The Anterior Interhemispheric Transcallosal Approach to the Ventricles: How We Do It.

Intraventricular tumors of the lateral and third ventricles are relatively rare, accounting for 1-2% of all primary brain tumors in most large series [1-4]. They can be uniquely challenging to approach due to their deep location, propensity to become large before they are discovered, and association with hydrocephalus [5, 6]. The surgeon's goal is to develop a route to these deep lesions that will cause the least morbidity, provide adequate working space, and achieve a complete resection. This must be performed with minimal manipulation of the neural structures encircling the ventricles, avoiding functional cortical areas, and acquiring early control of feeding vessels [7, 8].

Prophylactic removal of a migratory missile from the cerebral ventricles: case report.

BACKGROUND AND IMPORTANCE: Nonpowder firearm injuries to the head pose major health risks, with retained fragments potentially causing harmful sequelae that require neurosurgical intervention. CLINICAL PRESENTATION: We report the case of 2-year-old girl who sustained an accidental gunshot wound to the head. She sustained a penetrating ballistic intracranial injury caused by a BB shot from a rifle. At presentation, she was neurologically intact with a punctate laceration on her left forehead. Head CT demonstrated a small depressed left frontal skull fracture, a small intracerebral hematoma, and a 5-mm metallic bullet fragment in the deep left frontal lobe near the frontal horn of the left lateral ventricle. She was admitted to the hospital and managed nonoperatively with levetiracetam and intravenous antibiotics, and discharged home in good condition. Follow-up CT in 1 week showed slight migration of the metallic bullet fragment to the left, placing it at the anterior horn of the lateral ventricle. Six weeks later, follow-up CT showed migration of the bullet to the temporal horn of the left lateral ventricle. Intraventricular migration of the bullet raised concern that it could move further to obstruct the foramen of Monro or cerebral aqueduct. Therefore, we removed the bullet through a small left temporal craniotomy with image guidance using a microsurgical approach through a translucent tube. CONCLUSION: The authors discuss the rationale and technique for removing a nonpowder firearm bullet that has migrated within the cerebral ventricles.

An unusual finding of an anaplastic meningioma in NF2-related schwannomatosis.

NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features.

Relative anemia and perioperative stroke in children with moyamoya.

OBJECTIVES: Surgical revascularization for moyamoya arteriopathy decreases long-term stroke risk but carries a risk of perioperative ischemic complications. We aimed to evaluate modifiable stroke risk factors in children undergoing surgical revascularization for moyamoya. MATERIALS AND METHODS: In this exploratory, single-center, retrospective cohort study, medical records of pediatric patients undergoing surgical revascularization for moyamoya arteriopathy at our center between 2003 and 2021 were reviewed. Candidate modifiable risk factors were analyzed for association with perioperative stroke, defined as ischemic stroke ≤7 days after surgery. RESULTS: We analyzed 53 surgeries, consisting of 39 individual patients undergoing indirect surgical revascularization of 74 hemispheres. Perioperative ischemic stroke occurred following five surgeries (9.4%). There were no instances of hemorrhagic stroke. Larger pre-to-postoperative decreases in hemoglobin (OR 3.90, p=0.017), hematocrit (OR 1.69, p=0.012) and blood urea nitrogen (OR 1.83, p=0.010) were associated with increased risk of perioperative ischemic stroke. Weight-adjusted intraoperative blood loss was not associated with risk of perioperative ischemic stroke (OR 0.94, p=0.796). Among children with sickle cell disease, all of whom underwent exchange transfusion within one week prior to surgery, none experienced perioperative stroke. CONCLUSIONS: Decreases in hemoglobin, hematocrit, and blood urea nitrogen between the preoperative and postoperative periods are associated with increased risk of perioperative stroke. These novel findings suggest that dilutional anemia, possibly due to standardly administered hyperhydration, may increase the risk of perioperative stroke in some children with moyamoya. Further work optimizing both mean arterial pressure and oxygen-carrying capacity in these patients, including consideration of alternative blood transfusion thresholds, is necessary.

