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Background Optic pathway gliomas (OPGs) are low-grade tumors of the white matter of the visual system with a highly variable clinical course. The aim of the study was to generate a magnetic resonance imaging (MRI)-based predictive model of OPG tumor progression using advanced image analysis and machine learning techniques. Methods We performed a retrospective case–control study of OPG patients managed between 2009 and 2015 at an academic children’s hospital. Progression was defined as radiographic tumor growth or vision decline. To generate the model, optic nerves were manually highlighted and optic radiations (ORs) were segmented using diffusion tractography tools. For each patient, intensity distributions were obtained from within the segmented regions on all imaging sequences, including derivatives of diffusion tensor imaging (DTI). A machine learning algorithm determined the combination of features most predictive of progression. Results Nineteen OPG patients with progression were matched to 19 OPG patients without progression. The mean time between most recent follow-up and most recently analyzed MRI was 3.5 ± 1.7 years. Eighty-three MRI studies and 532 extracted features were included. The predictive model achieved an accuracy of 86%, sensitivity of 89%, and specificity of 81%. Fractional anisotropy of the ORs was among the most predictive features (area under the curve 0.83, P < 0.05). Conclusions Our findings show that image analysis and machine learning can be applied to OPGs to generate a MRI-based predictive model with high accuracy. As OPGs grow along the visual pathway, the most predictive features relate to white matter changes as detected by DTI, especially within ORs.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Study design This is a level IV retrospective descriptive study at a single institution. Objective The objective of the study was to determine the preoperative signs or symptoms prompting cervicomedullary imaging in Jeune syndrome. Summary of background data Jeune syndrome is a rare autosomal recessive disorder that results in pulmonary compromise from abnormal development of the thorax. Multiple medical comorbidities complicate timely diagnosis of cervicomedullary stenosis, which neurologically jeopardizes this patient population with regards to improper cervical manipulation. Currently, explicit screening of the cervicomedullary junction is not advocated in national guidelines. Methods The User Reporting Workbench and Center for Thoracic Insufficiency Syndrome (CTIS) Safety Registry was queried for patients with Jeune syndrome under the age of 18 with cervicomedullary stenosis with or without suboccipital craniectomy/craniotomy evaluated at the authors' institution from January 1, 2007 to August 21, 2018. The primary outcome was the clinical reason for cervicomedullary screening. Secondary outcomes were: age at time of surgery, preoperative myelopathy (spasticity, urinary retention), hydrocephalus, postoperative deficits (respiratory, motor, swallowing difficulty), and need for cervical fusion. Results Of 32 patients with Jeune syndrome, four (12.5%) had cervicomedullary stenosis requiring decompression. The average age at surgery was 5.25 months (2-9 mo). Two patients underwent imaging due to desaturation events while the other two patients were diagnosed with cervical stenosis as an incidental finding. No patients exhibited clinical myelopathy. Two patients had baseline preoperative swallowing difficulties. None of the patients postoperatively required cervical fusions, nor did they exhibit respiratory deficits, motor deficits, or worsening swallowing difficulties. Conclusion Jeune patients should be routinely screened for cervicomedullary stenosis and undergo subsequent prophylactic decompression to minimize or eliminate the development of irreversible neurologic compromise. Level of evidence 4.

OBJECTIVE Cerebral ventricular shunt failure is common and presents with symptoms that range from headaches to death. The combination of Diamox (acetazolamide), Decadron (dexamethasone), and Zantac (ranitidine) (DDZ) is used at our institution to medically stabilize pediatric patients presenting with symptomatic shunt failure before shunt revision. We describe our experience of this drug combination as a temporizing measure to decrease symptoms associated with shunt failure. METHODS We performed a single-center retrospective chart review of patients younger than 18 years with ventricular shunt failure who underwent a shunt revision between January 2015 to October 2017 and received DDZ before surgery. The outcome variables evaluated included pre-DDZ and post-DDZ clinical symptoms, pain scores, and vital signs. RESULTS There were 112 cases that received DDZ before shunt revision. The 4 most commonly reported symptoms were analyzed. Headache was observed in 42 cases pre-DDZ, and post-DDZ there was a 71% reduction in headache (P < 0.0001); emesis was reported pre-DDZ in 76 cases, and post-DDZ there was an 83% reduction (P < 0.0001); irritability was noted pre-DDZ in 30 cases, and post-DDZ there was a 77% reduction (P = 0.0003); lethargy pre-DDZ was observed in 60 cases, and post-DDZ 73% demonstrated improvement (P < 0.0001). Maximum pain scores significantly decreased post-DDZ (P < 0.0001). Heart rate, systolic, and diastolic blood pressures significantly decreased post-DDZ (P < 0.0001, P < 0.0001, P = 0.0002, respectively). CONCLUSIONS The combination of Decadron, Diamox, and Zantac is a novel treatment for ventricular shunt failure that may temporarily improve symptoms in patients awaiting shunt revision. Future studies could compare efficacy with other medical treatments.

