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1.
Childs Nerv Syst ; 2024 Oct 04.
Artículo en Inglés | MEDLINE | ID: mdl-39365301

RESUMEN

PURPOSE: Encephaloceles are neural tube closure defects characterized by herniation of intracranial contents through the skull, with a mortality rate of 33.3%. Approximately 50% of patients who survived have some degree of neuropsychomotor developmental impairment or seizures. This study aimed to analyze the relationship between good neuropsychomotor development (NPMD) in patients undergoing fetal occipital encephalocele correction and the reversal of microcephaly, comparing these outcomes with those observed in patients who underwent postnatal surgery. METHODS: The 22 participants were categorized into two groups: 10 in the fetal group (FG) and 12 in the postnatal group (PNG). During the study, 1 patient was excluded from the FG and 2 patients were excluded from the PNG, totaling 19 patients in the study. All patients were diagnosed, evaluated, and monitored by the same healthcare service between July 2012 and July 2018. All participants were subjected to a careful developmental assessment using the Bayley Scale of Infant Development, Second Edition (BSID-II), up to 2 years and 11 months of age. Additionally, CP measurements were taken during the first year of life to monitor their progress. The relationship between microcephaly reversal and NPMD was studied. RESULTS: The CP adjusted for gestational age showed a tendency toward the reversal of progressive microcephaly after correction of encephaloceles in the FG. We found a statistically significant difference in the median BSID-II score between the PNG and FG. Patients in the FG maintained normal CP development in the first year of life, whereas those in the PNG remained microcephalic. CONCLUSION: The reversal of microcephaly in the FG directly influences good NPMD and can be considered a protective factor.

2.
Childs Nerv Syst ; 40(11): 3703-3708, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39017694

RESUMEN

OBJECTIVE: The objective of this study was to evaluate the prevalence of tethered cord among patients in the postoperative period of open and occult spina bifida. To identify warning signs for its early diagnosis, as well as outcomes after the new surgical approach. METHODS: Retrospective study of patients followed at the Pediatric Neurosurgery Department of the Federal University of São Paulo with spinal dysraphism. Signs and symptoms indicating reoperation were collected, and postoperative results were classified as improved, unchanged, or worsened. RESULTS: 222 medical records of patients diagnosed with spinal dysraphism were evaluated. Symptomatic Tethered Cord Syndrome (STCS) was identified in 30 patients (13.51%), with clinical manifestations related to orthopedic deformities (66.7%), neurological deficits (56.7%), urological dysfunction (50%), and intestinal dysfunction (40%). 20 cases underwent surgery for tethered cord release. The mean age at the time of surgery was 7.7 ± 4.9 years, with 13 female patients (65%). In the postoperative evaluation, improvement in low back pain (90.9%), urological pattern, and urinary tract infection episodes (45.4%) were particularly noteworthy. 3 patients (33.3%) with constipation showed improvement, and one worsened (11.1%). Improvement in ambulation was seen in two cases (16.7%). Low back pain was the first symptom to improve after surgery, with an average time of 1.3 months, followed by changes in the urological pattern at 15.6 months. Improvement in constipation was observed in the first month in 2 cases (66.7%), positive changes in ambulation were observed around 7 months after surgery, and only one case showed improvement in clubfoot correction. CONCLUSIONS: The prevalence of tethered cord recurrence after primary correction surgery for open or occult neural tube closure defects was similar to that found in the literature. The results were encouraging, with good postoperative evolution of patients, especially in the improvement of low back pain and urological symptoms.


