Cylinder tumor surgery in pediatric low-grade gliomas.

Background: Periventricular pediatric low-grade gliomas (pLGG) present a surgical challenge due to their deep-seated location, accessibility, and relationship with the subcortical network connections. Minimally invasive parafascicular approaches with tubular brain retractors (port brain surgery) have emerged, in recent years, as an alternative to conventional microsurgical and endoscopic approaches for removal of periventricular tumors.

Objectives: To describe the minimally invasive approach with tubular brain retractors for periventricular pLGG, its technique, applications, safety, and efficacy.

Urological outcome with the use of salvage continent vesicostomy in pediatric reconstructive surgery. A single tertiary center experience.

Purpose: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix.

Methods: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV).

Parental sensitivity and specificity to recognize shunt malfunction in their child: a single-center prospective study.

Objective: The objective of this study was to estimate the diagnostic performance (sensitivity, specificity, positive predictive value, and negative predictive value) for recognizing ventriculoperitoneal shunt (VPS) failure in the parents of patients 0-18 years of age who attended the hospital's emergency room (ER). The second objective was to identify the factors associated with the parents' ability to recognize the shunt blockage (true positives).

Methods: A prospective cohort study was conducted between 2021 and 2022 including all patients 0-18 years of age who had a VPS and attended the hospital's ER with symptoms that could correspond to VPS blockage.

Surgical treatment of segmental spinal dysgenesis: a report of 19 cases.

Study Design: Retrospective review.

Objective: To describe clinical presentation, surgical management, long-term results, and complications in patients with segmental spinal dysgenesis (SSD). In addition, we sought to emphasize early surgery for this complex congenital abnormality.

Pediatric Urodynamic Study Without a Preprocedural Urine Culture, Is It Safe in Clinical Practice?

Objective: To prove that incidence of UTI after a pediatric urodynamic study (UDS) is low, and that patients without urine culture (UC) analysis prior to a UDS will not have a significant increase in the incidence of UTI (post-UDS UTI).

Methods: Prospective cohort study including consecutive pediatric patients undergoing UDS in a single center for 1 year. Patients were divided in 2 groups: (G1) UDS with a previous negative UC and (G2) UDS without a previous UC analysis.

Ketogenic diet use in children with intractable epilepsy secondary to malformations of cortical development: A two- centre experience.

Purpose: To evaluate the efficacy and tolerability of the ketogenic diet (KD) as a treatment for drug-resistant epilepsy secondary to malformations of cortical development.

Methods: A two-centre retrospective analysis of 45 paediatric patients with refractory epilepsy due to malformation of cortical development was carried out. Patients were divided into three groups based on malformation type: abnormal neural proliferation (Group 1); abnormal neural migration (Group 2) and abnormal post-migrational development (Group 3).

Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome.

Objective: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis.

Study Design: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample.

Risk factors for morbidities and mortality in children following pneumonectomy.

Background And Objective: Pneumonectomy (PNE) is a procedure infrequently performed in children. A high morbidity/mortality rate associated with PNE has been described. Few series have been published in the last 15 years.

Epileptic spasms in clusters and associated syndromes other than West syndrome: A study of 48 patients.

Objective: To study the different epilepsy syndromes that included epileptic spasms (ES) in clusters without hypsarrhythmia (WoH).

Methods: Between 2/1990 and 7/2013, we registered 48 patients with the electroclinical diagnostic criteria of ES in clusters WoH.

Results: We recognized two subgroups.

Post-infectious bronchiolitis obliterans and mannose-binding lectin insufficiency in Argentinean children.

Background And Objective: Post-infectious bronchiolitis obliterans (PIBO) is a severe disorder following acute lower pulmonary infection in young children, especially caused by adenovirus. Mannose-binding lectin (MBL) deficiency arising from polymorphisms in the coding and non-coding region on the MBL2 gene has been associated with more frequent and severe respiratory infections. Our aim was to evaluate the influence of MBL variants in the susceptibility and evolution of children with PIBO.

Unilateral Eye Blinking Arising From the Ictal Ipsilateral Occipital Area.

We report on an 18-month-old boy with unilateral left eye blinking as a single ictal manifestation without facial twitching. The clinical onset of this phenomenon was first recorded (as an occasional event) at age 3 months, and it was overlooked. By age 6 months, the child's blinking increased to almost daily occurrence in clusters: during blinking the infant showed intact awareness and occasional jerks in the upper limbs and right leg.

Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES).

Purpose: In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add-on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs).

Neonatal lupus erythematosus: a report of four cases.

Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10% of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities.

A prospective, multicentric scoring system to predict mortality in febrile neutropenic children with cancer.

Background: Many studies have succeeded in identifying a subset of children with febrile neutropenia (FN) who are at lower risk of infectious complications and eventual death. Conversely, to the authors' knowledge, no scoring system has been published to date with which to assess the risk of mortality for the whole group of children with neutropenia and fever.

Methods: Between March 2000 and July 2004, 1520 episodes of FN in 981 children were included in a multicentric prospective study to evaluate a scoring system that was designed to identify high mortality risk at the onset of an FN episode in children with cancer.

Primary cutaneous nocardiosis in immunocompetent children.

Primary cutaneous nocardiosis is an infrequent opportunistic infection that mainly affects immunodepressed hosts. We describe two immunocompetent patients who exhibited two clinical forms of cutaneous nocardiosis, a mycetoma from walking barefoot in contaminated water and in the case of the other patient a lymphocutaneous form on the basis of a stabbing-cutting injury. In both cases the tests performed ruled out any immunodeficiency, particularly chronic granulomatose disease, since nocardiosis is regarded as an infection that acts as a marker for that illness.