Search

Background Surgical resection has been shown to prolong survival in patients with glioblastoma multiforme (GBM), although this benefit has not been demonstrated for reoperation following tumor recurrence. Laser interstitial thermal therapy (LITT) is a minimally invasive ablation technique that has been shown to effectively reduce tumor burden in some patients with intracranial malignancy. The aim of this study was to describe the safety and efficacy of LITT for recurrent and newly diagnosed GBM at a large tertiary referral center. Methods Patients with GBM receiving LITT were retrospectively analyzed. Overall survival from the time of LITT was the primary end point measured. Results There were 69 patients identified for inclusion in this study. The median age of the cohort was 56 years (range, 15–77 years). Median tumor volume was 10.4 cm3 (range, 1.0–64.0 cm3). A Kaplan-Meier estimate of median overall survival for the series from the time of LITT was 12 months (95% confidence interval 8–16 months). Median progression-free survival for the cohort from LITT was 4 months (95% confidence interval 3–7 months). Adjuvant chemotherapy significantly prolonged progression-free survival and overall survival (P Conclusions LITT can safely reduce intracranial tumor burden in patients with GBM who have exhausted other adjuvant therapies or are poor candidates for conventional resection techniques.

In this review, we examine the postulated mechanisms of therapeutic effect of ketogenic diets in the treatment of gliomas, review the completed clinical trials, and discuss further directions in this field. Cancers including gliomas are characterized by derangements in cellular metabolism. In vitro and animal studies have revealed that dietary interventions to reduce glucose and glycolytic pathways in gliomas may have a therapeutic effect. Early trials in patients with malignant gliomas have shown feasibility, but are not robust enough yet to demonstrate clinical applicability. Therapies for malignant gliomas of the brain are increasingly using a multi-targeted approach. The use of ketogenic diets and its variants may offer a unique and promising anti-glioma treatment by exploiting metabolic alterations seen in cancers including gliomas seen at the cellular level, which may work in concert with other therapies.

OBJECTIVEMesenchymal stem cells (MSCs) have been shown to localize to gliomas after intravascular delivery. Because these cells home to areas of tissue injury, the authors hypothesized that the administration of ionizing radiation (IR) to tumor would enhance the tropism of MSCs to gliomas. Additionally, they sought to identify which radiation-induced factors might attract MSCs.METHODSTo assess the effect of IR on MSC migration in vitro, transwell assays using conditioned medium (CM) from an irradiated commercially available glioma cell line (U87) and from irradiated patient-derived glioma stem-like cells (GSCs; GSC7-2 and GSC11) were employed. For in vivo testing, green fluorescent protein (GFP)-labeled MSCs were injected into the carotid artery of nude mice harboring orthotopic U87, GSC7-2, or GSC17 xenografts that were treated with either 0 or 10 Gy of IR, and brain sections were quantitatively analyzed by immunofluorescence for GFP-positive cells. These GSCs were used because GSC7-2 is a weak attractor of MSCs at baseline, whereas GSC17 is a strong attractor. To determine the factors implicated in IR-induced tropism, CM from irradiated GSC7-2 and from GSC11 was assayed with a cytokine array and quantitative ELISA.RESULTSTranswell migration assays revealed statistically significant enhanced MSC migration to CM from irradiated U87, GSC7-2, and GSC11 compared with nonirradiated controls and in a dose-dependent manner. After their intravascular delivery into nude mice harboring orthotopic gliomas, MSCs engrafted more successfully in irradiated U87 (p = 0.036), compared with nonirradiated controls. IR also significantly increased the tropism of MSCs to GSC7-2 xenografts (p = 0.043), which are known to attract MSCs only poorly at baseline (weak-attractor GSCs). Ionizing radiation also increased the engraftment of MSCs in strong-attractor GSC17 xenografts, but these increases did not reach statistical significance. The chemokine CCL2 was released by GSC7-2 and GSC11 after irradiation in a dose-dependent manner and mediated in vitro transwell migration of MSCs. Immunohistochemistry revealed increased CCL2 in irradiated GSC7-2 gliomas near the site of MSC engraftment.CONCLUSIONSAdministering IR to gliomas enhances MSC localization, particularly in GSCs that attract MSCs poorly at baseline. The chemokine CCL2 appears to play a crucial role in the IR-induced tropism of MSCs to gliomas.

