Prognostic risk factors for early outcomes of patients with myelomeningocele: a prospective study.

INTRODUCTION: Myelomeningocele (MMC) is a prevalent form of neural tube defect. Despite advancements in treatment, MMC still poses significant health risks, including complications leading to chronic disability and mortality. Identifying prognostic risk factors for early outcomes is crucial for tailored intervention strategies. METHODS: This prospective study involved newborns and infants diagnosed with MMC who underwent surgery between 2020 and 2023 at Urmia University of Medical Sciences. Demographic data and surgical outcomes were collected, and participants were followed up for six months. Statistical analyses were conducted using descriptive statistics, Chi-Square, and independent t-test. RESULTS: The study included 29 MMC cases, with an incidence rate of 1.4 per 10,000 live births. Lesions were predominantly located in the lumbar spine. Although mortality rates appeared to increase with ascending lesion sites, this trend was not statistically significant. Short-term outcomes revealed high morbidity and mortality rates, with neurological deficits being the most prevalent complication. Multivariable analysis identified head circumference as a significant predictor of adverse outcomes (IRR = 1.37, 95% CI = 1.02 to 1.86, p = 0.04). Furthermore, an increase in birth weight was associated with a reduction in the incidence of requiring a ventriculoperitoneal shunt (IRR = 0.99, 95% CI = 0.998 to 0.999, p = 0.02). CONCLUSION: This prospective study highlights prognostic risk factors for early outcomes in MMC patients, emphasizing the need for personalized intervention strategies. By addressing modifiable risk factors and implementing targeted interventions, healthcare providers can strive to improve outcomes and enhance the quality of life for MMC patients.

Perioperative Segmentation of the Posterior Fossa and the Keel of Goodrich in Surgical Outcomes of Chiari Malformations.

BACKGROUND: Chiari I malformation (CM- I) is defined as the extension of brain tissue into the spinal cord. This study aimed to refine the methodology for the acquisition of 3-dimensional measurements of the posterior fossa and introduce occipital keel size as a new marker and its impact in patients with CM. MATERIALS AND METHODS: In this retrospective study, all patients who underwent Chiari decompression surgery at Montefiore Medical Center from April 2012 to April 2022 were included. Perioperative clinical information was obtained in addition to maximal keel thickness (KT), foramen magnum area, and pre-and postoperative posterior fossa volumes for each patient and age-matched controls. Volumetric measurements were obtained using AI-based semiautomated segmentation. RESULTS: A total of 107 patients with CM including 37 males, and 70 females were studied with a mean age of 26.56±17.31 compared with 103 controls without CM. The comparison between the CM and the general population groups demonstrated a significantly increased keel size in Chiari patients. Keel size had a significant relationship with dysphagia, paresthesia, and intraoperative blood loss, while posterior volume change had a significant relationship with sex and early symptomatic improvement. The Foramen magnum area was related to tonsillar descent and more prominent in patients with spina bifida. CONCLUSION: The Keel of Goodrich is a new anatomical factor that should be taken into consideration when evaluating preoperative symptoms, and intraoperative complications in patients with CM-1. Volumetric analyses demonstrated that posterior fossa volume change had a significant impact on early symptom improvement in patients with Chiari, as did the choice of operative approach. The routine use of semi-automated segmentation of the posterior fossa may help stratify Chiari patients in the future and should be implemented in routine clinical care.

Investigating the Safety and Efficacy of Therapeutic Hypothermia in Pediatric Severe Traumatic Brain Injury: A Systematic Review and Meta-Analysis.

