The Use of External Ventricular Drainage to Reduce the Frequency of Wound Complications in Myelomeningocele Closure.

INTRODUCTION: Myelomeningocele (MMC) is an open neural tube defect routinely surgically closed within 48 h of birth to prevent secondary infection. Up to 18% of patients experience wound complications, and 85% require shunting for hydrocephalus. We hypothesized that wound complications could be reduced by cerebrospinal fluid (CSF) diversion at the time of closure. METHODS: Institutional review board approval was obtained to review records of the 88 patients who underwent MMC closure between January 2005 and June 2016 at the Children's Hospital of Pittsburgh. Twenty-three patients (26%) had an external ventricular drain (EVD) placed at the time of MMC closure and underwent 7-11 days of CSF drainage. Fourteen patients (16%) had a shunt placed at the time of MMC closure, and 51 (58%) had no form of CSF diversion at the time of MMC closure. RESULTS: Patients with an EVD or shunt placed at the time of closure had no wound complications. In contrast, 8 patients (16%) without CSF diversion at closure developed wound complications (p = 0.048). Seven of the 8 wound complications occurred in the 71 patients with evidence of hydrocephalus at birth (p = 0.98). Of patients with evidence of hydrocephalus at the time of MMC closure, wound complications had a higher rate of occurrence among patients who did not receive a shunt or EVD at closure (p = 0.01). When comparing only patients with evidence of hydrocephalus at birth, the EVD group alone had a lower rate of wound complications than patients who did not receive CSF diversion at the time of closure (p = 0.031). CONCLUSIONS: These results suggest that addressing hydrocephalus at the time of MMC closure significantly reduces the likelihood of wound complications and may justify temporary CSF diversion at birth, at least in those patients manifesting hydrocephalus.

The impact of mode of delivery on infant neurologic outcomes in myelomeningocele.

BACKGROUND: Controversy exists regarding the optimal route of delivery for fetuses who are diagnosed prenatally with myelomeningocele. Current recommendations are based partly on antiquated studies with questionable methods. All studies that have been published to date suffer from nonstandardized outcome measures, selection bias, and small sample size. The larger studies are >15 years old. OBJECTIVE: The purpose of this study was to provide information for evidence-based decision-making regarding the impact of route of delivery on motor outcomes for pediatric patients with prenatally were diagnosed myelomeningocele in a well-defined retrospective cohort. STUDY DESIGN: Medical records were reviewed retrospectively for all neonates who had been diagnosed with a myelomeningocele at birth from 1995-2015 within the University of Pittsburgh Medical Center system, as identified through the Children's Hospital of Pittsburgh Neurosurgery Department operative database. Records were matched with maternal records with the use of the Center for Assistance in Research that used eRecord. Data from 72 maternal-neonatal pairs were analyzed for multiple variables. The primary outcome measure was the difference between the functional and anatomic motor levels in the child at the age of 2 years, stratified by mode of delivery and presence or absence of labor. The sample size necessary to detect a difference between the groups with power of 0.8 and significance of .05 was calculated to be 52 subjects total (26 per group). RESULTS: Functional levels were slightly better than predicted by anatomic levels for all pediatric patient groups, regardless of mode of delivery or presence of labor. Anatomic levels were slightly lower (better), and defects were smaller for those infants who underwent vaginal delivery or a trial of labor, likely attributable to selection bias. Attempts to correct for this selection bias did not change the results. No other outcomes that were analyzed were associated significantly with mode of delivery or presence of labor. CONCLUSION: No benefit to motor function from delivery by cesarean section or avoidance of labor was demonstrated statistically in this mother-infant cohort.

Short-term neurocognitive outcomes following anterior temporal lobectomy.

