National 22-year epilepsy surgery landscape shows increasing open and minimally invasive pediatric epilepsy surgery.

OBJECTIVES: A surgical "treatment gap" in pediatric epilepsy persists despite the demonstrated safety and effectiveness of surgery. For this reason, the national surgical landscape should be investigated such that an updated assessment may more appropriately guide health care efforts. METHODS: In our retrospective cross-sectional observational study, the National Inpatient Sample (NIS) database was queried for individuals 0 to <18 years of age who had an International Classification of Diseases (ICD) code for drug-resistant epilepsy (DRE). This cohort was then split into a medical group and a surgical group. The former was defined by ICD codes for -DRE without an accompanying surgical code, and the latter was defined by DRE and one of the following epilepsy surgeries: any open surgery; laser interstitial thermal therapy (LITT); vagus nerve stimulation; or responsive neurostimulation (RNS) from 1998 to 2020. Demographic variables of age, gender, race, insurance type, hospital charge, and hospital characteristics were analyzed between surgical options. Continuous variables were analyzed with weight-adjusted quantile regression analysis, and categorical variables were analyzed by weight-adjusted counts with percentages and compared with weight-adjusted chi-square test results. RESULTS: These data indicate an increase in epilepsy surgeries over a 22-year period, primarily due to a statistically significant increase in open surgery and a non-significant increase in minimally invasive techniques, such as LITT and RNS. There are significant differences in age, race, gender, insurance type, median household income, Elixhauser index, hospital setting, and size between the medical and surgical groups, as well as the procedure performed. SIGNIFICANCE: An increase in open surgery and minimally invasive surgeries (LITT and RNS) account for the overall rise in pediatric epilepsy surgery over the last 22 years. A positive inflection point in open surgery is seen in 2005. Socioeconomic disparities exist between medical and surgical groups. Patient and hospital sociodemographics show significant differences between the procedure performed. Further efforts are required to close the surgical "treatment gap."

Vagus nerve stimulation and heart rate variability: A scoping review of a somatic oscillatory signal.

The goal of this review is to synthesize the literature on vagus nerve stimulator (VNS)-related changes in heart rate variability (HRV) in patients with drug-resistant epilepsy (DRE) and assess the role of these changes in seizure relief. A scoping literature review was performed with the following inclusion criteria: primary articles written in English, involved implantable VNS in humans, and had HRV as a primary outcome. Twenty-nine studies were retrieved, however with considerable heterogeneity in study methods. The overall depression in HRV seen in DRE patients compared to healthy controls persisted even after VNS implant, indicating that achieving "healthy" HRV is not necessary for VNS therapeutic success. Within DRE patients, changes in frequency domain parameters six months after VNS implant returned to baseline after a year. The mechanism of how VNS reduces seizure burden does not appear to be significantly related to alterations in baseline HRV. However, the subtlety of sympathetic/parasympathetic signaling likely requires a more structured approach to experimental and analytic techniques than currently found in the literature.

Pediatric neuromodulation for drug-resistant epilepsy: Survey of current practices, techniques, and outcomes across US epilepsy centers.

Neuromodulation via Responsive Neurostimulation (RNS) or Deep Brain Stimulation (DBS) is an emerging treatment strategy for pediatric drug-resistant epilepsy (DRE). Knowledge gaps exist in patient selection, surgical technique, and perioperative care. Here, we use an expert survey to clarify practices. Thirty-two members of the Pediatric Epilepsy Research Consortium were surveyed using REDCap. Respondents were from 17 pediatric epilepsy centers (missing data in one): Four centers implant RNS only while 13 implant both RNS and DBS. Thirteen RNS programs commenced in or before 2020, and 10 of 12 DBS programs began thereafter. The busiest six centers implant 6-10 new RNS devices per year; all DBS programs implant <5 annually. The youngest RNS patient was 3 years old. Most centers (11/12) utilize MP2RAGE and/or FGATIR sequences for planning. Centromedian thalamic nuclei were the unanimous target for Lennox-Gastaut syndrome. Surgeon exposure to neuromodulation occurred mostly in clinical practice (14/17). Clinically significant hemorrhage (n = 2) or infection (n = 3) were rare. Meaningful seizure reduction (>50%) was reported by 81% (13/16) of centers. RNS and DBS are rapidly evolving treatment modalities for safe and effective treatment of pediatric DRE. There is increasing interest in multicenter collaboration to gain knowledge and facilitate dialogue. PLAIN LANGUAGE SUMMARY: We surveyed 32 pediatric epilepsy centers in USA to highlight current practices of intracranial neuromodulation. Of the 17 that replied, we found that most centers are implanting thalamic targets in pediatric drug-resistant epilepsy using the RNS device. DBS device is starting to be used in pediatric epilepsy, especially after 2020. Different strategies for target identification are enumerated. This study serves as a starting point for future collaborative research.