BACKGROUND: Prior guidelines recommended maintaining normothermia following traumatic brain injury (TBI), but recent studies suggest therapeutic hypothermia as a viable option in pediatric cases. However, some others demonstrated a higher mortality rate. Hence, the impact of hypothermia on neurological symptoms and overall survival remains contentious. METHODS: We conducted a systematic review and meta-analysis to evaluate the effects of hypothermia on neurological outcomes in pediatric TBI patients. The PubMed/Medline, Scopus, and Web of Science databases were searched until 1 January 2024 and data were analyzed using appropriate statistical methods. RESULTS: A total of eight studies, comprising nine reports, were included in this analysis. Our meta-analysis did not reveal significant differences in mortality (RR = 1.58; 95% CI = 0.89-2.82, p = 0.055), infection (RR = 0.95: 95% CI = 0.79-1.1, p = 0.6), arrhythmia (RR = 2.85: 95% CI = 0.88-9.2, p = 0.08), hypotension (RR = 1.54: 95% CI = 0.91-2.6, p = 0.10), intracranial pressure (SMD = 5.07: 95% CI = -4.6-14.8, p = 0.30), hospital length of stay (SMD = 0.10; 95% CI = -0.13-0.3, p = 0.39), pediatric intensive care unit length of stay (SMD = 0.04; 95% CI = -0.19-0.28, p = 0.71), hemorrhage (RR = 0.86; 95% CI = 0.34-2.13, p = 0.75), cerebral perfusion pressure (SMD = 0.158: 95% CI = 0.11-0.13, p = 0.172), prothrombin time (SMD = 0.425; 95% CI = -0.037-0.886, p = 0.07), and partial thromboplastin time (SMD = 0.386; 95% CI = -0.074-0.847, p = 0.10) between the hypothermic and non-hypothermic groups. However, the heart rate was significantly lower in the hypothermic group (-1.523 SMD = -1.523: 95% CI = -1.81--1.22 p < 0.001). CONCLUSIONS: Our findings challenge the effectiveness of therapeutic hypothermia in pediatric TBI cases. Despite expectations, it did not significantly improve key clinical outcomes. This prompts a critical re-evaluation of hypothermia's role as a standard intervention in pediatric TBI treatment.

Predicting the Outcome and Survival of Patients with Spinal Cord Injury Using Machine Learning Algorithms: A Systematic Review.

BACKGROUND: Spinal cord injury (SCI) is a significant public health issue, leading to physical, psychological, and social complications. Machine learning (ML) algorithms have shown potential in diagnosing and predicting the functional and neurologic outcomes of subjects with SCI. ML algorithms can predict scores for SCI classification systems and accurately predict outcomes by analyzing large amounts of data. This systematic review aimed to examine the performance of ML algorithms for diagnosing and predicting the outcomes of subjects with SCI. METHODS: The literature was comprehensively searched for the pertinent studies from inception to May 25, 2023. Therefore, electronic databases of PubMed, Embase, Scopus, and Web of Science were systematically searched with individual search syntax. RESULTS: A total of 9424 individuals diagnosed with SCI across multiple studies were analyzed. Among the 21 studies included, 5 specifically aimed to evaluate diagnostic accuracy, while the remaining 16 focused on exploring prognostic factors or management strategies. CONCLUSIONS: ML and deep learning (DL) have shown great potential in various aspects of SCI. ML and DL algorithms have been employed multiple times in predicting and diagnosing patients with SCI. While there are studies on diagnosing acute SCI using DL algorithms, further research is required in this area.

Clinical Features and Management of Skull Base Fractures in the Pediatric Population: A Systematic Review.

Pediatric basilar skull fractures (BSFs) are a rare type of traumatic head injury that can cause debilitating complications without prompt treatment. Here, we sought to review the literature and characterize the clinical features, management, and outcomes of pediatric BSFs. We identified 21 relevant studies, excluding reviews, meta-analyses, and non-English articles. The incidence of pediatric BSFs ranged from 0.0001% to 7.3%, with falls from multi-level heights and traffic accidents being the primary causes (9/21). The median presentation age ranged from 3.2 to 12.8 years, and the mean age of patients across all studies was 8.68 years. Up to 55% of pediatric BSFs presented with intracranial hematoma/hemorrhage, along with pneumocephalus and edema. Cranial nerve palsies were a common complication (9/21), with the facial nerve injured most frequently (7/21). While delayed cranial nerve palsy was reported in a few studies (4/21), most resolved within three months post-admission. Other complications included CSF leaks (10/21) and meningitis (4/21). Management included IV fluids, antiemetics, and surgery (8/21) to treat the fracture directly, address a CSF leak, or achieve cranial nerve compression. Despite their rarity, pediatric skull base fractures are associated with clinical complications, including CSF leaks and cranial nerve palsies. Given that some of these complications may be delayed, patient education is critical.

Nuanced Management of a Skull Base Tumor in the Setting of Relapsed Acute Lymphoblastic Leukemia.