OBJECTIVEEvaluation of increased intracranial pressure (ICP) in the pediatric craniosynostosis population based solely on ophthalmological, clinical, and radiographic data is subjective, insensitive, and inconsistent. The aim of this study was to examine the intraoperative ICP before and after craniectomy in this patient population.METHODSThe authors measured the ICP before and after craniectomy using a subdural ICP monitor in 45 children. They regulated end-tidal carbon dioxide and the monitoring site under general anesthesia to record consistent ICP readings.RESULTSThe average age of the patient population was 29 months (range 3.8–180.5 months). Thirty-seven patients (82.2%) were undergoing initial craniosynostosis procedures. All craniosynostosis procedures were categorized as one of the following: frontoorbital advancement (n = 24), frontoorbital advancement with distraction osteogenesis (n = 1), posterior vault distraction osteogenesis (n = 10), and posterior vault reconstruction (n = 10). Nineteen of 45 patients (42.2%) had syndromic or multisuture craniosynostosis. The mean postcraniectomy ICP (8.8 mm Hg, range 2–18 mm Hg) was significantly lower than the precraniectomy ICP (16.5 mm Hg, range 6–35 mm Hg) (p < 0.001). Twenty-four patients (53%) had elevated ICP prior to craniectomy, defined as ≥ 15 mm Hg. Only 4 (8.9%) children had papilledema on preoperative funduscopic examination (sensitivity 17%, specificity 100%, negative predictive value 51%, and positive predictive value 100%). There were no significant differences in elevated precraniectomy ICP based on type of craniosynostosis (syndromic/multisuture or nonsyndromic) or age at the time of surgery. Patients undergoing initial surgery in the first 12 months of life were significantly less likely to have elevated precraniectomy ICP compared with patients older than 12 months (26.3% vs 73.1%, p = 0.005).CONCLUSIONSIn this study, the authors report the largest cohort of syndromic and nonsyndromic craniosynostosis patients (n = 45) who underwent precraniectomy and postcraniectomy ICP evaluation. A craniectomy or completed craniotomy cuts for distractors effectively reduced ICP in 43/45 patients. The authors’ findings support the notion that papilledema on funduscopy is a highly specific, however poorly sensitive, indicator of increased ICP, and thus is not a reliable screening method. These findings indicate that even nonsyndromic patients with craniosynostosis are at risk for increased ICP. Furthermore, patients who present prior to 12 months of age appear less likely to have elevated ICP on presentation. Further studies with other noninvasive imaging of the retina may be useful as an adjunct tool for determining elevated ICP.

Hydrocephalus is one of the most common congenital abnormalities affecting the nervous system, occurring in 0.3 to 2.5 per 1,000 live births. It results from obstruction of cerebrospinal fluid (CSF) pathways by a diverse range of developmental, genetic, and acquired abnormalities and can have negative consequences on the neurodevelopmental outcome of affected neonates. Historically, hydrocephalus was diagnosed after birth and managed with a shunt procedure; however, with the advent of advanced antenatal imaging techniques, it may now be detected and treated before delivery in some individuals. Moreover, surgical options for the treatment of hydrocephalus have increased over the past few decades, and temporary CSF diversion may prevent the need for permanent shunt placement. Posthemorrhagic hydrocephalus is the most common cause of hydrocephalus in the preterm newborn, but the timing of surgical intervention for this condition remains controversial. Recent evidence suggests that earlier, rather than later, intervention may have some benefits, but more data are needed to adequately inform clinical practice. Regardless of etiology or timing of diagnosis, a thorough understanding of the natural history of hydrocephalus and the range of treatment options available is needed for parental counseling, prognostication, and appropriate surgical management.

Endoscopic third ventriculostomy (ETV) has become a popular technique for the treatment of hydrocephalus, but small sample size has limited the generalizability of prior studies. We performed a large-scale review of all available studies to help eliminate bias and determine how outcomes have changed and been influenced by patient selection over time. A systematic literature search was performed for studies of ETV that contained original, extractable patient data, and a meta-analytic model was generated for correlative and predictive analysis. A total of 130 studies were identified, which included 11,952 cases. Brain tumor or cyst was the most common hydrocephalus etiology, but high-risk etiologies, post-infectious or post-hemorrhagic hydrocephalus, accounted for 18.4%. Post-operative mortality was very low (0.2%) and morbidity was only slightly higher in developing than in industrialized countries. The rate of ETV failure was 34.7% and was higher in the first months and plateaued around 20 months. As anticipated, ETV is less successful in high-risk etiologies of hydrocephalus and younger patients. Younger patient age and high-risk etiologies predicted failure. ETVs were performed more often in high-risk etiologies over time, but, surprisingly, there was no overall change in ETV success rate over time. This study should help to influence optimal patient selection and offer guidance in predicting outcomes.

OBJECTIVENeurogenic thoracic outlet syndrome (nTOS) is an uncommon compression syndrome of the brachial plexus that presents with pain, sensory changes, and motor weakness in the affected limb. The authors reviewed the clinical presentations and outcomes in their series of pediatric patients with surgically treated nTOS over a 6-year period.METHODSCases of nTOS in patients age 18 years or younger were extracted for analysis from a prospective database of peripheral nerve operations. Baseline patient characteristics, imaging and neurophysiological data, operative findings, and outcomes and complications were assessed.RESULTSTwelve patients with 14 cases of nTOS surgically treated between April 2010 and December 2016 were identified. One-third of the patients were male, and 2 male patients underwent staged, bilateral procedures. Disabling pain (both local and radiating) was the most common presenting symptom (100%), followed by numbness (35.7%), then tingling (28.6%). The mean duration of symptoms prior to surgery was 15.8 ± 6.6 months (mean ± SD). Sports-related onset of symptoms was seen in 78.6% of cases. Imaging revealed cervical ribs in 4 cases, prominent C-7 transverse processes in 4 cases, abnormal first thoracic ribs in 2 cases, and absence of bony anomalies in 4 cases. Neurophysiological testing results were normal in 85.7% of cases. Conservative management failed in all patients, with 5 patients reporting minimal improvement in symptoms with physical therapy. With a mean follow-up after surgery of 22 ± 18.3 months (mean ± SD), pain relief was excellent (> 90%) in 8 cases (57.1%), and good (improved > 50%) in 6 cases (42.9%). On univariate analysis, patients who reported excellent pain resolution following surgery at long-term follow-up were found to be significantly younger, and to have suffered a shorter duration of preoperative symptoms than patients who had worse outcomes. Lack of significant trauma or previous surgery to the affected arm was also associated with excellent outcomes. There were 4 minor complications in 3 patients within 30 days of surgery: 1 patient developed a small pneumothorax that resolved spontaneously; 1 patient suffered a transient increase in pain requiring consultation, followed by hiccups for a period of 3 hours that resolved spontaneously; and 1 patient fell at home, with transient increased pain in the surgically treated extremity. There were no new neurological deficits, wound infections, deep vein thromboses, or readmissions.CONCLUSIONPediatric nTOS commonly presents with disabling pain and is more frequently associated with bony anomalies compared with adult nTOS. In carefully selected patients, surgical decompression of the brachial plexus results in excellent pain relief, which is more likely to be seen in younger patients who present for early surgical evaluation.

