Intradural lumbosacral malignant extrarenal rhabdoid tumor: a case report.

BACKGROUND: Rhabdoid tumors are highly malignant tumors predominantly affecting the pediatric population. When these tumors occur outside of the kidneys, they are referred to as malignant extrarenal rhabdoid tumors (MERT), a rare highly aggressive subtype. Less commonly, these tumors involve the neuro-axis. OBJECTIVE: Here we present a case of a 15-year-old girl with intradural MERT of the lumbosacral spine who presented with back pain, sudden worsening of lower extremity strength, and complete loss of bowel and bladder control. RESULTS: The patient's tumor showed loss of INI-1 and negative staining for cytokeratin AE1AE3, CD99, and SOX10. CONCLUSIONS: To our knowledge, there are no previous case reports of MERT with intradural lumbosacral spinal involvement.

Migration of a ventriculo-peritoneal shunt catheter into a back incision of a patient with previous spinal fusion.

INTRODUCTION: This case examines a unique, longitudinal presentation of an abandoned, migrating VP shunt which presents as multiple complications, including a weeping abscess in the patients back. We believe that the latter complication was potentially caused by the wound from the patient's previous history of spinal fusion surgery. CASE PRESENTATION: The patient presents with an associated type 2 Chiari malformation, hydrocephalus, and a previous history of posterior spinal fusion (T4-L5 anterior fusion and T2-L5 posterior fusion) at age 11. The patient had undergone shunt revisions in early adolescence as well. At 22, the patient is admitted into emergency care due to recurrent infections caused by a migrating VP shunt. Due to complications in corrective surgery at the time, the shunt was forced to be abandoned. This resulted in the most recent presentation of a weeping abscess at the patient's spinal fusion surgery wound; the culprit was the abandoned, migrating VP shunt.. MANAGEMENT/OUTCOME: An initial course of broad-spectrum antibiotics was started. However, the abscess continued to recur. Eventually, the catheter was surgically removed, a tailored antibiotic regiment was started, and a 6-month patient follow-up was performed. The patient is no longer symptomatic and off of antibiotics. DISCUSSION: In abandoned VP shunts, migration into a non-sterile cavity dictates prompt removal, especially after symptoms of infection present. Additionally, careful monitoring for signs of peritonitis or other symptoms for a dedicated period of time is necessary. To the authors' best knowledge, this is the first case of an occult shunt migration through the patient's back that presented with a weeping abscess.

Desmoplastic infantile ganglioglioma with associated giant aneurysm­case report.

Desmoplastic infantile ganglioglioma (DIG) and supratentorial giant cerebral aneurysm are each extremely rare entities in infants. Here, we present the case of an 8-day old boy who had both of these conditions concurrently. To our knowledge, there is no previous case reported of a patient with coexisting DIG and giant aneurysm.

Symptomatic cerebral vasospasm following resection of a medulloblastoma in a child.

BACKGROUND: Vasospasm may occur following intracranial tumor resection but is uncommon following resection of tumors in the posterior fossa. METHODS: Case report. RESULTS: Here, we report an unusual pediatric case of symptomatic cerebral vasospasm following resection of a posterior fossa medulloblastoma in a 10-year-old child. CT angiogram and serial Transcranial Doppler (TCD) studies confirmed the presence of vasospasm and response to hemodynamic augmentation therapy, resulting in favorable outcome. CONCLUSION: This case illustrates an unusual complication of posterior fossa tumor resection, and the potential utility of TCD studies in the detection and management of vasospasm in pediatric neurocritical care.

Digital subtraction angiography in cerebrovascular disease: current practice and perspectives on diagnosis, acute treatment and prognosis.

Digital Subtraction Angiography (DSA) is the gold-standard imaging modality in acute cerebrovascular diagnosis. The role of DSA has become increasingly prominent since the incorporation of endovascular therapy in standards of care for acute ischemic stroke. It is used in the assessment of cerebral vessel patency; however, the therapeutic role of DSA from a prognostic standpoint merits further investigation. The current paper provides an update on current practice on diagnostic, therapeutic and prognostic use of DSA in acute cerebrovascular diseases and various indications and perspectives that may apply, or limit its use, in ongoing surveillance or prognosis. Pre-clinical and clinical studies on the aspects, including but not limited to the morphology of cerebrovasculature in acute ischaemic stroke, are required to delineate and inform its prognostic role.