Changes in cognitive function are a well established risk of anterior temporal lobectomy (ATL). Deficits in verbal memory are a common postoperative finding, though a small proportion of patients may improve. Postoperative evaluation typically occurs after six to 12months. Patients may benefit from earlier evaluation to identify potential needs; however, the results of a formal neuropsychological assessment at an early postoperative stage are not described in the literature. We compared pre- and postoperative cognitive function for 28 right ATL and 23 left ATL patients using repeated measures ANOVA. Changes in cognitive function were compared to ILAE seizure outcome. The mean time to postoperative neuropsychological testing was 11.1weeks (SD=6.7weeks). There was a side×surgery interaction for the verbal tasks: immediate memory recall (F(1,33)=20.68, p<0.001), short delay recall (F(1,29)=4.99, p=0.03), long delay recall (F(1,33)=10.36, p=0.003), recognition (F(1,33)=5.69, p=0.02), and naming (F(1,37)=15.86, p<0.001). This indicated that the left ATL group had a significant decrement in verbal memory following surgery, while the right ATL group experienced a small but significant improvement. For the right ATL group, there was a positive correlation between ILAE outcome and improvement in immediate recall (r=-0.62, p=0.02) and long delay recall (r=-0.57, p=0.03). There was no similar finding for the left ATL group. This study demonstrates that short-interval follow-up is effective in elucidating postoperative cognitive changes. Right ATL was associated with improvement in verbal memory, while left ATL resulted in a decrement in performance. Improvement in the right ATL group was related to improved seizure outcome. Short-interval follow-up may lend itself to the identification of patients who could benefit from early intervention.

Implantation accuracy and operative variables in robot-assisted stereoelectroencephalography.

OBJECTIVE: The stereoelectroencephalography (SEEG) procedure provides a unique 3D overview of the seizure-onset zone. Although the success of SEEG relies on the accuracy of depth electrode implantation, few studies have investigated how different implantation techniques and operative variables affect accuracy. This study examined the effect of two different electrode implantation techniques (external vs internal stylet) on implantation accuracy while controlling for other operative variables. METHODS: The implantation accuracy of 508 depth electrodes from 39 SEEG cases was measured after coregistration of postimplantation CT or MR images with planned trajectories. Two different implantation techniques were compared: preset length with internal stylet use and measured length with external stylet use. Correlations between implantation accuracy and technique type, entry angle, intended implantation depth, and other operative variables were determined statistically using multiple regression analysis. RESULTS: Multiple regression analysis showed that the internal stylet technique exhibited a larger target radial error (p = 0.046) and angular deviation (p = 0.039) with a smaller depth error (p < 0.001) than the external stylet technique. Entry angle and implantation depth were positively correlated with target radial error (p = 0.007 and < 0.001, respectively) only for the internal stylet technique. CONCLUSIONS: Better target radial accuracy was achieved when an external stylet was used to open the intraparenchymal pathway for the depth electrode. In addition, more oblique trajectories were equally accurate to orthogonal ones with the usage of an external stylet, while more oblique trajectories were associated with larger target radial errors with the usage of an internal stylet (without an external stylet).

Strength of default mode resting-state connectivity relates to white matter integrity in children.

A default mode network of brain regions is known to demonstrate coordinated activity during the resting state. While the default mode network is well characterized in adults, few investigations have focused upon its development. We scanned 9-13-year-old children with diffusion tensor imaging and resting-state functional magnetic resonance imaging. We identified resting-state networks using Independent Component Analysis and tested whether the functional connectivity between the medial prefrontal cortex (mPFC) and posterior cingulate cortex (PCC) depends upon the maturation of the underlying cingulum white matter tract. To determine the generalizability of this relationship, we also tested whether functional connectivity depends on white matter maturity between bilateral lateral prefrontal cortex (lateral PFC) within the executive control network. We found a positive relationship between mPFC-PCC connectivity and fractional anisotropy of the cingulum bundle; this positive relationship was moderated by the age of the subjects such that it was stronger in older children. By contrast, no such structure-function relationship emerged between right and left lateral PFC. However, functional and structural connectivity of this tract related positively with cognitive speed, fluency, and set-switching neuropsychological measures.

Effect of dopamine transporter genotype on caudate volume in childhood ADHD and controls.

Polymorphism of the dopamine transporter genotype (DAT1) confers a small but significant susceptibility to attention deficit hyperactivity disorder (ADHD). We examined whether the volume of the head of caudate, a striatal structure with high DAT expression that is important for inhibitory function, differs by DAT1 in children diagnosed with the disorder relative to age and IQ matched controls. Volume of the head of caudate was delineated in the right and left hemisphere and compared between 7- and 13-year-old children with and without ADHD (combined type) who were carriers of two (10/10) or one (9/10) copy of the 10-repeat DAT1 allele. Caudate volumes were overall smaller in 10/10 than 9/10 children, particularly in the left than right hemisphere. While DAT1 effects did not vary by ADHD diagnosis, overall caudate volumes were smaller in ADHD relative to control children. Altered caudate development associated with 10-repeat homozygosity of DAT1 may contribute susceptibility to ADHD.