Efficacy of vagus nerve stimulation in managing drug-resistant absence epilepsy syndromes.

PURPOSE: Around 11% of patients with absence epilepsy develop drug-resistant absence epilepsy (DRAE), and are at increased risk for developing psychiatric and neurologic comorbidities. Current therapeutic options for DRAE are limited. The purpose of this study was to assess the efficacy of vagus nerve stimulation (VNS) in treating DRAE. METHODS: Our institution maintains a database of patients who received VNS between 2010 and 2022. We identified DRAE patients who were <18 years of age at seizure onset, were electro-clinically diagnosed with an absence epilepsy syndrome (childhood absence, juvenile absence, or Jeavons Syndrome) by an epileptologist, and had normal brain imaging. The primary outcome measure was post-VNS absence seizure frequency. RESULTS: Twenty-six patients (M/F:14/12) were identified. Median age at seizure onset was 7 years (IQR 4-10) and patients experienced seizures for 6 years (IQR 4.3-7.6) before VNS. After VNS, the median absence seizure frequency reduced to 1.5 days (IQR 0.1-3.5) per week from 7 days (IQR 7-7), a 66% reduction seizure frequency. VNS responder rate was 80%, and seven patients achieved seizure freedom. There was no significant effect on VNS efficacy between the time from DRAE diagnosis to VNS placement (p = 0.067) nor the time from first seizure onset to VNS implant (p = 0.80). The median follow-up duration was 4.1 years (IQR 2.4-6.7), without any significant association between follow-up duration and VNS efficacy (r2=0.023) CONCLUSIONS: VNS is effective in managing DRAE. The responder rate was 80%; seizure improvement was independent of age at both seizure onset and latency to VNS after meeting DRAE criteria.

Super-selective Wada test for pre-surgical leg motor function assessment: A case report.

INTRODUCTION: Wada test is well-known to assess lateralization of memory and language functions; however, super-selective Wada (ss-Wada) to evaluate motor leg function is rare. We present a ss-Wada test within the anterior cerebral artery (ACA) to assess the motor function of the leg. METHODS: Retrospective chart review. RESULTS: Comprehensive phase-I/II surgical evaluation revealed an ictal focus around the left post-central gyrus with immediate involvement around the left para-central regions. To avoid potential right leg motor dysfunction with the surgery, the patient underwent a ss-Wada procedure. Angiography revealed bilateral ACAs were supplied by the left A1 segment. Super-selective microcatheter injection of amobarbital into the left ACA was performed to avoid cross-filling the contralateral ACA. The ss-Wada test confirmed no right leg motor impairment. Afterward, a craniotomy with direct cortical stimulation confirmed that the left-sided ictal/peri-ictal zone had no clear leg motor function. The patient underwent disconnection of that region and remained seizure-free at 10-month post-op follow-up without any motor or sensory deficits in the right limbs. CONCLUSION: This case demonstrates the proof of concept for ss-Wada in assessing lower extremity motor function. The ss-Wada procedure accurately predicted no motor deficits in the right leg, consistent with preserved motor function post-surgery.

Externalization of an infected vagus nerve stimulator generator without a break in anti-seizure therapy: a technical note.

