Epidemiology, Diagnostic Strategies, and Therapeutic Advances in Diffuse Midline Glioma.

Object: Diffuse midline glioma (DMG) is a highly aggressive and lethal brain tumor predominantly affecting children and young adults. Previously known as diffuse intrinsic pontine glioma (DIPG) or grade IV brain stem glioma, DMG has recently been reclassified as "diffuse midline glioma" according to the WHO CNS5 nomenclature, expanding the DMG demographic. Limited therapeutic options result in a poor prognosis, despite advances in diagnosis and treatment. Radiotherapy has historically been the primary treatment modality to improve patient survival. Methods: This systematic literature review aims to comprehensively compile information on the diagnosis and treatment of DMG from 1 January 2012 to 31 July 2023. The review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement and utilized databases such as PubMed, Cochrane Library, and SciELO. Results: Currently, molecular classification of DMG plays an increasingly vital role in determining prognosis and treatment options. Emerging therapeutic avenues, including immunomodulatory agents, anti-GD2 CAR T-cell and anti-GD2 CAR-NK therapies, techniques to increase blood-brain barrier permeability, isocitrate dehydrogenase inhibitors, oncolytic and peptide vaccines, are being explored based on the tumor's molecular composition. However, more clinical trials are required to establish solid guidelines for toxicity, dosage, and efficacy. Conclusions: The identification of the H3K27 genetic mutation has led to the reclassification of certain midline tumors, expanding the DMG demographic. The field of DMG research continues to evolve, with encouraging findings that underscore the importance of highly specific and tailored therapeutic strategies to achieve therapeutic success.

Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature.

INTRODUCTION AND OBJECTIVE: Arachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment. MATERIAL AND METHODS: The patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed. RESULTS: After analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment. CONCLUSIONS: Although it is necessary to design a prospective study to establish causality, the results of our research and the available literature suggest that there is no causal relationship between the presence of ACs and epilepsy. The study and treatment of these patients should be carried out in a multidisciplinary epilepsy surgery unit, without initially assuming that the AC is the cause of epilepsy.

Usefulness of ipsilateral translaminar C2 screws insertion for cervical fixation in children with a low laminar profile: a technical note.

PURPOSE: The fusion of the upper cervical spine in children is demanding due to its reduced size, its anatomical complexity, or a severe pathology of the cranio-cervical junction. In some pediatric patients with today's more popular C1-C2 or, occipito-C2 techniques, it is impossible, or very risky to perform a short upper cervical fixation. METHODS: We present the utility and good results of ipsilateral laminar C2 screws insertion technique in two pediatric cases with low C2 laminar profile. Case 1: a 5-year-old child with a C1-C2 unstable subluxation, and a condylar assimilation of C1, where we performed an occipito-C2 fixation using an occipital plate and this modified translaminar C2 screw technique. Case 2: a 8-year-old Down syndrome boy who suffered an unstable subluxation of C1-C2 upon whom we performed a C1-C2 screw fixation using the same technique. RESULTS: Both cases have been followed up over a period of 7 and 2 years respectively, with good clinical results. CONCLUSIONS: We describe the technique and the feasibility of the ipsilateral insertion of translaminar C2 screws in children with low laminar profiles, as a good alternative to other techniques when the anatomy of the C2 does not permit its use or make them very risky.