The association of transradial access and transfemoral access with procedural outcomes in acute ischemic stroke patients receiving endovascular thrombectomy: A meta-analysis.

OBJECTIVE: There is an ongoing debate regarding the benefits of using transradial access (TRA) over transfemoral access (TFA) in endovascular therapies including endovascular thrombectomy (EVT) for acute ischemic stroke (AIS) patients. This study sought to investigate the association of TRA and TFA with procedural success, access-site complications, first-pass reperfusion (FPR), puncture-to-recanalisation (PTR) time and hemorrhagic transformation (HT) by performing a meta-analysis. MATERIALS AND METHODS: PubMed, EMBASE and Scopus were searched. Studies with patients aged ≥ 18 years and head-to-head TRA vs TFA comparisons were included. Random-effects modeling was performed to obtain summary effects and forest plots were plotted to study the association of TFA with access site complications, FPR, HT, PTR time and procedural success. RESULTS: Six studies encompassing 945 patients (347 TRA and 598 TFA) were included in the meta-analysis. Meta-analysis revealed that in AIS patients receiving EVT, TRA was significantly associated with a decreased risk of access-site complications (RR 0.17, 95% CI 0.05 0.54; p = 0.003, z = -2.957) and HT (RR 0.07, 95% CI 0.02 0.27; p < 0.0001, z = -3.8841). However, TRA was not significantly associated with procedural success (RR 0.96, 95% CI 0.90 1.01; p = 0.141, z = -1.473), FPR (RR 0.91, 95% CI 0.79 1.05; p = 0.194, z = -1.299) and PTR time (SMD -0.14, 95% CI -0.42 -0.14; p = 0.323, z = -0.989). CONCLUSION: Our meta-analysis demonstrated that TRA is a safe alternative to TFA, in AIS patients receiving EVT, with significantly decreased access-site complications and HT with TRA, albeit with comparable procedural success, FPR and PTR time to TFA. DATA AVAILABILITY STATEMENT: The original contributions presented in the study are included in the article/Supplementary information, further inquiries can be directed to the corresponding author.

Middle Cerebral Artery Aneurysm Clipping With Immersive 360° Virtual Reality Model: 2-Dimensional Operative Video.

This is a 50-yr-old female who presented with severe headaches and was found to have an unruptured middle cerebral artery (MCA) aneurysm. She was taken to the operating room for microsurgical clipping. Prior to surgery, an interactive 360° virtual reality (VR) model (Surgical Theatre Inc) rendered from the patient's volumetric computed tomography (CT) angiography (CTA) was used for patient education and operative planning. The aneurysm was located at the MCA trifurcation, and the VR model was used to rehearse clip options prior to surgery. A right pterional craniotomy was performed with a dissection through the sylvian fissure down the M1 segment of the MCA for proximal control. The surgical clips are placed and highlighted in the virtual platform simultaneously. Doppler and indocyanine green (ICG) confirmed patent flow through the MCA trifurcation. A postoperative angiogram confirmed no aneurysm filling. The immersive 360° VR models of pre- and postoperative CTA were shown to the patient at the 1-mo follow-up. The case presented here demonstrates the utilization of the 360° VR model for patient education, preoperative planning, and intraoperative visualization. It is imperative to remain flexible during surgery to change the strategy based on the aneurysm anatomy intraoperatively. Importantly, the interactive 360° VR model allowed for a detailed evaluation of the patient-specific anatomy prior to surgery and helped understand the complex anatomy in high resolution. The patient consented to the procedure and publication of this video for educational purposes.

Acute Neurological Care in the COVID-19 Era: The Pandemic Health System REsilience PROGRAM (REPROGRAM) Consortium Pathway.