Introduction Intradural spinal arachnoid cysts (SACs) are among many etiologies for syringomyelia. Consequentially, neurologic symptoms arise such as pain, gait disturbance, and bladder dysfunction. Identification of SAC on magnetic resonance imaging (MRI) can be challenging, as SACs can be fenestrated or in the form of fine webs. Methods Imaging and clinical data for 7 patients who underwent surgical treatment for SAC associated with syringomyelia were reviewed. All previous publications of this pathology were reviewed via MEDLINE search. Results Seven patients with a mean age 59 years were found to have a SAC causing syringomyelia. Intraoperative exploration confirmed SAC appearances of fine webs or a fluid-filled loculation impinging on the spinal cord. Common presentations were back pain, gait disturbance, and bladder incontinence. Diagnosis was made by MRI, although in 3 cases, the SAC was not identified on the initial review. Computed tomography myelogram was performed in one case due to the enlarged syringomyelia and lack of obvious spinal cord compression. Thoracic laminectomy/laminoplasty was performed for all patients, centered at the level of a subtle indentation of the cord; the syringomyelia proper was not directly addressed. Postoperatively, all patients had complete resolution of their symptoms with MRI demonstrating resolution of the syringomyelia. Conclusions Careful evaluation of the MRI can demonstrate subtle indentation of the cord at the caudal or cephalad end of the syringomyelia and may obviate the need for additional imaging. Meticulous arachnoid dissection and establishment of good CSF flow is sufficient for resolution of the syringomyelia, averting the need for more aggressive procedures.

Chordomas and chondrosarcomas of the spine share several key features that make them distinct from other malignant primary bone tumors. They manifest more indolent behavior characterized by slow but persistent local progression. They are best addressed with aggressive surgical resection, ideally with margins, as radiation and chemotherapy display little efficacy in their treatment. Chordomas can occur anywhere along the axial skeleton and skull base, with relatively equal predilection for skull base, mobile spine, or sacrum. Spinal chondrosarcomas constitute 10% of all chondrosarcomas, with most occurring in the thoracic spine. For both chordoma and chondrosarcoma, en bloc resection should be pursued if technically feasible as it reduces the risk of local recurrence compared to intralesional resection. In cases of suspected spinal chordoma or chondrosarcoma, a computed tomographic–guided core biopsy is recommended to establish the diagnosis, which will allow for subsequent careful interdisciplinary surgical planning. The Enneking and the Weinstein/Boriani/Biagini surgical staging systems can help determine if a patient is a candidate for a wide en bloc resection.

Intradural spinal malignant peripheral nerve sheath tumors (MPNST) are extremely rare, with only 20 adult patients reported to our knowledge, and only four primary tumors arising from the cauda equina. A 49-year-old man presented with back pain, constipation, and lower extremity weakness and was found to have a large intradural lesion involving the cauda equina. Imaging of the rest of his neuraxis revealed additional small left temporal lobe, cervical, and thoracic lesions. The patient underwent laminectomy for tumor debulking and biopsy, as gross total resection was not possible due to envelopment of the cauda equina. Histopathology revealed a MPNST with high cellularity, elevated proliferative indices, and nerve fascicle invasion. After the debulking, the patient reported improvement in his symptoms. However, 6 weeks later, the patient began having severe headaches, and his left temporal lobe lesion was found to have increased significantly in size, requiring craniotomy for palliative resection. The authors report the first adult patient with sporadic spinal MPNST with craniospinal metastasis to our knowledge. Imaging of the entire neuraxis is recommended for initial workup of these lesions, which are capable of intradural spread. The best treatment approach is unclear, but total surgical resection should be attempted, barring infiltration and engulfment of the nerve roots or widespread dissemination.

Objective To evaluate the results of endoscopic transnasal resection of tuberculum sellae meningiomas (TSMs) as compared with transcranial approaches. Design We retrospectively analyzed five patients who underwent endoscopic endonasal resection of TSM and performed a comprehensive review of articles published between 2000 and 2012 describing the operative treatment of TSMs. Results Gross total resection (GTR) was achieved in four patients (80%). Transient diabetes insipidus occurred in three patients (60%). Preoperative visual field deficit resolved in all patients. Cerebrospinal fluid (CSF) leak occurred in one patient. Analysis of published studies included 1,026 transcranial and 144 transnasal cases. GTR was achieved in 85% of transcranial and 72% of transnasal cases. Visual field deficit improved in 65% of transcranial and 82% of transnasal cases. Rate of diabetes insipidus and CSF leak was higher in the transnasal series. Rate of GTR and visual improvement was higher in endoscopic endonasal as compared with microsurgical transnasal series. Conclusion The literature supports transsphenoidal surgery for the resection of TSMs with significant optic nerve compromise and limited lateral extension. This approach may have an equivalent if not superior outcome over transcranial surgery in visual outcome. CSF leaks are still a challenge but may improve with the use of vascularized nasoseptal flaps.

