Management of Total Frontal Bone Loss After Surgery for Craniosynostosis: The Modified Visor Bone Flap With Brain Cage.

Complications after craniosynostosis surgery occur in 11% to 36% of cases and may be precipitated by poor soft tissue coverage and concomitant exposure of non-sterile regions; sequelae may result in infection, osteomyelitis, and bone loss requiring complex reconstruction. In the pediatric population, autologous cranioplasty remains the gold standard due to growth potential and a more favorable complication profile than synthetic cranioplasty. Virtual surgery planning (VSP) and computer-assisted design (CAD)/computer-assisted manufacturing (CAM) technology can be utilized to create innovative, patient-specific autologous solutions, similar to the approach with synthetic cranioplasty. A novel surgical approach using VSP was used for an 18-month-old female with near total bifrontal bone loss. Surface area measurements were used to determine the amount of bone available to replace the infected frontal bone. VSP was utilized to determine the most efficient construct configuration possible to achieve maximal coverage via calculation of cranial bone surface area measurements. Surgical reconstruction of the defect was planned as a Modified Visor Bone Flap with Posterior Brain Cage. A construct was fashioned from available cranial bone struts to obtain widespread coverage. 3D Recon images from before and after surgery demonstrate almost complete re-ossification of the cranial vault with significant resulting clinical improvement. Reconstruction of total frontal bone loss is possible by utilizing this technique. VSP can improve the safety and efficiency of complex autologous cranial bone reconstructions. We propose a treatment algorithm to address the problem of near total frontal bone loss in young children for whom alloplastic implants are not suitable.

A multi-stem cell basis for craniosynostosis and calvarial mineralization.

Craniosynostosis is a group of disorders of premature calvarial suture fusion. The identity of the calvarial stem cells (CSCs) that produce fusion-driving osteoblasts in craniosynostosis remains poorly understood. Here we show that both physiologic calvarial mineralization and pathologic calvarial fusion in craniosynostosis reflect the interaction of two separate stem cell lineages; a previously identified cathepsin K (CTSK) lineage CSC1 (CTSK+ CSC) and a separate discoidin domain-containing receptor 2 (DDR2) lineage stem cell (DDR2+ CSC) that we identified in this study. Deletion of Twist1, a gene associated with craniosynostosis in humans2,3, solely in CTSK+ CSCs is sufficient to drive craniosynostosis in mice, but the sites that are destined to fuse exhibit an unexpected depletion of CTSK+ CSCs and a corresponding expansion of DDR2+ CSCs, with DDR2+ CSC expansion being a direct maladaptive response to CTSK+ CSC depletion. DDR2+ CSCs display full stemness features, and our results establish the presence of two distinct stem cell lineages in the sutures, with both populations contributing to physiologic calvarial mineralization. DDR2+ CSCs mediate a distinct form of endochondral ossification without the typical haematopoietic marrow formation. Implantation of DDR2+ CSCs into suture sites is sufficient to induce fusion, and this phenotype was prevented by co-transplantation of CTSK+ CSCs. Finally, the human counterparts of DDR2+ CSCs and CTSK+ CSCs display conserved functional properties in xenograft assays. The interaction between these two stem cell populations provides a new biologic interface for the modulation of calvarial mineralization and suture patency.

Comparative Analysis of 2D and 3D Metrics for Evaluation of Postoperative Outcomes Following Endoscopic Suturectomy for Sagittal Craniosynostosis.

