- Atypical presacral masses in Currarino syndrome: a case series. [Case Reports]
- CONCLUSIONS: These cases highlight the considerable heterogeneity of presacral lesions in Currarino syndrome and identify two underrecognized or previously unreported tumor types: myxopapillary ependymoma and neuroendocrine tumor. Surgical management must be individualized and often requires a multidisciplinary approach. Future studies will further clarify genotype-phenotype relationships and improve diagnostic and management strategies in this complex syndrome.
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- Timing of radiotherapy and autologous stem cell transplantation in embryonal tumor with multilayered rosettes: a critical narrative review. [Review]
- CONCLUSIONS: Current evidence indicates that RT deferral beyond the HDCT/ASCT consolidation window is associated with inferior local control and worse long-term outcomes in ETMR. PNOC031, a prospective but nonrandomized trial that assigns RT-eligible patients to early focal RT and RT-ineligible patients to HDCT/ASCT in parallel cohorts, together with future prospective studies, should help to resolve this. Pending those results, and recognizing that the supporting data are observational and subject to selection bias, early focal RT following gross-total resection appears a reasonable and defensible strategy in eligible patients. The relationship between RT timing, HDCT/ASCT, pseudoprogression, and neurodevelopmental toxicity is not yet well understood and warrants further study.
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- Barometric pressure variability and shunt-related hospital encounters in the pediatric population: a retrospective analysis. [Journal Article]
- CONCLUSIONS: This study documents an association between fluctuations in atmospheric pressure and daily hydrocephalus census at a single tertiary medical center. Weather-related influences should be considered when evaluating shunt malfunction in patients with chronic shunted hydrocephalus.
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- Safe electrophysiology-guided resection of an epileptogenic non-exophytic hamartoma at the floor of the fourth ventricle in a child. [Case Reports]
- Hamartoma of the floor of fourth ventricle (HFFV) is an extremely rare entity known to be causing focal movement disorders in children. According to the few reported cases in literature, the only effective treatment is disconnection or resection of the lesion which can be very challenging in this anatomic area. We report a 15-year-old child with a fourth ventricle floor's limited nonexophytic ham…
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- Predictors and causes of 30-day failure after shunt placement in pediatric hydrocephalus: a systematic review and meta-analysis. [Systematic Review]
- CONCLUSIONS: VP shunt failure affects approximately one in eight pediatric patients in the first 30 days following placement, and occurs significantly more frequently following secondary shunt insertion. Among the pooled predictors, ventricular catheter placement quality represents the most significant modifiable factor associated with early shunt survival, although this finding was based on limited data. Several predictors showed estimates suggestive of increased risk; however, prospective studies with standardized reporting are necessary to define the size of these associations.
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- Pediatric spasticity in Brazil: insights from a neurosurgical referral center. [Journal Article]
- CONCLUSIONS: Late referral (median age 8.5 years) contributes to progressive musculoskeletal complications despite stable neurological lesions. Systematic use of GMFCS and FMS provides a reproducible screening tool for surgical candidacy in resource-limited settings. The high prevalence of dystonia (41.1%), characterizing a late-stage mixed hypertonia profile due to delayed referrals, highlights the need for expanded neuromodulation access in public healthcare systems.
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- Endoscopic third ventriculostomy for diencephalosynapsis-related hydrocephalus in MPDZ gene syndrome: a case report and review of the literature. [Case Reports]
- CONCLUSIONS: This case provides proof of concept that ETV is both mechanistically justified and clinically effective in selected patients with MPDZ-related obstructive hydrocephalus, potentially avoiding lifelong shunt dependency. Patient selection should nonetheless remain individualized, based on ventricular anatomy, third ventricular floor thickness, and associated structural anomalies.
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- Isolated clival Langerhans cell histiocytosis in a pediatric patient: systematic review and illustrative case report. [Systematic Review]
- CONCLUSIONS: Isolated clival LCH is an exceptionally rare presentation that can radiologically mimic aggressive skull base neoplasms. Histopathological confirmation is essential. Early recognition and appropriate multidisciplinary management are associated with favorable outcomes.
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- Frameless VarioGuide biopsy for pediatric brainstem lesions in the molecular era: diagnostic performance and workflow characteristics in a comparative single-center series. [Journal Article]
- CONCLUSIONS: In this exploratory single-center cohort, frameless VarioGuide biopsy achieved high diagnostic and molecular diagnostic yields without permanent procedure-related morbidity and was associated with shorter preoperative setup time. These findings support further evaluation of frameless VarioGuide biopsy as a workflow-efficient approach for tissue acquisition in pediatric brainstem lesions.
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- Occipitocervical fusion and decompression in Morquio syndrome: a case report and literature review. [Case Reports]
- Mucopolysaccharidosis IVA, also known as Morquio syndrome, is a rare autosomal recessive lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Spinal involvement, particularly at the craniocervical junction, is a major contributor to morbidity and mortality due to atlantoaxial instability and cervical spinal stenosis. Early diagnosis and timely surgical i…
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- Fatal spontaneous bilateral extradural hematoma with swirl sign in a child with sickle cell disease: lessons from delayed surgical access at a tertiary center. [Case Reports]
- CONCLUSIONS: This is, to our knowledge, the first description of the swirl sign in spontaneous SCD-related EDH, suggesting that this radiological marker may also indicate active bleeding in this rare clinical entity. The unusual macroscopic appearance and friability of the calvarial bone raise the possibility of a bone marrow-related perioperative complication, including fat embolism, although the cause of death could not be established. The documented neurological deterioration during the delay to surgery highlights the potential impact of delayed access to emergency neurosurgical care in resource-limited settings such as ours.
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- Evaluation of nationwide variation in post-haemorrhagic ventricular dilatation management following national guideline implementation in the Netherlands. [Multicenter Study]
- CONCLUSIONS: Following implementation of a national guideline, PHVD management in Dutch NICUs demonstrates high consistency in core diagnostics and treatment principles, showing that national standardisation of PHVD care is feasible. Residual variation is concentrated in areas where evidence remains limited, identifying priorities for future collaborative research.
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