Functional connectivity of the inferior frontal cortex changes with age in children with autism spectrum disorders: a fcMRI study of response inhibition.

Unmasking the neural basis of neurodevelopmental disorders, such as autism spectrum disorders (ASD), requires studying functional connectivity during childhood when cognitive skills develop. A functional connectivity magnetic resonance imaging (fcMRI) analysis was performed on data collected during Go/NoGo task performance from 24 children ages 8-12 years (12 with ASD; 12 controls matched on age and intellectual functioning). We investigated the connectivity of the left and right inferior frontal cortex (IFC; BA 47), key regions for response inhibition, with other active regions in frontal, striatal, and parietal cortex. Groups did not differ on behavioral measures or functional connectivity of either IFC region. A trend for reduced connectivity in the right IFC for the ASD group was revealed when controlling for age. In the ASD group, there was a significant negative correlation between age and 2 right IFC correlation pairs: right IFC-bilateral presupplementary motor area (BA 6) and right IFC-right caudate. Compared with typical controls, children with ASD may not have gross differences in IFC functional connectivity during response inhibition, which contrasts with an adult study of ASD that reported reduced functional connectivity. This discrepancy suggests an atypical developmental trajectory in ASD for right IFC connectivity with other neural regions supporting response inhibition.

Deep Brain Stimulation of the Subthalamic Nucleus and Globus Pallidus for Parkinson's Disease.

The concept of deep brain stimulation (DBS) for Parkinson's disease (PD) was introduced over 20 years ago, but our understanding of the nuances of this procedure continues to improve. The average motor outcomes of internal segment of the globus pallidus (GPi) and subthalamic nucleus (STN) DBS appear to be similar, although GPi DBS may allow greater recovery of verbal fluency and may provide greater relief of depression symptoms and improvement in the quality of life, and STN DBS appears more likely to result in decrease in levodopa equivalent doses. Despite the lack of consensus on whether STN or GPi DBS is most appropriate for a given clinical phenotype, the general expansion of patient selection criteria to include younger and older patients and the advent of real-time imaging-confirmed that DBS electrode placement are making life-changing treatment available to greater numbers of movement disorder patients.

Outcomes of Interventional-MRI Versus Microelectrode Recording-Guided Subthalamic Deep Brain Stimulation.

In deep brain stimulation (DBS) of the subthalamic nucleus (STN) for Parkinson's disease (PD), there is debate concerning the use of neuroimaging alone to confirm correct anatomic placement of the DBS lead into the STN, versus the use of microelectrode recording (MER) to confirm functional placement. We performed a retrospective study of a contemporaneous cohort of 45 consecutive patients who underwent either interventional-MRI (iMRI) or MER-guided DBS lead implantation. We compared radial lead error, motor and sensory side effect, and clinical benefit programming thresholds, and pre- and post-operative unified PD rating scale scores, and levodopa equivalent dosages. MER-guided surgery was associated with greater radial error compared to the intended target. In general, side effect thresholds during initial programming were slightly lower in the MER group, but clinical benefit thresholds were similar. No significant difference in the reduction of clinical symptoms or medication dosage was observed. In summary, iMRI lead implantation occurred with greater anatomic accuracy, in locations demonstrated to be the appropriate functional region of the STN, based on the observation of similar programming side effect and benefit thresholds obtained with MER. The production of equivalent clinical outcomes suggests that surgeon and patient preference can be used to guide the decision of whether to recommend iMRI or MER-guided DBS lead implantation to appropriate patients with PD.

Interventional MRI-Guided Deep Brain Stimulation Lead Implantation.

Current knowledge of the functional anatomy of the subthalamic nucleus and globus pallidus, discovered through microelectrode recording and postoperative imaging, justifies purely anatomic targeting for deep brain stimulation (DBS). Interventional MRI (iMRI)-DBS is more anatomically accurate than traditional awake procedures and has similar clinical outcomes without increased risk or increased operative times. iMRI lead implantation allows patients to receive DBS therapy who cannot tolerate or do not agree to undergo an awake procedure. This article describes considerations for iMRI-DBS implantation in the subthalamic nucleus and globus pallidus, including patient selection, technique of electrode placement, expected outcomes, and potential complications.