To assess whether 3-dimensional (3D) volumetrics can be used to track and evaluate postoperative course of patients treated with endoscopic suturectomy for nonsyndromic sagittal synostosis, we compared changes in 2-dimensional (2D) measurements along with 3D volumetric correlates throughout the period of helmet therapy. Forty-six patients treated at our institution with endoscopic suturectomy for sagittal synostosis were retrospectively reviewed. Head circumference (HC), cephalic index (CI), and total cranial volumes (TCVs) were measured at 3 timepoints following surgery using optical surface scans obtained for helmet orthotics. All measurements showed significant differences between timepoints on the analysis of variance ( P <0.001). There was a significant correlation between CI and TCV (r=0.35, P =0.004) and between HC and TCV (r=0.81, P <0.001). The normalized rate of change over the course of treatment was significantly higher for TCV (36.7%) than for CI (8.8%) and HC (8.4%, P <0.001), with no difference between HC and CI. The authors conclude that 3D metrics were able to reliably follow the course of postoperative 2D metrics. There was a direct and linear correlation between HC and CI with TCV. Total cranial volumes showed the highest rate of sustained change at every timepoint. Although CI and HC plateau after the first measurement, TCV continues to adapt over the course of treatment. These results demonstrate the feasibility and value of volumetrics from 3D imaging to provide a more comprehensive evaluation of postoperative surgical outcomes than traditional 2D metrics without the ionizing radiation traditionally utilized for CT to obtain 3D metrics.

Complications in craniosynostosis surgery in patients with rickets: illustrative case and systematic review of literature.

BACKGROUND: Craniosynostosis (CSS) is the premature fusion of calvarial sutures associated with identified genetic mutations or secondary to alterations in intracranial pressure, brain, or bone growth patterns. Of the metabolic etiologies implicated in CSS, X-linked hypophosphatemic rickets (XLHR) is the most common, with dysfunctional bone mineralization leading to progressive hyperostosis and delayed synostosis. There is a paucity of literature discussing the unique surgical considerations for XLHR-related CSS. OBSERVATIONS: A 26-month-old male with XLHR-related sagittal CSS underwent cranial vault remodeling (CVR). Surgery was complicated by the presence of diploic hypertrophy with significant intraoperative estimated blood loss (EBL). EBL greatly exceeded reference ranges for CVR in all-cause CSS. As a result, the surgical goals were modified and the complete planned procedure aborted. Subsequent review of preoperative imaging revealed multiple fine vascular lacunae within the bone. A systematic literature review was conducted to identify reported complications relating to surgical intervention for rickets-associated CSS. LESSONS: Future considerations for patients with XLHR-related CSS should emphasize awareness of metabolic risk factors with associated complications, and the need for selection of approach and operative management techniques to avoid EBL. Further research is required to elucidate underlying mechanisms and determine whether the encountered phenomenon is characteristic across this patient population and potentially minimized by preoperative medical therapy.

Examining the Role of Early Diagnostic Imaging for Craniosynostosis in the Era of Endoscopic Suturectomy: A Single Institution Experience.

ABSTRACT: Endoscopic suturectomy is a minimally invasive surgical treatment for single-suture craniosynostosis in children between 1 and 4 months of age. This study sought to characterize the role played by diagnostic imaging in facilitating early surgical management with endoscopic suturectomy. The authors also characterized the overall diagnostic utility of imaging in patients assessed for abnormal head shape at their institution, regardless of surgical status. A retrospective cohort of children diagnosed with singlesuture synostosis undergoing either primary endoscopic suturectomy or open calvarial reconstruction at the authors' institution from 1998 to 2018 was first reviewed. Of 132 surgical patients, 53 underwent endoscopic suturectomy and 79 underwent open repair. There was no difference in the proportion of endoscopic and open surgery patients imaged preoperatively before (24.5% versus 35.4%; P = 0.24) or after (28.3% versus 25.3%; P = 0.84) craniofacial assessment. Stratifying by historical epoch (1998-2010 versus 2011-2018), there was also no difference found between preoperative imaging rates (63.6% versus 56.4%; P = 0.35). In another cohort of 175 patients assessed for abnormal head shape, 26.9% were imaged to rule out craniosynostosis. Positive diagnostic imaging rates were recorded for suspected unicoronal (100%), metopic (87.5%), lambdoidal (75.0%), sagittal (63.5%), multisuture (50%), and bicoronal (0%) synostosis. The authors conclude that the use of diagnostic imaging at their institution has not increased despite higher utilization of endoscopic suturectomy and need for expedient identification of surgical candidates.However, their results suggest that imaging may play a greater diagnostic role for suspected bicoronal, sagittal, and multi-sutural synostosis among sutural subtypes of synostosis.

