Pediatric Neurosurgery

INTRODUCTION: Human Herpes Virus-6 (HHV-6) is a ubiquitous virus but can lead to deleterious clinical manifestations due to its predilection for the pediatric central nervous system. Despite significant literature describing its common clinical course, it is rarely considered as a causative agent in CSF pleocytosis in the setting of craniotomy and external ventricular drainage device. Identification of a primary HHV-6 infection allowed for timely treatment with an antiviral agent along with earlier discontinuation of antibiotic regimen and expedited placement of a ventriculoperitoneal shunt...

BACKGROUND: Incidental intradural tumors of the spine in the pediatric population are rare lesions whose management remains unclear. Surgeons must balance the risks of iatrogenic deficits and complications after surgical resection against the risks from progressive growth of the tumor. Moreover, the natural history of an incidental finding can be difficult to predict. Here we review the literature on incidental intradural tumors of the spine and present considerations for their management...

INTRODUCTION: Encephaloduroarteriosynangiosis (EDAS) for moyamoya is predominantly performed using a branch of the superficial temporal artery (STA) as the donor artery. At times, other branches of the external carotid artery (ECA) are better suited for EDAS than is the STA. There is little information in the literature concerning using the posterior auricular artery (PAA) for EDAS in the pediatric age group. In this case series, we review our experience using the PAA for EDAS in children and adolescents...

Most people who are infected with the novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) are asymptomatic or present with mild upper respiratory symptoms. This is especially true in the pediatric population; however, rarely, a massive cytokine storm can develop causing multisystem inflammatory syndrome associated with COVID (MIS-C). Furthermore, children may also suffer from acute ischemic strokes secondary to SARS-CoV-2 infection. Here, we present a 2-year-old male who was admitted to the hospital with MIS-C and evidence of a previous SARS-CoV-2 infection...

INTRODUCTION: Resection of brain lesions associated with refractory epilepsy to achieve seizure control is well-accepted. However, concurrent behavioral effects of these lesions such as changes in mood, personality, and cognition and the effects of surgery on behavior have not been well characterized. We describe 5 such children with epileptogenic lesions and significant behavioral abnormalities which improved after surgery. CASE DESCRIPTIONS: Five children (ages 3-14 years) with major behavioral abnormalities and lesional epilepsy were identified and treated at our center...

INTRODUCTION: Brain arteriovenous malformations (AVMs) are increasingly being treated with Onyx liquid embolic agent (Onyx, Medtronic, Inc.). The phenomenon of delayed Onyx migration is not well documented in the literature. Moreover, the clinical presentation associated with Onyx migration is not well understood. CASE PRESENTATION: A pediatric patient with a history of neonatal seizures was referred to our institution upon experiencing daily headaches with photophobia, phonophobia, and sleep disturbance...

Introduction Pediatric Hydrocephalus is a common disease in sub-Saharan Africa. In Mali, 350-400 new cases are diagnosed in our center yearly. With a total land mass of 1.241.000 km2, patients in remote areas must travel up to 1500km to access neurosurgical care. Hence, treatment and follow-ups of "shunted" patients are difficult. In this context, endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) provides an opportunity for an affordable and less-constraining treatment for hydrocephalus children under 12 months of age...

BACKGROUND: Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors. SUMMARY: Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet is also associated with non-vestibular schwannoma, meningioma, and ependymoma...

The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension and hydrocephalus is widely described in the literature, especially in children with pediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association and several theories have been proposed. However, whatever the pathophysiological mechanism, it is still unclear what is the best management and treatment of CM and hydrocephalus in multisutural craniosynostosis patients...

PURPOSE: Peri-insular hemispherotomy (PIH) is a hemispheric separation technique under the broader hemispherotomy group, a surgical treatment for patients with intractable epilepsy. Hemispherotomy techniques such as the PIH, vertical parasagittal hemispherotomy (VPH), and modified-lateral hemispherotomy are commonly assessed together, despite significant differences in anatomical approach and patient selection. We aim to describe patient selection, outcomes, and complications of PIH in its own right...

