Development of a New Score Based on Image Defined Risk Factors to Standardize Surgical Risk in Neuroblastoma Resection - A SIOPEN Collaborative Study.

Background And Aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability.

Survey of Latin American ketogenic diet centers: Challenges and success for "La Terapia Cetogénica".

Objective: Despite growing recognition of the efficacy of ketogenic diet therapies (KDT) in reducing seizure frequency and improving the quality of life for individuals with epilepsy, several factors hinder their widespread adoption across Latin America. Specific challenges in the region have been discussed but not formally studied. Currently, no data exist on the availability and operation of KDT teams in the region.

Vagus nerve stimulation in pediatric patients with drug-resistant epilepsy: a step-by-step video.

Vagus nerve stimulation (VNS) is a neuromodulatory treatment involving chronic intermittent electrical stimulation of the left vagus nerve, administered through a programmable pulse generator implanted subcutaneously in the chest. This generator connects to a bipolar lead, with electrodes wrapped around the vagus nerve in the neck. Primarily used as an adjunct therapy for patients with refractory epilepsy who cannot undergo or have not benefitted from resective surgery, VNS is generally well tolerated with few severe side effects.

Surgical Anatomy and Technique of Peri-Insular Hemispherotomy in Pediatric Epilepsy.

Background And Objectives: Hemispherotomy is a highly complex procedure that demands a steep learning curve. An incomplete brain disconnection often results in failure of seizure control. The purpose of this article was to present a step-by-step guide to the surgical anatomy of this procedure.

Pediatric hemispheric cerebellar low-grade gliomas: clinical approach, diagnosis, and management challenges-experience at a tertiary care children's hospital.

Purpose: This study aims to provide an exhaustive analysis of pediatric low-grade gliomas (pLGGs) in the cerebellar hemispheres, focusing on incidence, clinical characteristics, surgical outcomes, and prognosis. It seeks to enhance understanding and management of pLGGs in the pediatric population.

Methods: We conducted an observational, descriptive, retrospective, and cross-sectional study at a pediatric hospital, reviewing medical records of 30 patients with cerebellar hemispheric pLGGs treated from December 2014 to January 2023.

Giant brainstem cavernoma in pediatrics: diagnosis and treatment-case report.

Introduction: Brain cavernomas or cavernous angiomas are a rare vascular malformation in the general population, even more so in pediatric patients. Their incidence in this group is less than 5% of all vascular malformations. They are typically found in the cerebral hemispheres in cortico-subcortical locations and, more rarely, in the brainstem.

Presence of Epstein-Barr virus (EBV) antigens detected by sensitive methods has no influence on local immune environment in diffuse large B cell lymphoma.

EBV+ diffuse large B cell lymphoma (DLBCL) not otherwise specified (NOS) is a new entity confirmed by the World Health Organization (WHO) in 2017. In this new entity, the virus may contribute to a tolerogenic microenvironment. Traces of the virus have been described in DLBCL with more sensitive methods, in cases that were originally diagnosed as negative.

Brivaracetam as add-on therapy in children with developmental epileptic encephalopathies: A study of 42 patients.

Objective: Here we present a multicenter series of patients with developmental epileptic encephalopathies (DEE) who were treated with brivaracetam (BRV) as add-on therapy.

Methods: Medical records of 42 patients with DEE treated with add-on BRV seen at four pediatric neurology centers in Argentina between January 2021 and July 2023 were retrospectively analyzed.

Results: We included 42 patients (26 males, 16 females) with a mean age of 7 years (SD, ± 3.

Safe First-Time use of Stereotactic Headband Fixation in a 5-Month-Old Child: An Economical and Easily Accessible Method.

Objective: We present a low-cost and easily accessible adaptation system to perform stereotactic procedures in infants.

Methods: We used an adaptive device consisting of a headband with a plaster bandage, cotton bandage roll, and gauze bandages. Prior to its clinical application, the device was tested in our neuroscience laboratory using a simulation model of a size similar to that of a 5-month-old infant, during which no complications arose.

Clinical Utility of Definitive Diagnostic Tests for Choledocholithiasis in Pediatric Patients with Mild Gallstone Pancreatitis.

Background: Gallstone pancreatitis was historically considered a risk factor for choledocholithiasis (CD). However, recent studies of adult patients evidenced a weak association between gallstone pancreatitis and CD. The aim of this study was to analyze this association in pediatric patients.

Presence of EBV antigens detected by a sensitive method in pediatric and adult Diffuse Large B-cell lymphomas.

