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. Data collection included demographics, clinical presentation, imaging findings, surgical approach, postoperative complications, histopathological diagnosis, hydrocephalus management, and follow-up. Molecular markers and adjuvant therapies were also analyzed. RESULTS: The cohort predominantly presented with cerebellar symptoms, with 60% showing hydrocephalus at diagnosis. MRI with gadolinium was crucial for diagnosis. Surgical focus was on achieving gross total resection (GTR), accomplished in 70% of cases. Postsurgical hydrocephalus was less common, and cerebellar mutism was not reported. While a complete molecular analysis was not performed in all cases, available data suggest significant influence of molecular markers on prognosis and therapeutic options of pLGGs. CONCLUSIONS: This study highlights the unique clinical and molecular characteristics of cerebellar hemispheric pLGGs in children. The lower incidence of postoperative hydrocephalus and absence of cerebellar mutism are notable findings. Emphasizing a multidisciplinary approach, our findings contribute to a deeper understanding of pediatric pLGGs, underscoring the need for personalized treatment strategies and vigilant follow-up.

How I do it: operative nuances of the Encephalo-Duro-Mio-Synangiosis (EDMS) technique for pediatric moyamoya disease and syndrome.

BACKGROUND: Moyamoya disease, a progressive occlusive arteriopathy mainly affecting the supraclinoid internal carotid artery, leads to abnormal "Moyamoya vessels" and ischemic events in children due to decreased cerebral blood flow. Surgery, especially indirect revascularization, is suggested for pediatric Moyamoya cases. METHOD: We present the Encephalo-Duro-Mio-Synangiosis (EDMS) technique, illustrated with figures and videos, based on 14 years' experience performing 71 surgeries by the senior author (SGJ) and the Moyamoya Interdisciplinary Workteam at "Prof. Dr. J. P. Garrahan" Pediatric Hospital. CONCLUSION: EDMS is a simple and effective treatment for Moyamoya disease, enhancing procedure precision and safety, reducing associated risks, complications, and improving clinical outcomes.

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. METHODS: We present the case of a 3-day-old girl with an antenatal diagnosis of hydrocephalus who was diagnosed with double dermal sinus tracts of the cervical and thoracic regions at admission. The patient presented signs of elevated intracranial pressure (ICP), which imposed a challenge in the management of the case. RESULTS: Our patient was successfully treated initially with a lumbar puncture in order to discard a cerebrospinal fluid (CSF) infection. With negative CSF cultures, a ventriculoperitoneal shunt (VPS) was placed. Nine days after the VPS surgery and without signs of infection, the DST was excised in a single procedure, without follow-up complications. CONCLUSION: To our knowledge, this is the first description of a patient with multiple midline neural tube defects (NTDs) associated with congenital intracranial pathology. Although there are no guidelines regarding the best treatment for this complex associated pathology, the patient was treated, without follow-up complications.

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.

INTRODUCTION: The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND: The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION: Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS: The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT: Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION: IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

Abusive head trauma, protocols, and legal concerns in a South American Country.

The abusive head trauma (AHT) is a form of child abuse and is a frequent entity all over de world. It is particularly unique among medical diagnoses because of the legal implications imposed by the diagnosis. Therefore, it has been the subject of much legal controversy over the decades. Knowledge of the clinical signs and imaging findings of abusive head trauma is vitally important for early diagnosis. An oriented anamnesis, as well as a complete physical examination and obtaining adequate images of the central nervous system, play a significant role in confirming the presumptive diagnosis. The interdisciplinary approach (pediatricians, neurosurgeons, neuroradiologists, social workers, and other specialists) is the key in the management of these patients. The purpose of this article is to familiarize the pediatric neurosurgeon with some of the more common medicolegal issues surrounding AHT as well as to discuss legal commitments and ethical obligations of the neurosurgeon in Argentina (South America) based on 2 clinical cases.

Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift. / Tumores del sistema nervioso central. Clasificación de la Organización Mundial de la Salud 2021. Hacia un cambio de paradigma.

The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published. The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.

El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.

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. Herein is presented an educational surgical video providing a detailed, step-by-step technical description of VNS implantation. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID244.

New CNS tumor classification: The importance in pediatric neurosurgical practice.