OBJECTIVE: To demonstrate that a novel non-invasive index of intracranial pressure (ICP) derived from diffuse optics-based techniques is associated with intracranial hypertension. STUDY DESIGN: We compared non-invasive and invasive ICP measurements in infants with hydrocephalus. Infants born term and preterm were eligible for inclusion if clinically determined to require cerebrospinal fluid (CSF) diversion. Ventricular size was assessed preoperatively via ultrasound measurement of the fronto-occipital (FOR) and fronto-temporal (FTHR) horn ratios. Invasive ICP was obtained at the time of surgical intervention with a manometer. Intracranial hypertension was defined as invasive ICP ≥15 mmHg. Diffuse optical measurements of cerebral perfusion, oxygen extraction, and non-invasive ICP were performed preoperatively, intraoperatively, and postoperatively. Optical and ultrasound measures were compared with invasive ICP measurements, and their change in values after CSF diversion were obtained. RESULTS: We included 39 infants; 23 had intracranial hypertension. No group difference in ventricular size was found by FOR (p=0.93) or FTHR (p=0.76). Infants with intracranial hypertension had significantly higher non-invasive ICP (p=0.02) and oxygen extraction fraction (p=0.01) compared with infants without intracranial hypertension. Increased cerebral blood flow (p=0.005) and improved oxygen extraction fraction (P < .001) after CSF diversion were only observed in infants with intracranial hypertension. CONCLUSIONS: Non-invasive diffuse optical measures (including a non-invasive ICP index) were associated with intracranial hypertension. The findings suggest impaired perfusion from intracranial hypertension was independent of ventricular size. Hemodynamic evidence of the benefits of CSF diversion was seen in infants with intracranial hypertension. Non-invasive optical techniques hold promise for aiding the assessment of CSF diversion timing.

Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations. Historically, this condition has been treated with closure of the MMC defect shortly after birth. The goal of postnatal closure is to cover the exposed spinal cord and prevent infection. However, postnatal surgery does not reverse or prevent the neurologic injury seen in MMC, reverse hindbrain herniation, or prevent hydrocephalus. The neurologic defects result from primary incomplete neurulation and secondary chronic prenatal damage to the exposed neural elements through mechanical and chemical trauma. In a hope to reduce the numerous surgical procedures, medical complications, and lifelong disabilities associated with MMC, the proposal of prenatal closure was put forth more than two decades ago. After promising results in animal models and some clinical series, a randomized controlled trial, the Management of Myelomeningocele Study (MOMS), was conducted. The MOMS trial demonstrated that closure during the prenatal period could be performed relatively safely and can result in significant benefit to the child. Specifically, prenatal closure results in improved motor function, reduced hindbrain herniation, and reduced need for a cerebral spinal fluid diversion. Long-term outcomes of the patients in the MOMS trial continues in the MOMS 2 study as these children grow. Additionally, investigations are underway on modifications to the open fetal MMC closure techniques.

OBJECTIVE Fetal ventriculomegaly (FV), or enlarged cerebral ventricles in utero, is defined in fetal studies as an atrial diameter (AD) greater than 10 mm. In postnatal studies, the frontooccipital horn ratio (FOHR) is commonly used as a proxy for ventricle size (VS); however, its role in FV has not been assessed. Using image analysis techniques to quantify VS on fetal MR images, authors of the present study examined correlations between linear measures (AD and FOHR) and VS in patients with FV. METHODS The authors performed a cross-sectional study using fetal MR images to measure AD in the axial plane at the level of the atria of the lateral ventricles and to calculate FOHR as the average of the frontal and occipital horn diameters divided by the biparietal distance. Computer software was used to separately segment and measure the area of the ventricle and the ventricle plus the subarachnoid space in 2 dimensions. Segmentation was performed on axial slices 3 above and 3 below the slice used to measure AD, and measurements for each slice were combined to yield a volume, or 3D VS. The VS was expressed as the absolute number of voxels (non-normalized) and as the number of voxels divided by intracranial size (normalized). A Pearson correlation coefficient was used to measure the strength of the relationships between the linear measures and the size of segmented regions in 2 and 3 dimensions and over various gestational ages (GAs). Differences between correlations were compared using Steiger's z-test. RESULTS Fifty FV patients who had undergone fetal MRI between 2008 and 2014 were included in the study. The mean GA was 26.3 ± 5.4 weeks. The mean AD was 18.1 ± 8.3 mm, and the mean FOHR was 0.49 ± 0.11. When using absolute VS, the correlation between AD and 3D VS (r = 0.844, p < 0.0001) was significantly higher than that between FOHR and 3D VS (r = 0.668, p < 0.0001; p = 0.0004, Steiger's z-test). However, when VS was normalized, correlations were not significantly different between AD and 3D VS (r = 0.830, p < 0.0001) or FOHR and 3D VS (r = 0.842, p < 0.0001; p = 0.8, Steiger's z-test). For GAs of 24 weeks or earlier, AD correlated more strongly with normalized 3D VS (r = 0.902, p < 0.0001) than with FOHR (r = 0.674, p < 0.0001; p < 0.0001, Steiger's z-test). After 24 weeks, there was no difference in correlations between linear measures (AD or FOHR) and 3D VS (r > 0.9). Correlations of linear measures with VS in 2 and 3 dimensions were similar, and inclusion of the subarachnoid space did not significantly alter results. CONCLUSIONS Findings in the study support the use of AD as a measure of VS in fetal studies as it correlates highly with both absolute and relative VS, especially at early GAs, and captures the preferential dilation of the occipital horns in patients with FV. Compared with AD, FOHR similarly correlates with normalized VS and, after a GA of 24 weeks, can be reported in fetal studies to provide continuity with postnatal monitoring.

Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children’s hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children’s Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58–64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

Preterm infants with post-hemorrhagic hydrocephalus (PHH) are often treated with temporizing measures such as ventricular access devices (VADs) in order to drain cerebrospinal fluid (CSF) prior to permanent diversion with ventriculoperitoneal shunt (VPS) placement. There is little consensus on the timing and management of VADs and VPSs. This leads to marked practice variations among treating services that can adversely affect patient outcomes. This is a quality improvement study evaluating practices from February 2011 to September 2017 including infants with PHH in a single level IV NICU. A multidisciplinary team created a local clinical pathway modified from the Hydrocephalus Clinical Research Network’s Shunting Outcomes in Post-Hemorrhagic Hydrocephalus protocol to manage infants with PHH. Methods of CSF diversion and shunt timing were based on weight. Neonatal care providers performed VAD aspiration; timing was guided by imaging and clinical exam criteria. Surgical procedures were performed in the NICU. There were 78 patients eligible for the study. Prior to pathway implementation, infections occurred in 4% of VAD and 3% of VPS patients. There have been no infections since inception of the pathway. With pathway implementation, treatment compliance improved from 55 to 86% while conversion compliance rate improved from 89 to 100%. Standardization of care for PHH infants leads to improvement in patient outcomes such as a decrease in time to VAD placement. Reservoir aspirations by the neonatology team did not result in an increase in infection rate.

We present pilot results on the validation of non-invasive assessment of elevated intracranial pressure with optical measurement of critical closing pressure. A strong correlation (r=0.85) between optical measurements of critical closing pressure and invasive measurements of intracranial pressure was observed in 5 infants with hydrocephalus, and 1 adult patient with diffuse hypoxic ischemic brain injury. By facilitating timely detection of intracranial hypertension, this approach has potential to reduce risk of brain damage in hydrocephalus and other vulnerable patient populations.

OBJECTIVE To determine whether the presence of a myelomeningocele (MMC) sac and sac size correlate with compromised lower-extremity function in fetuses with open spinal dysraphism. METHODS A radiology database search was performed to identify cases of MMC and myeloschisis (MS) diagnosed prenatally in a single center from 2013 to 2017. All cases were evaluated between 18 and 25 weeks. Ultrasound reports were reviewed for talipes and impaired lower-extremity motion. In MMC cases, sac volume was calculated from ultrasound measurements. Magnetic resonance imaging reports were reviewed for hindbrain herniation. The association of presence of a MMC sac and sac size with talipes and impaired lower-extremity motion was assessed. Post-hoc analysis of data from the multicenter Management of Myelomeningocele Study (MOMS) randomized controlled trial was performed to confirm the study findings. RESULTS In total, 283 MMC and 121 MS cases were identified. MMC was associated with a lower incidence of hindbrain herniation than was MS (80.9% vs 100%; P < 0.001). Compared with MS cases, MMC cases with hindbrain herniation had a higher rate of talipes (28.4% vs 16.5%, P = 0.02) and of talipes or lower-extremity impairment (34.9% vs 19.0%, P = 0.002). Although there was a higher rate of impaired lower-extremity motion alone in MMC cases with hindbrain herniation than in MS cases, the difference was not statistically significant (6.6% vs 2.5%; P = 0.13). Among MMC cases with hindbrain herniation, mean sac volume was higher in those associated with talipes compared with those without talipes (4.7 ± 4.2 vs 3.0 ± 2.6 mL; P = 0.002). Review of the MOMS data demonstrated similar findings; cases with a sac on baseline imaging had a higher incidence of talipes than did those without a sac (28.2% vs 7.5%; P = 0.007). CONCLUSIONS In fetuses with open spinal dysraphism, the presence of a MMC sac was associated with fetal talipes, and this effect was correlated with sac size. The presence of a larger sac in fetuses with open spinal dysraphism may result in additional injury through mechanical stretching of the nerves, suggesting another acquired mechanism of injury to the exposed spinal tissue. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. A multidisciplinary, retrospective cohort study from seven children’s hospitals was performed including asymptomatic children with MMC

Better understanding the incidence and patterns of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to alter neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retina can non-invasively diagnose elevated ICP, and has demonstrated high sensitivity and specificity among patients with craniosynostosis. This study sought to characterize patterns of elevated ICP among patients with craniosynostosis. Quantitative retinal parameters were prospectively assessed in both eyes of patients with craniosynostosis using spectral-domain OCT. Based on retinal OCT thresholds associated with elevated ICP (> 15 mmHg), subjects were assigned an OCT diagnosis of elevated or non-elevated ICP which was analyzed relative to clinical characteristics and craniosynostosis patterns. Eighty subjects (aged 0.2–18 years) with craniosynostosis were enrolled; among these, 67 (84%) were nonsyndromic. OCT evaluation was performed at initial vault expansion in 56 (70%) patients. Among this subset, 27 (48%) patients had peri-papillary changes suggestive of elevated ICP, reflecting a 44% incidence in nonsyndromic and 83% in syndromic patients. The median age at initial vault expansion was higher among those with elevated ICP (11.1 months) than those without (7.8 months; p = 0.04.) Multi-suture synostosis was associated with changes consistent with elevated ICP in 9 (75%) patients compared with 18 (41%) with single suture synostosis (p = 0.05). OCT of the retina produces a potentially sensitive indicator of ICP in craniosynostosis patients. Elevated ICP may be associated with number of involved sutures and older patient presentation, and refining appropriate “cutoffs” will be important as the technology becomes more widespread.

