Association of Thromboelastography with Progression of Hemorrhagic Injury in Children with Traumatic Brain Injury.

INTRODUCTION: Progression of hemorrhagic injury (PHI) in children with traumatic brain injury portends poor outcomes. The association between thromboelastography (TEG), functional coagulation assays, and PHI is not well characterized in children. METHODS: This was a retrospective cohort study of children presenting with PHI at a pediatric level I academic trauma center from 2015 to 2020. Inclusion criteria were as follows: age less than 18 years, intracranial hemorrhage on admission head computed tomography scan, and admission rapid TEG assay and conventional coagulation tests. PHI was defined by the following radiographic criteria: any expansion of or new intracranial hemorrhage on subsequent head computed tomography scan. Rapid TEG values included Activated Clotting Time (ACT), alpha angle, maximum amplitude, and lysis at 30 min. Wilcoxon rank-sum test was used to assess baseline differences between groups with PHI and without PHI, including laboratory assays. Univariate analysis was performed to examine the association between variables of interest and PHI. Patients were dichotomized on the basis of this cut point to generate a "low ACT" group and a "high ACT" group. These variables were included in a multivariable logistic regression model to determine independent association with traumatic brain injury progression. RESULTS: In total, 219 patients met criteria for analysis. In this cohort, the median (interquartile range [IQR]) age = 6 (2-12) years, median (IQR) Injury Severity Score = 21 (11-27), 68% were boys, and 69% sustained blunt injury. The rate of PHI was 25% (54). Median (IQR) time to PHI was 1 (0-4) days. Children with PHI had a higher Injury Severity Score (p < 0.001), lower Glasgow Coma Scale (p < 0.001), greater incidence of shock (p = 0.04), and lower admission hemoglobin (p = 0.02) compared with those without PHI. Children with PHI had a higher International Normalized Ratio (INR) and longer TEG-ACT; other TEG values (alpha angle, maximum amplitude, and lysis at 30 min) were not associated with PHI. In the logistic regression model accounting for other covariates associated with PHI, elevated ACT remained an independent predictor of progression (odds ratio = 2.25, 95% confidence interval 1.09-4.66; p = 0.03; area under the receiver operating characteristic curve = 0.76). After adjusting for confounders, INR fell out of the model and was not an independent predictor of progression (odds ratio = 1.32, 95% confidence interval 0.60-2.93; p = 0.49). CONCLUSIONS: Although INR was elevated in children with PHI and has been associated with poor clinical outcomes, only admission TEG-ACT was independently associated with PHI. Further study is warranted to determine whether TEG-ACT reflects an actionable therapeutic target.

Impact of therapeutic regimen and clinical presentation on overall survival in CNS lymphoma.

BACKGROUND: The authors present a retrospective analysis of 45 patients who underwent treatment of CNS lymphoma (both primary and secondary) at a single institution between 2005 and 2012. METHODS: This study involves 21 female and 24 male patients with a mean age of 59.2 years. All medical records and pathology reports were reviewed for each patient. Univariate and multivariate analyses of overall survival were performed. RESULTS: Presentation with altered mental status was a significant risk factor for worse overall survival. An HIV infection, deep lesion location, and age over 60 did not impact survival. A survival benefit was demonstrated with the use of systemic therapy, specifically rituximab, and radiation. The CNS Lymphoma Score was derived from this cohort, which proved a powerful predictive tool for overall survival. The surgical complication rate in this series was 17.8 %. CONCLUSIONS: This study highlights the prognostic importance of presenting mental status on outcomes in CNS lymphoma and demonstrates a summative benefit of rituximab and whole brain radiation therapy. Considering these factors together provides an easily applicable and meaningful stratification for this patient population. The surgical complication rate in this patient population is not negligible. The high percentage of wound-related surgical complications suggests the need for a waiting period between surgery and initiation of chemotherapy to allow for wound healing.

Infant Rudimentary Meningocele with Tethering of the Cervical Cord: A Case Report.

