Resection of the quadrangular lobule of the cerebellum to increase exposure of the cerebellomesencephalic fissure: an anatomical study with clinical correlation.

OBJECTIVE: The lateral aspect of the cerebellomesencephalic fissure frequently harbors vascular pathology and is a common surgical corridor used to access the pons tegmentum, as well as the cerebellum and its superior and middle peduncles. The quadrangular lobule of the cerebellum (QLC) represents an obstacle to reach these structures. The authors sought to analyze and compare exposure of the cerebellar interpeduncular region (CIPR) before and after QLC resection and provide a case series to evaluate its clinical applicability. METHODS: Forty-two sides of human brainstems were prepared with Klingler's method and dissected. The exposure area before and after resection of the QLC was measured and statistically studied. A case series of 59 patients who underwent QLC resection for the treatment of CIPR lesions was presented and clinical outcomes were evaluated at 1-year follow-up. RESULTS: The anteroposterior surgical corridor of the CIPR increased by 10.3 mm after resection of the QLC. The mean exposure areas were 42 mm2 before resection of the QLC and 159.6 mm2 after resection. In this series, ataxia, extrapyramidal syndrome, and akinetic mutism were found after surgery. However, all these cases resolved within 1 year of follow-up. Modified Rankin Scale score improved by 1 grade, on average. CONCLUSIONS: QLC resection significantly increased the exposure area, mainly in the anteroposterior axis. This surgical strategy appears to be safe and may help the neurosurgeon when operating on the lateral aspect of the cerebellomesencephalic fissure.

The long-term effect on functional outcome of endoscopic brainwashing for intraventricular hemorrhage compared to external ventricular drainage alone: A retrospective single-center cohort study.

Background: Intraventricular hemorrhage (IVH) is a complex condition with both mechanical and chemical effects, resulting in mortality rates of 50-80%. Recent reports advocate for neuroendoscopic treatment, particularly endoscopic brainwashing (EBW), but long-term functional outcomes remain insufficiently explored. This study aims to outline the step-by-step procedure of EBW as applied in our institution, providing results and comparing them with those of external ventricular drainage (EVD) alone. Methods: We performed a retrospective analysis of adult patients with IVH who underwent EBW and patients submitted to EVD alone at our institution. All medical records were reviewed to describe clinical and radiological characteristics. Results: Although both groups had similar baseline factors, EBW patients exhibited a larger hemoventricle (median Graeb score 25 vs. 23 in EVD, P = 0.03) and a higher prevalence of chronic kidney disease and diabetes. Short-term mortality was lower in EBW (52% and 60% at 1 and 6 months) compared to EVD (80% for both), though not statistically significant (P = 0.06). At one month, 16% of EBW patients achieved a good outcome (Modified Rankin scale < 3) versus none in the EVD group (P = 0.1). In the long term, favorable outcomes were observed in 32% of EBW patients and 11% of EVD patients (P = 0.03), with no significant difference in shunt dependency. Conclusion: Comparing EBW and EVD, patients submitted to the former treatment have the highest modified Graeb scores and, at a long-term follow-up, have better outcomes, demonstrated by the improvement of the patients in the follow-up.

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. Methods: Since 2013, 15 patients with a diagnosis of NGGCT by histopathology and/or serum/cerebrospinal fluid (CSF) tumor markers, ßHCG >200mlU/ml and/or positive alpha-fetoprotein were treated with neoadjuvant chemotherapy with carboplatin, cyclophosphamide and etoposide followed by ventricular radiotherapy (RTV) of 18Gy with boost (32Gy) to the primary site. Metastatic patients underwent craniospinal irradiation (CSI) and "slow responders" to the four initial cycles of CT, to autologous stem cell transplantation (ASCT) followed by CSI. Results: Mean age, 13.1 years. Thirteen males. Primary sites: pineal (n=12), suprasellar (n=2) and bifocal (n=1). Four patients were metastatic at diagnosis. Eight patients had CSF and/or serum alpha-fetoprotein levels > 1,000ng/ml. Tumor responses after chemotherapy demonstrated complete in six cases and partial in seven, with "second-look" surgery being performed in five cases, and two patients presenting viable lesions being referred to ASCT. The main toxicity observed was hematological grades 3/4. Two patients with metastatic disease, one with Down Syndrome and AFP > 1,000ng/ml and the other with choriocarcinoma and pulmonary metastases, developed progressive disease resulting in death, as well as two other patients without evidence of disease, due to endocrinological disorders. Event-free and overall survival at 2 and 5 years were 80% and 72.7%, respectively, with a mean follow-up of 48 months (range, 7-107). Conclusions: Despite the small number of patients, in our series, treatment with six cycles of chemotherapy and RTV with focal boost for localized disease (n=11) and ACST for identified slow responders (n=2) seem to be effective strategies contributing to the overall effort to improve outcomes of this group of patients.