Introduction Relapsed acute lymphoblastic leukemia (ALL) involving the central nervous system (CNS) is a significant issue that contributes to both morbidity and mortality. Given the poor outcomes in patients with CNS relapse, understanding how ALL involving intracranial relapse presents and is treated is critical. Here, we present a complex case of relapsed recurrent ALL in a pediatric patient. Case Report An 11-year-old patient presented with double relapse of ALL in the form of an extensive skull base lesion and again with leptomeningeal disease. For the skull base lesion, she was treated nonsurgically with chemotherapy and radiation, which led to a remarkable reduction in the size of the lesion. However, she was found to have early recurrence with leptomeningeal enhancement resulting in hydrocephalus 5 months after completing therapy. A shunt was placed successfully. Currently, she is being managed with monthly intrathecal chemotherapy with cerebrospinal fluid sampling and bone marrow biopsies every 2 months. Discussion We report the significant effect of chemotherapy and radiotherapy in reducing the size of the extensive skull base lesion, saving the patient from the risks associated with surgery. This patient's initial relapse, with a large skull base lesion that had intracranial involvement, is an unusual presentation of relapsed ALL. The additional early recurrence of leptomeningeal disease further makes this case unique and the management even more nuanced. Here, we demonstrate a multidisciplinary approach for the successful treatment of our patient, which can help guide the management of similar patients in the future.

Clear cell meningiomas-case presentation, review of radiographic identifiers, and treatment approaches.

Spinal clear cell meningiomas (CCMs) are a rare histological subtype of meningiomas that pose preoperative diagnostic challenges due to their radiographic similarities with other lesions. They are also more aggressive, exhibiting higher rates of recurrence, particularly in pediatric patients. Overcoming diagnostic challenges of these tumors can improve patient outcomes. In this report, we describe a case of a pediatric patient presenting with a lumbar CCM in whom we were able to obtain gross total resection. Our report reviews previously identified predictors of CCM recurrence, including the Ki-67 proliferation index, number of spinal segments involved, and hormonal influences related to age and sex. We describe the characteristic radiographic features that differentiate spinal CCMs from other tumors to improve pre-operative diagnosis. Furthermore, we provide our rationale for adjuvant therapy for pediatric patients to refine treatment protocols for these rare tumors.

Volumetric segmentation in the context of posterior fossa-related pathologies: a systematic review.

BACKGROUND: Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature. METHODS: A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria. RESULTS: The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation. CONCLUSIONS: Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.

A comprehensive systematic review and meta-analysis study in comparing decompressive craniectomy versus craniotomy in patients with acute subdural hematoma.

There are two controversial surgery methods which are traditionally used: craniotomy and decompressive craniectomy. The aim of this study was to evaluate the efficacy and complications of DC versus craniotomy for surgical management in patients with acute subdural hemorrhage (SDH) following traumatic brain injury (TBI). We conducted a comprehensive search on PubMed, Scopus, Web of Science, and Embase up to July 30, 2023, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Relevant articles were reviewed, with a focus on studies comparing decompressive craniectomy to craniotomy techniques in patients with SDH following TBI. Ten studies in 2401 patients were reviewed. A total of 1170 patients had a craniotomy, and 1231 had decompressive craniectomy. The mortality rate was not significantly different between the two groups (OR: 0.46 [95% CI: 0.42-0.5] P-value: 0.07). The rate of revision surgery was insignificantly different between the two groups (OR: 0.59 [95% CI: 0.49-0.69] P-value: 0.08). No significant difference was found between craniotomy and decompressive craniectomy regarding unilateral mydriasis (OR: 0.46 [95% CI: 0.35-0.57] P-value < 0.001). However, the craniotomy group had significantly lower rates of non-pupil reactivity (OR: 0.27 [95% CI: 0.17-0.41] P-value < 0.001) and bilateral mydriasis (OR: 0.59 [95% CI: 0.5-0.66] P-value: 0.04). There was also no significant difference in extracranial injury between the two groups, although the odds ratio of significant extracranial injury was lower in the craniotomy group (OR: 0.58 [95% CI: 0.45-0.7] P-value: 0.22). Our findings showed that non-pupil and bilateral-pupil reactivity were significantly more present in decompressive craniectomy. However, there was no significant difference between the two groups regarding mortality rate, extracranial injury, revision surgery, and one-pupil reactivity.

The complex treatment paradigms for concomitant tethered cord and scoliosis: illustrative case.

BACKGROUND: Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections. OBSERVATIONS: A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3-4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved. LESSONS: Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.