Background: The management of the central nervous system (CNS) tumors in the pediatric population is crucial in neurosurgical practice. The World Health Organization (WHO) has evolved its classification of CNS tumors from the 19th century to the 5th edition, published in 2021, incorporating molecular advancements. This transition from morphology to molecular characterization is ongoing. Methods: This manuscript analyzes the modifications introduced in the 5th edition of WHO's CNS tumor classification, particularly focusing on pediatric tumor families. The paper integrates clinical, morphological, and molecular information, aiming to guide pediatric neurosurgeons in their daily practice and interdisciplinary discussions. Results: The 5th edition of the WHO classification introduces a hybrid taxonomy that incorporates both molecular and histological components. The terminology shifts from "entity" to "type" and "subtype," aiming to standardize terminology. Tumor grading experiences changes, integrating molecular biomarkers for prognosis. The concept of integrated layered diagnosis is emphasized, where molecular and histological information is combined systematically. Conclusion: The 5th edition of the WHO CNS classification signifies a paradigm shift toward molecular characterization. The incorporation of molecular advances, the layered diagnostic approach, and the inclusion of clinical, morphological, and molecular information aim to provide comprehensive insights into pediatric CNS tumors. This classification offers valuable guidance for pediatric neurosurgeons, aiding in precise diagnosis and treatment planning for these complex neoplasms.

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. It is composed of a 7-stage approach, enhancing access to and improving visualization of deep structures. METHODS: A retrospective analysis of 39 pediatric patients with refractory epilepsy who underwent this technique was conducted. Engel scores were assessed 1 year postsurgery. Cadaveric dissections were performed to illustrate the procedure. RESULTS: Between 2015 and 2022, 39 patients were surgically treated using the peri-insular technique. The technique involved 7 stages: patient positioning, operative approach, opercular resection, transventricular callosotomy, fronto-orbital disconnection, anterior temporal disconnection, and posterior temporal disconnection. Most of the patients (92.30%) were seizure-free (Engel class I) at 1 year postoperative, 5.13% were nearly seizure-free (Engel II), and 2.56% showed significant improvement (Engel III). Complications occurred in 8% of cases, including 1 infection, 2 cases of aseptic meningitis, and 1 non-shunt-requiring acute hydrocephalus. CONCLUSION: The peri-insular hemispherotomy technique offers excellent seizure control with a low complication rate. Our visual documentation of surgical anatomy, complemented by detailed descriptions of surgical nuances, significantly contributes to a comprehensive understanding of this technique.

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. Parents were interviewed on admission and patients were assessed over time to discover potential VPS malfunction by surgery or follow-up. Consent was obtained from all participants. RESULTS: Ninety-one patients were surveyed, and 59.3% showed evidence of a confirmed VPS blockage. Parental sensitivity was 66.7%, with a specificity of 21.6%. An association was found between parents who could correctly identify their child's shunt block and the number of symptoms of shunt failure that the parent could name (OR 2.4, p < 0.05) as well as parents who reported vomiting and headache as symptoms of shunt malfunction (OR 6, p < 0.05). Parents who knew the first and last name of their primary neurosurgeon (OR 3.5, p < 0.05) also had better diagnostic sensitivity. CONCLUSIONS: Parents who are more knowledgeable of their child's disease, as well as parents who have good communication with their neurosurgeon, were found to have better diagnostic sensitivity.

Results of training with a low-cost simulation model for endoscope-assisted scaphocephaly repair.

