Child's Nervous System最新文献

Purpose: We aimed to present our surgical experience and the impact of a solid or cystic morphology of cerebellar pilocytic astrocytoma (cPA) on surgery and the risk for a re-resection.

Methods: We retrospectively analyzed all children operated at our institution between 2009 and 2023 for cPA. Tumours were categorized into 4 groups: (i) cystic PA without cyst wall enhancement, (ii) cystic PA with cyst wall enhancement, (iii) solid tumour, (iv) and solid tumour with central necrosis.

Results: Forty-two children with a median age at surgery of 7.1 years (range 0.7-14 years; male to female ratio 1.5) were identified. The median follow-up time was 3.1 years (0.6-14 years). Twenty-eight patients (66.6%) presented with cystic PA (20 without and 8 with cyst wall enhancement), 9 patients (21.4%) exhibited a solid tumour with central necrosis and 5 (11.9%) had a solid tumour without central necrosis. Gross total resection could be achieved in 31 patients (73.8%), near total resection in 6 (14.3%), and subtotal resection in 5 (11.9%). Progression occurred in 11 cases with 9 patients having a 2nd resection after a mean time of 3.4 years. The highest risk for a 2nd resection was seen in the group of solid tumours with a necrotic centre (odds ratio = 2.3), progression of enhancing cyst wall remnants was seen in one out of two patients with remnants needing reoperation.

Conclusion: Surgery in cerebellar PA should aim for gross total resection of the solid-enhancing tumour.

Introduction: Endoscopic Third Ventriculostomy (ETV) is a well-established treatment for pediatric hydrocephalus, particularly in cases of aqueductal stenosis. The ETV Success Score (ETVSS) is a predictive tool widely used to estimate the likelihood of ETV success based on factors like age. Its accuracy, especially in infants under 3 months, is still debated.

Patients and methods: This study evaluates the age-dependency of ETV success in 54 pediatric patients compared to ETVSS predictions. Patients were divided into age and pathology groups according to Kulkarni. Success was defined according the ETVSS criteria. Minimum follow-up was 12 months and included MRI to demonstrate a flow void at the floor or the third ventricle.

Results: Our institutional data revealed a higher overall success rate SR (88%) compared to the ETVSS-predicted rate of 73%. Despite small numbers within subgroups, especially in very young children < 1 month, the success rate was higher than predicted by ETVSS.

Discussion: Our results show significantly higher actual SR across all age groups compared to ETVSS predictions (p = 0.035) when selected and performed by an experienced physician. The age groups > 1 year had significantly higher SR close to 100% (p < 0.0001 and p = 0.0038, respectively). This suggests that ETV may be underutilized, particularly in infants, where predicted success rates are pessimistic.

Conclusion: ETVSS is a useful tool for counseling of parents, but differences in institution-specific outcomes should not be neglected. Depending on that, physicians might opt in favor of ETV as primary treatment in occlusive hydrocephalus of very young children, counterbalancing risks and sequalae of VP-shunting.

Purpose: Pediatric patients undergoing neurosurgery pose risk of perioperative hemorrhage and clotting dysfunction which is increased in tumors with high vascularity, endothelial exposure, and necrosis. Lesions affecting the ventricular system may arise from several etiologies, including rare tumors. The present study aimed to study the preoperative coagulation and transfusion profile of pediatric patients undergoing neurosurgery for intraventricular lesions.

Methods: A retrospective study of all paediatric patients who underwent surgery for intraventricular lesion was included from June 2014 to June 2022. Demographic data, preoperative and postoperative investigations, intraoperative blood loss, blood product transfusion, and duration of surgery were noted. Postoperative outcomes were also assessed.

Results: A total of 98 patients underwent surgery, the median age was 11.1 years (1.0 to 17.0), with a male predominance (50.9%). The median blood loss during surgery was 500 mL (300-812.5). The incidence of preoperative coagulopathy was 30.7%. The intraoperative packed blood cell and fresh frozen plasma transfusion requirement was noted in 50.9% and 5%, respectively. The median ICU stay and hospital stay was 6 days (3-10) and 14 days (4-77), respectively. The mortality rate in the study population was 7.7%.