William Macewen and the first documented successful resection of a brain tumor.

William Macewen was a visionary fearless Scottish surgeon who performed the first documented successful resection of a brain tumor on July 27, 1879. The pioneering operation received little attention at the time, and even today Macewen is under-recognized for his historic accomplishment. In this vignette, the author discusses details of Macewen's landmark procedure and describes two other groundbreaking brain tumor operations, one performed by Rickman Godlee 5 years later and another performed by Zanobi Pecchioli almost half a century earlier.

Intracranial arachnoid cysts.

PURPOSE: The purpose of this review article is to outline the natural history, pathogenesis, anatomic considerations and surgical decision-making in caring for patients with intracranial arachnoid cysts. METHODS: A review of the literature for intracranial arachnoid cysts was performed using Embase, PubMed, and Web of Science databases, including review of the bibliographies of eligible articles and the author's own experience. RESULTS: Among those reviewed, 59 relevant original articles were included as well as illustrative cases from the authors own experience. CONCLUSIONS: Arachnoid cysts are congenital lesions characterized by split arachnoid membrane, thick collagen in the cyst wall, absent traversing trabecular processes within the cyst, and hyperplastic arachnoid cells in the cyst wall. The underlying etiology is not entirely known, and they occur in greater proportion in males and in greater incidence with various genetic conditions including Down syndrome, mucopolysaccharidosis, schizencephaly, neurofibromatosis, autosomal dominant polycystic kidney disease (ADPKD), acrocallosal syndrome, and Aicardi syndrome. Most intracranial arachnoid cysts are incidentally found and occur in the middle cranial fossa, with the remaining occurring in the cerebellopontine angle, suprasellar cistern, quadrigeminal cistern, convexity, and posterior fossa/cisterna magna. The current article outlines the natural history, prevalence, demographic factors, and treatment decisions in managing patients with intracranial arachnoid cysts.

The Gordian knot of hydrocephalus: constrictive entanglement of distal ventriculoperitoneal shunt catheter.

We recently cared for a patient with a distal ventriculoperitoneal shunt malfunction. The peritoneal catheter was coiled in a scarred pseudocyst, and we encountered strong resistance while attempting to remove the distal shunt catheter. The catheter was successfully removed under laparoscopic guidance and was found to be tightly coiled in a constrictive knot. We describe a technique for managing this unusual presentation to minimize the risk of complications.

Nephrogenic rest vs immature teratoma associated with lumbosacral lipomyelomeningocele: a case report and review of the literature.

BACKGROUND: Lipomyelomeningoceles (LMMs) are subcutaneous lipomas with dural penetration that often present with spinal cord tethering and may lead to neurological deterioration if untreated. This report describes a rare case of an LMM associated with immature nephroblastic tissue, representing a nephrogenic rest (NR) or, less likely, an immature teratoma. CLINICAL PRESENTATION: An 8-day-old infant girl presented to the clinic with a sacral dimple. Imaging demonstrated a tethered spinal cord with low-lying conus medullaris and an LMM. A firm mass was noted in the subcutaneous lipoma. Detethering surgery and removal of the lipoma and mass were performed at the age of 6 months. Pathological examination identified the mass as cartilage, fat, and immature nephroblastic tissue consistent with NR tissue or, less likely, a teratoma with renal differentiation. CONCLUSION: This presentation of an LMM associated with an immature teratoma or NR poses a risk of malignant transformation in patients. As a result, careful surgical dissection, resection, and close clinical follow-up are recommended for these patients.

H3 K27-altered diffuse midline glioma presenting as massive cerebellopontine hemorrhage.