OBJECTIVE: Endoscope-assisted repair of sagittal craniosynostosis is an effective technique that requires a learning curve. Surgical simulation models can be applied to acquire the necessary skills for this procedure. Several models with a wide range of costs have been described for training in this technique. The aim of this work was to present the results of training with a low-cost simulation model for endoscope-assisted sagittal craniosynostosis repair. METHODS: A simulation model for sagittal craniosynostosis was developed using low-cost materials. The model is easily assembled and allows successive uses. Three neurosurgery residents, 3 fellows, and 2 neurosurgeons performed a 4-session training program in sagittal craniosynostosis repair. The Global Rating Scale (GRS) score, number of errors, and the time required to perform the task were reported by 2 independent evaluators using a checklist. Measurements were compared between the first and last training using the Wilcoxon signed-rank test. All participants completed a questionnaire (5-point Likert scale) regarding the realism of the simulation model. RESULTS: A model was developed to recreate the steps required to perform an endoscope-assisted scaphocephaly repair with the patient in a simulated sphinx position. All participants improved their GRS performance between the first and final training. The median time needed to perform the initial training was 47.5 minutes (interquartile range [IQR] 44.5-48 minutes, interrater difference [IRD] p = 0.77), and for the last training was 40.5 minutes (IQR 35.5-43 minutes, IRD p > 0.99). The median number of errors reported in the initial training was 5.5 (IQR 3-7.75 errors, IRD p = 0.8), and in the last training was 1 (IQR 0.75-2.25 errors, IRD p = 0.35). There was a statistically significant difference regarding the time and number of errors between the initial and final training (p < 0.001). More than 85% of the participants found that the surface anatomy, skull and anterior fontanel, fused sagittal suture, and epidural space of the model were realistic and had appropriate detail required to perform the surgery. All respondents agreed or strongly agreed that the endoscope handling was realistic, and that the steps and skills required to complete the task were representative of those required for the real procedure. CONCLUSIONS: A low-cost sagittal craniosynostosis simulation model was developed, allowing successive uses. The acquisition of skills within the simulation was demonstrated for all participants regarding the GRS score and the number of errors and time needed to perform the task. In addition, the model was found to be realistic in terms of anatomical references and the procedural steps required for this minimally invasive technique.

Cranial dermal sinuses in pediatrics: Presentation, diagnosis, complications, and management. Experience at a tertiary care children's hospital. / Senos dérmicos craneales en pediatría: presentación, diagnóstico, complicaciones y tratamiento. Experiencia de un hospital pediátrico de nivel III.

INTRODUCTION: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications. There have been isolated reports of this condition. OBJECTIVE: A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complications. There have been isolated reports of this condition. POPULATION AND METHODS: Observational, descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. RESULTS: Observational, descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. CONCLUSIONS: In this series, dermal sinuses presented as specific lesions on the scalp. The most common site was the occipital midline, and more than 50% of these extended through the bone. The treatment of choice was complete resection of dermal sinus and associated lesions. An emergency surgery was performed when the dermal sinus was symptomatic or associated with intracranial lesions.

Introducción. El seno dérmico cefálico es un tracto tubular formado por una separación incompleta entre el ectodermo neural y el ectodermo epitelial; puede tener complicaciones infecciosas. Existen reportes aislados de esta patología. OBJETIVO: describir una serie de pacientes con seno dérmico craneal, las formas de presentación, el diagnóstico, el tratamiento y las complicaciones. Población y métodos. Estudio observacional descriptivo de una serie de pacientes pediátricos con seno dérmico cefálico atendidos en un hospital pediátrico de nivel III entre 2014 y 2019. RESULTADOS: se incluyeron 18 pacientes. La clínica de presentación fue, en 12 casos, una lesión puntual en el cuero cabelludo, hipertensión endocraneana en 4 casos, ataxia en 1 caso y lesión puntual con fístula en 1 caso. La mitad presentó síntomas de infección. La localización fue en la línea media sobre el hueso occipital en 13 casos, en la línea media sobre el hueso frontal en 3 casos y en la línea media interparietal en 2 casos. En 5 casos se encontró asociada una imagen extracraneana y, en 11 casos, una complicación intracraneana. Ningún paciente presentó recidiva de la lesión y en todos se realizó un solo procedimiento quirúrgico. CONCLUSIONES: Los senos dérmicos en esta serie se presentaron como lesiones puntuales en cuero cabelludo. La localización más frecuente fue a nivel occipital sobre línea media y, en más de la mitad, atravesaba el hueso. El tratamiento de elección fue la exéresis completa del seno dérmico y las lesiones asociadas. Ante la presencia de senos dérmicos sintomáticos o asociados a lesiones intracraneanas, la cirugía se realizó de urgencia.

Tumores del sistema nervioso central. Clasificación de la Organización Mundial de la Salud 2021. Hacia un cambio de paradigma / Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift

El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.

The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19 th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published.The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.