A Case Report of Infant-Type Hemispheric Glioma with a Novel GAB1-ABL2 Kinase Fusion Treated with Dasatinib.

INTRODUCTION: Infant-type hemispheric glioma (IHG) is a rare form of cancer that affects newborns and infants. It is classified as a pediatric-type high-grade glioma and typically harbors receptor tyrosine kinase (RTK) gene fusions. Here, we present the finding of a novel gene fusion IHG treated with a targeted therapy that has yet to be implemented for any other IHG case to date. CASE PRESENTATION: We report the case of a 12-month-old boy with IHG who presented with obstructive hydrocephalus due to a large mass in the right frontal lobe. The patient initially underwent mass resection, but subsequent imaging showed rapid interval progression of the residual tumor. Comprehensive molecular analysis of the tumor tissue revealed a novel GAB1-ABL2 gene fusion, and the patient was started on dasatinib, an ABL kinase inhibitor. Shortly after initiation of dasatinib treatment, there was a significant reduction in tumor size and enhancement, followed by stabilization of disease. DISCUSSION: The patient's robust response to treatment suggests that dasatinib is an effective targeted therapy for IHG harboring a GAB1-ABL2 gene fusion. This finding may inform future investigations into the disease processes of IHG and help guide the diagnosis and treatment of IHG in the absence of previously identified gene fusions, improving clinical management of this vulnerable patient population.

Case Report: Treatment of the rare B-cell lymphoblastic lymphoma with scalp lesion using rotation flap.

Introduction: Leukemia is the most frequently occurring cancer in children, and lymphoblastic lymphoma (LBL) is a rare subtype. LBL are lymphoid neoplasms of B or T cell origin and are primarily treated with chemotherapy. Although cure rates among children are excellent, these patients must be monitored for relapse. Cutaneous lesions involving B-cell LBL (B-LBL) are extremely rare and here we present a patient with a worsening B-LBL scalp mass who required radical surgical excision. Case report: A 6-year-old female patient with a history of a nontender scalp mass discovered at approximately 2-3 years of age was evaluated for resection of the nodule due to its size and treatment history. The patient was originally diagnosed with follicular lymphoma by punch biopsy; excision was successfully performed on this 4 cm lesion and upon examination of the skin biopsy did we get a diagnosis of B-LBL. Reconstruction of the scalp was done through the rotation flap method. The patient's scalp healed well, and adjuvant chemotherapy was continued. There has been no reoccurrence. Discussion: Here we report the rarity of B-LBL cases involving extranodal involvement in the scalp. The most common reconstruction of scalp lesions has been using free flap from the anterolateral thigh (ALT) and latissimus dorsi (LD). Our case used the rotation flap, which has its functional and cosmetic benefits. The importance of monitoring this patient is emphasized due to the dangerous consequences of B-LBL relapse. Ultimately, our successful treatment and care of this rare case can be used as guidance for similar patients in the future.

Can Neutrophil to Lymphocyte Ratio Predict Early Outcome in Patients with Spinal Cord Injury?

BACKGROUND: Spinal cord injury is a frequent debilitating neurologic condition with increasing prevalence and related morbidity over the last decades. The neutrophil-to-lymphocyte ratio is a promising biomarker for determining different medical conditions' disease course and outcome such as traumatic brain injury (TBI). This study aimed to investigate the predictive value of neutrophil to lymphocyte ratio (NLR) in the outcome of SCI. METHOD: In a retrospective cross-sectional study from April 2019 to April 2022, all patients 18 to 65 years old, following spinal cord injury who were referred to Imam Khomeini Hospital and met inclusion and exclusion criteria enrolled in the study. A checklist including demographic data, lab, and clinical findings at admission, 24h, 48 h, and discharge were recorded. IBM SPSS Statistics software was used to analyze the data. A P-value of less than 0.05 was considered significant. RESULTS: Six hundred patients met our inclusion criteria and enrolled in the study. The mean age of the patients was 40.93 ± 12.77, with 75% male and 25% female. There was a significant correlation between the N/L ratio at different time points (p.value=0.001), injury type, and ASIA score at admission and discharge (0.001). Furthermore, the NLR had approached significant value alone to predict outcomes in patients enrolled in the study (0.06). CONCLUSIONS: A high NLR is unequivocally linked with poor outcomes in patients suffering from acute SCI and should be considered a negative prognostic factor; however, the NLR had approached significant predicting value in patients enrolled in the study.