We report the case of a 14-year-old boy who presented with extensive cerebellar and brainstem hemorrhage. Our presumptive diagnosis was a ruptured arteriovenous malformation (AVM), but two cerebral angiograms showed no significant vascular abnormalities. The patient underwent posterior fossa craniotomy and microsurgical evacuation of the hematoma. Pathological analysis of the hemorrhagic tissue made the diagnosis of diffuse midline glioma, H3 K27-altered (WHO grade 4), based on immunohistochemistry. He subsequently developed diffuse craniospinal leptomeningeal disease and progressed rapidly, with respiratory failure followed by severe neurologic decline without further hemorrhage. He was compassionately extubated at the request of the family and died before initiation of adjuvant therapy. This unusual case of a diffuse midline glioma presenting with massive hemorrhage underscores the importance of searching for an underlying etiology of hemorrhage in a child when a vascular lesion cannot be identified.

Same-day versus staged revascularization of bilateral moyamoya arteriopathy in pediatric patients.

PURPOSE: To compare the outcomes of conducting left and right hemisphere surgical revascularization on the same day versus different days for bilateral pediatric moyamoya arteriopathy patients. METHODS: We retrospectively analyzed mortality, stroke, and transient neurologic event (TNE) rates in North American bilateral pediatric moyamoya arteriopathy patients who underwent bilateral cerebral revascularization. RESULTS: A total of 38 pediatric (≤ 18 years old) patients at our institution underwent bilateral cerebral revascularization for moyamoya arteriopathy. Of these patients, 24 (63.2%) had both operations on the same day and 14 (36.8%) had the two operations on different days. The average length of stay for patients who underwent same-day bilateral revascularization was 6.9 ± 2.0 days and the average length of stay for each operation for patients who underwent staged bilateral revascularization was 4.5 ± 1.4 days, p = 0.001. While there were 7 (14.6%) postoperative strokes in patients who had both hemispheres revascularized on the same day, 0 (0%) strokes occurred in hemispheres after they had been operated on in the staged cohort, p = 0.042. Additionally, the postoperative stroke-free survival time in the ipsilateral hemisphere and TNE-free survival time were significantly longer in patients in the staged revascularization cohort. CONCLUSION: Same-day bilateral revascularization was associated with longer length of stay per operation, higher rate of ipsilateral stroke, and shorter postoperative TNE-free and stroke-free survival time in the revascularized hemisphere.

An update on the US adult thalassaemia population: a report from the CDC thalassaemia treatment centres.

Thalassaemia is caused by genetic globin defects leading to anaemia, transfusion-dependence and comorbidities. Reduced survival and systemic organ disease affect transfusion-dependent thalassaemia major and thalassaemia intermedia. Recent improvements in clinical management have reduced thalassaemia mortality. The therapeutic landscape of thalassaemia may soon include gene therapies as functional cures. An analysis of the adult US thalassaemia population has not been performed since the Thalassemia Clinical Research Network cohort study from 2000 to 2006. The Centers for Disease Control and Prevention supported US thalassaemia treatment centres (TTCs) to compile longitudinal information on individuals with thalassaemia. This dataset provided an opportunity to evaluate iron balance, chelation, comorbidities and demographics of adults with thalassaemia receiving care at TTCs. Two adult cohorts were compared: those over 40 years old (n = 75) and younger adults ages 18-39 (n = 201). The older adult cohort was characterized by higher numbers of iron-related comorbidities and transfusion-related complications. By contrast, younger adults had excess hepatic and cardiac iron and were receiving combination chelation therapy. The ethnic composition of the younger cohort was predominantly of Asian origin, reflecting the demographics of immigration. These findings demonstrate that comprehensive care and periodic surveys are needed to ensure optimal health and access to emerging therapies.

The neurosurgical management of Severe Hemophilia A and Moyamoya (SHAM): challenges, strategies, and literature review.

