Pediatric Neurosurgery

Traumatic injuries to the cervical spine or spinal cord are uncommon pathologies in the pediatric population. Unlike adults, children possess unique features such as incomplete ossification of vertebrae, synchondroses, pseudo-subluxation, horizontal alignment of ligaments, and absence of lordosis, which results in greater mobility and flexibility in the pediatric spine. These features are prominent in the cervical spine, which accounts for the most common area of traumatic spinal injuries in children. In this review, we summarize injury patterns, diagnosis, and treatment of traumatic cervical spine injuries in the pediatric population...

INTRODUCTION: Cerebral venous sinus thrombosis (CVST) is a rare but serious condition in both adults and children. Risk factors include thrombophilias, dehydration, and certain inherited conditions like sickle cell trait. We present a case of CVST in a pediatric patient with sickle cell trait to highlight key considerations in diagnosis and management. CASE PRESENTATION: A 14-year-old male with sickle cell trait presented with worsening headache and vomiting after prolonged sun exposure and dehydration during athletic camp...

OBJECTIVE: Rubinstein-Taybi syndrome (RTS) is a rare genetic condition with a distinctive set of physical features. This case series reports a single institutional experience of RTS cases, highlighting the role of neurosurgery in the comprehensive management of RTS patients. METHODS: A retrospective review of patients with genetically confirmed RTS presenting between 2010-2023 at Children's Hospital of Pittsburgh was performed. Patient demographics, genetic profile, clinical symptoms, radiographic characteristics, and neurosurgical management were recorded for all patients...

Introduction Penttinen premature aging syndrome is caused by mutations in the PDGFRB gene. We describe the case of a 10-year-old girl with a de novo c.1994T>C variant in PDGFRB who developed multiple cranial, intracranial, and spinal manifestations, including macrocephaly, enlarged convexity subarachnoid spaces crossed by numerous vascularized arachnoid trabeculae, hydrocephalus, spinal epidural lipomatosis, a low conus medullaris, calvarial thinning with large anterior fontanelle, and a skull fracture with bilateral epidural hematomas...

INTRODUCTION: In children and adolescents brain and central nervous system (CNS) tumors are the leading types of cancers. Past studies have found differing rates of intracranial cancers among races and identified additional cancer risk factors. This study aims to see if these differences can be substantiated with further investigation of the latest version (2019) of the Kids' Inpatient Database (KID). METHODS: A total of 7,818 pediatric patients <21 years old in KID with ICD-10 codes consisting of malignant neoplasms of the brain, brainstem, and cerebral meninges (C700, C709-C719) were queried...

INTRODUCTION: This cohort study aims to elucidate the caregiver burden of Helmet Therapy (HT) following Endoscopic Strip Craniectomy (ESC) to treat craniosynostosis in an effort to inform clinicians and future caregivers navigating this therapeutic option. METHODS: Fourteen caregivers of children with positional plagiocephaly (6) and craniosynostosis treated by ESC (8) undergoing HT at a single center were recruited via convenience sampling. Using a phenomenological qualitative approach, semi-structured interviews were conducted to understand the experience of HT for caregivers...

INTRODUCTION: Pediatric cavum cysts are a rare yet complicated pathology to manage. The literature is scarce, primarily consisting of case series, and lacking a consensus regarding clear management. In this scoping review, we aim to compile existing information in the literature regarding the management of pediatric cavum cysts across the last 10 years. We also present our management of 19 patients, the largest case series to date, highlighting knowledge gaps surrounding the management of this salient pathology...

INTRODUCTION: At our institution, revascularization after indirect moyamoya surgery is routinely evaluated using magnetic resonance angiography (MRA) rather than catheter angiography. This study reviews how revascularization can be visualized on axial MRA versus catheter angiography and compares clinical outcomes of surgeries evaluated by routine postoperative MRA versus routine catheter angiography. METHODS: We reviewed the records of all patients treated at our institution who underwent unilateral encephaloduroarteriosynangiosis (EDAS)/pial synangiosis 2004-2021 at 1-21 years of age...

Introduction The association between trigonocephaly and sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature [1,2]. However, the real incidence of this association and its clinical relevance remain unknown. Methods The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS from January 2014 to June 2023...

INTRODUCTION: Injury and subsequent thrombosis of the cerebral venous sinuses may be caused by closed head injuries secondary to a variety of different mechanisms. Skull fractures can lacerate or otherwise disrupt adjacent dural sinuses. The sequelae of such injuries may include thrombosis and either partial or total occlusion of the sinus, ultimately resulting in significant venous congestion. Sagittal sinus injury is associated with a more serious outcome due to the obligatory flow into the sinus, especially posterior to the coronal suture...

INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases...

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm that affects patients, predominantly males aged 40-70 years, with very heterogeneous clinical presentation and prognosis. In 2020, Goyal et al. proposed consensus recommendations for the management of patients with ECD, remarking on the exceptional presentation of the disease in the pediatric population. CASE PRESENTATION: The first patient, a 20-year-old male, underwent cervical laminectomy and partial removal of a cervical spine lesion, initially apparently consistent with cervical schwannomas...

INTRODUCTION: Pitt-Hopkins syndrome (PTHS) is a rare genetic syndrome associated with neurodevelopmental disorders and craniofacial dysmorphisms caused by variations in the TCF4 transition factor. The aim of this article is to report the case of two twin infants diagnosed with PTHS, confirmed by the identification of a heterozygous pathogenic variant in the TCF4 gene through DNA extracted from a buccal swab. CASE PRESENTATION: Both infants presented with craniofacial asymmetry with a metopic crest and cranial deformity...

INTRODUCTION: Open-lip-type schizencephaly is characterized by trans-cerebral clefts filled with cerebrospinal fluid (CSF) between the subarachnoid space at the hemisphere surface and the lateral ventricles. Disorders related to CSF retention, including hydrocephalus and arachnoid cysts, have reportedly been associated with open-lip schizencephaly and have induced intracranial hypertension in some cases. However, detailed neuroimaging and surgical treatment findings have rarely been described...

INTRODUCTION: Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution's experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined. METHODS: We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children's National Hospital (CNH) from 2005 to 2021...

INTRODUCTION: Intracranial arachnoid cysts (IAC) in children are a common incidental finding on imaging. Most IACs are asymptomatic and can be monitored; however, a small percentage may enlarge and require surgical intervention. This study aimed to identify clinical risk factors in patients with IAC who underwent surgery versus those who did not. METHODS: We conducted a retrospective chart review from 2009 to 2021 at a free-standing children's hospital. A total of 230 patients diagnosed with an IAC aged 0-21 years of age were included in the study...

BACKGROUND: Middle meningeal artery (MMA) embolization has been increasingly applied in adult populations for the treatment of chronic subdural hematomas (cSDH). There is a paucity of literature on the indications, safety, and outcomes of MMA embolization in the pediatric population. SUMMARY: A systematic literature review on pediatric patients undergoing MMA embolization was performed. We also report the case of successful bilateral MMA embolization for persistent subdural hematomas following resection of a juvenile pilocytic astrocytoma...

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...

INTRODUCTION: Pediatric Rhabdoid Meningioma (RM) are the rarest, but most aggressive subtypes of meningioma, related to a severe prognosis. They account for 1-3% of all intracranial meningiomas. CASE PRESENTATIONS: We report an institutional experience of 3 cases through which we discuss clinical, histological and therapeutic features of this tumour. Two of our patients were female-gendered (3 years old and 1 year and 6 months old), and one male-gendred (16 years old)...

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