Asunto(s)
Defectos del Tubo Neural , Disrafia Espinal , Humanos , Defectos del Tubo Neural/cirugía , Defectos del Tubo Neural/epidemiología , Femenino , Masculino , Niño , Estudios Retrospectivos , Preescolar , Disrafia Espinal/cirugía , Disrafia Espinal/complicaciones , Disrafia Espinal/epidemiología , Resultado del Tratamiento , Adolescente , Estudios de Seguimiento , Recurrencia , Lactante , Procedimientos Neuroquirúrgicos/métodos
3.
Childs Nerv Syst ; 40(4): 1213-1219, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38157046

RESUMEN

PURPOSE: The management of depressed skull fractures in infants can be either conservative or surgical. This study aimed to examine the outcomes of management with a negative-pressure vacuum device on depressed skull fractures in newborns. METHODS: Twenty-eight patients (aged 1-6 days) with simple depressed skull fractures underwent skull elevation using negative-pressure vacuum devices. A protocol for nonsurgical management was adopted for infants with such fractures between 2010 and 2023. All patients were initially evaluated with neurological examination and complementary assessments-hematological and coagulation studies, transfontanel transcranial ultrasound, skull radiography, and computed tomography scanning with three-dimensional reconstruction-according to availability and clinical needs. Gentle (negative) extraction pressure was applied with one of several devices (according to institutional availability) for a maximum duration of 60 s; this was performed as soon as possible after diagnosis, preferably within 72 h. Follow-up data, available in the clinical records, were reported. RESULTS: All patients exhibited satisfactory elevation of the depressed bone without associated injuries, except one patient who presented with an associated cephalohematoma which prevented optimal device coupling to generate sufficient vacuum pressure for correction. Neither neurological deficits nor development of epilepsy was noted; normal neurological assessment and oral alimentation tolerance were confirmed within 24 h post procedure. CONCLUSIONS: According to our data, ping-pong skull fracture elevation using the vacuum method is a safe and satisfactory treatment in the neonatal period. Early treatment allows for quick resolution, and in our opinion is the strategy of choice for depressed skull fractures in newborns.


Asunto(s)
Fractura Craneal Deprimida , Fracturas Craneales , Lactante , Humanos , Recién Nacido , Vacio , Cráneo , Cabeza
4.
Childs Nerv Syst ; 39(12): 3361-3369, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37878057

RESUMEN

PURPOSE: The study aimed to summarize all published cases of intrinsic brainstem epidermoid cysts in a timeline to highlight the specific characteristics and individualize the disease, in addition to discussing the best treatment used. METHODS: The scientific literature on pediatric cases of intrinsic epidermoid cysts of the brainstem was analyzed. We present the case of a 1.5-year-old male with incidental presentation, who was treated with gross total resection. We summarize all previously published cases to individualize the disease. RESULTS: We identified 21 patients, including 10 boys and 11 girls, with a mean age of 4.85 (1-15) years at the time of surgery. The most frequent symptoms were cranial nerve palsy (71.4%), pyramidal tract deficit (57.14%), and headache (52.38%). Among the affected cranial nerves, VII was the most frequently reported in 10 patients. CONCLUSION: Brainstem epidermoid cysts are extremely rare pathologies with relevant age involvement in young children. The treatment objective should be the maximum resection of the lesion through a careful approach and with the appropriate tools for the functional preservation of the patient.


Asunto(s)
Quiste Epidérmico , Masculino , Femenino , Humanos , Niño , Preescolar , Lactante , Quiste Epidérmico/diagnóstico por imagen , Quiste Epidérmico/cirugía , Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/cirugía , Tronco Encefálico/patología , Nervios Craneales
5.
Childs Nerv Syst ; 39(10): 2899-2927, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37606832

RESUMEN

Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal period. To perform fetal neurosurgical procedures, there is a need for specialized centers that have experts in the diagnosis of fetal pathologies and a highly complex obstetrics service with specialized maternal-fetal teams associated with a pediatric neurosurgery center with expertise in the diverse pathologies of the fetus and the central nervous system that offers multidisciplinary follow-up during postnatal life. Services that do not have these characteristics should refer their patients to these centers to obtain better treatment results. It is essential that the fetal neurosurgical procedure be performed by a pediatric neurosurgeon with extensive experience, as he will be responsible for monitoring these patients in the postnatal period and for several years. The objective of this manuscript is to demonstrate the diagnostic and treatment possibilities, in the fetal period, of some neurosurgical diseases such as hydrocephalus, tumors, occipital encephalocele, and myelomeningocele.