Cranial spring hardware is generally removed 3 months after placement for spring-mediated cranioplasty. Spring removal is performed as an outpatient procedure under general anesthesia in approximately 15 minutes through the incision locations of the index procedure. Herein, the authors provide a multimedia demonstration of cranial spring hardware removal after spring-mediated cranioplasty for sagittal craniosynostosis. The video can be found here: https://vimeo.com/511179695

Spring-mediated cranioplasty is a useful treatment modality for correcting scaphocephalic head shape in sagittal craniosynostosis because it is less invasive than whole-vault cranioplasty and offers durable morphologic outcomes. Herein, the authors provide a multimedia demonstration of alternative operative approaches for spring-mediated cranioplasty for sagittal craniosynostosis. The video can be found here: https://vimeo.com/511256259

There is no clear consensus for the optimal treatment of sagittal craniosynostosis; however, recent studies suggest that improved neurocognitive outcomes may be obtained when surgical intervention imparts active cranial expansion or remodeling and is performed before 6 months of age. The authors consider spring-mediated cranioplasty (SMC) to optimally address these imperatives, and this is an investigation of how helmet orthoses before or after SMC affect aesthetic outcomes.The authors retrospectively evaluated patients treated with SMC and adjunct helmeting for sagittal synostosis. Patients were stratified into 4 cohorts based on helmet usage: preop, postop, both, and neither. The cephalic index (CI) was used to assess head shape changes and outcomes. Twenty-six patients met inclusion criteria: 6 (23%) had preop, 11 (42%) had postop, 4 (15%) had preop and postop, and 5 (19%) had no helmeting. Average age at surgery was 3.6 months. Overall, CI improved from a mean 69.8 to 77.9 during an average 7-month course of care. Mean preoperative change in CI showed greater improvement with preop helmet (1.3) versus not (0.0), (P = 0.029), despite similar initial CI in these cohorts (70.4 and 69.6 respectively, P = 0.69). Nonetheless, all patient cohorts regardless of helmeting status achieved similar final CIs (range 76.4-80.4; P = 0.72).In summary, preoperative molding helmet therapy leads to improved CI at the time of spring-mediated cranioplasty. However, this benefit does not necessarily translate into overall improved CI after surgery and in follow-up, calling into question the benefits of molding helmet therapy in this setting.

There are a variety of imaging modalities for evaluation of peripheral nerves. Of these, ultrasonography (US) is often underused. There are several advantages of this imaging modality, including its cost-effectiveness, time-efficient assessment of long segments of peripheral nerves, ability to perform dynamic maneuvers, lack of contraindications, portability, and noninvasiveness. It can provide diagnostic information that cannot be obtained by electrophysiologic or, in some cases, magnetic resonance imaging studies. Ideally, the neurosurgeon can use US as a diagnostic adjunct in the preoperative assessment of a patient with traumatic, neoplastic, infective, or compressive nerve injury. Perhaps its most unique use is in intraoperative surgical planning. In this article, a brief description of normal US nerve anatomy is presented followed by a description of the US appearance of peripheral nerve disease caused by trauma, tumor, infection, and entrapment.

Neonatal brachial plexus avulsion injury (BPAI) commonly occurs as a consequence of birth trauma and can result in lifetime morbidity; however, little is known regarding the evolving neuropathological processes it induces. In particular, mechanical forces during BPAI can concomittantly damage the spinal cord and may contribute to outcome. Here, we describe the functional and neuropathological outcome following BPAI, with or without spinal cord injury, in a novel pediatric animal model. Twenty-eight-day-old piglets underwent unilateral C5–C7 BPAI with and without limited myelotomy. Following avulsion, all animals demonstrated right forelimb monoparesis. Injury extending into the spinal cord conferred greater motor deficit, including long tract signs. Consistent with clinical observations, avulsion with myelotomy resulted in more severe neuropathological changes with greater motor neuron death, progressive axonopathy, and persistent glial activation. These data demonstrate neuropathological features of BPAI associated with poor functional outcome. Interestingly, in contrast to adult small animal models of BPAI, a degree of motor neuron survival was observed, even following severe injury in this neonatal model. If this is also the case in human neonatal BPAI, repair may permit functional restoration. This model also provides a clinically relevant platform for exploring the complex postavulsion neuropathological responses that may inform therapeutic strategies.

OBJECT Mutations in the sodium channel alpha 1 subunit gene (SCN1A) have been associated with a wide range of epilepsy phenotypes including Dravet syndrome. There currently exist few histopathological and surgical outcome reports in patients with this disease. In this case series, the authors describe the clinical features, surgical pathology, and outcomes in 6 patients with SCN1A mutations and refractory epilepsy who underwent focal cortical resection prior to uncovering the genetic basis of their epilepsy. METHODS Medical records of SCN1A mutation-positive children with treatment-resistant epilepsy who had undergone resective epilepsy surgery were reviewed retrospectively. Surgical pathology specimens were reviewed. RESULTS All 6 patients identified carried diagnoses of intractable epilepsy with mixed seizure types. Age at surgery ranged from 18 months to 20 years. Seizures were refractory to surgery in every case. Surgical histopathology showed evidence of subtle cortical dysplasia in 4 of 6 patients, with more neurons in the molecular layer of the cortex and white matter. CONCLUSIONS Cortical resection is unlikely to be beneficial in these children due to the genetic defect and the unexpected neuropathological finding of mild diffuse malformations of cortical development. Together, these findings suggest a diffuse pathophysiological mechanism of the patients’ epilepsy which will not respond to focal resective surgery.