INTRODUCTION: Vagus nerve stimulation (VNS) is a widely used treatment for drug-resistant epilepsy. An infection of the VNS generator pocket occurs in 3-8% of cases. Current standard of care requires the removal of the device, antibiotic therapy, and then replacement of the device. The consequential break in VNS therapy leaves patients highly susceptible to seizures. METHODS: Retrospective case report. RESULTS: The externalized generator continued to provide electroceutical coverage of the patient's seizures while the pocket was sterilized with IV antibiotics as well as betadine and local antibiotics. The externalized generator was kept safe against the patient's chest with ioban and an entriely new system was implanted on post-externalization day 5. The patient is now 7 months post-operative and without any evidence of infection. CONCLUSIONS: We report successful management of an infected VNS generator with externalization and short interval replacement of the entire system without any break in anti-seizure therapy.

Brief pump externalization without ITB wean to treat a probable pocket infection.

BACKGROUND AND IMPORTANCE: Intrathecal baclofen (ITB) pumps are an effective management tool for spasticity and dystonia. In the pediatric population, ITB complications including infection are common, and strategies to minimize their impact are important. Current standard of care for pump infection involves a wean of the ITB therapy followed by explantation of the hardware, which can be a three to 6-month process. CLINICAL PRESENTATION: We present a 20-year-old female who developed an pump pocket infection after revision. The pump was removed from the body but left connected. The pocket was debrided, thoroughly washed, and packed with multiple microbicidal agents. Nine days later, a new pump and proximal catheter was re-implanted without complications or interruption of medication administered intrathecally. One year later, the patient is doing well without any infectious problems. CONCLUSION: To our knowledge, this is the first report of an externalized pump and expedited reimplantation without a break in ITB therapy. Further studies are required in order to best characterize this technique as a potential alternative for infected baclofen pump pockets.

Evidence for Thalamic Responsive Neurostimulation in Treatment of Adult and Pediatric Epilepsy.

Responsive neurostimulation (RNS) has well-established efficacy in patients with identifiable seizure foci. Emerging evidence suggests the feasibility of expanding this treatment to patients with nonfocal or multifocal epileptic profiles with thalamic targeting. Our institution performed two successful implantations of thalamic RNS (tRNS) targeting the centromedian nucleus of the thalamus (CMT), and 1-year postoperative outcomes are provided. Additionally, a literature review of all reported tRNS was conducted. Publications were excluded if they did not include demographic data and/or epilepsy outcomes at follow-up. In the literature, 19 adult and 3 pediatric cases were identified. These cases were analyzed for outcome, indications, previous operations, and surgical practice variations. Both of our patients had failed multiple previous pharmacological and neurosurgical interventions for epilepsy. Case #1 underwent tRNS with bilateral CMT stimulation. Case #2 underwent tRNS with simultaneous right CMT and right insular stimulation, although an additional lead was placed in the left CMT and left capped for potential future use. Each has achieved ≥90% reduction in seizure burden and approach seizure freedom. 71% of patients in the literature review had multifocal, bilateral, or cryptogenic seizure onset. Three patients were implanted for Lennox Gastaut (2 of 3 are pediatric). 16 patients underwent an average of 1.6 failed procedures prior to successful tRNS implantation. Taken together, the 21 adult patients reviewed have experienced an average seizure reduction of 77% at the latest follow-up. 95% of the adult patients reported in the literature experienced >50% reduction in seizure activity following tRNS and 52% experienced ≥90% reduction in seizure burden following tRNS. Pediatric patients have experienced 70-100% improvement.

Epidural Effusion as Allergic Reaction Following Polyetheretherketone Cranioplasty: An Illustrative Case and Review of the Literature.

Well-described complications of polyetheretherketone (PEEK) cranioplasty in pediatric patients include surgical site infection, post-operative hematoma, cerebral edema, and implant fracture. We present a rare case of hypersensitivity to PEEK presenting as an epidural effusion in a 7-year-old male receiving a PEEK cranioplasty following a decompressive craniectomy. Within three weeks, the patient experienced fever and emesis. Erythrocyte sedimentation rate (ESR) was high (>130 mm/Hr) as well as C-reactive protein (CRP) (6.4 mg/dL). A brain MRI with contrast demonstrated both subgaleal and epidural fluid collections with T2 isointense columns projecting from the galeal surface, through the holes in the implant to the dural surface. The patient appeared clinically well. A sterile tap of the pericranial fluid showed no growth, b2-transferrin was negative, but the IgG level was high (>129.2 mg/dL) in the tap fluid. High-dose steroids reduced the epidural collection, but then the collection returned with steroid wean. A second cranioplasty operation replaced the PEEK flap with autologous bone. Postoperative imaging demonstrated markedly reduced fluid collections and a decreased midline shift. The patient remained clinically intact throughout the experience. PEEK allergy following cranioplasty is a rare entity and must be distinguished from infection or hematoma. Medical treatment with steroids can be attempted, but, if refractory, then appropriate treatment may necessitate removal of the offending PEEK implant.

