Oberlin transfer and partial radial to axillary nerve neurotization to repair an explosive traumatic injury to the brachial plexus in a child: case report.

PURPOSE: Explosive injuries to the pediatric brachial plexus are exceedingly rare and as such are poorly characterized in the medical literature. METHODS: Herein, we describe an 8-year-old who was struck in the neck by a piece of shrapnel and suffered multiple vascular injuries in addition to a suspected avulsion of the cervical 5 and 6 ventral rami. The patient had a complete upper brachial plexus palsy and failed to demonstrate any clinical improvement at 6-months follow-up. He was taken to the operating from for a partial ulnar to musculocutaneous nerve neurotization as well as a partial radial to axillary nerve neurotization. RESULTS: The patient's motor exam improved from a Medical Research Council scale 1 to 4+ for biceps brachii and 0 to 4 deltoid function with greater than 90° of shoulder abduction. CONCLUSIONS: This outcome supports complex neurotization techniques as viable treatment options for persistent motor deficits following an upper brachial plexus injury in older, non-infant age, children.

Resection of an inferior pontine cavernous malformation using a far-lateral transcondylar approach.

BACKGROUND: When considering an approach to remove a symptomatic brainstem cavernous malformation, exposure and adequate visualization of the lesion with minimal morbidity should be the primary goals. METHOD: We describe the use of the far-lateral transcondylar technique to access a cavernous malformation in the lower anterior pons. RESULTS: This approach accesses the inferior belly of the pons in an inferior-to-superior direction, where the cavernous malformation approaches the surface between the root exit zones of the abducens (VI) cranial nerves. CONCLUSION: We recommend adding this technique to the repertoire of approaches used to resect these technically challenging lesions in selected cases.

Comparing Outcomes and Cost of 3 Surgical Treatments for Sagittal Synostosis: A Retrospective Study Including Procedure-Related Cost Analysis.

BACKGROUND: Neurosurgical techniques for repair of sagittal synostosis include total cranial vault (TCV) reconstruction, open sagittal strip (OSS) craniectomy, and endoscopic strip (ES) craniectomy. OBJECTIVE: To evaluate outcomes and cost associated with these 3 techniques. METHODS: Via retrospective chart review with waiver of informed consent, the last consecutive 100 patients with sagittal synostosis who underwent each of the 3 surgical correction techniques before June 30, 2013, were identified. Clinical, operative, and process of care variables and their associated specific charges were analyzed along with overall charge. RESULTS: The study included 300 total patients. ES patients had fewer transfusion requirements (13% vs 83%, P < .001) than TCV patients, fewer days in intensive care (0.3 vs 1.3, P < .001), and a shorter overall hospital stay (1.8 vs 4.2 d, P < .001), and they required fewer revisions (1% vs 6%, P = .05). The mean charge for the endoscopic procedure was $21 203, whereas the mean charge for the TCV reconstruction was $45 078 (P < .001). ES patients had more preoperative computed tomography scans (66% vs 44%, P = .003) than OSS patients, shorter operative times (68 vs 111 min, P < .001), and required fewer revision procedures (1% vs 8%, P < .001). The mean charge for the endoscopic procedure was $21 203 vs $20 535 for the OSS procedure (P = .62). CONCLUSION: The ES craniectomy for sagittal synostosis appeared to have less morbidity and a potential cost savings compared with the TCV reconstruction. The charges were similar to those incurred with OSS craniectomy, but patients had a shorter length of stay and fewer revisions.

Management of subaxial cervical instability in very young or small-for-age children using a static single-screw anterior cervical plate: indications, results, and long-term follow-up.

OBJECTIVE Subaxial cervical instability in very young or small-for-age children is uncommon and typically arises from trauma or skeletal dysplasia. Various operative techniques have been used to achieve stabilization in pediatric patients with evidence of instability, including anterior, posterior, and combined approaches. In this study, the authors report their results with subaxial cervical instability in this patient population treated using a static single-screw anterior cervical plate (ACP) system and allograft fusion. METHODS In a retrospective chart review, the authors identified all patients 6 years of age or younger who underwent an anterior cervical fusion procedure using a static single-screw ACP system either as a stand-alone construct or as part of an anterior-posterior stabilization procedure. Reasons for fusion included trauma, tumor, and congenital anomalies. RESULTS Five patients 6 years of age or younger underwent anterior cervical fusion using a static single-screw system during the 19-year study period. Follow-up ranged from 12 to 51 months (mean 26.8 months). Two patients underwent repeat surgery, one 7 days after and the other 21 months after their initial procedure. At last follow-up, a mean vertical growth of 22.8% was seen across the fused segments, with no evidence of kyphotic or lordotic abnormalities. CONCLUSIONS In very young or small-for-age children, the use of a static single-screw ACP system appears to be a safe and effective option to manage subaxial cervical instability. Bony fusion and continued longitudinal growth occur within the fused segments, with no evidence of long-term cervical malalignment.