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. 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.

Intrinsic epidermoid cyst of the brainstem in children-review and case report.

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.

Fetal neurosurgery.

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.

Survival analysis and prognostic factors in posterior fossa ependymomas in children and adolescents.

OBJECTIVE: In this study, the authors aimed to analyze the overall survival (OS) and progression-free survival (PFS) of patients younger than 18 years of age who were diagnosed with posterior fossa ependymomas, and to identify prognostic factors such as the degree of resection, tumor topography, and involvement of the lesion in the hindbrain. METHODS: The authors performed a retrospective cohort study of patients younger than 18 years of age, treated beginning in 2000, with a diagnosis of posterior fossa ependymoma. Ependymomas were separated into three groups: tumors restricted to the fourth ventricle, tumors inside the fourth ventricle and exiting from the foramen of Luschka, and tumors inside the fourth ventricle and completely surrounding the hindbrain. Furthermore, the tumors were classified by molecular group using the staining method for H3K27me3. Statistical analysis was performed using Kaplan-Meier survival curves, with p < 0.05 considered statistically significant. RESULTS: Of 1693 patients who underwent surgical treatment between January 2000 and May 2021, 55 patients who met the inclusion criteria were included. The median age at diagnosis was 2.98 years. The median OS was 44 months, and the survival rates at 1, 5, and 10 years were 92.5%, 49.1%, and 38.3%, respectively. The cases were assigned to two posterior fossa ependymoma molecular groups: 35 (63.6%) cases to group A and 8 (14.5%) to group B. The median ages in groups A and B were 2.94 and 2.85 years and the median OS values were 44 and 38 months, respectively (p = 0.9245). Statistical analysis was performed on multiple variables, including age, sex, histological grade, Ki-67 expression, tumor volume, extent of resection, and adjuvant therapies. The median PFS of patients with dorsal-only involvement was 28 months; for dorsolateral involvement, it was 15 months; and for total involvement, it was 9.5 months (p = 0.0464). No statistically significant difference was found for OS. There was a statistically significant difference between the proportion of patients in whom gross-total resection was achieved in the dorsal-only involvement group (73.1%, 19/26) and those in the total involvement group (0%, 0/6) (p = 0.0019). CONCLUSIONS: This study confirmed that the extent of resection has an impact on OS and PFS. The authors found that adjuvant radiotherapy resulted in a higher OS but did not prevent progression, that the pattern of involvement of the brainstem in the tumor at diagnosis could elicit important information regarding the patient's prognosis regarding PFS, and that the total involvement of the rhombencephalon impaired the gross-total resection of these tumors.

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. METHODS: A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy. RESULTS: The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas. CONCLUSIONS: The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.

Time course of hemispheric cerebral volume after decompressive craniectomy in malignant middle cerebral artery stroke.

BACKGROUND: Brain edema is the leading cause of death in patients with malignant middle cerebral artery (MCA) infarction. Midline shift (MLS) has been used as a monohemispheric brain edema marker in several studies; however, it does not precisely measure brain edema. It is now possible to directly measure hemisphere brain volume. Knowledge about the time course of brain edema after malignant middle cerebral artery infarction may contribute to the condition's management. OBJECTIVE: Therefore, our goal was to evaluate the course of brain edema in patients with malignant MCA infarction treated with decompressive craniectomy (DC) using hemispheric volumetric measurements. METHODS: Patients were selected consecutively from a single tertiary hospital between 2013 and 2019. All patients were diagnosed with malignant middle cerebral artery infarction and underwent a decompressive craniectomy (DC) to treat the ischemic event. All computed tomography (CT) exams performed during the clinical care of these patients were analyzed, and the whole ischemic hemisphere volume was calculated for each CT scan. RESULTS: We analyzed 43 patients (197 CT exams). Patients' mean age at DC was 51.72 [range: 42-68] years. The mean time between the ischemic ictus and DC was 41.88 (range: 6-77) hours. The mean time between the ischemic event and the peak of hemisphere volume was 168.84 (95% confidence interval [142.08, 195.59]) hours. CONCLUSION: In conclusion, the peak of cerebral edema in malignant MCA infarction after DC occurred on the 7th day (168.84 h) after stroke symptoms onset. Further studies evaluating therapies for brain edema even after DC should be investigated.