Infectious Complications and Operative Management of Intrathecal Baclofen Pumps in the Pediatric Population: A Systematic Review and Meta-Analysis of 20 Years of Pooled Experience.

BACKGROUND: Intrathecal baclofen (ITB) is a treatment modality used to improve the quality of life of patients with intractable spasticity and dystonia. Although it is an effective solution in patients failing oral interventions, it is associated with potential infectious complications. It is known that pediatric patients with ITB have significantly higher infection rates compared with adult patients. The cause of these higher rates in pediatric patients remains unclear. In the present study, we performed a meta-analysis focusing on the incidence of infection, and clarification of potential risk factors for infection in pediatric patients with ITB. METHODS: This meta-analysis was performed in accordance with the PRISMA guidelines. An electronic database search was performed through PubMed, Web of Science, Embase, and Cochrane Library databases. Eligibility criteria and bias assessment were applied before statistical analysis. RESULTS: The 17 studies identified yielded 2238 pediatric patients treated with implanted ITB pumps between 1994 and 2014. Infection comprised 34% of observed complications, second only to catheter malfunction. Pediatric ITB primary infection ranged between 0% and 44% among included studies (interquartile range, 4.85%-18.85%). A linear mixed-effects regression model showed that subfascial implantation had 12% lower primary infection rates compared with subcutaneous implantations across the literature. The relative risk of infection was 56% lower in pediatric patients with subfascially implanted ITB pumps. CONCLUSIONS: Surgeons and clinicians should use these data to better assess patient risk-benefit when considering ITB pump implantation.

Risk of occult spinal dysraphism based on lumbosacral cutaneous manifestations.

OBJECTIVES: Occult spinal dysraphism is a congenital failure of fusion of the posterior vertebral arches with intact skin overlying the defect. Lumbosacral cutaneous manifestations are associated with a variable risk of occult spinal dysraphism. Tethered cord syndrome is a type of occult spinal dysraphism that puts abnormal traction on the spinal cord. This study analyzed neonates and infants who were referred to our pediatric urology practice and had evidence of lumbosacral cutaneous manifestation on physical examination. METHODS: We reviewed the presence of lumbosacral cutaneous manifestations in neonates and infants evaluated in our pediatric urology clinic at our Institution over a 6-year period (1 March, 2015-28 February, 2021) with no prior diagnosis of lumbosacral cutaneous manifestation. All patients underwent a spinal ultrasound. RESULTS: The most common lumbosacral cutaneous manifestations were bifurcated/duplicated gluteal folds (33%), gluteal asymmetry (19%), and sacral dimples (14%). A total of 34 (24%) patients had an abnormal spinal ultrasound; 15 (44%) of these infants underwent a lumbar magnetic resonance imaging. A coccygeal pit was statistically marginally higher in abnormal versus normal spinal ultrasound (p = 0.07). Patients with only one lumbosacral cutaneous manifestation (N = 121) were significantly more likely to have a normal spinal ultrasound compared to those with two or more lumbosacral cutaneous manifestation (N = 17) (79% vs 53%, p = 0.03). CONCLUSION: Due to the varying risk of certain lumbosacral cutaneous manifestations with occult spinal dysraphism, all patients with a lumbosacral cutaneous manifestation should undergo spinal ultrasound. This study also highlights the importance of urodynamic studies when there are abnormal cutaneous findings. Routine physical examinations of the lumbar region for cutaneous manifestations of occult spinal dysraphism are vital to ensure prompt management of tethered cord syndrome and avoid potentially devastating consequences.