Asunto(s)
Hidrocefalia , Meningomielocele , Neurocirugia , Masculino , Embarazo , Femenino , Humanos , Niño , Feto/cirugía , Procedimientos Neuroquirúrgicos/métodos , Hidrocefalia/cirugía , Meningomielocele/cirugía , Meningomielocele/complicaciones
6.
Childs Nerv Syst ; 39(9): 2329-2339, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37202535

RESUMEN

PURPOSE: This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period. MATERIAL AND METHODS: Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types. RESULTS: Germinoma was found in 33.1%, with an overall 60-month survival rate of 88%; the female sex was the only factor of a worse prognosis. Non-germinomatous germ cell tumors were found in 27.1%, with an overall 60-month survival rate of 67.2%; metastasis upon diagnosis, residual tumor, and the absence of radiotherapy were associated with a worse diagnosis. Pineoblastoma was found in 22.5%, with an overall 60-month survival rate of 40.7%; the male sex was the only factor of a worse prognosis; a tendency toward a worse outcome was found in patients < 3 years of age and those with metastasis upon diagnosis. Glioma was identified in 12.5%, with an overall 60-month survival rate of 72.6%; high-grade gliomas were associated with a worse prognosis. Atypical teratoid rhabdoid tumors was found in 3.3%, and all patients died within a 19-month period. CONCLUSION: Pineal region tumors are characterized by the heterogeneity of histological types, which exert an influence on the outcome. Knowledge of the prognostic factors for each histological types is of extreme importance to the determination of guided multidisciplinary treatment.


Asunto(s)
Neoplasias Encefálicas , Glioma , Glándula Pineal , Pinealoma , Niño , Humanos , Masculino , Femenino , Pinealoma/cirugía , Pronóstico , Glándula Pineal/cirugía , Neoplasias Encefálicas/cirugía , Glioma/patología
7.
Childs Nerv Syst ; 39(5): 1115-1122, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-36752912

RESUMEN

PURPOSE: Russel described a rare clinical entity known as diencephalic syndrome (DS) in 1951, which was traditionally caused by a neoplasm in the hypothalamic-optic chiasmatic region. DS is characterized by severe emaciation despite adequate or slightly reduced caloric intake, locomotor hyperactivity, euphoria and other minor features. Current evidence suggests that a rare population of children with a similar phenotype may have their tumor located in the posterior fossa instead, defining the DS-like presentation, a rare entity with few cases reported in the literature. METHODS: A thorough search of three databases (PubMed, Ovid Medline, and Ovid Embase) was conducted to identify relevant papers reporting children with DS associated with brainstem tumors. To our knowledge, only seven cases have been documented in the literature. Moreover, we present four of our own cases, focusing on the unusual clinical presentation, the diagnosis process, and the lag time between the initial symptoms and the definitive diagnosis. RESULTS: In this review, the mean lag time between the onset of symptoms and diagnosis was 20.9 months (median: 16 months; range: 1.5-72 months), whereas in our series of cases, the time was 32.5 months (median: 33 months; range: 7-57 months). CONCLUSION: Despite recent significant advances in neuro-oncology diagnostic tools, this mean lag time did not improve when compared with the previous literature review from 1976. Throughout these data, we aim to raise awareness in the hopes of detecting intracranial neoplasms earlier in cases of children with profound emaciation of unknown cause.