ANTECEDENTES: O edema cerebral é a principal causa de morte em pacientes com infarto maligno de artéria cerebral média. O desvio da linha média tem sido utilizado como marcador de edema cerebral mono-hemisférico em alguns estudos; porém, ele não mede de forma precisa o edema cerebral. Atualmente é possível mensurar diretamente o volume do hemisfério cerebral. O conhecimento sobre a evolução temporal do edema cerebral após infartos malignos da artéria cerebral média pode contribuir para o cuidado clínico desta condição. OBJETIVO: Nosso objetivo é avaliar o edema hemisférico ao longo do tempo, em pacientes com infarto maligno da artéria cerebral média, tratados com craniectomia descompressiva. MéTODOS: Os pacientes foram selecionados de forma consecutiva, em um hospital terciário, entre 2013 e 2019. Todos os pacientes apresentavam diagnóstico de infarto maligno de artéria cerebral média e foram submetidos a craniectomia descompressiva. Todas as tomografias computadorizadas de crânio destes pacientes foram analizadas, e o volume do hemisfério cerebral infartado foi mensurado. RESULTADOS: Analisamos 43 pacientes (197 tomografias de crânio). A idade média dos pacientes na craniectomia descompressiva foi 51,72 (42­68) anos. O tempo médio entre o ictus e a craniectomia descompressiva foi 41,88 (6­77) horas. O tempo médio entre o ictus e o pico do volume hemisférico foi 168,84 (142,08­195,59) horas. CONCLUSãO: O pico do volume cerebral em pacientes com infarto maligno de artéria cerebral média submetidos a craniectomia descompressiva ocorreu no 7o dia (168,84 horas) após o infarto. Mais estudos avaliando terapêuticas direcionadas ao edema cerebral seriam úteis neste contexto.

Prognostic factors of pediatric pineal region tumors at a single institution.

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.

Endoscopic Approach for Resection of Frontal Forehead Osteoma: Technical Case Report Instruction.

Forehead osteomas are benign bone tumors. They are frequently associated with exophytic growth in the outer table of the skull, causing cosmetic disfigurement of the face.1-9 The objective of this study was to present the efficacy and feasibility of the endoscopic treatment of forehead osteomas by presenting a case report with details of the surgical technique. A 40-year-old female patient presented with aesthetic complains of a progressing bulge in the forehead. A computed tomography scan with 3-dimensional reconstruction showed bone lesions on the right side of the forehead. The patient underwent surgery under general anesthesia with no noticeable incision, which was planned 2 cm behind the hairline in the midline because the osteoma was close to the midline plane on the forehead (Video 1). A retractor coupled with a 4-mm channel for endoscopy and a 30-degree optic was used to dissect, elevate the pericranium, and locate the 2 bone lesions in the forehead. The lesions were removed using a chisel, endoscopic facelifting raspatory, and a 3-mm burr drill. The tumors were resected completely, resulting in good cosmetic outcomes. The endoscopic approach for treating forehead osteomas is less invasive and facilitates complete removal of tumors, which results in good cosmetic outcomes. Neurosurgeons should consider and add this feasible approach to enhance their surgical armamentarium.

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 HCGß 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. RESULTS: Mean age 13.2 years (range, 4.7-25.5 years); 29 were males. Diagnosis was made by tumor markers (n = 6), surgery (n = 25), or both (n = 10). Two bifocal cases with negative tumor markers were treated as germinoma. Primary tumor location was pineal (n = 18), suprasellar (n = 14), bifocal (n = 10), and basal ganglia/thalamus (n = 1). Fourteen had ventricular/spinal spread documented by imaging studies. Second-look surgery occurred in three patients after chemotherapy. Thirty-five patients achieved complete responses after chemotherapy, and eight showed residual teratoma/scar. Toxicity was mostly grade 3/4 neutropenia/thrombocytopenia during chemotherapy. At a median follow-up of 44.5 months, overall and event-free survivals were 100%. CONCLUSION: The treatment is tolerable, and WVFI dose reduction to 18 Gy preserves efficacy; we have demonstrated the feasibility of successfully conducting a prospective multicenter trial in a large MIC despite resource disparity.