Metastatic Adenoid Cystic Carcinoma Mimicking Butterfly Glioblastoma: A Rare Presentation in the Splenium of the Corpus Callosum.

BACKGROUND: Intracranial spread of an adenoid cystic carcinoma (ACC) of the parotid gland is rare, and metastatic ACC to the splenium of the corpus callosum mimicking butterfly glioblastoma (GBM) has not been reported previously. We report a rare case of metastasis to the splenium of the corpus callosum from ACC of the parotid gland. CASE DESCRIPTION: The tumor occupied the splenium and mimicked the presentation of a butterfly glioma. The patient had undergone parotidectomy 5 years before presentation with this intracranial lesion. On magnetic resonance imaging, the lesion was separate from the pineal gland and displaced the internal cerebral veins downward. Ventricular obstruction and increased cellularity were also suggested, and multiple fluid-filled cystic spaces were observed. The tumor was partially resected, because the extreme lateral boundary could not be visualized. Histological analysis with anti-c-kit antibody showed strong expression of the epithelial component; immunohistochemistry with anti-p63 antibody revealed nests of positive tumor cells, highlighting the myoepithelial component. The tumor also stained positive for anti-Myb antibody. CONCLUSIONS: The treatment for this lesion is surgical debulking followed by radiation therapy; however, the overall prognosis remains grim because of limited chemotherapy options and a propensity for recurrence in both local and distant fashions. When a tumor with adenoid histological features and a "butterfly" phenotype grows in the corpus callosum in a patient with known parotid ACC, both metastasis and adenoid variant GBM should be considered. Careful clinical and radiological correlation is required to diagnose and treat this rare lesion.

Immune checkpoint blockade as a potential therapeutic target: surveying CNS malignancies.

BACKGROUND: Expression of programmed cell death protein 1 (PD-1)/programmed death ligand 1 (PD-L1) across glioma grades is undocumented, and their interactions with commonly expressed genetic and epigenetic alterations are undefined but nonetheless highly relevant to combinatorial treatments. METHODS: Patients with CNS malignancies were profiled by Caris Life Sciences from 2009 to 2016. Immunohistochemistry findings for PD-1 on tumor-infiltrating lymphocytes (TIL) and PD-L1 on tumor cells were available for 347 cases. Next-generation sequencing, pyrosequencing, immunohistochemistry, fragment analysis, and fluorescence in situ hybridization were used to determine isocitrate dehydrogenase 1 (IDH1), phosphatase and tensin homolog (PTEN), and tumor protein 53 mutational status, O(6)-DNA methylguanine-methyltransferase promoter methylation (MGMT-Me) status, PTEN expression, plus epidermal growth factor receptor variant III and 1p/19q codeletion status. RESULTS: PD-1+ TIL expression and grade IV gliomas were significantly positively correlated (odds ratio [OR]: 6.363; 95% CI: 1.263, 96.236)-especially in gliosarcomas compared with glioblastoma multiforme (P = .014). PD-L1 expression was significantly correlated with tumor grade with all PD-L1+ cases (n = 21) being associated with grade IV gliomas. PD-1+ TIL expression and PD-L1 expression were significantly correlated (OR: 5.209; 95% CI: 1.555, 20.144). Mutations of PTEN, tumor protein 53, BRAF, IDH1, and epidermal growth factor receptor or MGMT-Me did not associate with increased intratumoral expression of either PD-1+ TIL or PD-L1 in glioblastoma multiforme even before false discovery rate correction for multiple comparison. CONCLUSIONS: Targeting immune checkpoints in combination with other therapeutics based on positive biomarker selection will require screening of large patient cohorts.

Occipital aneurysmal bone cyst rupture following head trauma: case report.