The management of acute neurological conditions, particularly acute ischemic stroke, in the context of Coronavirus disease 2019 (COVID-19), is of importance, considering the risk of infection to the healthcare workers and patients and emerging evidence of the neuroinvasive potential of the virus. There are variations in expert guidelines further complicating the picture for clinicians in acute settings. In this light, there is a compelling need for further formulation of recommendations that compile these variations seen in the numerous guidelines present. Health system protocols for managing ongoing acute neurological care and intervention need consideration of safety and well-being of the frontline healthcare workers and the patients. We examine existing pathways and their efficacy to mitigate viral exposure to the healthcare workers and patients and synthesize a systemic approach to manage patients with acute neurological conditions in the COVID-19 scenario. Early experiences with a COVID-19 positive stroke patient treated with endovascular thrombectomy is presented to highlight the urgent need for adequate personal protective equipment (PPE) during acute neuro-interventional procedures.

Rapidly Expanding Pediatric Post Radiation Brainstem Cavernoma Presenting with Singultus.

Here we present a pediatric patient status post resection of a primitive neuroectodermal tumor (PNET) with cranial/spinal radiation and development of a medullary cavernoma seven years after radiation therapy. The patient's cavernoma demonstrated rapid symptomatic growth in six weeks resulting in the presentation of intractable hiccups (singultus). The patient underwent resection of the cavernoma with cessation of the hiccups. We also review the pathology and possible mechanisms of such rapid growth of this post-radiation cavernoma as well as advise surveillance for patients with such lesions, as their course may be different from that of sporadic cavernomas.

Treatment of ruptured lenticulostriate artery aneurysms.

OBJECT: Lenticulostriate artery (LSA) aneurysms are rarely reported in the literature, making management decisions challenging. Conservative, endovascular, and surgical treatments have been described primarily through case reports and reports of individual authors' experiences. The purpose of this study is to report neurological outcomes in a single-institution experience of ruptured lenticulostriate aneurysms treated surgically. METHODS: The authors have conducted a retrospective review of all cases involving patients with ruptured LSA aneurysms who presented to the Mt. Sinai Hospital neurosurgical service between September 2001 and January 2007. RESULTS: Over 5.4 years, the authors treated 6 patients with 7 LSA aneurysms-6 ruptured and 1 unruptured. The Hunt and Hess grade on admission ranged from I to IV, with subarachnoid hemorrhage in 5 of the 6 patients. Catheter angiography confirmed the presence of the aneurysms, and all patients underwent a pterional craniotomy and clipping or resection of the aneurysm, performed by a single surgeon. Associated risk factors in our series of patients included hypertension, cocaine abuse, and intracranial occlusive disease suggestive of moyamoya disease. Two types of LSA aneurysms were identified. The mean size of the 6 ruptured aneurysms was 3.2 mm. The LSA was preserved in 3 of 6 patients, but LSA preservation did not correlate with development of a postoperative infarct, clinically or radiologically. In patients with ruptured aneurysms, the mean modified Rankin Scale score at discharge was 1.7. The 3 patients in whom the LSA was sacrificed had good outcomes, suggesting that loss of the artery is clinically well tolerated. CONCLUSIONS: This case series demonstrates that surgical treatment of ruptured LSA aneurysms can be an appropriate, effective, and safe therapy.

Sporadic Meningioangiomatosis: A Series of Three Pediatric Cases.

Meningioangiomatosis (MA) is a rare benign, hamartomatous lesion within the leptomeninges and cerebral cortex. Three percent of intractable epileptic patients with tumor develop MA. It may be accompanied with neurofibromatosis type II, or it may occur sporadically. Three patients, age range of 2-16 years old, presented with episodes of seizure. The patients demonstrated no family history or stigmata of neurofibromatosis type II. Electroencephalogram (EEG) was unremarkable for epileptiform activity. Magnetic resonance imaging (MRI) revealed enhancing lesions within the frontal gyrus, the anterior cingulate gyrus, and the parietal lobe. Incomplete resection led to recurrence in one patient, and later, intraoperative ultrasound was used to achieve total resection in another patient. Each patient was seizure free on follow-up, and managed with anti-epileptic medication. Resection is the only curative treatment in 85% of MA cases. Complete resection is necessary for symptomatic treatment in cases of MA, as recurrence has been documented in this lesion. Intraoperative ultrasound is an effective imaging modality to ensure gross total resection of MA.