This chapter takes a case-based approach to the diagnosis and management of adult brachial plexus injury involving the upper trunk. The clinical presentation and differential diagnosis associated with this injury pattern are reviewed, as well as the findings of electrodiagnostic and imaging studies. Preoperative considerations include the timing from initial injury and the difference between pre- and postganglionic injury. Options for nerve reconstruction include nerve grafting, nerve transfer, or a combination of both. The options are compared, and a detailed description of each surgical procedure is provided, along with related complications, alternative repair strategies, and outcomes.

BACKGROUND The effect of modifications in fetal myelomeningocele (fMMC) closure techniques has not been extensively studied. OBJECTIVE To study the effect of a modified closure technique on fMMC postnatal patient outcomes: hydrocephalus, hindbrain herniation, and cyst development. METHODS We performed single-center retrospective study of a subset of post-MOMS (Management of Myelomeningocele Study) trial patients who underwent fMMC closure. After January 2015, the fetal myofascial closure technique was modified. Needlepoint monopolar cautery was used to raise dural lined myofascial flaps to create a more robust closure. Outcomes between the pre- and postmodification groups were compared with regard to hindbrain herniation, hydrocephalus, and cyst development. Families who transitioned care to local institutions were contacted via telephone for outcome information. RESULTS From January 2011 to May 2016, data were reviewed from 119 fMMC closure patients. Patients without full follow-up data were excluded from the final analysis. Cerebrospinal fluid diversion was seen in 32 of 74 patients with the standard technique compared to 14 of 45 with the modified closure and was significantly decreased in postmodification when compared to that of the MOMS trial (P = .01). Hindbrain herniation resolution was significantly decreased in both the pre- and postmodification groups compared to that of the MOMS trial (P

OBJECTIVEComplications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.METHODSThe Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).RESULTSSeventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.CONCLUSIONSA modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

Objective Post-ictal EEG alterations have been identified in studies of intracranial recordings, but the clinical significance of post-ictal EEG activity is undetermined. The purpose of this study was to examine the relationship between peri-ictal EEG activity, surgical outcome, and extent of seizure propagation in a sample of pediatric epilepsy patients. Methods Intracranial EEG recordings were obtained from 19 patients (mean age = 11.4 years, range = 3–20 years) with 57 seizures used for analysis (mean = 3.0 seizures per patient). For each seizure, 3-min segments were extracted from adjacent pre-ictal and post-ictal epochs. To compare physiology of the epileptic network between epochs, we calculated the relative delta power (Δ) using discrete Fourier transformation and constructed functional networks based on broadband connectivity (conn). We investigated differences between the pre-ictal (Δpre, connpre) and post-ictal (Δpost, connpost) segments in focal-network (i.e., confined to seizure onset zone) versus distributed-network (i.e., diffuse ictal propagation) seizures. Results Distributed-network (DN) seizures exhibited increased post-ictal delta power and global EEG connectivity compared to focal-network (FN) seizures. Following DN seizures, patients with seizure-free outcomes exhibited a 14.7% mean increase in delta power and an 8.3% mean increase in global connectivity compared to pre-ictal baseline, which was dramatically less than values observed among seizure-persistent patients (29.6% and 47.1%, respectively). Significance Post-ictal differences between DN and FN seizures correlate with post-operative seizure persistence. We hypothesize that post-ictal deactivation of subcortical nuclei recruited during seizure propagation may account for this result while lending insights into mechanisms of post-operative seizure recurrence.

BACKGROUND: Ventricular shunt complications in children can be severe and life-threatening if not identified and treated in a timely manner. Evaluation for shunt obstruction is not without risk, including lifetime cumulative radiation as patients routinely receive computed tomography (CT) scans of the brain and shunt series (multiple radiographs of the skull, neck, chest, and abdomen). METHODS: A multidisciplinary team collaborated to develop a clinical pathway with the goal of standardizing the evaluation and management of patients with suspected shunt complication. The team implemented a low-dose CT scan, specifically tailored for the detection of hydrocephalus and discouraged routine use of shunt series with single-view radiographs used only when specifically indicated. RESULTS: There was a reduction in the average CT effective dose (millisievert) per emergency department (ED) encounter of 50.6% (confidence interval, 46.0–54.9; P ≤ .001) during the intervention period. There was a significant reduction in the number of shunt surveys obtained per ED encounter, from 62.4% to 5.32% (P < .01). There was no significant change in the 72-hour ED revisit rate or CT scan utilization rate after hospital admission. There were no reports of inadequate patient evaluations or serious medical events. CONCLUSIONS: A new clinical pathway has rapidly reduced radiation exposure, both by reducing the radiation dose of CT scans and eliminating or reducing the number of radiographs obtained in the evaluation of patients with ventricular shunts without compromising clinical care.

Fetal ventriculomegaly (VM) refers to the enlargement of the cerebral ventricles in utero. It is associated with the postnatal diagnosis of hydrocephalus. VM is clinically diagnosed on ultrasound and is defined as an atrial diameter greater than 10 mm. Because of the anatomic detailed seen with advanced imaging, VM is often further characterized by fetal magnetic resonance imaging (MRI). Fetal VM is a heterogeneous condition with various etiologies and a wide range of neurodevelopmental outcomes. These outcomes are heavily dependent on the presence or absence of associated anomalies and the direct cause of the ventriculomegaly rather than on the absolute degree of VM. In this review article, we discuss diagnosis, work-up, counseling, and management strategies as they relate to fetal VM. We then describe imaging-based research efforts aimed at using prenatal data to predict postnatal outcome. Finally, we review the early experience with fetal therapy such as in utero shunting, as well as the advances in prenatal diagnosis and fetal surgery that may begin to address the limitations of previous therapeutic efforts.