Aneurysmal bone cysts (ABCs) are benign, expansile, osteolytic lesions that represent 1%-2% of primary bone tumors. Cranial ABCs are even more rare and represent 3%-6% of these unique lesions. The authors describe the case of a 3-year-old girl who presented with an acute posterior fossa epidural hematoma after minor trauma. Imaging workup revealed a previously undiagnosed suboccipital ABC that appeared to have ruptured as a result of her trauma, leading to a life-threatening hemorrhage. To the authors' knowledge, a ruptured ABC has never before been presented in the pediatric literature. In this case report, the authors review the imaging findings, natural history, clinical course, and treatment of these rare lesions.

Pediatric spinal pilomyxoid astrocytoma.

Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5-11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.

Recurrent, delayed hemorrhage associated with edoxaban after deep brain stimulation lead placement.

Factor-Xa inhibitors like edoxaban have been shown to have comparable or superior rates of stroke and systemic embolization prevention to warfarin while exhibiting lower clinically significant bleeding rates. The authors report a case of a man who presented with delayed, recurrent intracranial hemorrhage months after successful deep brain stimulator placement for Parkinson disease while on edoxaban for atrial fibrillation. Further reports on the use of novel anticoagulants after intracranial surgery are acutely needed to help assess the true relative risk they pose.

Comparing fourth ventricle shunt survival after placement via stereotactic transtentorial and suboccipital approaches.

OBJECT: Fourth ventricle hydrocephalus, or a "trapped" fourth ventricle, presents a treatment challenge in pediatric neurosurgery. Fourth ventricle hydrocephalus develops most commonly as a result of congenital anomalies, intraventricular hemorrhage, or infection. Standard management of loculated fourth ventricle hydrocephalus consists of fourth ventricle shunt placement via a suboccipital approach. An alternative approach is stereotactic-guided transtentorial fourth ventricle shunt placement via the nondominant superior parietal lobule. In this report, the authors compare shunt survival after placement via the suboccipital and stereotactic parietal transtentorial (SPT) approaches. METHODS: A retrospective chart review was performed to find all patients with a fourth ventricle shunt placed between January 1, 1998, and December 31, 2011. Time to shunt failure was quantified as the number of days from shunt placement to first shunt revision or removal. Other variables studied included patient age and sex, origin of hydrocephalus, comorbidities, number of existing supratentorial catheters at the time of fourth ventricle shunt placement (as a proxy for complexity), operating surgeon, and number of previous shunt revisions. The crossover rate from one technique to the other after shunt failure from the original approach was also investigated. RESULTS: In the 29 fourth ventricle shunts placed during the study period, 18 were placed via the suboccipital approach (62.1%) and 11 via the SPT approach (37.9%). There was a statistically significant difference in time to shunt failure, with the SPT shunts lasting an average of 901 days and suboccipital shunts lasting 122 days (p = 0.04). In addition, there was a significant difference in the rate of crossover from one technique to another, with 1 SPT shunt changed to a suboccipital shunt (5.6%) and 5 suboccipital shunts changed to SPT shunts (45.5%). CONCLUSIONS: Fourth ventricle shunt placement using an SPT approach resulted in significantly longer shunt survival times and lower rates of revision than the traditional suboccipital approach, despite a higher rate of crossover from previously failed shunting procedures. Stereotactic parietal transtentorial shunt placement may be considered for patients with loculated fourth ventricle hydrocephalus, especially when shunt placement via the standard suboccipital approach fails. It is therefore reasonable to offer this procedure either as a first option for the treatment of fourth ventricle hydrocephalus or when the need for fourth ventricle shunt revision arises.

Treatment of indolent, nonencapsulated cryptococcal meningitis associated with hydrocephalus.

Infection with cryptococcal meningitis is uncommon in immunocompetent patients. The major virulence factor is the polysaccharide capsule, while nonencapsulated mutants are generally considered nonpathogenic. The authors present a case of hydrocephalus caused by meningitis from an indolent, nonencapsulated Cryptococcus sp. requiring placement and multiple revisions of a ventriculoperitoneal shunt (VPS). The patient presented with progressively worsening occipital headaches. Computed tomography and magnetic resonance imaging showed significant hydrocephalus with no apparent cause. Her symptoms initially resolved after placement of a VPS, but returned four months later. Cultures of the shunt tubing and cerebrospinal fluid (CSF) showed no bacterial infection. When the symptoms failed to resolve, CSF fungal culture revealed Cryptococcus-like yeast, although the organisms were nonencapsulated, and the cryptococcal antigen was negative. After antibiotic therapy, the symptoms resolved. The unusual clinical presentation delayed the diagnosis, highlighting the importance of understanding the detection, diagnosis, and treatment of meningeal infections caused by C. neoformans.