Arachnoid cysts: using prenatal imaging and need for pediatric neurosurgical intervention to better understand their natural history and prognosis.

OBJECTIVES: Intracranial arachnoid cysts are commonly characterized as congenital. Evidence to support a congenital origin is scant and documented evolution during infancy also calls into question the genesis of these lesions. To improve our understanding of the natural history and the clinical significance of arachnoid cysts on prenatal ultrasound, we conducted a study to describe the fate of these cysts after initial diagnosis. METHODS: We conducted a retrospective descriptive review of all prenatal ultrasounds with reported intracranial arachnoid cysts at a tertiary care center from 2010 to 2016 and cohort study comparing patients with additional ultrasound abnormalities to those with an isolated finding of arachnoid cyst. Data collected included gestational age at cyst diagnosis, cyst evolution on follow-up imaging, cyst size and cyst location, postnatal imaging and neurosurgical consultation and intervention. Statistical analysis including Chi-square and Fisher's exact tests and univariate logistic regressions were performed using Stata v. 13 (StataCorp 2013. Stata Statistical Software: Release 13. College Station, TX: StataCorp LP). Confidence intervals were reported at 95% and a p-value < .05 was considered significant. RESULTS: A total of 33,621 anatomical ultrasound scans were performed from 2010 to 2016. Seventy patients (0.2%) had prenatal findings of arachnoid cyst, of which the mean gestational age was 21.46 weeks. Ventriculomegaly was observed in one patient (1.4%) at the time of diagnosis and other abnormalities were found in twelve patients (17%). Complete sonographic follow-up prior to delivery was obtained in 53 (75.7%) patients with 5(9.4%) progression, 5(9.4%) regression, and 43(81.1%) no longer visualized. Forty-three cysts (81.1%) were no longer visualized on follow-up imaging, all of which were interhemispheric in location. All of the cysts that progressed in size on prenatal ultrasounds were > 2 cm in size on initial diagnosis. Cyst fenestration was performed in two patients (2.9%) that had arachnoid cysts measuring at least 3 cm in each dimension on prenatal imaging, one at eight months and one at six months of age. Patients with and without additional sonographic abnormalities had similar rates of AMA (23.1% vs 26.3%, p = .56), nulliparity (61.5% vs 70.2%, p = .38), obesity (0.0% vs 12.3%, p = .221), major medical comorbidities (38.5% vs 33.3%, p = .48) and pregnancy achieved via Assisted Reproductive Technology (38.5% vs 24.6%, p = .25), respectively. Patients with additional sonographic abnormalities were significantly more likely to have prenatal Magnetic Resonance Imaging (OR: 8.28, CI: 1.84-40.4, p = .07), prenatal neurosurgery consultation (OR: 8.25, CI: 1.23-69.05, p = .04) and invasive diagnostic genetic testing (OR: 11.25, CI: 2.33-64.35, p = .003). CONCLUSIONS: Arachnoid cysts are infrequently found on prenatal screening. Size greater than 2 cm on second trimester ultrasound and location outside of the interhemispheric fissure may indicate the need for further evaluation and eventual fenestration. Prenatally diagnosed arachnoid cysts are not typically associated with other anatomic or genetic abnormalities, although the presence of additional abnormalities usually leads to more intensive prenatal and postnatal investigations.

Outcomes of a virtual craniofacial clinic for assessing plagiocephaly during the COVID-19 pandemic.