Conclusion: This study highlights the multifactorial nature of perioperative blood loss and transfusion requirements in pediatric patients undergoing surgery for intraventricular lesions. Factors such as tumor histopathology, preoperative hemoglobin levels, and surgical duration play a crucial role in determining transfusion needs.

Background and aim: Osteoid osteoma (Oo) and osteoblastoma (Ob) are rare primary bone tumors with a higher prevalence in the second decade of life. Treatment can be conservative, but in cases of spinal location, resective surgery is of great importance but may be challenging.

Material and methods: We report four pediatric cases of Oo and Ob managed in our unit, with different locations at the level of the cervical spine. All four patients had a common complaint of persistent and progressive neck pain, two reported worsening pain at night, and three underwent NSAID treatment attempts without significant response. The radiological examination revealed a lesion of bone origin in the cervical spine in all cases: two at the pediculolaminar complex, one at the laminar, and one at the level of the C2 body and odontoid. A comprehensive literature review was carried out by comparing the data and a discussion of the cases.

Results: A complete surgical resection was performed by a posterior approach in three patients and a transoral in one. A hard neck collar under molding ensured postoperative stability in all cases. Control imaging studies demonstrated satisfactory lesion resection. Histologically, three cases were an osteoblastoma and one an osteoid osteoma. Control imaging studies demonstrated satisfactory lesion resection with good late bone reconstruction. Ultimately, none showed late instability requiring fixation. The mean follow-up time is 36 (16-63) months. None had a recurrence nor late instability requiring fixation.

Conclusion: Treatment of pediatric cervical spine of osteoid osteoma and osteoblastoma should seek a complete resection. In regions with a risk for secondary instability, the preservation of capsule-ligamental structures and temporary orthopedic immobilization can allow bone regeneration with no need for fixation. Extended follow-up is recommended especially in very young children to avoid late instability.

Introduction: Diffuse intrinsic pontine glioma (DIPG) in children comprises 80% of brainstem gliomas. In 2021, 5th edition of WHO CNS tumor classification defined H3K27M altered diffuse midline gliomas (DMGs) which replaced this entity. Lesion location precludes resection and the only current option available is radiotherapy. Patient age, duration of symptoms, histone subtype mutation etc. may helpl in prognostication but the disease remains incurable with a median overall survival of 9-12 months.

Method: This is a prospective observational study from a tertiary health care center in a low to middle-income country. We included patients with DIPG (radiological and/or histopathological H3K27M altered) from June 2018 to April 2023. Clinical, radiological, histology, and molecular features were reviewed and prognostic factors for 3 months, 6 months, and overall survival was analysed for all patients.

Results: We included 92 pediatric patients. The median age of our study population was 8.5 years. Median LPS was 80. Cranial nerve palsy was the most common presenting complaint. Hydrocephalus requiring CSF diversion was present in 38 patients (41.3%). Lesion biopsy was performed in 36 patients (39.1%) and exophytic component decompression was done in 11 patients (11.9%). Seven patients were lost to follow-up. Adjuvant therapy was received by 51 patients (51/85, 60%). Radiotherapy was the only significant prognostic indicator of 3 months, 6 months, and overall survival (HR: 0.39). The presence of necrosis on histopathology was also an indicator of poor prognosis (HR: 2.38). There were 7 long-term survivors in our study but we did not find any significant survival prognostic indicator amongst this group.

Conclusion: Conventional adjuvant therapy has not proven of much benefit. With the advancement in molecular understanding of the entity, there is an upsurge in the development of targeted therapy but with no promising results so far. In this study, we have attempted to explore the prognostic factors and unique challenges we face in a resource-limited setting against this disease.

Introduction: Adamantinomatous craniopharyngiomas (ACP) are rare epithelial tumors, which by the WHO are classified as non-malignant tumors. Despite radical tumor regression, almost 57% of patients develop a craniopharyngioma recurrence. The pathogenesis of epithelial cancers involves a process called epithelial-mesenchymal transition (EMT), which is involved in tumor progression and its invasion, and the loss of E-cadherin is crucial for this process. Undoubtedly, EMT also plays a role in the progression of ACP, but there are no studies that would examine its role in predicting postoperative tumor recurrence. Therefore, in our study, we aimed to compare the expression of EMT inducers and their markers, namely E-cadherin and vimentin, in material from two groups of pediatric patients, first with postoperative ACP relapse and second without relapse.