Radiation therapy continues to play an extremely valuable role in the treatment of malignancy. The effects of radiation therapy on normal tissue can present in a delayed fashion, resulting in localized damage with pseudomalignant transformation, producing a compressive effect on the spinal cord or exiting nerve roots. Infiltration of inflammatory cells and the subsequent fibrotic response can result in the development of an inflammatory pseudotumor (benign tumor-like lesion) with subsequent mass effect. Herein, the authors present a rare case of inflammatory pseudotumor with fulminant cervicothoracic cord compression, developing 7 years after radiation therapy for breast cancer. The lesion recurred following resection but subsequently displayed complete and rapid resolution following steroid therapy. To the best of the authors' knowledge, no previous studies have reported such an incident.

Object Over 85% of patients with myelomeningoceles require placement of a ventriculoperitoneal shunt for hydrocephalus, and between 25% and 85% of these patients develop scoliosis. Although most patients undergo repeated shunt series radiography to evaluate for device malfunction, scoliosis radiographs are less consistently obtained. The authors sought to determine if a correlation exists between these 2 radiographic techniques for a given patient, as shunt series are obtained with the patient supine, whereas scoliosis radiographs are acquired with the patient standing upright. The authors also endeavored to study if shunt series radiographs can reliably detect significant scoliosis. Methods The authors retrospectively reviewed a single institution's series of 593 patients with myelomeningoceles and identified all patients in whom a shunt series and scoliosis radiographs were obtained within a 6-month period. They reviewed the medical records and radiographs of these patients for demographic and radiographic parameters. They then applied a linear regression model and determined shunt series curve cutoffs to detect scoliotic curves greater than 20° and 50°. Results Of the 593 patients identified, 116 did not have radiographs available for interpretation. Of the remaining 477 patients, 201 had radiographic evidence of scoliosis (42%), and 66 had both a shunt series and a scoliosis radiographs acquired within a 6-month interval. In 4 patients, both end vertebrae of the scoliotic curve could not be visualized on a single radiograph. The mean age of the remaining cohort was 10.6 ± 5.2 years and the mean curve magnitude was 58° ± 37°. Using identical end vertebrae, the mean shunt series curve magnitude was 49° ± 35°. The mean interval between both radiographs was 2.3 ± 3.3 months. The regression model showed a strong linear association between shunt series and scoliosis series curves. A curve greater than 19° on shunt series radiographs would detect significant curves of greater than 20° on scoliosis series with 91% sensitivity and 78% specificity. A shunt series curve greater than 37° had 100% sensitivity and 93% specificity in identifying significant scoliotic curves greater than 50°. Conclusions Although shunt series radiographs may not precisely depict scoliotic curve magnitude because the impact of gravity is negated, they may be useful in helping to confirm clinical suspicion of scoliosis. The authors' results suggest a strong correlation between both types of radiographs.

Object Outside of the patient population with achondroplasia, neurogenic claudication is rare in the pediatric age group. Neurogenic claudication associated with posterior vertebral rim fracture is even more uncommon but nonetheless causes pain and disability in affected children and adolescents. The purpose of this study was to describe the surgical results of 3 adolescents presenting with neurogenic claudication and posterior vertebral rim fracture when treated with laminectomy alone. Methods The medical and operative records of the 3 pediatric patients were retrospectively reviewed. Presenting signs and symptoms and CT findings, such as the interpedicular distances between T-12 and L-5, were obtained. Perioperative results were assessed, including operative time, blood loss, length of hospital stay, and complications. Findings at latest follow-up were also recorded, including a patient satisfaction survey. Results The 3 patients (1 girl and 2 boys) had a mean age of 14.7 years (range 14–15 years) and underwent follow-up for a mean of 11.3 months (range 5–18 months). Notable preoperative signs and symptoms included back pain (all patients), leg pain (all patients), leg numbness (1 patient), and leg weakness (1 patient). No patient presented with bowel and/or bladder dysfunction. The mean blood loss during laminectomy was 123 ml (range 20–300 ml), and the mean length of hospital stay was 4.3 days (range 3–6 days). On average, decompression was performed at 2.2 levels (range 2–2.5 levels). All 3 patients reported at most recent follow-up that they were “satisfied” with the surgery. There was 1 complication of instability from an iatrogenic pars fracture, which required reoperation and posterior instrumented fusion. Conclusions To the best of the authors' knowledge, this report represents the first surgical series of pediatric neurogenic claudication associated with posterior vertebral rim fractures. Pediatric neurosurgeons may infrequently encounter neurogenic claudication associated with a posterior vertebral rim fracture in children. To treat children with neurogenic claudication associated with posterior vertebral rim fractures, a simple laminectomy may be a safe and efficacious alternative to discectomy and removal of fracture fragments.