OBJECTIVE: The aim of this study was to carry out a quantitative analysis of a virtual craniofacial clinic during the COVID-19 pandemic. METHODS: The charts of 90 patients from a single institution were reviewed. Of these patients, 45 visited the virtual clinic during the COVID-19 pandemic. The other 45 patients visited the clinic in the 3 months prior to COVID-19. Demographics including the mean age at the visit, chief complaint, visit diagnosis, appointment duration, helmet usage, accuracy of the diagnosis, need for a CT scan, and the need for a follow-up appointment were assessed. Diagnostic accuracy, the frequency of follow-up appointments, and patient satisfaction (via survey), as well as additional associated factors, were analyzed to determine the efficacy and satisfaction associated with the virtual clinic approach. RESULTS: The mean patient age at time of the visit was 5.6 and 7.3 months (p = 0.244), and the mean time from referral to appointment was 19.2 and 19 days (p = 0.934), in the in-person and virtual cohorts, respectively. There was no significant difference in the variety of chief complaints between the in-person and virtual visits, with 97.8% and 93.3% of patients' parents reporting abnormal head shape, respectively, and the remainder reporting more infrequent complaints (p = 0.435). The visit diagnosis was plagiocephaly in 93.3% of the in-person cohort and 80.0% of the virtual cohort (p = 0.118). The final diagnosis exhibited a similar pattern, with 95.6% of the in-person cohort and 88.9% of the virtual cohort observed as positional plagiocephaly; the remaining diagnoses were more infrequent (p = 0.434). The most common alternative diagnosis in the virtual visit cohort was a metopic ridge (8.4%). In the in-person visit cohort, the most common alternative diagnosis was equally a benign enlargement of the subarachnoid space in infancy, scalp mass, and skull lesion (2.2% each). None of the patients in either cohort were diagnosed with synostosis. Eighty percent of the in-person visits were 15 to 30 minutes in duration, with the remaining 20% being 31 minutes or longer; virtual visits were all 30 minutes or less, with 95.6% being 15 to 30 minutes (p = 0.002). Helmets were prescribed for 2 patients in the in-person cohort and no patients in the virtual cohort (p = 0.494). Alterations in diagnosis were made in 2.2% of in-person visits and 6.7% of virtual visits (p = 0.616). Follow-up was required in 15.6% of the in-person visits and 31.1% of the virtual visits (p = 0.134). CT was only utilized twice, once in the in-person visit cohort and once in the virtual visit cohort. CONCLUSIONS: Virtual clinic encounters resulted in comparable diagnostic accuracy. The trend toward frequent follow-up assessments and changes in the final diagnosis in the virtual clinic cohort has indicated a level of diagnostic uncertainty via the virtual interface, which required in-person assessment for confirmation. This finding did not contribute toward diagnostic inaccuracy with respect to missed synostosis. The study results have indicated that telemedicine can be an effective modality in assessing craniofacial pathology.

Closed-loop vagal nerve stimulation for intractable epilepsy: A single-center experience.

PURPOSE: A new class of heart-rate sensing, closed-loop vagal nerve stimulator (VNS) devices for refractory epilepsy may improve seizure control by using pre-ictal autonomic changes as an indicator for stimulation. We compared our experience with closed- versus open-loop stimulator implantation at a single institution. METHODS: We conducted a retrospective chart review of consecutive VNS implantations performed from 2004 to 2018. Bivariate and multivariable analyses were performed to compare changes in seizure frequency and clinical outcomes (Engel score) with closed- versus open-loop devices. Covariates included age, duration of seizure history, prior epilepsy surgery, depression, Lennox Gastaut Syndrome (LGS), tonic seizures, multiple seizure types, genetic etiology, and VNS settings. We examined early (9-month) and late (24-month) outcomes. RESULTS: Seventy subjects received open-loop devices, and thirty-one received closed-loop devices. At a median of 8.5 months, there was a greater reduction of seizure frequency after use of closed-loop devices (median 75% [IQR 10-89%]) versus open-loop (50% [0-78%], p < 0.05), confirmed in multivariable analysis (odds ratio 2.72 [95% CI 1.02 - 7.4]). Similarly, Engel outcomes were better after closed-loop compared to open-loop confirmed in the multivariable analysis at the early timepoint (OR 0.26 [95% CI 0.09 - 0.69]). These differences did not persist at a median of 24.5 months. CONCLUSIONS: This retrospective single-center study suggests the use of closed-loop VNS devices is associated with greater seizure reduction and more favorable clinical outcomes than open-loop devices at 9-months though not at 24-months. Expansion of this study to other centers is warranted to increase the generalizability of our study.

Analysis of the Sonopet Ultrasonic Bone Aspirator to Traditional Instrumentation for Endoscopic Suturectomy for Craniosynostosis.