Methods: A total of 35 formalin-fixed, paraffin-embedded tissue blocks of pediatric patients (19 girls and 16 boys, from 2 to 17 years old) were included. The material was collected during craniopharyngioma resection in the years 2000-2019 and after then examined by the Department of Pathomorphology. Gene expression analysis was done using qRT-PCR.

Results: In the studied group of 35 patients, high levels of E-cad and low levels of vimentin expression were found in patients who did not experience relapse (n = 25, p < 0.0001). The opposite was observed in patients who experienced a recurrence (n = 10, p < 0.0001). In contrast, analysis of the recurrent tissue itself showed low levels of vimentin and re-expression of E-cadherin (n = 10, p < 0.0001). Furthermore, our study shows that Snail is a key inducer of EMT in ACP.

Conclusion: We believe that the evaluation of the EMT profile in ACP could be a prognostic marker for predicting tumor recurrence in children, which would certainly contribute to a better prognosis for these patients.

Purpose: This study presents a MATrix LABoratory (MATLAB)-based methodology for calculating intracranial volumes from head computed tomography (CT) data and compares it with established methods.

Methods: Regions of interest (ROI) were manually segmented on CT images using a stylus pen, facilitated by mirroring a computer desktop onto a tablet. The volumetric process involved three main steps: (1) calculating the volume of a single voxel, (2) counting the total number of voxels within the segmented ROI, and (3) multiplying this voxel count by the single-voxel volume. This method was applied to 83 pediatric head CT scans from patients with minor head trauma, and the volumetric results were compared with those obtained from OsiriX.

Results: A paired t-test revealed a statistically significant difference (p < 0.001) between volumes obtained with our MATLAB-based method and those from OsiriX, with our method measuring 0.32% higher. However, an unpaired t-test found no statistically significant differences between the volumetric population groups (p = 0.84).

Conclusion: The significant difference identified by the paired t-test likely reflects statistical distinctions arising from differences in the calculation methods of the two approaches. Conversely, the unpaired t-test suggests no statistically detectable differences between the volumetric populations. Although this does not imply that the two methods produce identical results, the volumetric populations derived from our method may originate from the same underlying population as those obtained using OsiriX. By taking these points into account, our method has the potential to serve as a valuable tool for volumetric measurements.

Background: Goldenhar syndrome is a clinically heterogeneous disorder defined by a rare combination of congenital anomalies-an eye abnormality, in addition to two of the following three: ear anomalies, mandibular malformations, and vertebral defects. Notably, children with Goldenhar syndrome present with a high incidence of cervical spine malformations.

Clinical case: In this report, we present an unusual case of a 15-year-old child with Goldenhar syndrome, who additionally presents with some clinical features of VACTERL syndrome. The patient was scheduled for mandibular distraction surgery with oral maxillofacial surgery. It was noted on pre-operative three-dimensional computed tomography (3D CT) that there was mild basilar invagination and stenosis of the foramen magnum. A magnetic resonance imaging (MRI) study was recommended and showed cervicomedullary compression, for which a suboccipital craniectomy and C1 laminectomy were performed.

Conclusion: Considering the cervical spine anomalies in Goldenhar syndrome, we describe precautions to manage the risk of cervical spinal cord injury during general anesthesia, including pre-operative evaluation for cervical spine compression and/or instability and minimizing neck movement during intubation. For the latter, we recommend a controlled, neutral neck position during intubation, operation, and recovery.

Multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) is a rare tumefactive lesion requiring differentiation from glial tumors. Multinodular and vacuolating posterior fossa lesions of unknown significance (MV-PLUS) were initially reported in 2019 as posterior fossa lesions with characteristics similar to MVNT. MV-PLUS is often considered as a "leave-me-alone lesion" not necessitating surgical intervention. However, no studies have reported on MV-PLUS with long-term follow-up. In this report, we presented six new cases of MV-PLUS and reviewed existing literature on the condition. The median observation period for our cases was 93 months (range 79-124 months), significantly longer than previously reported. None of the patients underwent surgical treatment or showed any imaging changes during the observation period. We emphasize the importance of understanding the imaging characteristics of MV-PLUS to accurately differentiate these infratentorial lesions and avoid unnecessary surgical interventions.