Management of Pre-existing Ventriculoperitoneal Shunt in Posterior Vault Distraction for Lambdoid Craniosynostosis: A Case Report and Technical Note.

Posterior vault distraction osteogenesis (DO) is an emerging treatment option for craniosynostosis. Operative nuances detailing surgical management are being described with increasing use and experience. In this article, we discuss the surgical management of an 8-month-old male with a ventriculoperitoneal shunt (VPS) diagnosed with bilateral lambdoid craniosynostosis and Chiari I malformation. The patient underwent successful bilateral posterior fossa DO without surgical re-implantation of the shunt. Pre- and post-operative imaging confirmed no migration of the VPS. Intracranial volume increased by 20.1% and posterior fossa volume increased by 39.9%. Our experience illustrates that posterior vault DO can be done safely in the setting of a parieto-occipital VPS, in a single operative setting, without the need of additional procedures.

Three-Dimensional Volumetric Changes in Posterior Vault Distraction With Distraction Osteogenesis.

OBJECT: Distraction osteogenesis (DO) may allow for maximal volumetric expansion in the posterior vault (PV) by overcoming viscoelastic forces of overlying soft tissues. Little evidence exists regarding surgical planning and anticipated 3D volumetric changes pre- and post-operatively. We aim to study the volumetric changes occurring in PV distraction in lambdoid craniosynostosis. METHODS: From 2007 to 2019, a single institution retrospective review revealed 232 craniosynostosis patients. Fourteen demonstrated lambdoid synostosis (6%), and of those, 11 patients were included in the study due to treatment with PVDO or representative sample. Six patients had unilateral synostosis and 5 had bilateral synostosis. Imaging protocol for PVDO patients included preoperative head CT within 1 month of surgery and 8 weeks following distraction cessation with weekly skull plain films. 3D volumetric analyses were performed on pre and postoperative head CT using 3D Slicer software. RESULTS: Posterior fossa volume (PFV) increased by 38.7% and foramen magnum area increased by 26.9% postoperatively. Unilateral lambdoid craniosynostosis patients had greater increases in PFV versus bilateral lambdoid craniosynostosis patients (63.5% versus 8.9%, P = 0.007). Osteotomy to the asterion was more effective in increasing PFV versus osteotomy to foramen magnum (P = 0.050). Placement of distractor in the inferior third of the lambdoid suture is more effective in increasing PFV versus placement in the middle or top third of the suture (P = 0.041). CONCLUSIONS: Highest volumetric increases are seen in unilateral lambdoid synostosis. Extending osteotomy beyond the asterion is not necessary for maximal PV volumetric gain. Placement of distractor in the inferior third of the suture leads to maximal PV volumetric gains.

Protocol-driven prevention of perioperative hypothermia in the pediatric neurosurgical population.

OBJECTIVE: Perioperative hypothermia (PH) is a preventable, pathological, and iatrogenic state that has been shown to result in increased surgical blood loss, increased surgical site infections, increased hospital length of stay, and patient discomfort. Maintenance of normothermia is recommended by multiple surgical quality organizations; however, no group yet provides an ergonomic, evidence-based protocol to reduce PH for pediatric neurosurgery patients. The authors' aim was to evaluate the efficacy of a PH prevention protocol in the pediatric neurosurgery population. METHODS: A prospective, nonrandomized study of 120 pediatric neurosurgery patients was performed. Thirty-eight patients received targeted warming interventions throughout their perioperative phases of care (warming group-WG). The remaining 82 patients received no extra warming care during their perioperative period (control group-CG). Patients were well matched for age, sex, and preparation time intraoperatively. Hypothermia was defined as < 36°C. The primary outcome of the study was maintenance of normothermia preoperatively, intraoperatively, and postoperatively. RESULTS: WG patients were significantly warmer on arrival to the operating room (OR) and were 60% less likely to develop PH (p < 0.001). Preoperative forced air warmer use both reduced the risk of PH at time 0 intraoperatively and significantly reduced the risk of any PH intraoperatively (p < 0.001). All patients, regardless of group, experienced a drop in core temperature until a nadir occurred at 30 minutes intraoperatively for the WG and 45 minutes for the CG. At every time interval, from preoperatively to 120 minutes intraoperatively, CG patients were between 2 and 3 times more likely to experience PH (p < 0.001). All patients were warm on arrival to the postanesthesia care unit regardless of patient group. CONCLUSIONS: Preoperative forced air warmer use significantly increases the average intraoperative time 0 temperature, helping to prevent a fall into PH at the intraoperative nadir. Intraoperatively, a strictly and consistently applied warming protocol made intraoperative hypothermia significantly less likely as well as less severe when it did occur. Implementation of a warming protocol necessitated only limited resources and an OR culture change, and was well tolerated by OR staff.