Surgical treatment of sagittal synostosis by extended strip craniectomy: cranial index, nasofrontal angle, reoperation rate, and a review of the literature.

BACKGROUND: Sagittal synostosis is the most common non-syndromic single suture craniosynostosis. Different techniques of surgical correction, including extended strip craniectomy (ESC), have been used to treat this condition. The aim of this study is to evaluate radiologic changes and rate of symptomatic restenosis after ESC in a large group of patients less than 12 months of age with non-syndromic sagittal synostosis. METHODS: A retrospective study of patients from 1990 to 2012 was performed comparing cranial index (CI) and nasofrontal angle (NFA) before and after surgical correction by ESC. Also, the frequency of subsequent reoperations for symptomatic restricted head growth was determined. RESULTS: A total of 238 patients underwent ESC. Follow-up information was available for 182 patients. The average age at the time of the operation was 4.5 months and the mean duration of follow-up was 49.6 months. The average post procedure radiologic follow-up (22 patients) was 40.7 months. CONCLUSIONS: The mean CI increased from 0.68 to 0.75 (p < 0.001) after ESC. Also, mean NFA increased from 127 to 133° (p < 0.001). Five patients (2.7%) required a second operation due to symptomatic cranial growth restriction. Reoperation occurred at an average of 26.5 months after the initial procedure. The most common symptom reported was headache. ESC is effective in treating non-syndromic sagittal synostosis. It significantly improved NFA without the need for direct frontal bone resection or frontal orbital osteotomy and significantly increased CI without adjunctive helmet treatment. Patients should be followed for at least 5 years after surgical correction as symptomatic restenosis, although rare, can occur.

Factors associated with hemispheric hypodensity after subdural hematoma following abusive head trauma in children.

Abusive head trauma (AHT) is a unique form of pediatric TBI with increased mortality and neurologic sequelae. Hemispheric hypodensity (HH) in association with subdural blood after AHT has been described. Though risk factors for HH are not understood, we hypothesized that risk factors could be identified. We retrospectively enrolled children under 5 years with TBI secondary to AHT (child advocacy diagnosis) who had undergone initial and interval brain imaging. Records were interrogated for prearrival and in-hospital physiologic and radiographic findings. HH was determined by a blinded observer. Twenty-four children were enrolled and 13 developed HH. HH was not significantly associated with age, initial Glascow Coma Scale, or mortality. Pediatric Intensity Level of Therapy (PILOT) scores (p=0.01) and daily maximal intracranial pressure (ICPmax; p=0.037) were higher in HH. Hypoxia, hypotension, cardiopulmonary arrest, need for blood transfusion, and daily blood glucoses tended to be greater in HH. Whereas all children with HH had acute subdural hematoma (SBH), many children without HH also had subdural blood; the presence of skull fracture was more likely in the children who did not develop HH (p=0.04), but no other intracranial radiographic pattern of injury was associated with HH. Surgical intervention did not appear to protect against development of HH. A variety of insults associated with ischemia, including intracranial hypertension, ICP-directed therapies, hypoxia, hypotension, and cardiac arrest, occurred in the children who developed HH. Given the morbidity and mortality of this condition, larger studies to identify mechanisms leading to the development of HH and mitigating clinical approaches are warranted.

Atypical neural substrates of Embedded Figures Task performance in children with Autism Spectrum Disorder.

Superior performance on the Embedded Figures Task (EFT) has been attributed to weak central coherence in perceptual processing in Autism Spectrum Disorder (ASD). The present study used functional magnetic resonance imaging to examine the neural basis of EFT performance in 7- to 12-year-old ASD children and age- and IQ-matched controls. ASD children activated only a subset of the distributed network of regions activated in controls. In frontal cortex, control children activated left dorsolateral, medial and dorsal premotor regions whereas ASD children only activated the dorsal premotor region. In parietal and occipital cortices, activation was bilateral in control children but unilateral (left superior parietal and right occipital) in ASD children. Further, extensive bilateral ventral temporal activation was observed in control, but not ASD children. ASD children performed the EFT at the same level as controls but with reduced cortical involvement, suggesting that disembedded visual processing is accomplished parsimoniously by ASD relative to typically developing brains.