Image guidance for brain metastases resection.

The primary goal in removing a metastatic brain tumor is to maximize surgical resection while minimizing the risk of neurological injury. Intraoperative image guidance is frequently used in the resection of both primary and metastatic brain tumors. Stereotactic volumetric techniques allow for smaller craniotomies, facilitate lesion localization, and help neurosurgeons avoid eloquent structures. In turn, this leads to decreased patient morbidity and shorter hospitalizations. Image guidance is not without shortcomings, however, perhaps the most significant of which is inaccuracy of tumor resection associated with intraoperative brain shifts. The goal of this review is to expound on the uses of image guidance and discuss avoidance of technical pitfalls in the resection of cerebral metastatic lesions.

Neurosurgical complications of direct thrombin inhibitors--catastrophic hemorrhage after mild traumatic brain injury in a patient receiving dabigatran.

Dabigatran etexilate is an oral anticoagulant that acts as a direct, competitive thrombin inhibitor. Large randomized clinical trials have shown higher doses of dabigatran (150 mg taken twice daily) to be superior to warfarin in terms of stroke and systemic embolism rates in patients with nonvalvular atrial fibrillation. As a result, in 2010 the US FDA approved the use of dabigatran for the prevention of stroke and systemic embolism in patients with atrial fibrillation. Dabigatran is especially attractive in the outpatient setting because patients do not require routine monitoring with prothrombin times or international normalized ratios. To date, no effective reversal agent for dabigatran in the event of catastrophic hemorrhage has been identified. The authors report a case of an elderly patient, being treated with dabigatran for atrial fibrillation, who presented with a rapidly expanding intracranial hemorrhage after a ground-level fall. This case highlights an impending neurosurgical quandary of complications secondary to this new anticoagulation agent and suggests potential options for management.

Comparison of Three-Dimensional Fluoroscopy versus Postoperative Computed Tomography for the Assessment of Accurate Screw Placement after Instrumented Spine Surgery.

While intraoperative three-dimensional fluoroscopy does not possess the resolution and image quality of computed tomography (CT), it may provide adequate information about screw placement to guide intra- and postoperative decision making. We compared the accuracy of intraoperative three-dimensional fluoroscopy visualization of proper screw placement with that of postoperative CT. We retrospectively reviewed spinal instrumentation procedures done using the O-arm (Medtronic, Minneapolis, MN, USA) that also had postoperative CT. All screws were assessed for placement accuracy on O-arm and CT images on a 4-point scale. In this study, 20 cases met the inclusion criteria. Thirteen breaches (11 grade 1 and 2 grade 2) were identified on O-arm images, and 14 breaches (10 grade 1, 3 grade 2, and 1 grade 3) were identified on CT. Sensitivity, specificity, and positive and negative predictive values were 93, 99, 99, and 98%, respectively. The Kappa value (0.96) suggested a very high degree of agreement between three-dimensional fluoroscopy and CT in determining accuracy of screw placement. These findings may allow less frequent use of postoperative CT scans, improving cost effectiveness in patients who require spinal instrumentation procedures and potentially decreasing the number of patients who require replacement of an inappropriately positioned screw.

Spinal arteriovenous fistulas in children with hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant angiodysplasia with high penetrance and variable expression. The manifestations of HHT are often age related, and spinal arteriovenous fistula (AVF) may be the initial presentation of HHT in young children. Because spinal AVFs are rarely reported, however, screening is not incorporated into current clinical recommendations for the treatment of patients with HHT. The authors describe 2 cases of children younger than 2 years of age with acute neurological deterioration in the context of a spinal AVF and in whom HHT was subsequently diagnosed. One patient presented with intraventricular and subarachnoid hemorrhage and the other with acute thrombosis of an intramedullary varix. These cases highlight the potential for significant neurological morbidity from a symptomatic AVF in very young children with HHT. Given the lack of data regarding the true incidence and natural history of these lesions, these cases raise the question of whether spinal cord imaging should be incorporated into screening recommendations for patients with HHT.