Endoscopic corpus callosotomy and hemispherotomy.

OBJECT: Corpus callosotomy and hemispherotomy are conventionally performed via a large craniotomy with the aid of a microscope for children with intractable epilepsy. Primary technical considerations include completeness of disconnection and blood loss. The authors describe an endoscopic technique performed through a microcraniotomy for these procedures. METHODS: Four patients with drop attacks and 2 with intractable seizures related to a neonatal stroke underwent endoscopic complete corpus callosotomy and hemispherotomy, respectively. The surgeries were performed through a 2- to 3-cm precoronal microcraniotomy. Interhemispheric dissection to the corpus callosum was done using the standard technique. Subsequently, the bimanual technique with a suction device mounted on an endoscope was used to perform a complete corpus callosotomy, including interforniceal and anterior commissure disconnection. In patients who had hemispherotomy, the fornix was resected posteriorly and lateral disconnection was done by unroofing the temporal horn. Anteriorly, endoscopic corticectomy was done along the ipsilateral anterior cerebral artery to reach the bifurcation of the internal carotid artery to complete the anterior disconnection. Postoperative MRI and diffusion tensor imaging (DTI) of the brain were performed to confirm complete disconnection. RESULTS: The procedure was accomplished successfully in all patients, with excellent visualization secured. None of the patients required a blood transfusion. Postoperative MRI and DTI confirmed completeness of the disconnection. Patients who underwent corpus callosotomy had complete resolution of drop attacks at a mean follow-up of 6 months, and patients who underwent hemispherotomy became seizure free. CONCLUSIONS: Endoscopic corpus callosotomy and hemispherotomy are surgically feasible procedures associated with minimal blood loss, minimal risk, and excellent visualization.

Epidural spinal cord compression in a patient with blue rubber bleb nevus syndrome.

Blue rubber bleb nevus syndrome (BRBNS) can present with vascular malformations throughout the body, especially in the gastrointestinal tract. Spinal cord compression from these lesions is rare, particularly in the pediatric population. The authors report a case of BRBNS involving an 18-year-old female patient who presented with back pain and an epidural thoracic mass with cord compression. She underwent an uncomplicated thoracic laminectomy and decompression, with removal of what appeared to be a venous malformation. Postoperatively her pain improved, and imaging revealed resolution of cord compression. Pathological analysis highlighted dilated venous channels with myxoid degeneration in the wall with clot, characteristic of BRBNS. The early age of presentation and location are unique based on the literature search of BRBNS. The present report highlights the multiplicity of venous malformations in BRBNS, and the management of this case.

Pallidal deep brain stimulation for primary dystonia in children.

BACKGROUND: Deep brain stimulation (DBS) at the internal globus pallidus (GPi) has replaced ablative procedures for the treatment of primary generalized dystonia (PGD) because it is adjustable, reversible, and yields robust clinical improvement that appears to be long lasting. OBJECTIVE: To describe the long-term responses to pallidal DBS of a consecutive series of 22 pediatric patients with PGD. METHODS: Retrospective chart review of 22 consecutive PGD patients, ≤21 years of age treated by one DBS team over an 8-year period. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate symptom severity and functional disability, pre- and post-operatively. Adverse events and medication changes were also noted. RESULTS: The median follow-up was 2 years (range, 1-8 years). All 22 patients reached 1-year follow-up; 14 reached 2 years, and 11 reached 3 years. The BFMDRS motor subscores were improved 84%, 93%, and 94% (median) at these time points. These motor responses were matched by equivalent improvements in function, and the response to DBS resulted in significant reductions in oral and intrathecal medication requirements after 12 and 24 months of stimulation. There were no hemorrhages or neurological complications related to surgery and no adverse effects from stimulation. Significant hardware-related complications were noted, in particular, infection (14%), which delayed clinical improvement. CONCLUSION: Pallidal DBS is a safe and effective treatment for PGD in patients <21 years of age. The improvement appears durable. Improvement in device design should reduce hardware-related complications over time.