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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article presents the discussion on brain tumors being the most common neoplasms and the leading cause of death from cancer in children. Topics include introducing major changes in brain tumor taxonomy, emphasizing molecular diagnostic features; and risk factors for progression being young age, incomplete resection, fibrillary histologic features, and hypothalamic or chiasmatic location.

Summary: 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. [ABSTRACT FROM AUTHOR]

Background Refinements in optics and instrumentation have enabled surgeons to approach deep-seated intracranial diseases with reduced exposure and brain retraction. The anterior subtemporal keyhole approach is a minimally invasive route to the posterolateral aspect of the suprasellar area and petroclival region. Objective In this cadaveric study, we show the benefits of endoscope-assisted microsurgical techniques in the anterior subtemporal keyhole approach. Methods The anterior subtemporal keyhole approach was performed bilaterally on 5 formalin-fixed human specimens using standard microneurosurgical methods. Dissections compared the exposure afforded by the microsurgical route with the endoscope-assisted technique, using 0° and 30° angled rod-lenses. Anatomic relationships among the surgical target and the surrounding neurovascular structures were described. Results Endoscope-assisted maneuvers enhanced the operative view afforded by the microscope and helped to minimize parenchymal retraction and neurovascular injury. Endoscope-assisted microsurgery provides a panoramic picture of the surgical target and its surroundings. It enables the operator to see into hidden corners beyond the microsurgical view. The endoscope provides enhanced visualization of the neurovascular structures located in the posterolateral suprasellar region, basal cisterns, upper clivus and midclivus, and interpeduncular region. Conclusions Endoscope-assisted microsurgery provides a means to approach selected lesions located in the posterolateral suprasellar region, perimesencephalic cisterns, and upper clivus and midclivus. The operative view can be maximized by introducing the 0° and 30° angled endoscopes. Minimally invasive techniques help to optimize surgical exposure and lessen parenchymal retraction, thus improving control of the surrounding neurovascular structures. [ABSTRACT FROM AUTHOR]

From its inception in ancient Egyptian rituals, neuroendoscopy always promised a minimally invasive route to the cerebrum. Early visionaries, however, hit the proverbial wall of technical development until the 20th century, when new technologies allowed for light to be transmitted across a tube for visualization of intracranial structures. Despite a hiccupping start, with surgical microscopy hampering initial excitement, the development and transformation of neuroendoscopy continued, and today it is a widespread and reliable surgical option for the treatment of numerous varied and complex pathologies. [ABSTRACT FROM AUTHOR]

Transcranial Doppler (TCD) With Transfusions Changing to Hydroxyurea (TWiTCH) trial is a randomized, open-label comparison of hydroxycarbamide (also termed hydroxyurea) versus continued chronic transfusion therapy for primary stroke prevention in patients with sickle cell anaemia (SCA) and abnormal TCD. Severity and location of iron overload is an important secondary outcome measure. We report the baseline findings of abdominal organ iron burden in 121 participants. At enrollment, patients were young (9·8 ± 2·9 years), predominantly female (60:40), and previously treated with transfusions (4·1 ± 2·4 years) and iron chelation (3·1 ± 2·1 years). Liver iron concentration (LIC; 9·0 ± 6·6 mg/g dry weight) and serum ferritin were moderately elevated (2696 ± 1678 μg/l), but transferrin was incompletely saturated (47·2 ± 23·6%). Spleen R2* was 509 ± 399 Hz (splenic iron ~13·9 mg/g) and correlated with LIC (r² = 0·14, P = 0·0008). Pancreas R2* was increased in 38·3% of patients but not to levels associated with endocrine toxicity. Kidney R2* was increased in 80·7% of patients; renal iron correlated with markers of intravascular haemolysis and was elevated in patients with increased urine albumin-creatinine ratios. Extra-hepatic iron deposition is common among children with SCA who receive chronic transfusions, and could potentiate oxidative stress caused by reperfusion injury and decellularized haemoglobin. [ABSTRACT FROM AUTHOR]

Presents a case study of a mass in the forehead. Clinical history of the patient; Radiology; Differential diagnosis.