Rudimentary meningoceles of the spine with dural extension are very rare and warrant surgical excision to prevent infection and long-term neurological deficits in pediatric patients. We present the case of a 5-month-old infant with a tethered spinal cord secondary to a rudimentary meningocele. The patient presented shortly after birth with a midline cervical dimple that was evaluated for a suspected dermal sinus tract. Magnetic resonance imaging scan of the spine showed a sinus tract with intradural extension to C2-3 and external opening at the level of spinous process C5. En bloc surgical excision and spinal cord release were successfully performed. Histological analysis of the specimen confirmed the presence of two blunt sinus tracts and staining was consistent with a rudimentary meningocele. Intradural rudimentary meningoceles in infants can successfully be managed with surgical intervention. Surgery is indicated to prevent future motor complications from spinal cord tethering and neoplastic growth from the rudimentary meningocele.

Nasal Embryonal Rhabdomyosarcoma in the Pediatric Population: Literature Review and Report of Midline Presentation.

BACKGROUND: Congenital midline nasal masses are rare anomalies and are typically benign nasal dermoid sinus cysts (NDSCs). Rhabdomyosarcomas (RMSs) are even less common, and only a fraction affect sites like the external nose, nasal cavity, nasopharynx, and paranasal sinuses. We review the clinical presentation and treatment of nasal, nasopharyngeal, and paranasal RMSs and report the first documented midline presentation. METHODS: We queried PubMed for articles with titles containing the terms rhabdomyosarcoma or sarcoma botryoides and nose, nasal, paranasal, sinonasal, nasopharynx, or nasopharyngeal. We then searched the references of each included article using the same parameters and continued this process iteratively until no new articles were found. RESULTS: The paranasal sinuses were the most commonly affected site, followed by the nasopharynx, nasal cavity, and external nose. Two patients presented with involvement of the external nose, but each presented with involvement of the right ala rather than a midline mass. The rates of intracranial extension and/or skull base involvement were comparable to those of NDSCs. The alveolar subtype was most common, followed by the embryonal subtype. CONCLUSIONS: Most midline nasal masses are benign; however, we report the first documented presentation of an RMS as a midline nasal mass. Accordingly, RMS should be included in the differential diagnosis of midline nasal masses in the pediatric population. Surgery for midline nasal masses is sometimes delayed due to the risks of interfering with developing structures and early anesthesia. However, early surgical treatment should be considered given this new differential and its predilection for early metastasis.

Parasitic rachipagus conjoined twin: case report.

A parasitic twin represents a rare developmental anomaly in which an asymmetrical, nonviable conjoined twin is attached to the host body at the time of birth. Rachipagus is among the rarest of conjoined twin subtypes and typically features a parasitic twin mass attached at the spine. Herein, the authors review the literature and describe the case of a 9-month-old girl presenting with a rachipagus parasitic twin consisting of a fully developed set of lower extremities originating from the midline upper back. After a complete workup to delineate parasite and autosite anatomy, the parasitic twin mass was successfully excised by a multidisciplinary team of surgeons, and the resulting defect was closed in a single stage.

Pigmented ganglioglioma in a patient with chronic epilepsy and cortical dysplasia.

We report a rare case of a 22-year-old woman with biopsy-proven pigmented ganglioglioma. The patient initially underwent a right temporal lobectomy for intractable seizures at the age of 9 and remained seizure free for several years but subsequently developed complex partial seizures. Due to enhancement of a left mesial occipital lesion on preoperative MRI of the brain, the patient underwent a left subdural electrode placement and simultaneous biopsy of the left mesial occipital lesion. Biopsy results revealed a rare pigmented ganglioglioma, World Health Organization Grade I. The seizure focus was identified in the left mesial occipital lobe and the patient underwent tumor resection. An extensive literature search revealed that our patient is the fourth case of pigmented ganglioglioma described in the literature and was positive for BRAF V600E mutation by molecular studies.

On-demand pulsatile intracerebral delivery of carisbamate with closed-loop direct neurostimulation therapy in an electrically induced self-sustained focal-onset epilepsy rat model.