Repair of a large primary subtemporal encephalocele in a 3-year-old child: case report.

Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.An 8-month-old boy presented to the authors' service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed using a preauricular infratemporal approach when the patient was 3 years old. Gliotic tissue was resected and amygdala, hippocampus, and middle cerebral artery were preserved.The immediate results of the operation showed good immediate outcome. Seizure freedom and neurodevelopment outcomes remain to be seen at follow-up.

Importance of Physical Examination and Imaging in the Detection of Tethered Cord Syndrome.

Tethered cord syndrome (TCS) is a type of occult spinal dysraphism that may lead to permanent neurologic and orthopedic deficits. Infants with TCS may have lumbosacral cutaneous malformations (LsCMs). We studied 67 infants referred to a single pediatric urology practice for a urological concern unrelated to occult spinal dysraphism with no prior diagnosis of LsCM between March 1, 2015 and September 30, 2018. Each infant underwent a spinal ultrasound. If an abnormality was detected, they were referred to a pediatric neurosurgeon. The most common cutaneous manifestations were duplicated or bifurcated (46%) gluteal folds and gluteal asymmetry (16%). Fourteen (21%) of the 67 patients had an abnormal spinal ultrasound; 5 of the 14 infants underwent a lumbar magnetic resonance imaging. One infant had urodynamics studies and a tethered cord release. Pediatricians should be familiar with TCS and perform lumbar physical examinations for LsCMs suggestive of TCS to ensure prompt diagnosis and management and avoid potentially devastating complications.

Demographics, clinical features, and response to conventional treatments in pediatric Pseudotumor Cerebri syndrome: a single-center experience.

OBJECTIVE: The goal of this study was to better understand pediatric Pseudotumor Cerebri syndrome, and its relationship to age, obesity, and other medical conditions; and to evaluate response to conventional treatments. METHODS: A retrospective chart review was performed on consecutive patients who were diagnosed with PTCS between January 1, 2007, and July 31, 2014. A total of 78 patients were included in this study: 54 female (69.3%) and 24 male (30.7%). Variables including age, sex, body mass index, concomitant medical conditions, secondary causes, associated symptoms, physical exam findings, imaging results, recurrence of symptoms, and treatment modalities were analyzed. Patients were grouped into "pre-kindergarten," "elementary," and "adolescent" based on their age; and weight categories of underweight, normal weight, overweight, moderately, and severely obese. RESULTS: Mean age of symptom onset was 11.92 ± 4.09 years. Elementary and adolescent age patients were more likely to be overweight, moderately obese, and severely obese, while this finding was not found for patients in pre-kindergarten group. Headache (83.3%) and visual disturbances (48.7%) were the most common presenting complaints. Asthma (16.6%) was the most common associated concomitant medical condition. Medical management resulted in resolution in 84% of population, 15% required surgical interventions, and the recurrence rate was found to be 20.5%. There was a statistically significant trend in success with medical management in younger patients (p = 0.04), while medically refractory PTCS was seen in adolescent females. Recurrence of PTCS had a linear trend with increased occurrence in adolescent age group with higher BMI. Asthma was observed to be frequently associated with PTCS in our cohort. Obesity is strongly associated with PTCS, not only in the adolescent group but also in the younger elementary age group. Treatment remains similar to management in the adults with a good response (84%) to medical management and a low relapse rate.

Neoadjuvant chemotherapy for atypical teratoid rhabdoid tumors: case report.