Diffuse intrinsic pontine gliomas (DIPG) are high grade glioma (HGG) that occur primarily in children, and represent a leading cause of death in pediatric patients with brain tumors with a median overall survival of only 8-11 months. While these lesions were previously thought to behave similarly to adult HGG, emerging data have demonstrated that DIPG are a biologically distinct entity from adult HGG frequently driven by mutations in the histone genes H3.3 and H3.1 not found in adult glioma. While biopsy of DIPG was historically felt to confer unacceptable risk of morbidity and mortality, multiple studies have demonstrated that stereotactic biopsy of DIPG is safe, allowing not only for improved understanding of DIPG but also forming the basis for protocols for personalized medicine in DIPG...

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BACKGROUND: Periodic updates to the World Health Organization (WHO) classification system for central nervous system (CNS) tumors reflect advances in the pathological diagnosis, categorization, and molecular underpinnings of primary brain, spinal cord, and peripheral nerve tumors. The 5th edition of the WHO Classification of CNS Tumors was published in 2021. This review discusses the guiding principles of the revision, introduces the more common new diagnostic entities, and describes tumor classification and nomenclature changes that are relevant for pediatric neurological surgeons...

INTRODUCTION: Chordoma is a rare, aggressive tumor that is believed to originate from notochord remnants. It can occur anywhere from the clivus to the sacrum and often recurs even after resection and radiotherapy. We present a unique case that initially suggested a different pathology based on imaging and presentation but was found to be a chordoma on gross and pathological analysis. CASE PRESENTATION: An 11-year-old girl presented outpatient for scoliosis evaluation and was found to have what appeared to be a right L4 peripheral nerve sheath tumor on MRI, causing dextroconvex scoliosis...

Background Brain tumors are the most common solid tumors and the leading cause of cancer-related deaths in children. Incidence in the US has been on the rise for the last 2 decades. While therapeutic advances in diagnosis and treatment have improved survival and quality of life in many children, prognosis remains poor and current treatments have significant long-term sequelae. Summary There is a substantial need for the development of new therapeutic approaches, and since the introduction of immunotherapy by immune checkpoint inhibitors there has been an exponential increase in clinical trials to adopt these and other immunotherapy approaches in children with brain tumors...

INTRODUCTION: Craniosynostosis is a common pediatric presentation in which the premature fusion of one or more cranial sutures results in a misshapen skull. This birth defect is often associated with comorbidities due to structural impacts on nearby anatomical features. While there is some evidence for a male-predominance among craniosynostosis patients, little has been investigated regarding sex differences in comorbidities of this condition. This study seeks to explore potential sexual dimorphisms in craniosynostosis patients at the time of presentation...

INTRODUCTION: Optimal material and timing of cranioplasty in the pediatric population continues to be debated. Autologous and alloplastic materials have various indications for use and risk factors for complications. METHODS: A single-center retrospective cohort study was undertaken of all pediatric patients who underwent cranioplasty with any material from 1991-2021. RESULTS: 149 cranioplasty implants were included. Younger age (6 years old or under), a diagnosis of craniosynostosis as reason for implant, use of autologous bone, and shorter times to cranioplasty were predictive of need for revision surgery...

INTRODUCTION: An extension of the dermoid cyst below the nasal bone has been identified in 10% of patients in a large series of nasal dermoid cysts, but these are generally easily identifiable and connected to the tract. To date, no previous reports have documented a case with intraosseous dermoid cyst which was completely hidden in the nasal bone. CASE PRESENTATION: An 8 year-old previously healthy female was initially found to have a small pit on her nasal dorsum...

INTRODUCTION: Prevalence of intracranial aneurysms in children with Apert syndrome has not been described, and development of an aneurysm as a complication secondary to craniofacial surgery has never been reported. CASE PRESENTATION: We report the rare case of a 10-year-old boy with Apert syndrome who underwent craniofacial reconstruction surgery consisting of subcranial Le Fort III osteotomies, bilateral lateral canthopexies, and nasal nares dilations for midfacial hypoplasia and resultant obstructive sleep apnea and on routine follow-up magnetic resonance imaging (MRI) 1 year later was found to have a large left ophthalmic internal carotid artery (ICA) aneurysm that was not seen on MRI obtained 2 years prior...

INTRODUCTION: The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARM) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord are suspected to vary, with consideration to type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the United States in terms of diagnosis and management of tethered cord and specifically, patients with tethered cord and ARM...