A 5-year-old Asian male presented with episodes of dystonia involving the right upper extremity during vigorous crying. He was diagnosed with moyamoya disease. Initial laboratory evaluation revealed positive anticardiolipin and antinuclear antibodies. [Copyright &y& Elsevier]

The objective of the present study is to describe the diagnosis and treatment of intracranial complications of frontal sinusitis (Pott’s puffy tumor) in a series of pediatric patients at our institution. A rare entity, Pott’s puffy tumor has been reported in only 21 pediatric cases in the literature of the antibiotic era. The hospital records and radiographic files at Rainbow Babies and Childrens Hospital, Cleveland, Ohio, USA, over the previous 16 years were retrospectively reviewed in a search for patients with the diagnosis of Pott’s puffy tumor, defined as scalp swelling and associated intracranial infection. There were 6 male patients and 1 female patient. Ages ranged from 11 to 18 years (median 14.5 years). Intracranial infections consisted of epidural abscess in 5 patients, subdural empyema in 4 and brain abscess in 1. Intraoperative cultures grew anaerobic organisms in 1 patient, microaerophilic streptococcus in 5 patients, Klebsiella species in 1 patient and Streptococcus pneumoniae in another. All patients presented with frontal scalp swelling, and other common symptoms included headache, fever, nasal drainage and frontal sinus tenderness. Five patients were treated with antibiotics prior to their presentation. Four patients presented with neurologic decompensation characterized by varying degrees of hemiparesis, obtundation, pupillary dilatation or aphasia. All patients underwent craniotomy and evacuation of the intracranial infection. Even severely impaired patients demonstrated full neurologic recovery. Despite the widespread use of antibiotics, neurosurgical complications of sinusitis continue to occur. A high degree of suspicion, along with prompt neurosurgical intervention and the use of appropriate antibiotics, can result in favorable outcomes in even the sickest patients.Copyright © 2001 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune-driven connective tissue disorder that results in fibrosis of the skin and internal organs such as the lung. Fibroblasts are known as the main effector cells involved in the progression of SSc through the induction of extracellular matrix (ECM) proteins and myofibroblast differentiation. Here, we demonstrate that 4'-(cyclopropylmethyl)-N2-4-pyridinyl-[4,5'-bipyrimidine]-2,2'-diamine (PIK-III), known as class III phosphatidylinositol 3-kinase (PIK3C3/VPS34) inhibitor, exerts potent antifibrotic effects in human dermal fibroblasts (HDFs) by attenuating transforming growth factor-beta 1 (TGF-β1)-induced ECM expression, cell contraction and myofibroblast differentiation. Unexpectedly, neither genetic silencing of PIK3C3 nor other PIK3C3 inhibitors (e.g., SAR405 and Autophinib) were able to mimic PIK-III-mediated antifibrotic effect in dermal fibroblasts, suggesting that PIK-III inhibits fibroblast activation through another signaling pathway. We identified that PIK-III effectively inhibits p38 activation in TGF-β1-stimulated dermal fibroblasts. Finally, PIK-III administration significantly attenuated dermal and lung fibrosis in bleomycin-injured mice. [ABSTRACT FROM AUTHOR]

We describe a simple midline approach to posterior fossa lesions in children that involves removal and replacement of a bone flap. This approach offers several advantages over conventional suboccipital craniectomy. Elevation of the bone flap is facilitated by the relatively small size of the midline bony keel in children. This method is simple, safe and expeditious, and restores normal anatomical planes and improved protection to the contents of the posterior fossa. While this approach has been described previously for individual cases, the authors employ craniotomy as the standard method of access to the posterior fossa in pediatric patients, and suggest a straightforward technique for removing and replacing the bone flap. [ABSTRACT FROM AUTHOR]

Estimates the counterbalance effects of ethnic group immigrations to the high prevalence of homozygous beta-thalassemia in younger patients. Increasing mean ages of thalassemia groups; Estimated number of patients with thalassemia in North America; Effectiveness of modern therapies; Increasing incidence of thalassemia in younger adults.