OBJECTIVE: The goal of endoscopic treatment for craniosynostosis is to remove the fused suture and achieve calvarial remodeling with external orthosis. To reduce the need for secondary surgery and to minimize blood loss, instruments that maximize bone removal while minimizing blood loss and risk of dural injury are evolving. The authors therefore assess the safety and efficacy of the Sonopet Ultrasonic Bone Aspirator (UBA) (Stryker, Kalamazoo, MI) for endoscopic suturectomy compared to traditional instrumentation at our institution. METHODS: Retrospective chart review of consecutive endoscopic suturectomies performed from 2011 to 2019 at Weill Cornell Medical Center was conducted, including demographics, cephalic index, surgical indications, operative time, cosmetic and functional results, complications, estimated blood loss (EBL), re-operation rate, length of stay, and length of helmet therapy. These variables were then compared between the Sonopet and non-Sonopet cohorts. RESULTS: Of the 60 patients who underwent endoscopic suturectomy, 16 cases (26.7%) utilized the Sonopet. Mean operative time was 2.8 ±â€Š0.4 hours in the Sonopet group, compared to 3.2 ±â€Š1.2 hours (P = 0.05) without the Sonopet. EBL was 17.8 ±â€Š23.9 cc versus 34.7 ±â€Š75.5 cc (P = 0.20) with versus without the Sonopet respectively. Length of stay and duration of helmet therapy were similar in both groups, ranging from 1 to 3 days (P = 0.68) and 7.25 to 12 months (P = 0.30) respectively. There were no reoperations in the Sonopet group with a mean follow up of 9.18 months. There were 3 reoperations in the non-Sonopet group with a mean follow up of 11.3 months. Among the cases utilizing the Sonopet, 13 (81%) were metopic and three (19%) were coronal synostoses. Of the non-Sonopet cases, 27 (61%) were sagittal, 8 (18%) were metopic, 7 (16%) were coronal, and 2 (5%) were lambdoid synostoses. CONCLUSIONS: The use of the Sonopet resulted in a mean decrease in operative time at our institution (P = 0.18). Lower EBL and reoperation rates with comparable LOS and helmet therapy duration were also seen. This modality should be considered a safe and effective adjunct in appropriate endoscopic craniosynostosis cases.

Utility of invasive electroencephalography in children 3 years old and younger with refractory epilepsy.

OBJECTIVE: Early surgical intervention for pediatric refractory epilepsy is increasingly advocated as surgery has become safer and data have demonstrated improved outcomes with early seizure control. There is concern that the risks associated with staged invasive electroencephalography (EEG) in very young children outweigh the potential benefits. Here, the authors present a cohort of children with refractory epilepsy who were referred for invasive monitoring, and they evaluate the role and safety of staged invasive EEG in those 3 years old and younger. METHODS: The authors conducted a retrospective review of children 3 years and younger with epilepsy, who had been managed surgically at two institutions between 2001 and 2015. A cohort of pediatric patients older than 3 years of age was used for comparison. Demographics, seizure etiology, surgical management, surgical complications, and adverse events were recorded. Statistical analysis was completed using Stata version 13. A p < 0.05 was considered statistically significant. Fisher's exact test was used to compare proportions. RESULTS: Ninety-four patients (45 patients aged ≤ 3 [47.9%]) and 208 procedures were included for analysis. Eighty-six procedures (41.3%) were performed in children younger than 3 years versus 122 in the older cohort (58.7%). Forty-two patients underwent grid placement (14 patients aged ≤ 3 [33.3%]); 3 of them developed complications associated with the implant (3/42 [7.14%]), none of whom were among the younger cohort. Across all procedures, 11 complications occurred in the younger cohort versus 5 in the older patients (11/86 [12.8%] vs 5/122 [4.1%], p = 0.032). Two adverse events occurred in the younger group versus 1 in the older group (2/86 [2.32%] vs 1/122 [0.82%], p = 0.571). Following grid placement, 13/14 younger patients underwent guided resections compared to 20/28 older patients (92.9% vs 71.4%, p = 0.23). CONCLUSIONS: While overall complication rates were higher in the younger cohort, subdural grid placement was not associated with an increased risk of surgical complications in that population. Invasive electrocorticography informs management in very young children with refractory, localization-related epilepsy and should therefore be used when clinically indicated.