Asunto(s)
Neoplasias del Tronco Encefálico , Enfermedades Hipotalámicas , Enfermedades de la Hipófisis , Humanos , Enfermedades Hipotalámicas/complicaciones , Emaciación/complicaciones , Neoplasias del Tronco Encefálico/complicaciones , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Quiasma Óptico , Síndrome
8.
Childs Nerv Syst ; 39(11): 3131-3136, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-36840750

RESUMEN

PURPOSE: The recent history of myelomeningocele has shown that treatment during the fetal life may reduce the risk of developing hydrocephalus in individuals by approximately 50%. Thus, a significant advancement involves fetal surgery performed through an endoscopic technique in which portals are placed to introduce the forceps and laparoscopic instruments. However, the development of this technique requires training; therefore, this study aimed to develop a training model for fetal myelomeningocele repair technique with multi-portal endoscopy. METHODS: Two stages of endoscopic technique development were performed. The first stage consisted of exercises in order to familiarize the surgeon with 2D-vision endoscopic surgery, associated with the application of exercises focused on surgical skills, such as the development of laparoscopic knots in a synthetic model. The second stage involved the creation and application of the stages of myelomeningocele closure with a non-living animal model consisting of a chicken breast to simulate the myelomeningocele and a basketball to simulate the gravid uterus, in which perforations were made to introduce vascular introducers (portals) that, as in vivo, are used as portals (trocars) for the introduction of laparoscopic instruments. Overall, two different scenarios with three portals and two portals were tested. RESULTS: In three-portal simulator, the triangular apex trocar was used for the introduction of 4-mm 0° or 30° optics or even Minop type neurodoscope (Aesculap®, Germany) that was operated by the assistant surgeon; the other two portals are used for the introduction of laparoscopic instruments. Thus, the surgeon is able to perform maneuvers bimanually since dissection to laparoscopic sutures. In two-portal simulator, the surgeon and assistant stay side by side and one of the portals is used for the optic and the other for the laparoscopic instruments. There is no possibility of bimanual dissection in this method. CONCLUSION: Realistic simulation models for endoscopic fetal surgery for myelomeningocele correction are easily performed and help develop the necessary skills for fetal surgery teams.


Asunto(s)
Hidrocefalia , Laparoscopía , Meningomielocele , Humanos , Embarazo , Femenino , Animales , Meningomielocele/cirugía , Feto/cirugía , Atención Prenatal , Hidrocefalia/cirugía
9.
Childs Nerv Syst ; 39(7): 1881-1887, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-36715744

RESUMEN

PURPOSE: The possibility that ventricular opening generates postoperative complications after surgical tumor treatment often restricts the degree of tumor resection. This study aims to determine whether the ventricular opening is associated with more complications in surgeries for resectioning supratentorial intra-axial brain tumors in the pediatric population. METHODS: A retrospective review analysis was performed of patients treated at IOP/GRAACC between 2002 and 2020 under 19 years of age and underwent surgery for supratentorial intra-axial primary brain tumor resection. Data were collected from 43 patients. RESULTS: Glial tumor was more common than non-glial (65% vs. 35%, p = 0.09). The ventricular opening was not related to neoplastic spreads to the neuroaxis (6% vs. 0, p > 0.9) or leptomeningeal (3% vs. 0, p > 0.9). Of the patients whose ventricle was opened, 10% developed hydrocephalus requiring treatment, while none of the patients in the group without ventricular opening developed hydrocephalus (p = 0.5). There was also no statistical difference regarding ventriculitis. Postoperative subdural hygroma formation correlated with the ventricular opening (43% vs. 0, p = 0.003). The survival at 1, 5, and 10 years of cases with the ventricular opening was 93.2%, 89.7%, and 75.7%, respectively, while in cases without ventricular opening, it was 100%, 83%, and 83%, respectively, respectively, with no statistical difference between the mortality curves. CONCLUSION: Our study demonstrated that ventricular violation was not associated with the occurrence of significant complications. It was related to the formation of subdural hygroma, which did not require additional treatment.