Time course of hemispheric cerebral volume after decompressive craniectomy in malignant middle cerebral artery stroke / Evolução temporal do volume hemisférico cerebral após craniectomia descompressiva em infarto maligno de artéria cerebral média

Abstract Background Brain edema is the leading cause of death in patients with malignant middle cerebral artery (MCA) infarction. Midline shift (MLS) has been used as a monohemispheric brain edema marker in several studies; however, it does not precisely measure brain edema. It is now possible to directly measure hemisphere brain volume. Knowledge about the time course of brain edema after malignant middle cerebral artery infarction may contribute to the condition's management. Objective Therefore, our goal was to evaluate the course of brain edema in patients with malignant MCA infarction treated with decompressive craniectomy (DC) using hemispheric volumetric measurements. Methods Patients were selected consecutively from a single tertiary hospital between 2013 and 2019. All patients were diagnosed with malignant middle cerebral artery infarction and underwent a decompressive craniectomy (DC) to treat the ischemic event. All computed tomography (CT) exams performed during the clinical care of these patients were analyzed, and the whole ischemic hemisphere volume was calculated for each CT scan. Results We analyzed 43 patients (197 CT exams). Patients' mean age at DC was 51.72 [range: 42-68] years. The mean time between the ischemic ictus and DC was 41.88 (range: 6-77) hours. The mean time between the ischemic event and the peak of hemisphere volume was 168.84 (95% confidence interval [142.08, 195.59]) hours. Conclusion In conclusion, the peak of cerebral edema in malignant MCA infarction after DC occurred on the 7th day (168.84 h) after stroke symptoms onset. Further studies evaluating therapies for brain edema even after DC should be investigated.

Resumo Antecedentes O edema cerebral é a principal causa de morte em pacientes com infarto maligno de artéria cerebral média. O desvio da linha média tem sido utilizado como marcador de edema cerebral mono-hemisférico em alguns estudos; porém, ele não mede de forma precisa o edema cerebral. Atualmente é possível mensurar diretamente o volume do hemisfério cerebral. O conhecimento sobre a evolução temporal do edema cerebral após infartos malignos da artéria cerebral média pode contribuir para o cuidado clínico desta condição. Objetivo Nosso objetivo é avaliar o edema hemisférico ao longo do tempo, em pacientes com infarto maligno da artéria cerebral média, tratados com craniectomia descompressiva. Métodos Os pacientes foram selecionados de forma consecutiva, em um hospital terciário, entre 2013 e 2019. Todos os pacientes apresentavam diagnóstico de infarto maligno de artéria cerebral média e foram submetidos a craniectomia descompressiva. Todas as tomografias computadorizadas de crânio destes pacientes foram analizadas, e o volume do hemisfério cerebral infartado foi mensurado. Resultados Analisamos 43 pacientes (197 tomografias de crânio). A idade média dos pacientes na craniectomia descompressiva foi 51,72 (42-68) anos. O tempo médio entre o ictus e a craniectomia descompressiva foi 41,88 (6-77) horas. O tempo médio entre o ictus e o pico do volume hemisférico foi 168,84 (142,08-195,59) horas. Conclusão O pico do volume cerebral em pacientes com infarto maligno de artéria cerebral média submetidos a craniectomia descompressiva ocorreu no 7o dia (168,84 horas) após o infarto. Mais estudos avaliando terapêuticas direcionadas ao edema cerebral seriam úteis neste contexto.

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. 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.

Training model for the fetal myelomeningocele correction with multiportal endoscopic technique.

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.

Posterior C1-C2 fusion for atlantoaxial rotatory fixation after posterior fossa craniotomy in a 4-year old: a case report.

INTRODUCTION: This study aimed to highlight that atlantoaxial rotatory fixation (AARF) can be related to neurosurgery procedures in children, with an afterward demonstration of good results after halo-gravity traction and C1-C2 stabilization using the Harms technique. CASE DESCRIPTION: To the best of our knowledge, this is the first case to report a 4-year-old boy who presented with AARF after a posterior fossa craniotomy to treat a cerebellar astrocytoma. At our medical facility, AARF was diagnosed using plain radiography and computed tomography imaging. The patient was treated with continuous cranial traction for 14 days. Initially, we detected that the patient had no C1 posterior arch or C2 spinous process; therefore, the best option was to perform the Harms technique. Postoperatively, the patient was placed in a cervical collar for 4 weeks. At the 4-year postoperative follow-up, the patient was doing well and had not developed any complications. CONCLUSION: Herein, we report a case in which AARF can be developed after neurosurgical procedure. Surgical techniques used for atlantoaxial subluxation should be carefully selected. In our case, the Harms technique after cranial traction was an excellent option for correcting and stabilizing the abnormal neck position. However, further studies are required to determine the best technique to use in the pediatric population.