The International Subarachnoid Aneurysm Trial heralded a paradigm shift in the treatment of intracranial aneurysms. During this same time frame, neurosurgical training programs increased in size and scope. The present study examines the impact of trends in surgical clipping and the endovascular treatment of intracranial aneurysms, over one decade, and the neurosurgical resident complement on the resident teaching environment using the Nationwide Inpatient Sample (NIS).The NIS was used to estimate the number of aneurysms treated with either surgical clipping and endovascular methods from 2002 through 2011 at teaching institutions. Teaching opportunities per year per resident or chief resident were calculated as the ratio of the number of specified cases to the average number of neurosurgical trainees by year. Annualized trends were assessed.Over the study period, the percent change in odds of occurrence of a clipped ruptured aneurysm was -15.6% per year (P0.001) and of ruptured aneurysms undergoing endovascular treatment was 18.7% per year (P 0.001) within teaching institutions. This corresponded to a decline in teaching opportunities for clipped ruptured aneurysms for both residents and chief residents (P 0.001). In contrast, teaching opportunities for endovascular treatment of both ruptured and unruptured aneurysms increased dramatically over the study period.There has been a significant decrease in opportunity for operative exposure to craniotomy for ruptured aneurysm clipping over the past decade, whereas the volume of endovascular procedures for aneurysms has dramatically increased, highlighting the need for a shift in training strategy for those neurosurgeons graduating from residency desiring to subspecialize in neurovascular neurosurgery.

Intraoperative neuromonitoring (IONM) has transformed the fields of pediatric surgery and anesthesia since its introduction in 1979, and is an important diagnostic and preventative tool to potentially decrease neurological complications in children after surgical procedures. Its value in minimizing neurological injury during surgery has been well established in adults, with a 10–30% difference in injury rates in neurosurgical historical cohorts, but this risk reduction has yet to be systematically studied in children. The goal of neuromonitoring during surgery is to detect changes in neural signaling as early as possible, allow surgical teams to prevent further injury by monitoring for and potentially reversing the electrophysiological changes that are detected. The adaptation of various neuromonitoring techniques from the adult population to pediatrics has proved crucial in assessing and optimizing surgical outcomes across numerous subspecialty fields. Early uses of IONM included the use of brainstem auditory evoked potentials (BAERs) and monitoring effects on the spinal cord during spine deformity correction. These early applications demonstrated the powerful utility of IONM and its applicability to children.

Myelomeningocele (MMC) is one of the most common serious congenital malformations. Typically this condition has been treated with closure of the MMC defect shortly after birth. In general, surgery for MMC aims to provide a multilayered closure to provide protection to the neural elements, prevent leakage of spinal fluid and reduce infection risks. A randomized controlled trial, the Management of Myelomeningocele Study (MOMS), has shown that closure during the fetal period can be performed relatively safely and can result in significant benefit to the child. Whereas the surgical technique of prenatal closure of an MMC defect is similar to a postnatal closure, there are some important technical differences. The goal of this paper is to describe the technique of fetal closure of MMC defects, highlight the unique steps that are needed for this surgery and delineate some potential pitfalls.

Object Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring. Methods The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord. Results During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level. Conclusions All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

Object Neonatal brachial plexus palsy (NBPP) represents a significant health problem with potentially devastating consequences. The most common form of NBPP involves the upper trunk roots. Currently, primary surgical repair is performed if clinical improvement is lacking. There has been increasing interest in “early” surgical repair of NBPPs, occurring within 3–6 months of life. However, early treatment recommendations ignore spontaneous recovery in cases of Erb's palsy. This study was undertaken to evaluate the optimal timing of surgical repair in this group with respect to quality of life. Methods The authors formulated a decision analytical model to compare 4 treatment strategies (no repair or repair at 3, 6, or 12 months of life) for infants with persistent NBPPs. The model derives data from a critical review of published studies and projects health-related quality of life and quality-adjusted life years over a lifetime. Results When evaluating the quality of life of infants with NBPP, improved outcomes are seen with delayed surgical repair at 12 months, compared with no repair or repair at early and intermediate time points, at 3 and 6 months, respectively. ANOVA showed that the differences among the 4 groups are highly significant (F = 8369; p < 0.0001). Pairwise post hoc comparisons revealed that there are highly significant differences between each pair of strategies (p < 0.0001). Meta-regression showed no evidence of improved outcomes with more recent treatment dates, compared with older ones, for either nonsurgical or for surgical treatment (p = 0.767 and p = 0.865, respectively). Conclusions These data support a delayed approach of primary surgical reconstruction to optimize quality of life. Early surgery for NBPPs may be an overly aggressive strategy for infants who would otherwise demonstrate spontaneous recovery of function by 12 months. A randomized, controlled trial would be necessary to fully elucidate the natural history of NBPP and determine the optimal time point for surgical intervention.