OBJECT: The authors evaluated the preclinical feasibility of acutely stabilizing an active bihemispheric limbic epileptic circuit using closed-loop direct neurostimulation therapy in tandem with "on-demand'" convection-enhanced intracerebral delivery of the antiepileptic drug (AED) carisbamate. A rat model of electrically induced self-sustained focal-onset epilepsy was employed. METHODS: A 16-contact depth-recording microelectrode was implanted bilaterally in the dentate gyrus (DG) of the hippocampus of Fischer 344 rats. The right microelectrode array included an integrated microcatheter for drug delivery at the distal tip. Bihemispheric spontaneous self-sustained limbic status epilepticus (SSLSE) was induced in freely moving rats using a 90-minute stimulation paradigm delivered to the right medial perforant white matter pathway. Immediately following SSLSE induction, closed-loop right PP stimulation therapy concurrent with on-demand nanoboluses of the AED [(14)C]-carisbamate (n = 4), or on-demand [(14)C]-carisbamate alone (n = 4), was introduced for a mean of 10 hours. In addition, 2 reference groups received either closed-loop stimulation therapy alone (n = 4) or stimulation therapy with saline vehicle only (n = 4). All animals were sacrificed after completing the specified therapy regimen. In situ [(14)C]-autoradiography was used to determine AED distribution. RESULTS: Closed-loop direct stimulation therapy delivered unilaterally in the right PP aborted ictal runs detected in either ipsi- or contralateral hippocampi. Freely moving rats receiving closed-loop direct stimulation therapy with ondemand intracerebral carisbamate delivery experienced a significant reduction in seizure frequency (p < 0.001) and minimized seizure frequency variability during the final 50% of the therapy/recording session compared with closed-loop stimulation therapy alone. CONCLUSIONS: Unilateral closed-loop direct stimulation therapy delivered to afferent hippocampal white matter pathways concurrent with on-demand ipsilateral intracerebral delivery of nano-bolused carisbamate can rapidly decrease the frequency of electrographic seizures in an active bihemispheric epileptic network. Additionally, direct pulsatile delivery of carisbamate can stabilize seizure frequency variability compared with direct stimulation therapy alone.

Hyperdense suprasellar mass: An unusual radiological presentation of intracranial dermoid cyst.

We report an exceedingly rare patient with a hyperdense suprasellar dermoid cyst and a pertinent review of the literature. Intracranial dermoid tumors are rare congenital lesions of the brain that account for less than 1% of all intracranial tumors. Even though they are rare, typical CT scan and MRI features and location allow radiological diagnosis in the majority of patients. Radiologically, dermoid cysts typically present as low density masses on CT scan and are generally hyperintense on T1-weighted MRI sequences with variable signal on T2-weighted sequences. The recognition of atypical features can avoid diagnostic pitfalls and is clinically relevant for overall surgical management.

Optimizations and Nuances in Neurosurgical Technique for the Minimization of Complications in Subdural Electrode Placement for Epilepsy Surgery.

BACKGROUND: Surgical intervention is an important therapeutic option for patients with intractable epilepsy and a well-characterized epileptogenic focus. Invasive monitoring with subdural electrodes is an effective technique for localizing epileptogenic foci. Previous studies reported varying complication rates, and these may deter more widespread adoption. We present potentially valuable technical nuances that may be associated with low complication rates. We assess the potential contribution of specific aspects of surgical technique to the reduction of complication rates. METHODS: We retrospectively reviewed patients from the Rush University Surgical Epilepsy database who underwent craniotomies for invasive electroencephalography monitoring for medically intractable epilepsy using our technique. We analyzed and compared complication rates and techniques with those reported elsewhere. RESULTS: The sample group comprised 127 consecutive patients who underwent electrode implantation. The average monitoring period was 6 days. There were 5 complications (3.9%), including 1 subdural hematoma requiring surgery (0.8%), 1 infection (0.8%), 2 pulmonary emboli (1.6%), and 1 deep vein thrombosis (0.8%). There were no symptomatic cerebrospinal fluid leaks or permanent neurologic complications. These results compare favorably with published results. Analysis and comparison of our technique anecdotally suggest the importance of use of a subgaleal drain throughout the monitoring period, postoperative antibiotic coverage for 1 week, meticulous hemostasis, and secure suturing of the electrodes to the dura mater to minimize trauma to superficial vessels as potential contributors to improved complication rates. CONCLUSIONS: A very low incidence of major morbidity can be achieved in invasive electroencephalography monitoring with this protocol.

Selective excision of cerebral metastases from the precentral gyrus.