Asunto(s)
Neoplasias Encefálicas , Hidrocefalia , Linfangioma Quístico , Efusión Subdural , Neoplasias Supratentoriales , Humanos , Niño , Efusión Subdural/complicaciones , Linfangioma Quístico/complicaciones , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Neoplasias Supratentoriales/cirugía , Estudios Retrospectivos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Neoplasias Encefálicas/cirugía
10.
Childs Nerv Syst ; 39(6): 1485-1493, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36454311

RESUMEN

PURPOSE: Low-grade gliomas compose 30% of pediatric central nervous system tumors and outcomes of disease-free progression, and survival is directly correlated to the extent of resection. The use of sodium fluorescein (Na-Fl) is an intraoperative method in the localization of tumor cells in adult patients to optimize resection. Our purpose is to describe the use of Na-Fl in pediatric low-grade gliomas and its outcomes. METHODS: Patients under 18 years of age with low-grade gliomas at the author's institution underwent resection with the use of Na-Fl, with review of preoperative imaging findings, intraoperative results, and follow-up. Then, a comprehensive, narrative literature review of the use of Na-Fl in pediatric low-grade glioma was performed. RESULTS: Our single-institution use of Na-Fl in pediatric patients with suspected low-grade glioma demonstrated excellent results of intraoperative enhancement of tumor cells as well as gross total resection. The literature demonstrated 84% Na-Fl staining and 59.2% of gross total resection in pediatric low-grade gliomas with few small case studies, a range of reported findings, and few side effects. CONCLUSION: Na-Fl has a promising use in low-grade glioma resection in the pediatric patient population. Further research is warranted, such as randomized controlled studies, to assess Na-Fl as a potential tool in improving resection and long-term favorable outcomes.


Asunto(s)
Neoplasias Encefálicas , Glioma , Adulto , Humanos , Niño , Adolescente , Fluoresceína , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Glioma/diagnóstico por imagen , Glioma/cirugía , Glioma/patología , Procedimientos Neuroquirúrgicos/métodos
11.
Childs Nerv Syst ; 39(2): 481-489, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36258049

RESUMEN

Schistosomiasis is an endemic parasitic disease in several tropical countries. In Brazil, the only prevalent species of parasite responsible for schistosomiasis is Schistosoma mansoni. Neuroschistosomiasis is the second most frequent form of infection and the primary ectopic manifestation, with predominant involvement of the lower thoracic spinal cord and lumbar and lumbosacral regions. The frequent contact of children with contaminated ponds and the immaturity of their immune systems make this age group especially susceptible to infection by this parasite. Therefore, neuroschistosomiasis mansoni should always be considered in cases of transverse myelitis in children from endemic regions. The treatment for this condition is quite simple and effective, resulting in total recovery of neurological deficits if the diagnosis is made early.


Asunto(s)
Neuroesquistosomiasis , Enfermedades de la Médula Espinal , Animales , Niño , Humanos , Neuroesquistosomiasis/diagnóstico , Neuroesquistosomiasis/parasitología , Neuroesquistosomiasis/patología , Schistosoma mansoni , Enfermedades de la Médula Espinal/diagnóstico , Brasil
12.
Pediatr Blood Cancer ; 69(5): e29637, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35233919

RESUMEN

Optic pathway gliomas (OPG) can cause elevated cerebrospinal fluid (CSF) protein concentrations. We report on two patients with suprasellar low-grade gliomas and high CSF protein levels (590 and 551 mg/dl) that precluded shunt implantation. After two and three doses of bevacizumab, respectively, the levels dropped dramatically to 191 and 178 mg/dl, respectively. Bevacizumab treatment was associated with a decrease in CSF protein level, allowing successful shunt placement. Our results are consistent with the pharmacological mechanism of bevacizumab, which decreases protein leakage from blood vessels to the ventricles.