Foundations of the Diagnosis and Surgical Treatment of Epilepsy.

The diagnostic and surgical management of epilepsy has made enormous strides over the past 3 decades, concomitant with advances in technology and electrophysiologic understanding of neuronal connectivity. Distinct zones have been identified within this network that each communicate and play a role in the genesis of seizures. Invasive and noninvasive modalities for defining the epileptogenic lesion or region have been able to more accurately determine which patients are optimal candidates for treatment when their seizures are refractory to conventional conservative management. Ablative, palliative, and disconnecting procedures have been developed as alternatives for traditional open resection techniques, and in recent studies, they have shown excellent seizure control and mitigation of complications. In this review, we discuss the evolution of these advancements in the management of epilepsy and provide an overview of current and future neurosurgical therapeutic modalities.

Challenges of Epilepsy Surgery.

Though frequently effective in the management of medically refractory seizures, epilepsy surgery presents numerous challenges. Selection of the appropriate candidate patients who are likely to benefit from surgery is critical to achieving seizure freedom and avoiding neurocognitive morbidity. Identifying the seizure focus and mapping epileptogenic networks involves an interdisciplinary team dedicated to formulating a safe and effective surgical plan. Various strategies can be employed either to eliminate the epileptic focus or to modulate network activity, including resection of the focus with open surgery or laser interstitial thermal therapy; modulation of epileptogenic firing patterns with responsive neurostimulation, deep brain stimulation, or vagus nerve stimulation; or non-invasive disconnection of epileptic circuits with focused ultrasound, which is also discussed in greater detail in the subsequent chapter in our series. We review several challenges of epilepsy surgery that must be thoughtfully addressed in order to ensure its success.

Innovations in the Neurosurgical Management of Epilepsy.

Technical limitations and clinical challenges have historically limited the diagnostic tools and treatment methods available for surgical approaches to the management of epilepsy. By contrast, recent technological innovations in several areas hold significant promise in improving outcomes and decreasing morbidity. We review innovations in the neurosurgical management of epilepsy in several areas, including wireless recording and stimulation systems (particularly responsive neurostimulation [NeuroPace]), conformal electrodes for high-resolution electrocorticography, robot-assisted stereotactic surgery, optogenetics and optical imaging methods, novel positron emission tomography ligands, and new applications of focused ultrasonography. Investigation into genetic causes of and susceptibilities to epilepsy has introduced a new era of precision medicine, enabling the understanding of cell signaling mechanisms underlying epileptic activity as well as patient-specific molecularly targeted treatment options. We discuss the emerging path to individualized treatment plans, predicted outcomes, and improved selection of effective interventions, on the basis of these developments.

Cavernous malformation hemorrhage due to trans-mural pressure alterations after cerebrospinal fluid diversion: a case report.

BACKGROUND: Cavernous malformations are rare cerebral pseudo-vascular lesions with annualized bleeding rates of 0.5-3% in most studies. Of the various explored risk factors for bleeding to date, only prior hemorrhage has shown significant correlation. CASE PRESENTATION: In this case, we describe a 65-year old man with a peri-ventricular atrial cavernous malformation that hemorrhaged after CSF diversion via ventriculoperitoneal shunting. Serial imaging showed that bleeding continued until the shunt was revised with a programmable valve set at maximum resistance with the addition of a gravitational unit, thereby lowering the trans-mural pressure differential across the cavernous malformation. CONCLUSIONS: Given that other vascular lesions are subject to hemorrhage from alterations in trans-mural pressure dynamics, we hypothesize that cavernous malformations are similarly affected by trans-mural pressure gradients as they are composed of primitive vascular elements. This hypothesis is corroborated by the temporal correlation of interventions, imaging, and exam findings in the present case, and suggests a potentially important risk factor for hemorrhage in CM patients that affects prognostication and management.