Factores de riesgo asociados a la persistencia de hidrocefalia posterior a la resección de tumores de fosa posterior / Risk factors associated with hydrocephalus persistence after posterior fossa tumors resection

Introducción. Las neoplasias de fosa posterior son los tumores de sistema nervioso central más frecuentes en la población pediátrica y una causa frecuente de hidrocefalia. El objetivo del presente trabajo es analizar los factores de riesgo asociados a hidrocefalia luego de las cirugías de resección de tumores de fosa posterior en una población pediátrica. Material y métodos. Se realizó un estudio observacional retrospectivo de pacientes pediátricos operados de tumores de fosa posterior en un único hospital. Se analizaron potenciales factores de riesgo pre y post quirúrgicos. Se consideró como variable respuesta la necesidad de derivación definitiva para tratar la hidrocefalia a los 6 meses de la resección tumoral. Resultados. En el análisis univariado se detectaron múltiples factores de riesgo significativos. Sin embargo, solamente 3 se mantuvieron en el modelo multivariado: grado de resección (Subtotal: OR 7.86; Parcial: OR 20.42), infección postoperatoria (OR 17.31) y ausencia de flujo de salida postoperatorio en IV ventrículo (OR 4.29). Éste modelo presentó una buena capacidad predictiva (AUC: 0.80, Sensibilidad 80.5%, Especificidad 76.3%). Conclusión. La realización de tercer ventriculostomía endoscópica preoperatoria no redujo la incidencia de hidrocefalia postoperatoria. El grado de resección tumoral, la presencia de infección postoperatoria y la obstrucción de salida del IV ventrículo fueron los factores de riesgo más importantes para el requerimiento de sistema derivativo definitivo luego de la resección de un tumor de fosa posterior. Ésto podría influir en la toma de decisiones respecto al tratamiento en este grupo de pacientes pediátricos

Background. Posterior fossa tumors are the most frequent central nervous system neoplasms in the pediatric population and a frequent cause of hydrocephalus. The objective of this study is to analyze the risk factors associated with hydrocephalus after posterior fossa tumors resection in a pediatric population. Methods. A retrospective observational study was conducted on pediatric patients who underwent posterior fossa tumor resection in a single hospital. Potential pre- and post-operative risk factors were analyzed. The need for definitive shunt placement to treat hydrocephalus at 6 months after tumor resection was considered as the outcome variable. Results. Univariate analysis identified multiple significant risk factors. However, only 3 factors remained in the multivariate model: extent of resection (subtotal: OR 7.86; partial: OR 20.42), postoperative infection (OR 17.31), and absence of postoperative outflow of the fourth ventricle (OR 4.29). This model showed good predictive capacity (AUC: 0.80, Sensitivity 80.5%, Specificity 76.3%). Conclusion. Preoperative endoscopic third ventriculostomy did not reduce the incidence of postoperative hydrocephalus. The extent of tumor resection, presence of postoperative infection, and obstruction of fourth ventricle outflow were the most important risk factors for the requirement of a definitive shunt system after posterior fossa tumor resection. This could influence treatment decisions in this group of pediatric patients

Senos dérmicos craneales en pediatría: presentación, diagnóstico, complicaciones y tratamiento. Experiencia de un hospital pediátrico de nivel III / Cranial dermal sinuses in pediatrics: Presentation, diagnosis, complications, and management. Experience at a tertiary care children's hospital

Introducción. El seno dérmico cefálico es un tractotubular formado por una separación incompleta entre el ectodermo neural y el ectodermo epitelial;puede tener complicaciones infecciosas. Existen reportes aislados de esta patología. Objetivo: describir una serie de pacientescon seno dérmico craneal, las formas depresentación, el diagnóstico, el tratamiento y las complicaciones. Población y métodos. Estudio observacionaldescriptivo de una serie de pacientes pediátricos con seno dérmico cefálico atendidos en unhospital pediátrico de nivel III entre 2014 y 2019. Resultados. se incluyeron 18 pacientes. La clínicade presentación fue, en 12 casos, una lesión puntual en el cuero cabelludo, hipertensiónendocraneana en 4 casos, ataxia en 1 caso y lesión puntual con fístula en 1 caso. La mitad presentó síntomas de infección. La localización fue en la línea media sobre el hueso occipital en 13 casos, en la línea media sobre el hueso frontal en 3 casos y en la línea media interparietal en 2 casos. En 5 casos se encontró asociada una imagen extracraneana y, en 11 casos, una complicación intracraneana. Ningún paciente presentó recidiva de la lesión y en todos se realizó un solo procedimiento quirúrgico. Conclusiones. Los senos dérmicos en esta serie se presentaron como lesiones puntuales en cuerocabelludo. La localización más frecuente fue a nivel occipital sobre línea media y, en más de la mitad, atravesaba el hueso. El tratamiento de elección fue la exéresis completa del seno dérmicoy las lesiones asociadas. Ante la presencia de senos dérmicos sintomáticos o asociados alesiones intracraneanas, la cirugía se realizó de urgencia.

