniques were used. These techniques allowed for separation of the twins at an early age and harnessed the regenerative capacity of their young brains.

Hydrocephalus is a disorder of cerebral spinal fluid (CSF) physiology that results in increased intracranial pressure (ICP). It is commonly treated via surgical placement of a shunt in the ventricles to divert CSF. Diffuse optical measurements of cerebral perfusion and oxygen extraction were acquired before and after surgical shunt placement in neonates with hydrocephalus. An invasive ICP measurement was made at the time of shunt placement. Shunting increased cerebral perfusion and decreased oxygen extraction only in infants with elevated ICP. This suggests abnormally low perfusion in patients with elevated ICP, and normal perfusion in patients without elevated ICP.

Peripheral nerves are vulnerable to a wide array of inflammatory, infiltrative, hyperplastic, degenerative, and neoplastic processes, the treatment of which depends on accurate clinical and histopathologic assessment. As they are rarely encountered in the pediatric population, peripheral nerve tumors (PNTs) often prove a diagnostic challenge in this cohort. Although many of the tumors seen in the adult population can also present in childhood, their relative contribution to morbidity is divergent between the two cohorts. In fact, lesions that dominate in adults are supplanted by lesser known but highly aggressive pathologies in children. Familiarity with the clinical presentation, diagnostic adjuncts, and therapeutic indications of pediatric PNTs is a requirement for achieving clinical success. It is the goal of this review to summarize the key features of the most commonly encountered pediatric PNTs, highlighting the key considerations in their management and recommended surgical techniques. Lesions will be presented in the context of three broad categories: benign, malignant, and reactive or hyperplastic lesions.

Neurogenic thoracic outlet syndrome (nTOS) describes a set of disorders related to symptomatic compression of the brachial plexus as it passes through the thoracic outlet into the arm. In children, nTOS is a rare and frequently delayed diagnosis due to the difficulty of communicating symptoms with adults and healthcare providers and decreased prevalence of the syndrome compared to adult nTOS. Additionally, nTOS is an uncommon variant of pediatric thoracic outlet syndrome, which is predominantly arterial or vascular in nature. In this chapter we review the presentation, associated radiographic and exam findings, and management for pediatric nTOS. We summarize the outcomes for pediatric nTOS reported in the literature to date, and also present our institutional experience managing these challenging and uncommon patients.

Congenital hydrocephalus (CH), characterized by enlarged brain ventricles, is considered a disease of excessive cerebrospinal fluid (CSF) accumulation and thereby treated with neurosurgical CSF diversion with high morbidity and failure rates. The poor neurodevelopmental outcomes and persistence of ventriculomegaly in some post-surgical patients highlight our limited knowledge of disease mechanisms. Through whole-exome sequencing of 381 patients (232 trios) with sporadic, neurosurgically treated CH, we found that damaging de novo mutations account for >17% of cases, with five different genes exhibiting a significant de novo mutation burden. In all, rare, damaging mutations with large effect contributed to similar to 22% of sporadic CH cases. Multiple CH genes are key regulators of neural stem cell biology and converge in human transcriptional networks and cell types pertinent for fetal neuro-gliogenesis. These data implicate genetic disruption of early brain development, not impaired CSF dynamics, as the primary pathomechanism of a significant number of patients with sporadic CH.

BACKGROUND Patients undergoing cranial expansion including spring-mediated cranioplasty (SMC) and cranial vault remodeling (CVR) receive costly and high acuity post-operative intensive care (ICU) given concerns over neurologic and hemodynamic vulnerability. The authors analyzed perioperative and post-operative events for patients presenting with sagittal craniosynostosis (CS) undergoing SMC and CVR in order to compare complication profiles. METHODS The authors performed a single center retrospective cohort study of patients undergoing SMC and CVR for the treatment of nonsyndromic, isolated sagittal CS from 2011 to 2018. Perioperative and post-operative factors were collected, focusing on hemodynamic instability and events necessitating ICU care. Mann-Whitney U and Fisher exact tests were used to compare data with significance defined as P