The author comments on an article published within the issue which explored the value of the current practice of measuring the hemoglobin level at 1 year of age to test for iron deficiency or anemia. Topics covered include the prevalence of anemia and low ferritin levels at 12 to13 months of age, the need of further studies to confirm the value of the serum ferritin level as a screening test, and key determinants of the clinical usefulness of a screening test.

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. [ABSTRACT FROM AUTHOR]

An infant sustained a significant depressed skull fracture from a fall. Before the planned surgical repair of the fracture could be carried out, the child sustained a second fall that led to the complete resolution of the depressed fracture. Mechanisms to explain this unusual phenomenon are discussed. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Rhombencephalosynapsis is a rare congenital anomaly, characterized by partial or total agenesis of the cerebellar vermis with midline fusion of the cerebellar hemispheres, dentate nuclei, and the superior cerebellar peduncles, creating the distinctive keyhole appearance of the fourth ventricle. Rhombencephalosynapsis can be isolated or can occur in association with other congenital anomalies and syndromes such as Gómez-López-Hernández syndrome (GLHS) or VACTERL: vertebral anomalies (V), anal atresia (A), cardiovascular defects (C), esophageal atresia and/or tracheoesophageal fistula (TE), and renal (R) and limb/radial (L) anomalies. Recent advances in prenatal imaging have resulted in an increasing rate of prenatal diagnosis of abnormalities of the posterior fossa including rhombencephalosynapsis. Patients with rhombencephalosynapsis may present with motor developmental delay, ataxia, swallowing difficulties, muscular hypotonia, spastic quadriparesis, abnormal eye movements, and a characteristic "figure-of-eight" head shaking. Cognitive outcome varies from severe intellectual disability to normal intellectual function. Rhombencephalosynapsis with VACTERL is often associated with severe cognitive disabilities, whereas patients with GLHS may have better cognitive function. The most common associated findings with rhombencephalosynapsis include hydrocephalus, mesencephalosynapsis, holoprosencephaly, pontocerebellar hypoplasia, corpus callosum dysgenesis, and absence of septum pellucidum. Patients can be categorized into 4 groups: 1) rhombencephalosynapsis associated with GLHS; 2) rhombencephalosynapsis with VACTERL; 3) rhombencephalosynapsis with atypical holoprosencephaly, and 4) isolated rhomboencephalosynapsis. The etiology of rhombencephalosynapsis is unknown. Here, we discuss several hypotheses about its etiology. [ABSTRACT FROM AUTHOR]

Abstract. Hematologic abnormalities in childhood lead poisoning may be due, in part, to the presence of other disorders, such as iron deficiency or thalassemia minor. In order to reassess increased lead burden as a cause of microcytic anemia, we studied 58 children with class III or IV lead poisoning, normal iron stores, and no inherited hemoglobinopathy. Anemia occurred in 12% and microcytosis in 21% of these children. The combination of anemia and microcytosis was found in only one of 58 patients (2%). When only children with class IV lead poisoning were studied, the occurrence of microcytosis increased to 46%. However, the combination of microcytosis and anemia was found in only one of these 13 more severely affected patients. Microcytic anemia was similarly uncommon in children with either blood lead concentration Is greater than or equal to 50 micro g/100 mi or erythrocyte protoporphyrin concentration Is greater than or equal to micro g/100 mi. These data indicate that microcytosis and anemia occur much less commonly than previously reported in childhood lead poisoning uncomplicated by other hematologic disorders. Pediatrics 67: 904-906, 1981; lead poisoning, anemia, microcytosis, erythrocyte protoporphyrin. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Editorial. Comments on the best strategy to prevent childhood iron deficiency. Usefulness of the reticulocyte hemoglobin content (CHr) in identifying children with iron deficiency or iron deficiency anemia; Call for implementation of CHr testing to identify iron deficiency before it begins; Suggestion that public health initiatives, and not merely improved technology, are what is necessary to prevent iron deficiency anemia.