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS. The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up. The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

Object Significant lumbar kyphosis is frequently observed in patients with myelomeningocele and has been associated with increasing functional impairment, decreased abdominal volume, respiratory impairment, discomfort, and skin ulcerations overlying the prominent gibbus. Treatment of severe kyphotic deformities can include kyphectomy, with or without ligation of the thecal sac, with posterior spinal fixation. However, most series have reported a high rate of morbidity and complications associated with surgical intervention for correction of kyphosis in patients with myelomeningocele. The authors describe a technique in which pedicle screw (PS)–only constructs are used without transection of the thecal sac to treat severe kyphosis successfully, with minimal morbidity. Methods The authors retrospectively reviewed medical records and radiographic images in 2 patients with myelomeningoceles in whom kyphectomies had been performed at the authors' institution between January 2007 and July 2010. They also reviewed the existing literature for case reports or published series of patients with myelomeningocele treated with kyphectomies, to evaluate the outcomes. Results Both patients were male and had thoracic-level myelomeningoceles that had been repaired at birth, with associated paraplegia. Neither patient had any significant scoliotic deformity associated with the kyphosis, and both had fixation from T-9 to the ilium, which was performed using PS constructs, along with L1–2 kyphectomies. The patient in Case 1 was 20 years old and was treated for progressive kyphosis and an ulcerated nonhealing wound over the gibbus. The patient in Case 2 was 10 years old and was treated for progressive pain and functional impairment. The 2 patients had a mean correction of 63%, with a mean correction of kyphotic deformity from 136° to 51°. Neither patient developed any complication in the short term postoperatively, whereas published series have reported high complication rates, including wound infection, poor wound healing, CSF leakage, pseudarthrosis, and shunt malfunction. Conclusions Severe kyphotic deformities in patients with myelomeningocele can be safely treated using PS-only constructs without ligation of the thecal sac. Further evaluation with a larger sample and longer follow-up are needed to detect any associated complications, such as proximal junctional kyphosis. Further evaluation may also validate whether PS-only constructs permit successful outcomes with a shorter construct and fewer instrumented levels.

Object Lumbar disc herniation is rare in the pediatric age group, but may still cause a significant amount of pain and disability. Whereas minimally invasive surgery (MIS) for lumbar disc herniation is routinely performed in adults, it has not yet been described in the pediatric population. The purpose of this study was to describe the surgical results of pediatric MIS-treated lumbar disc disease. Methods The authors retrospectively reviewed a series of 6 consecutive cases of lumbar microdiscectomy performed using MIS techniques between April 2008 and July 2010. Presenting symptoms, physical examination findings, and preoperative MR imaging results were obtained from medical records. Perioperative results, including blood loss, length of hospital stay, and complications were assessed. Findings at latest follow-up evaluation were also recorded. Results This report represents the first surgical series regarding pediatric lumbar microdiscectomies performed using MIS. The mean patient age was 16 years (range 14–17 years); there were 4 girls and 2 boys. Preoperative signs and symptoms of radiculopathic pain were notable in 100% of patients, and myotomal weakness was noted in 33% of patients. The first line of treatment in all patients was a period of conservative management lasting an average of 11.5 months (range 6–12 months). The mean intraoperative blood loss was 10.8 ml, and the mean postoperative length of stay was 1.3 days. There were no complications in this small series. Conclusions The treatment of pediatric lumbar disc herniation by using MIS techniques can be safe and efficacious. However, further study with a larger number of patients and longer follow-up is needed to compare outcomes between MIS and open microdiscectomies.

Dural arteriovenous fistulas (DAVF) of the cavernous sinus most commonly present with ocular symptoms and can be observed or treated with endovascular approaches, surgery, or radiosurgery. Combined surgical-endovascular approaches have been used for fistulas that are not amenable to standard endovascular approaches. A 40-year-old man presented with ocular symptoms from a cavernous sinus DAVF. Multiple previous transarterial and transvenous embolization attempts had failed. The patient underwent craniotomy for surgical exposure and cannulation of an arterialized sylvian vein. Subsequently he underwent coiling and onyx embolization of the DAVF. The intervention resulted in effective obliteration of the fistula. If a cavernous sinus DAVF is refractory to treatment, surgical exposure and cannulation of a cortical draining vein can facilitate transvenous endovascular treatments.