Magnetic resonance-guided focused ultrasound for ablation of mesial temporal epilepsy circuits: modeling and theoretical feasibility of a novel noninvasive approach.

OBJECTIVE: The authors tested the feasibility of magnetic resonance-guided focused ultrasound (MRgFUS) ablation of mesial temporal lobe epilepsy (MTLE) seizure circuits. Up to one-third of patients with mesial temporal sclerosis (MTS) suffer from medically refractory epilepsy requiring surgery. Because current options such as open resection, laser ablation, and Gamma Knife radiosurgery pose potential risks, such as infection, hemorrhage, and ionizing radiation, and because they often produce visual or neuropsychological deficits, the authors developed a noninvasive MRgFUS ablation strategy for mesial temporal disconnection to mitigate these risks. METHODS: The authors retrospectively reviewed 3-T MRI scans obtained with diffusion tensor imaging (DTI). The study group included 10 patients with essential tremor (ET) who underwent pretreatment CT and MRI prior to MRgFUS, and 2 patients with MTS who underwent MRI. Fiber tracking of the fornix-fimbria pathway and inferior optic radiations was performed, ablation sites mimicking targets of open posterior hippocampal disconnection were modeled, and theoretical MRgFUS surgical plans were devised. Distances between the targets and optic radiations were measured, helmet angulations were prescribed, and the numbers of available MRgFUS array elements were calculated. RESULTS: Tractograms of fornix-fimbria and optic radiations were generated in all ET and MTS patients successfully. Of the 10 patients with both the CT and MRI data necessary for the analysis, 8 patients had adequate elements available to target the ablation site. A margin (mean 8.5 mm, range 6.5-9.8 mm) of separation was maintained between the target lesion and optic radiations. CONCLUSIONS: MRgFUS offers a noninvasive option for seizure tract disruption. DTI identifies fornix-fimbria and optic radiations to localize optimal ablation targets and critical surrounding structures, minimizing risk of postoperative visual field deficits. This theoretical modeling study provides the necessary groundwork for future clinical trials to apply this novel neurosurgical technique to patients with refractory MTLE and surgical contraindications, multiple prior surgeries, or other factors favoring noninvasive treatment.

Neurosurgery Training Camp for Sub-Internship Preparation: Lessons From the Inaugural Course.

OBJECTIVE: Historically, medical student education in neurological surgery has generally limited student involvement to assisting in research projects with minimal formal clinical exposure before starting sub-internships and application for the neurosurgery match. Consequently, students have generally had little opportunity to acquire exposure to clinical neurosurgery and attain minimal proficiency. A medical student training camp was created to improve the preparation of medical students for the involvement in neurological surgery activities and sub-internships. METHODS: A 1-day course was held at Weill Cornell Medicine, which consisted of a series of morning lectures, an interactive resident lunch panel, and afternoon hands-on laboratory sessions. Students completed self-assessment questionnaires regarding their confidence in several areas of clinical neurosurgery before the start of the course and again at its end. RESULTS: A significant increase in self-assessed confidence was observed in all skill areas surveyed. Overall, rising fourth year students who were starting sub-internships in the subsequent weeks reported a substantial increase in their preparedness for the elective rotations in neurosurgery. CONCLUSIONS: The preparation of medical students for clinical neurosurgery can be improved. Single-day courses such as the described training camp are an effective method for improving knowledge and skill gaps in medical students entering neurosurgical careers. Initiatives should be developed, in addition to this annual program, to increase the clinical and research skills throughout medical student education.

Clinical Significance of Venous Anomalies in Syndromic Craniosynostosis.