In 2017, the World Health Organization (WHO) confirmed a new entity, Epstein Barr virus (EBV) + Diffuse large B cell lymphoma (DLBCL), not otherwise specified (NOS). Traces of EBV transcripts were described in lymphomas, including DLBCL, that were diagnosed as EBV negative by conventional methods. The aim of this study was to detect viral genome by qPCR, as well as LMP1 and EBNA2 transcripts, with a more sensitive method in DLBCL cases from Argentina.

Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

Purpose: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection.

Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.

Background/purpose: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.

Case report: double dermal sinus tracts of the cervical and thoracic spine associated with congenital intracranial pathology (hydrocephalus and an interhemispheric cyst) in a newborn patient.

Introduction: A dermal sinus tract (DST) is an uncommon type of spinal dysraphisms characterized by a tract lined with stratified squamous epithelium that extends from the subcutaneous tissue to the underlying thecal sac or neural tube. These developmental anomalies can present asymptomatically with cutaneous abnormalities or with devastating complications. Usually, it is presented as a unique lesion, and there are only a few reports that show multiple sinuses, and none of them associated with midline brain malformations.

Surgical treatment of a patient with a giant choroidal fissure cyst: a case report.

Introduction: Choroidal fissure cysts (CFC) are usually an incidental finding on imaging and lead to morbidity only in rare cases. The aim of this study was to present the case of a patient with a giant CFC and its treatment.

Case Report: The patient was a male infant of 9 days of life that presented with symptoms of intracranial hypertension.

Chronic Kidney Disease and Arterial Stiffness: A Two-Way Path.

The kidney-heart relationship has raised interest for the medical population since its vast and complex interaction significantly impacts health. Chronic kidney disease (CKD) generates vascular structure and function changes, with significant hemodynamic effects. The early arterial stiffening in CKD patients is a consequence of the interaction between oxidative stress and chronic vascular inflammation, leading to an accelerated deterioration of left ventricular function and alteration in tissue perfusion.

Leadership development for orthopaedic trauma surgeons in Latin America: opportunities for and barriers to skill acquisition.

Introduction: There is growing interest in leadership courses for physicians. Few opportunities are available in global regions with limited resources. This study describes orthopaedic trauma surgeons' desired leadership skill acquisition, opportunities, and barriers to course participation in Latin America.

Hereditary hypophosphatemic rickets and craniosynostosis.

Background: Craniosynostosis is an underdiagnosed complication associated with hypophosphatemic rickets. The study aims to describe the clinical and auxological characteristic of children with hypophosphatemic rickets and craniosynostosis, describe the usual treatment, and compare the characteristics with those of children without craniosynostosis.

Methods And Patients: An observational and retrospective cohort study was conducted.

Development of a risk score for choledocholithiasis in pediatric patients.

Purpose: The prevalence of gallstones in children has increased over the last years. Choledocholithiasis (CD) is present in up to 30% of the cases. There is a scarcity of studies on the management of choledocholithiasis in children.

Closure of a large lumbosacral myelomeningocele defect with a human pericardial graft: a case report.

Introduction: Myelomeningocele (MMC) is a complex congenital defect resulting from incomplete closure of the neural tube. The aim of this study is to present an unusual technique for the closure of a large defect.

Case Report: Here we report a patient that was prenatally diagnosed with MMC.

Case Report of Novel Genetic Variant in KCNT1 Channel and Pharmacological Treatment With Quinidine. Precision Medicine in Refractory Epilepsy.

In this work we present a female infant patient with epilepsy of infancy with migrating focal seizures (EIMFS). Although many pharmacological schemes were attempted, she developed an encephalopathy with poor response to antiepileptic drugs and progressive cerebral dysfunction. To present the pharmacological response and therapeutic drug monitoring of a paediatric patient with a severe encephalopathy carrying a genetic variant in KCNT1 gene, whose identification led to include quinidine (QND) in the treatment regimen as an antiepileptic drug.

Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia.

Purpose: Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure.

Choroid plexus cyst causing acute hydrocephalus and transtentorial herniation: report of a rare case and its successful neuroendoscopic treatment.

Choroid plexus cysts (CPC) are a frequent incidental neuroimaging finding and completely asymptomatic in the vast majority of cases. We hereby describe a rare case of acute hydrocephalus secondary to a CPC, atypical in size, location and presentation, which required urgent neuroendoscopic management. There are very few reported cases of CPC causing obstructive hydrocephalus.