Asunto(s)
Glioma del Nervio Óptico , Bevacizumab/uso terapéutico , Ventrículos Cerebrales , Ventrículos Cardíacos , Humanos
13.
J Cancer Res Clin Oncol ; 148(1): 107-119, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34626238

RESUMEN

PURPOSE: Gliomas represent the most frequent central nervous system (CNS) tumors in children and adolescents. However, therapeutic strategies for these patients, based on tumor molecular profile, are still limited compared to the wide range of treatment options for the adult population. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in gliomas of childhood and adolescence using the next-generation sequencing (NGS) strategy. METHODS: We selected 95 samples with initial diagnosis of glioma from patients treated at Pediatric Oncology Institute-GRAACC/UNIFESP. All samples were categorized according to the 2021 World Health Organization Classification of Tumors of the CNS, which included 39 low-grade gliomas (LGGs) and 56 high-grade gliomas (HGGs). Four HGG samples were classified as congenital glioblastoma (cGBM). NGS was performed to identify somatic genetic variants in tumor samples using the Oncomine Childhood Cancer Research Assay® (OCCRA®) panel, from Thermo Fisher Scientific®. RESULTS: Genetic variants were identified in 76 of 95 (80%) tumors. In HGGs, the most common molecular alteration detected was H3F3A c.83A > T variant (H3.3 K27M) and co-occurring mutations in ATRX, TP53, PDGFRA, MET, and MYC genes were also frequently observed. One HGG sample was reclassified as supratentorial ependymoma ZFTA-fusion positive after NGS was performed. In LGGs, four KIAA1549-BRAF fusion transcripts were detected and this alteration was the most recurrent genetic event and favorable prognostic factor identified. Additionally, genetic variants in ALK and NTRK genes, which provide potential targets for therapy with Food and Drug Administration-approved drugs, were identified in two different cases of cGBM that were classified as infant-type hemispheric glioma, a newly recognized subgroup of pediatric HGG. CONCLUSION: Molecular profiling by the OCCRA® panel comprehensively addressed the most relevant genetic variants in gliomas of childhood and adolescence, as these tumors have specific patterns of molecular alterations, outcomes, and effectiveness to therapies.


Asunto(s)
Biomarcadores de Tumor/genética , Neoplasias Encefálicas/genética , Variación Genética/genética , Glioma/genética , Adolescente , Neoplasias Encefálicas/patología , Niño , Preescolar , Variaciones en el Número de Copia de ADN/genética , Femenino , Predisposición Genética a la Enfermedad/genética , Glioma/patología , Secuenciación de Nucleótidos de Alto Rendimiento , Histonas/genética , Humanos , Lactante , Recién Nacido , Masculino , Proteínas de la Membrana/genética , Proteínas Proto-Oncogénicas B-raf/genética , Estudios Retrospectivos
14.
J Neurooncol ; 155(1): 13-23, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34570300

RESUMEN

PURPOSE: Ependymoma (EPN) accounts for approximately 10% of all primary central nervous system (CNS) tumors in children and in most cases, chemotherapy is ineffective and treatment remains challenging. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in EPNs of childhood and adolescence, using a next-generation sequencing (NGS) panel specific for pediatric neoplasms. METHODS: We selected 61 samples with initial diagnosis of EPN from patients treated at Pediatric Oncology Institute-GRAACC/UNIFESP. All samples were divided according to the anatomical compartment of the CNS - 42 posterior fossa (PF), 14 supratentorial (ST), and five spinal (SP). NGS was performed to identify somatic genetic variants in tumor samples using the Oncomine Childhood Cancer Research Assay® (OCCRA®) panel, from Thermo Fisher Scientific®. RESULTS: Genetic variants were identified in 24 of 61 (39.3%) tumors and over 90% of all variants were pathogenic or likely pathogenic. The most commonly variants detected were in CIC, ASXL1, and JAK2 genes and have not been reported in EPN yet. MN1-BEND2 fusion, alteration recently described in a new CNS tumor type, was identified in one ST sample that was reclassified as astroblastoma. Additionally, YAP1-MAMLD1 fusion, a rare event associated with good outcome in ST-EPN, was observed in two patients diagnosed under 2 years old. CONCLUSIONS: Molecular profiling by the OCCRA® panel showed novel alterations in pediatric and adolescent EPNs, which highlights the clinical importance in identifying genetic variants for patients' prognosis and therapeutic orientation.