Comments on the treatment of post-hemorrhagic ventricular dilatation resulting from periventricular hemorrhage. Reference to a study in this issue by the International Post-haemorrhagic Ventricular Dilatation Drug Trial Group comparing standard therapy with acetazolamide plus furosemide; Adverse side-effects; Drug therapy of children and regulations for safety and effectiveness; Solution to the problem of brain injury of preterm infants.

States that, in HIV-infected subjects with hemophilia, CD4 counts seem to fall more slowly in those on a high-purity factor VIII (FVIII) than on intermediate-purity product. Evaluation of whether risks for AIDS were associated with either product among 411 HIV-infected individuals.

In the modern era, neuroendoscopy has had an increasingly prominent role in neurosurgery. As attention has focused the development of minimally invasive surgical methods, neuroendoscopy has advanced both as an independent treatment modality for various neurologic disorders and as an adjunct to microneurosurgery. Neuroendoscopy is distinct from traditional surgery, and a thorough understanding of its unique attributes is required to attain maximal benefit. In addition to its advantages, neuroendoscopy is associated with unique obstacles that must be anticipated, appreciated, and accounted for to prevent complications. This article outlines techniques to reduce and manage complications during more common intraventricular neuroendoscopic procedures including endoscopic third ventriculostomy (ETV), colloid cyst resection, tumor biopsy and resection, and treatment of loculated hydrocephalus. [Copyright &y& Elsevier]

The article presents a letter to the editor about research by Olivieri and others concerning iron chelation with oral deferiprone in patients with thalassemia that was published in the August 13, 1998 issue.

Presents photographs of a case of thoracic scoliosis with cothoracic syrinx and herniation of the cerebellar tonsils below the foramen magnum.

This article describes the history of sickle cell disease and promising new treatments. In the 1940s, sickle cell anemia was established as the first molecular disease. From there others discovered the structure of hemoglobin and relationship with amino acid substitution. Stroke remains one of the most devastating complications of this disorder despite transfusion therapy. Leg ulcers, probable painful crises, and fatal pneumonia are still common as progress in the treatment of sickle cell disease has been slow.

The article presents a medical case study. Particular attention is given to a man who was admitted to the hospital because he had had a headache and was vomiting for 2 months. The author reports that the man had papilledema but otherwise normal results on neurological examination. Magnetic resonance imaging of the brain showed noncommunicating hydrocephalus due to occlusion of the anterior 3rd ventricle by a cystic mass. The article reveals that an endoscope directed into an asymmetrically enlarged left lateral ventricle through a single coronal burr hole disclosed a colloid cyst that obliterated the left foramen of Monro and blocked the passage of cerebrospinal fluid into the third ventricle. When the cyst was removed, the patient's headache and papilledema resolved postoperatively.

The article presents the case of a 37-year-old woman, who was admitted to the hospital with complain of headaches for two years. A neurologic examination was remarkable for the absence of retinal venous pulsations. Magnetic resonance imaging of the brain showed noncommunicating hydrocephalus due to stenosis of the caudal aqueduct of Sylvius, with enlargement of the third ventricle and attenuation of its floor. A fiberoptic neodymium:yttrium-aluminum-garnet laser was used to fenestrate the floor of the third ventricle anterior to the mamillary bodies. The endoscope was passed through the third ventriculostomy. The patient's headaches resolved, and the ventricular size decreased on follow-up magnetic resonance imaging.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The 113th Congress of the United States begins in January 2013. With each new Congress, there are many changes, not only in the faces of the newly elected, but also in the membership of committees and the staff serving the members. As agendas for the session are set, there is a resurgence of conflicting priorities. In the past, when these conflicts were resolved, children were rarely at the top of the list. Given the numerous pressing national issues, both domestic and foreign, the same trend will likely occur. [ABSTRACT FROM AUTHOR]