Ventriculoperitoneal Shunt Tap Task Trainer: A Technical Report.

This technical report describes the creation of a model of an infant with a ventriculoperitoneal shunt (VPS). This model is authentic, assembled easily, and reusable which allows for pediatric and neurosurgical practitioners to gain experience in performing VPS taps. Learning objectives have been provided to guide task training.

STAT5b gain-of-function disease in a child with mycobacterial osteomyelitis of the skull: rare presentation of an emerging disease entity.

PURPOSE: STAT proteins play a key role in several cellular functions related to cell development, differentiation, proliferation, and survival. Persistent STAT activation due to somatic STAT5bN642H gain-of-function mutation is a rare mechanism of STAT dysregulation that results in hypereosinophilia, frequent infections, leukemias, and pulmonary diseases. Herein, we describe a case of a child with a rare early onset STAT5b gain-of-function disease treated with targeted JAK inhibition who developed a cranial Mycobacterium avium osteomyelitis. METHODS: A 3-year-old male with a known STAT5b gain-of-function mutation presented with a 10-day history of a firm, immobile, non-painful cranial mycobacterium mass with dural infiltration located anterior to the coronal suture. Stepwise management finalized with complete resection of the lesion with calvarial reconstruction. A case-based literature review was performed evaluating all patients with this mutation who developed cranial disease. RESULTS: The patient was symptom and lesion-free at 1 year since surgical resection and initiation of triple mycobacterial pharmacotherapy. Our literature review demonstrated the rarity of this disease, as well as other presentations of this disease in other patients. CONCLUSION: Patients with STAT5b gain-of-function mutations have attenuated Th1 responses and are treated with medications, such as JAK inhibitors, which further inhibit other STAT proteins that regulate immunity against rare infectious entities, such as mycobacterium. Our case highlights the importance of considering these rare infections in patients on JAK inhibitors and with STAT protein mutations. Possessing a clear mechanistic understanding of this genetic mutation, its downstream effect, and the consequences of treatment may enhance a physician's diagnostic and clinical management of similar patients in the future.

Correlation Between Clinical Findings at Admission and Glasgow Outcome Scale Score in Children with Traumatic Brain Injury.

OBJECTIVE: Traumatic brain injury has different pathophysiology and outcomes in children and adults. This study investigated the relationship between clinical and laboratory findings at admission and Glasgow Outcome Scale (GOS) score in children with traumatic brain injury. METHODS: This prospective cross-sectional single-center study enrolled 444 children 1-16 years old admitted to the neurosurgery ward from 2016 to 2020. Clinical data and laboratory information were extracted from the records of these patients at admission, and the relationship with GOS score at discharge was investigated. RESULTS: The 444 patients include 249 (56.08%) boys and 195 (43.92%) girls with a mean age of 7.32 ± 4.4 years. There was no correlation between GOS score and sex (P = 0.12), age (P = 0.16), serum potassium level (P = 0.08), platelet level (P = 0.21), and blood glucose (P = 0.18). There was a significant relationship between GOS score and hypotension (P = 0.03), hyponatremia (P = 0.04), prothrombin time (P = 0.03), partial thromboplastin time (P = 0.03), pupil size (P = 0.02), pupil reaction to light (P = 0.04), and Glasgow Coma Scale score (P = 0.04). CONCLUSIONS: Clinical and laboratory findings such as hypotension, hyponatremia, prothrombin time, partial thromboplastin time, pupil size, pupil reaction to light, and Glasgow Coma Scale score at admission could affect GOS score at discharge and result in poor outcomes in children with traumatic brain injury.

Pediatric brain aneurysms: a review of 1458 brain MR angiograms.