Asunto(s)
Ependimoma , Secuenciación de Nucleótidos de Alto Rendimiento , Adolescente , Neoplasias del Sistema Nervioso Central/genética , Niño , Preescolar , Ependimoma/genética , Humanos , Lactante , Neoplasias Supratentoriales , Factores de Transcripción
15.
Childs Nerv Syst ; 37(11): 3407-3415, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34435215

RESUMEN

PURPOSE: To investigate certain aspects of hydrocephalus in patients with myelomeningocele. METHODS: We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6 h of life, group 2 consisted of patients who underwent surgery within the first 6 h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7 weeks of gestation. RESULTS: There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4 days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus. CONCLUSION: The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.


Asunto(s)
Hidrocefalia , Meningomielocele , Femenino , Humanos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Meningomielocele/complicaciones , Meningomielocele/cirugía , Procedimientos Neuroquirúrgicos , Embarazo , Estudios Retrospectivos , Derivación Ventriculoperitoneal
16.
Childs Nerv Syst ; 37(11): 3429-3436, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34297200

RESUMEN

INTRODUCTION: Spontaneous third ventriculostomy (STV) is characterized by the spontaneous rupture of one of the ventricle walls due to increased pressure in the third ventricle caused by obstructive hydrocephalus. Clinically, STV results in resolution of signs and symptoms of intracranial hypertension and head circumference stabilization. No spontaneous STV cases in patients with myelomeningocele have been reported in the literature. The objective of this study was to report three cases of STV in patients with type 2 Chiari malformation who underwent intrauterine treatment. CASE PRESENTATION: All patients presented clinically with increased head circumference during outpatient follow-up. Only one patient required a ventriculoperitoneal shunt implantation. The other patients did not require further intervention. CONCLUSION: STV is a rare entity that is difficult to diagnose and should always be suspected in spontaneous hydrocephalus resolution, especially in early childhood. STV is not synonymous with hydrocephalus resolution.


Asunto(s)
Hidrocefalia , Meningomielocele , Tercer Ventrículo , Preescolar , Femenino , Humanos , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/etiología , Hidrocefalia/cirugía , Meningomielocele/complicaciones , Meningomielocele/cirugía , Tercer Ventrículo/diagnóstico por imagen , Tercer Ventrículo/cirugía , Resultado del Tratamiento , Derivación Ventriculoperitoneal , Ventriculostomía
17.
Childs Nerv Syst ; 37(9): 2735-2741, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-34169385

RESUMEN

INTRODUCTION: Medullary neuroschistosomiasis is a severe complication of gastrointestinal infection by Schistosoma. There are several endemic areas, wherein the only causative species present is Schistosoma mansoni, which is responsible for the clinical manifestations of all cases in those areas. METHODS: We report the case of a 13-year-old female with lumbar pain and progressive lower limb weakness, with a delayed diagnosis of medullary involvement by the parasite. We also reviewed the literature on the disease. CONCLUSIONS: Although it is related to the less severe forms of schistosomiasis, one should pay attention to the diagnosis of neuroschistosomiasis in cases of transverse myelitis in patients who traveled to endemic areas. The delay in diagnosis and, consequently, the introduction of treatment may result in irreversible neurological sequelae.


Asunto(s)
Mielitis Transversa , Neuroesquistosomiasis , Adolescente , Animales , Progresión de la Enfermedad , Femenino , Humanos , Imagen por Resonancia Magnética , Neuroesquistosomiasis/diagnóstico por imagen , Schistosoma mansoni
18.
World Neurosurg ; 150: 17, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33741543

RESUMEN

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.