Externalized ventricular drains (EVDs) are commonly used in pediatric intensive care units (PICU) but few data are available regarding infection rates, infection risks, or factors associated with conversion to permanent cerebrospinal fluid (CSF) diversion. Retrospective observational study of patients managed with EVDs admitted to a tertiary care PICU from January 2005 to December 2009. Three hundred eighty patients were identified. Neurologic diagnostic groups were externalization of existing shunt in 196 patients (52 %), brain tumor in 122 patients (32 %), intracranial hemorrhage in 23 patients (6 %), traumatic brain injury in 17 patients (5 %), meningitis in 9 patients (2 %), or other in 13 patients (3 %). Six percent of all patients (24/380) had new infections associated with EVD management for an infection rate of 8.6 per 1,000 catheter days. The median time to positive cultures was 7 days (interquartile range 4.75, 9) after EVD placement. Patients with EVD infections had significantly longer EVD duration 6 versus 11.5 days (p = 0.0001), and higher maximum EVD outputs 1.9 versus 1.5 mL/kg/h (p = 0.0017). Need for permanent CSF diversion was associated with higher maximum EVD drainage (1.3 vs. 1.6 mL/kg/h p

Object No clear treatment guidelines for pediatric craniopharyngiomas exist. The authors developed a decision analytical model to evaluate outcomes of 4 surgical approaches for craniopharyngiomas in children, including attempted gross-total resection (GTR), planned subtotal removal plus radiotherapy, biopsy plus radiotherapy, and endoscopic resections of all kinds. Methods Pooled data, including the authors' own experience, were used to create evidence tables, from which incidence, relative risks, and summary outcomes in quality-adjusted life years (QALYs) were calculated for the 4 management strategies. Results Quality-adjusted life years at the 5-year follow-up were 2.3 ± 0.1 for attempted GTR, 2.9 ± 0.2 for planned subtotal removal plus radiotherapy, 3.9 ± 0.2 for biopsy plus radiotherapy, and 3.7 ± 0.2 for endoscopic resection (F = 17,150, p < 0.001). Similarly, QALYs at 10-year follow-up were 4.5 ± 0.2 for attempted GTR, 5.7 ± 0.5 for planned subtotal removal plus radiotherapy, and 7.8 ± 0.5 for biopsy plus radiotherapy (F = 6,173, p < 0.001). On post hoc pairwise comparisons, the differences between all pairs compared were also highly significant (p < 0.001). Since follow-up data at 10 years are lacking for endoscopic cases, this category was excluded from 10-year comparisons. Conclusions Biopsy with subsequent radiotherapy is the preferred approach with respect to improved overall quality of life. While endoscopic approaches also show promise in preserving quality of life at five-year follow-up, there are not sufficient data to draw conclusions about this comparison at 10 years.

OBJECTIVEThe Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons’ risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial.METHODSA cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons’ risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression.RESULTSCompared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either “very favorable” or “somewhat favorable,” and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts.CONCLUSIONSThe majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). Patients with MMC can face a lifetime of specific organ system dysfunction, chief among them spinal cord malformations, orthopedic issues, hydrocephalus, and urological disabilities. In addition, patients can experience specific patterns of neurobehavioral difficulties due to the changes in neuroanatomy associated with the open spinal defect. Although there is variability in these patterns, some trends have been described among MMC patients. It is thought that early recognition of these potential neurobehavioral deficits by treating neurosurgeons and other members of the treatment team could lead to earlier intervention and positively impact the overall outcome for patients. Neurodevelopmental and neurobehavioral follow-up assessments are recommended to help guide planning for relevant treatments or accommodations.

Myelomeningocele (MMC) affects approximately 2000 infants in the US yearly and causes long-term damage to the genitourinary system. There is a wide spectrum of effects on the bladder and urethral sphincter that do not correlate with the level of the spinal cord defect. The bladder changes can provide a safe storage pressure, often at the expense of continence, or can create a hostile environment leading to increased pressure on the kidneys and eventually to chronic kidney disease. The ultimate goals in management are to maintain a safe system that prevents renal deterioration, while providing social continence and independence in the long term. This review intends to provide an overview of the effects of MMC on the genitourinary system, regardless of intervention, in order to understand the pathophysiology of the disease.

Object Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature. Methods A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012. Results Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both N-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit. Conclusions Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.

Object Cerebral arteriovenous malformations (AVMs) have a higher postresection recurrence rate in children than in adults. The authors' previous study demonstrated that a diffuse AVM (low compactness score) predicts postresection recurrence. The aims of this study were to evaluate the intra- and interrater reliability of the AVM compactness score. Methods Angiograms of 24 patients assigned a preoperative compactness score (scale of 1–3; 1 = most diffuse, 3 = most compact) in the authors' previous study were rerated by the same pediatric neuroradiologist 9 months later. A pediatric neurosurgeon, pediatric neuroradiology fellow, and interventional radiologist blinded to each other's ratings, the original ratings, and AVM recurrence also rated each AVM's compactness. Intrarater and interrater reliability were calculated using the κ statistic. Results Of the 24 AVMs, scores by the original neuroradiologist were 1 in 6 patients, 2 in 16 patients, and 3 in 2 patients. Intrarater reliability was 1.0. The κ statistic among the 4 raters was 0.69 (95% CI 0.44–0.89), which indicates substantial reliability. The interrater reliability between the neuroradiologist and neuroradiology fellow was moderate (κ = 0.59 [95%CI 0.20–0.89]) and was substantial between the neuroradiologist and neurosurgeon (κ = 0.74 [95% CI 0.41–1.0]). The neuroradiologist and interventional radiologist had perfect agreement (κ = 1.0). Conclusions Intrarater and interrater reliability of the AVM compactness score were excellent and substantial, respectively. These results demonstrate that the AVM compactness score is reproducible. However, the neuroradiologist and interventional radiologist had perfect agreement, which indicates that the compactness score is applied most accurately by those with extensive angiography experience.

Severe pediatric traumatic brain injury (TBI) remains one of the leading causes of acquired disability and death in the USA, with the highest combined rates of TBI-related emergency room visits, hospitalizations, and deaths occurring in the youngest age. While the most effective treatment is preventing trauma in the first place, a large number of patients still present to the emergency rooms and trauma centers, and their outcome is greatly affected by prompt and effective pediatric neurocritical/neurosurgical care. There is an age-at-injury pattern. Falls and motor vehicle collisions are common accidental causes, while abuse in infants and toddlers and assaults in adolescents are unfortunate common non-accidental causes of TBI. Optimal management depends on detection of primary brain injury and preventing or limiting secondary brain injury.