Tubular single-port endoscope-assisted surgery for fetal myelomeningocele repair.

Objective: The authors aimed to describe a low-cost and easily reproducible alteration of the Bruner and Tulipan procedure to preserve uterine muscular fibers. They conducted a retrospective cohort study of 10 pregnant women whose fetuses developed lumbosacral myelomeningocele (MM). The MM was repaired through a fetal neurosurgical procedure using a tubular single-port endoscope-assisted technique.

Recurrent tethered cord: outcome and follow-up of 20 de-thetering for symptomatic spina bifida: choort study.

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.

Intracranial non-germinomatous germ cell tumors in children and adolescents: how can the experience from an uppermiddle-income country contribute to the worldwide effort to improve outcomes?

Background: Non-germinomatous germ cell tumors (NGGCT) accounts for one third of intracranial GCT. While the germinoma group have an excellent overall survival, the standard of practice for children with NGGCT is still under evaluation.

Aims: Describe the results of the of the Brazilian consortium protocol.

Treatment of depressed skull fractures with vacuum devices in the neonatal period: A case series.

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.

Outcomes and surgical approaches for pineal region tumors in children: 30 years' experience.

Objective: Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort.

Outcome of Children and Adolescents With Primary Intracranial Germinoma Treated With Chemotherapy and Reduced Dose-Field Irradiation: A Prospective Brazilian Experience.

Purpose: This prospective Brazilian single-arm trial was conducted to determine response to chemotherapy and survival after response-based radiotherapy in children with intracranial germinomas, in the setting of a multi-institutional study in a middle-income country (MIC) with significant disparity of subspecialty care.

Patients And Methods: Since 2013, 58 patients with histologic and/or serum and CSF tumor marker evaluations of primary intracranial germ cell tumors were diagnosed; 43 were germinoma with HCGβ levels ≤200 mIU/mL and five between 100 and 200 mIU/mL. The treatment plan consisted of four cycles of carboplatin and etoposide followed by 18 Gy whole-ventricular field irradiation (WVFI) and primary site(s) boost up to 30 Gy; 24 Gy craniospinal was prescribed for disseminated disease.

Diencephalic syndrome-like presentation of brainstem tumor: a series case-based review.

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.

Reduction in the cerebrospinal fluid protein level after bevacizumab treatment in patients with optic pathway low-grade gliomas.

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.

Gliomas in children and adolescents: investigation of molecular alterations with a potential prognostic and therapeutic impact.

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.

Molecular profiling of pediatric and adolescent ependymomas: identification of genetic variants using a next-generation sequencing panel.

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.

Hydrocephalus in myelomeningocele.

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.

Spontaneous third ventriculostomy in patients undergoing fetal surgery for myelomeningocele correction.

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.

Medullary neuroschistosomiasis in adolescence: case report and literature review.

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.

Endoscopic Laser Fenestration to Treat a Bobble-Head Doll Syndrome Caused by Suprasellar Cyst.

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.