Asunto(s)
Quistes Aracnoideos/cirugía , Quistes del Sistema Nervioso Central/cirugía , Discinesias/cirugía , Endoscopía , Terapia por Láser , Tercer Ventrículo/anomalías , Quistes Aracnoideos/etiología , Quistes del Sistema Nervioso Central/complicaciones , Niño , Discinesias/etiología , Endoscopía/instrumentación , Endoscopía/métodos , Humanos , Masculino , Tercer Ventrículo/cirugía , Resultado del Tratamiento
19.
World Neurosurg ; 146: 156, 2021 02.
Artículo en Inglés | MEDLINE | ID: mdl-33130131

RESUMEN

Arachnoid cysts are responsible for 1% of expansive lesions in the central nervous system. Usually, they do not cause neurologic symptoms unless they have expansion or hemorrhage. Intracystic bleeding is caused by trauma or may be spontaneous. There are few cases in the literature of spontaneous hemorrhage of arachnoid cysts. This 2-dimensional video (Video 1) demonstrates the case of a 6-year-old boy who presented with headache and diplopia. At the physical examination, he exhibited right sixth nerve palsy. The complementary examinations revealed a left middle fossa arachnoid cyst classified as Galassi 2. An urgent procedure was planned to fenestrate the cyst. Informed written consent was obtained from the patient's family. Due to the team experience, endoscope-controlled microsurgery was performed. The patient experienced remission of the headache and the diplopia. A CT scan was performed on the first postoperative day and revealed a lamina of acute blood at the subdural space. It was an asymptomatic thin lamina of blood; we opted to watch and follow. One month later, a control MRI revealed a left frontoparietal subdural hematoma. Despite being asymptomatic, the hematoma was determined to have significant mass effect; thus, it was evacuated by a parietal burr-hole. After 1 month, another MRI showed resolution of the frontoparietal hematoma and significant reduction in the arachnoid cyst. There are few cases described of spontaneous rupture of arachnoid cyst; beyond that, we would like to illustrate a step-by-step procedure that is not widely available as a video article.


Asunto(s)
Quistes Aracnoideos/cirugía , Microcirugia/métodos , Neuroendoscopía/métodos , Quistes Aracnoideos/complicaciones , Niño , Hematoma Subdural/etiología , Hematoma Subdural/cirugía , Humanos , Masculino , Rotura Espontánea/complicaciones , Rotura Espontánea/cirugía , Trepanación/métodos
20.
Childs Nerv Syst ; 36(11): 2757-2763, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32239293

RESUMEN

PURPOSE: The aim of this study was to analyze the skull base anatomy of patients who underwent intrauterine or postnatal myelomeningocele repair and to determine its relationship with hydrocephalus. METHODS: This was a retrospective cross-sectional study that analyzed three groups: the postnatal group, 57 patients who underwent myelomeningocele repair up to 48 h after birth; the fetal group, 70 patients who underwent myelomeningocele repair between 19 and 27 weeks of gestation; and a control group (65). We compared the rate of hydrocephalus treatment, the clivus-supraocciput angle (CSA), and the Welcher angle. RESULTS: The mean CSA in the fetal group was 87.6°, and the postnatal group was significantly different at 78.3° (p < 0.0001). The control group (89.1°) was significantly different from the postnatal group but not from the fetal group. The mean Welcher angle was not significantly different between the groups. There was an 8.5% rate of surgical treatment for hydrocephalus in the fetal group, compared with 73.6% in the postnatal group. CONCLUSIONS: The CSA in the fetal group was larger than that in the postnatal group, which may explain the decrease in the prevalence of hydrocephalus in the fetal group.


Asunto(s)
Hidrocefalia , Meningomielocele , Fosa Craneal Posterior , Estudios Transversales , Femenino , Humanos , Hidrocefalia/cirugía , Meningomielocele/cirugía , Estudios Retrospectivos
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