BACKGROUND: The pattern of cranial venous drainage in syndromic craniosynostosis is unpredictable and not adequately understood. Collateral channels substitute for stenotic venous sinuses and pose potential risk for surgical intervention. The purpose of this study was to analyze the patterns of venous drainage in patients with syndromic craniosynostosis and their influence on operative planning and morbidity. METHODS: A retrospective study of patients with syndromic craniosynostosis from 2000 to 2013 was performed. Demographic data were collected including phenotype and associated pathologies. Pre- and/or postoperative venous imaging was reviewed for venous sinus stenosis, collateral emissaries, and persistent fetal sinuses. Categorization of anomalous venous drainage was performed, and the relationship with surgical morbidity was assessed. RESULTS: Forty-one patients were identified. Anomalies were present in 31 patients (76%) consisting of dural sinus stenosis in 28 (68%), dilated emissaries in 26 (63%), and fetal sinuses in 7 (17%). Pfeiffer syndrome was most commonly associated with anomalous drainage (100%). Venous anomalies were associated with elevated intracranial pressure (ICP), shunted hydrocephalus, Chiari malformations, and sleep apnea. In 5 cases, the surgical plan was adjusted based on anomalous anatomy. No mortalities occurred. Intraoperative complication rate was 7.3%, all with anomalous drainage. Median estimated blood loss was 1,100 cc for patients with anomalies versus 400 cc without anomalies (P = 0.181). CONCLUSION: Cranial venous anomalies are commonly detected in patients with syndromic craniosynostosis and may affect surgical morbidity and outcome with a higher estimated blood loss, alteration of procedure, and postoperative morbidity. Detailed preoperative imaging of the venous drainage is therefore recommended in cases of syndromic synostosis.

Contemporary management and surveillance strategy after shunt or endoscopic third ventriculostomy procedures for hydrocephalus.

The management of hydrocephalus can be challenging even in expert hands. Due to acute presentation, recurrence, accompanying complications, the need for urgent diagnosis; a robust management plan is an absolute necessity. We devised a novel time efficient surveillance strategy during emergency, and clinic follow up settings which has never been described in the literature. We searched all articles embracing management/surveillance protocol on pediatric hydrocephalus utilizing the terms "hydrocephalus follow up" or "surveillance protocol after hydrocephalus treatment". The authors present their own strategy based on vast experience in the hydrocephalus management at a single institution. The need for the diagnostic laboratory testing, age and presentation based radiological imaging, significance of neuro-opthalmological exam, and when to consider the emergent exploration have been discussed in detail. Moreover, a definitive triaging strategy has been described with the help of flow chart diagrams for clinicians, and the neurosurgeons in practice. The triage starts from detail history, physical exam, necessary labs, radiological imaging depending on the presentation, and the age of the child. A quick head CT scan helps after shunt surgery while, a FAST sequence MRI scan (fsMRI) is important in post ETV patients. The need for neuro-opthalmological exam, and the shunt series stays vital in asymptomatic patients during regular follow up.

Rasmussen's encephalitis: advances in management and patient outcomes.

PURPOSE: Rasmussen's encephalitis (RE) is a hemispheric inflammatory disorder resulting in progressive epilepsy, hemiparesis, and cognitive decline. Controversy surrounds the most effective timing of surgery with respect to language dominance, functional status, and seizure outcome. We describe our experience with RE to inform treatment decisions. METHODS: A retrospective chart review was performed in children diagnosed with RE from 1983 to 2012. RESULTS: Thirteen consecutive cases were identified: six males and seven females with a mean age of 10.6 years (range 5-18). Nine patients received immunotherapy, with transient benefit in three, treatment-associated complications in two, and no difference in their mean time to treatment (5.38 vs 6.37 years p = 0.74) or long-term outcome. Mean follow-up was 5.6 years (range 0.58-12.25). There was no difference in outcome based on pre-operative duration of seizures. At last follow-up, 63 % of surgically treated patients achieved seizure freedom, 100 % had improved seizure control, 90 % had improved cognitive function, 36 % stopped medication, and 63 % tapered medication. Language improved in 83 % of patients with dominant disease. These findings were not associated with age at treatment. All surgical patients were ambulatory at last follow-up. CONCLUSIONS: Hemispherotomy achieves good seizure control with cognitive improvement and ambulatory status post-operatively. Time to surgery and dominant disease were not associated with outcome, suggesting that hemispherotomy can be offered early or late, with expectations of good seizure control and functional outcome, even with dominant disease.