Background: The aim of the present case report is to describe an unusual complication of foramen magnum decompression (FMD) for the Chiari 1 hindbrain malformation and its successful management with non-operative measures. Methods: A 2-year-old girl with the Chiari 1 malformation underwent FMD, including suboccipital craniotomy, C1 laminectomy and durotomy without opening the arachnoid. Results: After initial postoperative improvement, the patient deteriorated, developing subdural hygromas and hydrocephalus. These were treated successfully with observation and acetazolamide. Conclusions: Subdural hygromas may complicate FMD. A slit valve opening in the arachnoid might be part of the pathophysiology. While surgical intervention may be necessary in some circumstances, non-operative measures may be effective as well. Copyright © 2009 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Though a pastime rarely considered rough or dangerous, sledding is not risk-free. The presentation of several serious injuries to Rainbow Babies and Children’s Hospital in Cleveland, Ohio, led us to examine our emergency department and admitting records to determine the incidence of neurological injury among children involved in sledding accidents. We discovered 24 children who had sustained brain or spine injuries as sequelae to sledding mishaps during a 5-year period. The ages ranged from 3 months to 15 years. Injuries included multiple skull fractures, 2 epidural hematomas, 1 subdural hematoma, 1 odontoid fracture and 1 anoxic brain injury. There was 1 death. Twenty-one of 24 patients incurred their injuries by colliding with fixed objects. Both improvised crafts and models intended for sledding were involved in the injuries. Only 3 sleds possessed steering capability. Eight of the 9 children who sustained a serious neurosurgical injury were supervised by an adult. Only 2 children had received instruction before sledding. The lack of awareness of the potential for injury together with the dearth of instruction and lack of control over a vehicle dashing down a hill studded with obstacles can change a carefree pastime into a hazardous activity. Copyright © 2004 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

We describe our experience with the use of a polymeric biodegradable system for the correction of congenital pediatric craniofacial malformations. These fixation methods present several advantages over conventional metallic fixation systems. Our series consists of 51 patients that underwent craniofacial surgery, 46 for craniosysostosis, and 5 for encephalocele. The mean age of the patients was 3 years (median age 9 months). Patients with coronal or metopic craniosynostosis underwent a bifrontal craniotomy and anterior cranial vault and orbital reconstruction. Three patients with late sagittal synostosis underwent cranial vault reconstruction in two stages. Encephalocele defects were repaired with osteotomies, and/or onlay bone graft. Lactosorb (Lorenz Biomet, Warsaw, Ind.) plates (cut from a prefabricated mesh) and screws were employed using established fixation techniques. Cranial bone was the source of all bone graft when required. Pre- and postoperative clinical, radiographic and photographic examinations were performed on all patients. At 2 years follow-up, no evidence of infection, erythema, extrusion, instability of the bony fragments or relapse has been noted. The plates themselves were universally impalpable by the one year follow-up examination. The results in this series support the use of resorbable fixation systems in the correction of congenital craniofacial deformities.Copyright © 2001 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