INTRODUCTION: Severe Hemophilia A and Moyamoya arteriopathy (SHAM syndrome) is a rare genetic disorder caused by deletion of portions of the cytogenic band Xq28. A case of SHAM syndrome requiring bilateral cerebral revascularization is described with an emphasis on perioperative management. CASE REPORT: A 5-year-old boy with severe hemophilia A complicated by factor VIII inhibition presented with right-sided weakness. Imaging revealed multiple strokes and vascular changes consistent with Moyamoya disease. The patient underwent two-staged indirect cerebral bypass revascularizations, first on the left side and several months later on the right. Perioperative management required balancing the administration of agents to prevent coagulopathy and perioperative hemorrhage while mitigating the risk of thromboembolic events associated with bypass surgery. Despite a multidisciplinary effort by the neurosurgery, hematology, critical care, and anesthesiology teams, the post-operative course after both surgeries was complicated by stroke. Fortunately, the patient recovered rapidly to his preoperative functional baseline. CONCLUSION: We describe a rare case of SHAM syndrome in a pediatric patient who required bilateral revascularizations and discuss strategies for managing the perioperative risk of hemorrhage and stroke. We also review existing literature on SHAM syndrome.

Clinical predictors of survival for patients with atypical teratoid/rhabdoid tumors.

PURPOSE: Atypical teratoid/rhabdoid tumors (AT/RTs) are malignant central nervous system (CNS) neoplasms of the young. Our study analyzed a large AT/RT cohort from the National Cancer Database (NCDB) to elucidate predictors of short-term mortality and overall survival (OS). METHODS: Information was collected on patients with histologically confirmed AT/RT using the NCDB (2004-2016). Kaplan-Meier analysis indicated OS. Prognostic factors for 30-day mortality, 90-day mortality, and OS were determined via multivariate Cox proportional hazards (CPH) and logistic regression models. RESULTS: Our cohort of 189 patients had a median age of 1 year (IQR [1, 4]) and tumor size of 4.7 ± 2.0 cm at diagnosis. Seventy-two percent were under 3 years old; 55.6% were male and 71.0% were Caucasian. Fifty (27.2%) patients received only surgery (S) (OS = 5.91 months), 51 (27.7%) received surgery and chemotherapy (S + CT) (OS = 11.2 months), and 9 (4.89%) received surgery and radiotherapy (S + RT) (OS = 10.3 months). Forty-five (24.5%) received S + CT + RT combination therapy (OS = 45.4 months), 13 (17.1%) received S + CT + BMT/SCT (bone marrow or stem cell transplant) (OS = 55.5 months), and 16 (8.70%) received S + CT + RT + BMT/SCT (OS = 68.4 months). Bivariate analysis of dichotomized age (HR = 0.550, 95% CI [0.357, 0.847], p = 0.0067) demonstrated significantly increased patient survival if diagnosed at or above 1 year old. On multivariate analysis, administration of S + CT + RT, S + CT + BMT/SCT, or S + CT + RT + BMT/SCT combination therapy predicted significantly (p < 0.05) increased OS compared to surgery alone. CONCLUSION: AT/RTs are CNS tumors where those diagnosed under 1 year old have a significantly worse prognosis. Our study demonstrates that while traditional CT, RT, and BMT/SCT combination regimens prolong life, overall survival in this population is still low.

Caregiver Preferences for Three-Dimensional Printed or Augmented Reality Craniosynostosis Skull Models: A Cross-Sectional Survey.

BACKGROUND: Recent advances in three-dimensional (3D) printing and augmented reality (AR) have expanded anatomical modeling possibilities for caregiver craniosynostosis education. The purpose of this study is to characterize caregiver preferences regarding these visual models and determine the impact of these models on caregiver understanding of craniosynostosis. METHODS: The authors constructed 3D-printed and AR craniosynostosis models, which were randomly presented in a cross-sectional survey. Caregivers rated each model's utility in learning about craniosynostosis, learning about skull anatomy, viewing an abnormal head shape, easing anxiety, and increasing trust in the surgeon in comparison to a two-dimensional (2D) diagram. Furthermore, caregivers were asked to identify the fused suture on each model and indicate their preference for generic versus patient-specific models. RESULTS: A total of 412 craniosynostosis caregivers completed the survey (mean age 33 years, 56% Caucasian, 51% male). Caregivers preferred interactive, patient-specific 3D-printed or AR models over 2D diagrams (mean score difference 3D-printed to 2D: 0.16, P < 0.05; mean score difference AR to 2D: 0.17, P < 0.01) for learning about craniosynostosis, with no significant difference in preference between 3D-printed and AR models. Caregiver detection accuracy of the fused suture on the sagittal model was 19% higher with the 3D-printed model than with the AR model (P < 0.05) and 17% higher with the 3D-printed model than with the 2D diagram (P < 0.05). CONCLUSIONS: Our findings indicate that craniosynostosis caregivers prefer 3D-printed or AR models over 2D diagrams in learning about craniosynostosis. Future craniosynostosis skull models with increased user interactivity and patient-specific components can better suit caregiver preferences.