Objective Patients typically remain hospitalized for several days after transsphenoidal surgery for pituitary adenoma resection for reasons including pain control, serial neurological assessments, surveillance for cerebrospinal fluid leak, and management of endocrine issues. We sought to determine whether an evidence-based perioperative care protocol combined with an endoscopic approach could lead to routine and safe discharge on postoperative day 1. Methods Our multidisciplinary pituitary group prospectively implemented a perioperative care protocol that emphasizes patient education, early mobilization, and scheduled inpatient and outpatient endocrine assessments on 50 consecutive patients who underwent surgical resection of a pituitary adenoma (82% macroadenomas, 2.1 ± 0.8 cm, maximum 4.5 cm, 18% microadenomas). Endoscopic endonasal surgery characterized by aggressive tumor resection and avoidance of nasal packing and lumbar drains was used in all cases. Lengths of stay, readmissions, and postoperative outcomes were analyzed. Results Using the short-stay protocol, 92% (46 of 50) of patients were successfully discharged on postoperative day 1. The average length of stay for all patients was 1.16 ± 0.55 days (range 1 to 4). Postoperative diabetes insipidus occurred in 16% of patients (8 of 50), was effectively managed on an outpatient basis, and did not delay discharge. Readmission was required in 2 patients, in both cases for delayed presentation of a cerebrospinal fluid leak. Conclusions A short-stay protocol allows for an overnight hospital stay for patients after pituitary surgery, with a low rate of complications or readmission. This study offers evidence-based guidelines that may be used to avoid complications and facilitate early discharge after transsphenoidal surgery.

OBJECTIVE Glioblastoma (GBM) is the most common and deadly malignant primary brain tumor. Better surgical therapies are needed for newly diagnosed GBMs that are difficult to resect and for GBMs that recur despite standard therapies. The authors reviewed their institutional experience of using laser interstitial thermal therapy (LITT) for the treatment of newly diagnosed or recurrent GBMs. METHODS This study reports on the pre-LITT characteristics and post-LITT outcomes of 8 patients with newly diagnosed GBMs and 13 patients with recurrent GBM who underwent LITT. RESULTS Compared with the group with recurrent GBMs, the patients with newly diagnosed GBMs who underwent LITT tended to be older (60.8 vs 48.9 years), harbored larger tumors (22.4 vs 14.6 cm3), and a greater proportion had IDH wild-type GBMs. In the newly diagnosed GBM group, the median progression-free survival and the median survival after the procedure were 2 months and 8 months, respectively, and no patient demonstrated radiographic shrinkage of the tumor on follow-up imaging. In the 13 patients with recurrent GBM, 5 demonstrated a response to LITT, with radiographic shrinkage of the tumor following ablation. The median progression-free survival was 5 months, and the median survival was greater than 7 months. CONCLUSIONS In carefully selected patients with recurrent GBM, LITT may be an effective alternative to surgery as a salvage treatment. Its role in the treatment of newly diagnosed unresectable GBMs is not established yet and requires further study.

The treatment of patients with refractory epilepsy has always been challenging. Despite the availability of multiple antiepileptic medications and surgical procedures with which to resect seizure foci, there is a subset of epilepsy patients for whom little can be done. Currently available treatment options for these unfortunate patients include vagus nerve stimulation, the ketogenic diet, and electric stimulation, both direct and indirect, of brain nuclei thought to be involved in epileptogenesis. Studies of electrical stimulation of the brain in epilepsy treatment date back to the early 20th century, beginning with research on cerebellar stimulation. The number of potential targets has increased over the years to include the hippocampus, subthalamic nucleus, caudate nucleus, centromedian nucleus, and anterior nucleus of the thalamus (ANT). Recently the results of a large randomized controlled trial, the electrical Stimulation of the Anterior Nucleus of Thalamus for Epilepsy (SANTE) trial, were published, demonstrating a significant reduction in mean seizure frequency with ANT stimulation. Soon after, in 2011, the results of a second randomized, controlled trial—the NeuroPace RNS trial—were published. The RNS trial examined closed-loop, responsive cortical stimulation of seizure foci in patients with refractory partial epilepsy, again finding significant reduction in seizure frequency. In the present review, the authors examine the modern history of electrical stimulation of the brain for the treatment of epilepsy and discuss the results of 2 important, recently published trials, the SANTE and RNS trials.

Contralateral hematoma formation following acute subdural hematoma (ASDH) evacuation is a well-described complication. The most common type of contralateral hematoma is an epidural hematoma. Rarely, ASDH develops on the contralateral side. We report an elderly woman who presented with a post-traumatic ASDH and underwent ipsilateral hematoma evacuation by craniotomy and subsequently developed a contralateral ASDH. Because of the potential consequences of a delayed ASDH, there should be a low threshold for early post-operative imaging following ASDH evacuation, especially in elderly patients and those with additional associated intracranial injuries.