We have maintained a transgenic mouse model of hydrocephalus created to overproduce the cytokine, transforming growth factor-β1 (TGF-β1) in the central nervous system (CNS). The aim of the present study was to estimate the embryonic period when the transgenic mice would develop hydrocephalus, by investigating the chronological developmental changes of the cerebral ventricles. Qualitative analysis of ventricular size was performed on sections cut in the coronal plane of embryos at the 15th (E15) and 18th (E18) embryonic days, and postnatal mice aged 4 days (P4). The presence of the TGF-β1 transgene was determined by performing polymerase chain reaction (PCR) analysis. We have examined 24 embryos and 14 postnatal mice. By performing PCR analysis, the TGF-β1 transgene was determined to be present in 16 (42.1%). Five of 16 embryos at E15 carried the transgene, and showed a slight enlargement of the lateral ventricles. Three of 8 embryos at E18 carried the transgene, and had remarkable enlargement of the lateral ventricles. Eight of 14 pups at P4 carried the transgene, and 7 of 8 pups with the transgene developed hydrocephalus. In pups that were positive for the transgene, massive enlargement of the lateral ventricles was observed and there was an associated thinning of the overlying cerebral cortex. These results suggest that congenital hydrocephalus may develop at an important embryonic time period, which coincides with the stage of neural stem cell proliferation and differentiation in the CNS.Copyright © 2000 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Neurocytomas are uncommon intracranial neoplasms that have only recently been characterized pathologically. Neurocytomas are histologically benign neoplasms that are often quite large when diagnosed, and previous reports have commonly described presentation with mass effect or cerebrospinal fluid outflow obstruction. We describe the first case of neurocytoma presenting with intraparenchymal cerebral hemorrhage. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) is a disruptive pandemic that has continued to test the limits of health care system capacities. It is important to highlight the specific challenges facing US neurosurgery during these difficult circumstances. In the present study, we have described our neurosurgery department's unique experience during the COVID-19 pandemic. We analyzed the following data points both before and during the first months of the COVID-19 pandemic: the number of patients infected with COVID-19 at our institution, changes in neurosurgical operative workflow, changes in neurosurgical outpatient and inpatient clinic workflows, resident redeployment statistics and changes in call schedules, and changes in neurosurgical education. At our institution, the adult surgery numbers decreased from 120 during the week of March 4–11, 2020 (before the World Health Organization had classified the COVID-19 outbreak as a pandemic) to 17 during the week of April 13–17, 2020. The number of pediatric surgeries decreased from 15 to 3 during the same period. Significantly more surgeries were cancelled than were delayed (P < 0.0001). A drastic decline occurred in the number of in-person neurosurgery clinic visits (97.12%) between March and April 2020 (P = 0.0020). The inpatient census declined from mid-March to mid-April 2020 by 44.68% compared with a 4.26% decline during the same period in 2019 (P < 0.0001). Finally, neurosurgery education has largely shifted toward video-conferencing sessions rather than in-person sessions. By detailing our experience during the COVID-19 pandemic, we hope to have provided a detailed picture of the challenges facing neurosurgery within an academic medical center. [ABSTRACT FROM AUTHOR]

Many medical students find neurosurgery interesting, but few pursue it as a career. Reasons for this mismatch include lack of exposure and poor perceptions of its career demands, work–life balance, personalities, and patient outcomes. It is imperative to recruit promising students early in medical school to build a pipeline of future neurosurgeons. We aimed to recruit medical students to neurosurgery and improve perceptions of the field by hosting an optional cadaver laboratory event (Neuroanatomy Lab Initiative [NLI]) during first-year students' gross anatomy course. Five neurosurgery residents and a rotating faculty member led students through the hands-on performance of a retrosigmoid craniotomy on 4 anatomic specimens in the students' cadaveric laboratory. Questionnaires with 6-point Likert scores were distributed to students before and after the NLI. Thirty-nine students with broad specialty interests and previous experiences attended. They perceived neurosurgery to be demanding, competitive, and incongruent with work–life balance and family. At baseline, their interest in neurosurgery was high despite perceived lack of knowledge about the field. Students were eager to participate in neurosurgical procedures and interactions with neurosurgeons. After the NLI, students felt more knowledgeable about neurosurgery and perceived neurosurgery faculty and residents as more pleasant/friendly, approachable, and satisfied with their careers. An NLI during first-year medical students' anatomy course was an effective, relatively low-resource means of engaging students and improving their perceptions of neurosurgery. We provide a framework for scaling this initiative to other institutions to help recruit the next generation of neurosurgeons. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Surgical resection of craniopharyngiomas may be challenging sometimes because of the size, location and tenacity. Simultaneous endoscopic and microsurgical resection is a novel way to approach such lesions. The aim of this report is to discuss the usefulness of combined endoscopic and microsurgical approaches in treating complex craniopharyngiomas along with a review of the literature. Methods: A child with hydrocephalus and a large suprasellar craniopharyngioma underwent synchronous endoscopic and microsurgical resection of the lesion. Results: Gross total resection of the tumor was achieved. There were no postoperative complications. Hydrocephalus was also treated successfully. Conclusions: Neuroendoscopy can complement microsurgery in approaching complex craniopharyngiomas particularly when these tumors are large and accompanied by hydrocephalus. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]