Posterior ischemic optic neuropathy following supine craniotomy for epidural abscess in a child.

Post-operative vision loss (POVL) can be a devastating complication of neurosurgical procedures and is unusual in the pediatric population. Mechanisms of POVL include direct optic nerve injury, vascular occlusion, or indirect malperfusion resultant from surgeries with substantial blood loss or fluid shifts, with prone positioning being a major risk factor for these events. Posterior ischemic optic neuropathy (PION) is a rare cause of POVL and is associated with a poor prognosis for recovery of visual function. We present a case of PION following a supine bifrontal craniotomy for a frontal epidural abscess secondary to pan-sinusitis in a pediatric patient. This is an unusual reported case in that no additional traditional risk factors were identified. We present clinical and radiographic findings, diagnostic considerations, treatment strategies, and a literature review. The patient was managed medically and recovered substantial vision in the affected eye.

Meet in the middle: a technique for resecting nasocranial dermoids-technical note and review of the literature.

INTRODUCTION: Nasal dermoids with intracranial extension are benign lesions that can have life-threatening consequences, as a nasal punctum may serve as a portal of entry for infection into the central nervous system. The surgical management of these lesions can be challenging, as the extracranial and intracranial cysts and sinus tract must be resected to prevent recurrence, and the surgery must be carried out with an acceptable esthetic result. TECHNIQUE: The authors present a technique for the resection of nasocranial dermoids that eliminates the need to remove and replace the frontonasal segment. Working through a small frontal craniotomy enables the surgeon to drill out the sinus tract through the foramen cecum and inferiorly into the nose. This exposure eliminates the need to enter the frontal sinus, if it is present. CONCLUSION: The extracranial punctum and tract are resected from below in a minimally invasive fashion that permits removal of the lesion without a disfiguring scar.

Treatment-refractory Escherichia coli subdural empyema caused by infection of a chronic subdural hematoma in an infant.

INTRODUCTION: Subdural empyema (SDE) is a neurosurgical emergency that is typically treated with surgical drainage, either by burr hole or by craniotomy. Escherichia coli is an uncommon cause of SDE and is associated with infection of a pre-existing subdural hematoma. CASE REPORT: We report the case of an otherwise healthy, immunocompetent 4-month-old infant girl with an E. coli-infected subdural hematoma. The infection persisted despite aggressive neurosurgical treatment that included drainage of the subdural space through burr holes and, subsequently, a wide craniotomy was performed. Ultimately, after 30 days, the SDE resolved with good neurological outcome. A review of prior literature indicates that infected subdural hematomas (including those caused by E. coli) are typically less aggressive and respond to burr hole drainage. CONCLUSION: We illustrate the fulminant progression of the SDE in the face of neurosurgical treatment. Our experience suggests lowering the threshold for wide craniotomy in these incompletely understood cases.

Iliopsoas hematoma in a patient with sickle cell disease.

Although musculoskeletal pain in patients with sickle cell disease (SCD) is most frequently the result of vaso-occlusive episodes, clinicians often consider other etiologies including osteomyelitis, avascular necrosis, and trauma. In this study, we report the case of a young female with SCD with hip and back pain secondary to a nontraumatic iliopsoas periosteal hematoma with evidence of adjacent bone infarction. The pathophysiology, diagnostic considerations, and management of periosteal hematomas in SCD are reviewed. This case highlights the need for recognition of unusual causes of musculoskeletal pain in SCD.