Risk for hydrocephalus, hygroma, and tumor dissemination after ventricular opening during resection of supratentorial neoplasms in children.

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.

Ultrasound evaluation of uterine scar thickness after open fetal surgery for myelomeningocele.

PURPOSE: This study aimed to analyse the evolution of uterine scar thickness after open fetal surgery for myelomeningocele (MMC) by ultrasonography, and to establish a cut-off point for uterine scar thickness associated with high-risk of uterine rupture. METHODS: A prospective longitudinal study was conducted with 77 pregnant women who underwent open fetal surgery for MMC between 24 and 27 weeks of gestation. After fetal surgery, ultrasound follow-up was performed once a week, and the scar on the uterine wall was evaluated and its thickness was measured by transabdominal ultrasound. At least five measurements of the uterine scar thickness were performed during pregnancy. A receiver operating characteristics (ROC) curve was constructed to obtain a cut-off point for the thickness of the scar capable of detecting the absence of thinning. Kaplan-Meier curves were constructed to evaluate the probability of thinning during pregnancy follow-up. RESULTS: The mean ± standard deviation of maternal age (years), gestational age at surgery (weeks), gestational age at delivery (weeks), and birth weight (g) were 30.6 ± 4.5, 26.1 ± 0.8, 34.3 ± 1.2 and 2287.4 ± 334.4, respectively. Thinning was observed in 23 patients (29.9%). Pregnant women with no thinning had an average of 17.1 ± 5.2 min longer surgery time than pregnant women with thinning. A decrease of 1.0 mm in the thickness of the uterine scar was associated with an increased likelihood of thinning by 1.81-fold (95% confidence interval [CI]: 1.32-2.47; p < 0.001). The area below the ROC curve was 0.899 (95% CI: 0.806-0.954; p < 0.001), and the cut-off point was ≤ 3.0 mm, which simultaneously presented greater sensitivity and specificity. After 63 days of surgery, the probability of uterine scarring was 50% (95% CI: 58-69). CONCLUSION: A cut-off point of ≤ 3.0 mm in the thickness of the uterine scar after open fetal surgery for MMC may be used during ultrasonography monitoring for decision-making regarding the risk of uterine rupture and indication of caesarean section.

Response assessment in pediatric craniopharyngioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group.

BACKGROUND: Craniopharyngioma is a histologically benign tumor of the suprasellar region for which survival is excellent but quality of life is often poor secondary to functional deficits from tumor and treatment. Standard therapy consists of maximal safe resection with or without radiation therapy. Few prospective trials have been performed, and response assessment has not been standardized. METHODS: The Response Assessment in Pediatric Neuro-Oncology (RAPNO) committee devised consensus guidelines to assess craniopharyngioma response prospectively. RESULTS: Magnetic resonance imaging is the recommended radiologic modality for baseline and follow-up assessments. Radiologic response is defined by 2-dimensional measurements of both solid and cystic tumor components. In certain clinical contexts, response to solid and cystic disease may be differentially considered based on their unique natural histories and responses to treatment. Importantly, the committee incorporated functional endpoints related to neuro-endocrine and visual assessments into craniopharyngioma response definitions. In most circumstances, the cystic disease should be considered progressive only if growth is associated with acute, new-onset or progressive functional impairment. CONCLUSIONS: Craniopharyngioma is a common pediatric central nervous system tumor for which standardized response parameters have not been defined. A RAPNO committee devised guidelines for craniopharyngioma assessment to uniformly define response in future prospective trials.

SIOP PODC-adapted treatment guidelines for craniopharyngioma in low- and middle-income settings.

Pediatric craniopharyngioma is a rare tumor with excellent survival but significant long-term morbidities due to the loco-regional tumor growth or secondary to its treatment. Visual impairment, panhypopituitarism, hypothalamic damage, and behavioral changes are among the main challenges. This tumor should be managed under the care of a multidisciplinary team to determine the optimum treatment within the available resources. This is particularly important for low middle-income countries where resources are variable. This report provides risk-stratified management guidelines for children diagnosed with craniopharyngioma in a resource-limited setting.