PURPOSE: To evaluate clinical and imaging characteristics of pediatric brain aneurysms. MATERIALS AND METHODS: A retrospective review of 1458 MR angiograms of pediatric patients (≤18 years old) obtained between 2006 and 2021 was performed. A non-infundibular arterial luminal outpouching larger than 1mm in size was identified as an "Intracranial aneurysm." Patient demographics, clinical presentations, and predisposing risk factors, including family history and underlying medical conditions, were reviewed. MRA images were analyzed for aneurysm location, number, maximum diameter, and interval changes on follow-up. RESULTS: Forty-nine (3.3%) patients (30 females, 19 males) with 64 intracranial aneurysms were identified with an average age of 13.71 ± 3.67 years. Eleven (22.4%) patients had multiple aneurysms. An underlying systemic illness was observed in 81.6% (40/49) cases, with sickle cell disease as the most frequent (25/49, 51%) diagnosis. A first-degree family history of intracranial aneurysms was recognized in 36/1458 (2.5%) patients. However, no intracranial aneurysm was found in this group. While 02/49 (4%) patients presented with acute SAH, headache was the most common (16/49, 32.7%) symptom at presentation in unruptured cases. The majority (47/64, 73.4%) of the aneurysms were located in the anterior circulation, with the ICA ophthalmic segment being most frequently (24/47, 51%) involved. Most (54/64, 84.4%) aneurysms were smaller than 4mm in size at the time of diagnosis. At least one follow-up MRA was obtained in 72.3% (34/47) of the unruptured aneurysms cohort. There was no change in the aneurysm size and morphology in 31/34 (91.2 %) patients over an average imaging follow-up of 39.6 months. Three (6%) patients demonstrated an interval increase in the aneurysm size. SAH patients (n=2) and two unruptured aneurysm patients with an interval increase in size were successfully treated with endovascular techniques. CONCLUSION: Female predominance with a higher frequency of small and unruptured intracranial aneurysms was recognized in our cohort. A higher incidence of an underlying systemic illness, especially sickle cell disease, was also noted. Most intracranial aneurysms in children appear to remain stable. However, there seems to be the risk of an aneurysm size increase which warrants regular clinical and imaging follow-up.

A nine-month-old boy with regression of milestones and severe constipation: an unusual case of a large spinal NTRK1 fusion pilocytic astrocytoma.

INTRODUCTION: Pilocytic astrocytoma, a World Health Organization grade 1 tumor, is the most common brain tumor in children between 5 and 14 years of age and the second most common in children younger than 5 and older than 14. Although classical to the cerebellum and hypothalamic regions, it can also arise in the spinal cord. Larotrectinib, a selective inhibitor of tropomyosin receptor kinase, has been effective in pediatric tumors with NTRK fusion mutations in children as young as 1-month-old. CASE: We share the case of a 9-month-old boy who presented with a 4-month history of regression of his milestones and severe constipation who was found to have a large spinal pilocytic astrocytoma with multiple intracranial periventricular lesions.

Cochlear Implants: What the Neurosurgeon Needs to Know.

Patients with cochlear implants (CIs) commonly undergo neurosurgical interventions for concurrent pathologies. The neurosurgeon must be aware of the limitations these devices place on treating these patients and all pertinent interactions CIs have with common neurosurgical instruments and procedures. A literature search was performed utilizing the terms "cochlear implant" and "neurosurgery" or "neurosurgical" and all associated iterations. We reviewed the abstracts of 146 generated reports and eight published papers discussing the interaction and limitations of CI use in different neurosurgical procedures. Five realms were identified in which a CI may potentially interfere with standard neurosurgical care: Magnetic resonance imaging (MRI), radiotherapy, deep brain stimulation (DBS), intraventricular shunt placement, and intraoperative neuromonitoring (IONM). First, MRI use with CIs is limited due to thermal injury risk, imaging disruption, and implant damage. Secondly, high-dose >50 Gy single-fraction linear accelerator-based radiosurgery has been demonstrated to result in a loss of radio frequency link range in CIs, interfering with their function. Next, during surgery for DBS, the need for MRI and microelectrode recording requires CI magnet removal by neurotology and the surgeon must communicate with a non-hearing patient. Tunneling of shunts must accommodate CI position retroauricularly, if ipsilateral, and programmable valves must be placed >2 cm from the CI to prevent interference. Intraoperative neuromonitoring may produce voltages that interfere with CIs, and while monopolar cautery may pose the same risk, no study has proven this to date. Generally, bipolar cautery is safe and favored >1 cm from CIs. MRI use is limited in CI patients, although MRI-safer devices are in production. DBS electrodes may be successfully placed after CI magnet removal. Programmable shunt valves may be placed >2 cm away from CIs and radiosurgery <50 Gy has not demonstrated harm to these devices. IONM and monopolar cautery have not been demonstrated to directly affect CIs; however, more research is needed.