BACKGROUND: The surgical management of cerebral metastases to the eloquent cortex is a controversial topic. Precentral gyrus lesions are often treated with whole brain radiation therapy (WBRT) or stereotactic radiosurgery (SRS) because of the concern for causing new or worsened postoperative neurological deficits. However, there is evidence in the literature that radiation therapy carries significant risk of complication. We present a series of patients who were symptomatic from a precentral gyrus metastasis and underwent surgical excision. METHODS: During a 2-year period from 2010 to 2012, 17 consecutive patients harboring a cerebral metastasis within the precentral gyrus underwent microsurgical resection. All patients were discussed at a multi-disciplinary tumor board. The prerequisite for neurosurgical treatment was stable systemic disease and life expectancy greater than 6 months as determined by the patient's oncologist. Patients also were required to harbor a symptomatic lesion within the motor cortex, defined as the precentral gyrus. RESULTS: We present the 3-month neurological outcome for this group of patients. Surgery was uneventful and without any severe perioperative complications in all 17 patients. At 3 month follow up, symptoms had improved or been stabilized in 94.1% of patients and were worsened in 5.9%. CONCLUSION: Our results have shown that surgery for cerebral metastases in the precentral gyrus can be done safely and improve or stabilize the neurological function of most patients. Microsurgical resection of precentral gyrus metastases should be a treatment option for patients with single or multiple lesions who present a focal neurologic deficit. This can be performed safely and without intraoperative cortical mapping.

Massive cerebral involvement in fat embolism syndrome and intracranial pressure management.

Fat embolism syndrome (FES) is a common clinical entity that can occasionally have significant neurological sequelae. The authors report a case of cerebral fat embolism and FES that required surgical management of intracranial pressure (ICP). They also discuss the literature as well as the potential need for neurosurgical management of this disease entity in select patients. A 58-year-old woman presented with a seizure episode and altered mental status after suffering a right femur fracture. Head CT studies demonstrated hypointense areas consistent with fat globules at the gray-white matter junction predominantly in the right hemisphere. This CT finding is unique in the literature, as other reports have not included imaging performed early enough to capture this finding. Brain MR images obtained 3 days later revealed T2-hyperintense areas with restricted diffusion within the same hemisphere, along with midline shift and subfalcine herniation. These findings steered the patient to the operating room for decompressive hemicraniectomy. A review of the literature from 1980 to 2012 disclosed 54 cases in 38 reports concerning cerebral fat embolism and FES. Analysis of all the cases revealed that 98% of the patients presented with mental status changes, whereas only 22% had focal signs and/or seizures. A good outcome was seen in 57.6% of patients with coma and/or abnormal posturing on presentation and in 90.5% of patients presenting with mild mental status changes, focal deficits, or seizure. In the majority of cases ICP was managed conservatively with no surgical intervention. One case featured the use of an ICP monitor, while none featured the use of hemicraniectomy.

Stereotactic radiosurgery boost to the resection cavity for cerebral metastases: Report of overall survival, complications, and corticosteroid protocol.

BACKGROUND: This report focuses on the overall survival and complications associated with treatment of cerebral metastases with surgical resection followed by stereotactic radiosurgery (SRS). Management and complications of corticosteroid therapy are underreported in the literature but represent an important source of morbidity for patients. METHODS: Fifty-nine consecutive patients underwent surgical resection of a cerebral metastasis followed by SRS to the cavity. Patient charts were reviewed retrospectively to ascertain overall survival, local control, surgical complications, SRS complications, and corticosteroid complications. RESULTS: Our mean follow-up was 14.4 months (median 12.0 months, range 0.9-62.9 months). Median overall survival in this series was 15.25 months and local control was 98.3%. There was a statistically significant survival benefit conferred by Radiation Therapy Oncology Group recursive partitioning analysis Classes 1 and 2. The surgical complication rate was 6.8% while the SRS complication rate was 2.4%. Corticosteroid complications are reported and dependence at 1 month was 20.3%, at 3 months 6.8%, at 6 months 1.7%, and at 12 months no patients remained on corticosteroid therapy. CONCLUSIONS: Overall survival and local control with this treatment paradigm compare well to the other published literature. Complications associated with this patient population are low. A corticosteroid tapering protocol is proposed and demonstrated lower rates of steroid-related complications and dependence than previously reported.