Atypical teratoid rhabdoid tumors (ATRTs) are a rare pediatric brain tumor with high mortality rate. Several large series have reported achieving gross-total resection (GTR) in less than 50% of patients due to the lesions' large size, vascularity, and limited blood volume in young patients. While neoadjuvant chemotherapy for choroid plexus carcinomas in pediatric patients has become widely accepted, it has not been used as widely for other pediatric brain tumors. To the best of the authors' knowledge, there are only 3 published cases of neoadjuvant chemotherapy for ATRTs. In the present report, the authors present a fourth case of neoadjuvant chemotherapy for ATRT and review the available literature on this strategy. A 17-month-old child presented with a left ventricular ATRT for which imaging raised concern for a highly vascularized tumor. The authors undertook neoadjuvant chemotherapy with 2 cycles of Head Start II therapy, which reduced the size of the ventricular tumor by 35% and decreased the vascularity of the lesion on imaging. The estimated blood loss during resection was 425 ml and GTR was achieved. The patient continued with postoperative chemotherapy but suffered an on-therapy recurrence. While higher-quality data are necessary, available evidence suggests that neoadjuvant chemotherapy can reduce the size and vascularity of ATRTs and facilitate a surgical avenue for large or "inoperable" tumors.

Congenital myotonic dystrophy: ventriculomegaly and shunt considerations for the pediatric neurosurgeon.

PURPOSE: Ventriculomegaly in infants with congenital myotonic dystrophy (CDM) is common, and the neurosurgical determination of shunting is complex. The natural history of CDM-associated ventriculomegaly from prenatal to natal to postnatal stages is poorly known. The relationship between macrocephaly and ventriculomegaly, incidence of shunt necessity, and early mortality outcomes lack pooled data analysis. This study aims to review clinical features and pathophysiology of CDM, with emphasis on ventriculomegaly progression, ventriculomegaly association with macrocephaly, and incidence of shunting. METHODS: This is a literature review with pooled data analysis and case report. RESULTS: One hundred four CDM patients were reviewed in 13 articles that mentioned CDM with ventriculomegaly and/or head circumference. Data was very limited: only 7 patients had data on the presence or absence of prenatal ventriculomegaly, 97 on ventriculomegaly at birth, and 32 on whether or not the ventricles enlarged post-natally. Three patients of 7 (43 %) had pre-natally diagnosed ventriculomegaly, 43 of 97 (44 %) had ventriculomegaly at birth, and only 5 of 32 (16 %) had progressive enlargement of ventricles post-natally. Only 5 of 104 patients had a documented shunt placement: 1 for obstructive, 1 for a post-hemorrhagic communicating, 2 for a communicating hydrocephalus without hemorrhage, and 1 with unknown indication. Of 13 macrocephalic patients with data about ventricular size, 12 had ventriculomegaly. CONCLUSIONS: Ventriculomegaly occurs regularly with CDM but most often does not require CSF diversion. Decisions regarding neurosurgical intervention will necessarily be based on limited information, but shunting should only occur once dynamic data confirms hydrocephalus.

Management of a 3-year-old with an unstable C6-C7 diastasis without quadriplegia.

BACKGROUND: Caring for pediatric spine trauma patients places spine surgeons in situations that require unique solutions for complex problems. Recent case reports have highlighted a specific injury pattern to the lower cervical spine in very young children that is frequently associated with complete spinal cord injury. METHODS: This report describes the presentation and treatment of a C6-C7 dislocation in a 3-year-old patient with an incomplete spinal cord injury. The highly unstable cervical injury and the need to prevent neurologic decline added complexity to the case. RESULTS: A multi-surgeon team allowed for ample manpower to position the patient; with individuals with the requisite training and experience to safely move a patient with a highly unstable cervical spine. Initial closed reduction under close neurophysiologic monitoring, posterior fusion and immediate anterior stabilization lead to a successful patient outcome with preserved neurologic function. A traumatic cerebrospinal fluid leak, while a concern early on during the procedure, resolved without direct dural repair and did not complicate the patient's fusion healing. Additional anterior stabilization and fusion allowed long-term stability with bone healing that may not be achievable with posterior fixation and/or soft tissue healing alone. CONCLUSIONS: Familiarity with the challenges and solutions presented in the case may be useful to surgeons who could face a similar challenge in the future.