Introduction. A cranial dermal sinus is a tubular tract resulting from the incomplete separation of the epithelial ectoderm from the neuroectoderm which may lead to infectious complicationsThere have been isolated reports of this condition. Objective. To describe a series of patients with cranial dermal sinus, its presentation, diagnosis, management, and complications. Population and methods. Observational,descriptive study of a series of pediatric patients with cranial dermal sinus treated at a tertiary care children's hospital between 2014 and 2019. Results. A total of 18 patients were included. Theclinical presentation was a specific lesion on the scalp in 12 cases, intracranial hypertension in 4, ataxia in 1, and a specific lesion with fistula tract in 1. Half of patients had symptoms of infection. The lesion was located in the midline of the occipital bone in 13 cases; in the midline of the frontal bone in 3 cases; and in the interparietal midline in 2 cases. The dermal sinus was associated with anextracranial image in 5 cases and an intracranial complication in 11 cases. No patient hadrecurrence and only one surgery was performed in all of them. Conclusions. In this series, dermal sinusespresented as specific lesions on the scalp. The most common site was the occipital midline, and more than 50% of these extended through the bone. The treatment of choice was complete resection of dermal sinus and associated lesions. An emergency surgery was performed when the dermal sinus was symptomatic or associated with intracranial lesions.

Senos dérmicos cefálicos en pediatría: Presentación, diagnóstico, complicaciones y tratamiento / Cephalic dermal sinuses in pediatrics: Presentation, diagnosis, complications and treatment

Introducción: El seno dérmico cefálico es un tracto tubular, producido por una incompleta separación entre el ectodermo neural y el ectodermo epitelial que puede traer aparejado complicaciones infecciosas1.Hasta el momento, en la bibliografía internacional, existen casos reportados aislados de esta patología.Es por esto que realizamos un estudio descriptivo de una cohorte de 18 pacientes con seno dérmico cefálico que fueron operados en nuestra institución, con el objetivo de describir las formas de presentación, diagnóstico, tratamiento y complicaciones de esta patología poco frecuente.Metodología: Se realizó un estudio observacional descriptivo retrospectivo de una cohorte de pacientes pediátricos con seno dérmico cefálico.Resultados: Se analizaron 18 pacientes con seno dérmico cefálico. La clínica de presentación fue en 13 casos una lesión puntual en el cuero cabelludo, hipertensión endocraneana en 4 casos, ataxia en 1 caso y fístula en 1 caso. La mitad presentó síntomas de infección. Las localización fue en línea media sobre el hueso occipital en 13 casos, en línea media sobre el hueso frontal en 3 casos y en línea media interparietal en 2 casos.En 5 casos encontramos asociada una imagen extracraneana y en 11 casos una complicacion intracraneana. Ningún paciente presentó recidiva de la lesión y todos recibieron un solo procedimiento quirúrgico.Conclusiones: Los senos dérmicos pueden presentarse de diversas formas, desde un defecto puntual en un paciente asintomático, a una meningitis fulminante. El estudio de estas lesiones debe contar con una TC de cerebro y RM con DWI, si se sospecha una infección se debe solicitar contraste.El tratamiento es siempre la exéresis completa del seno dérmico y las lesiones asociadas. Ante el diagnóstico de una lesión asintomática se puede programar la cirugía, sin embargo sí encontramos senos dérmicos sintomáticos o asociadas a lesiones intracraneanas la cirugía debe realizarse sin demasiada demora.