Pediatric Neurosurgery最新文献

Introduction: Surgical revascularization is very effective in patients with moyamoya angiopathy (MMA) and leads to improvements in cortical perfusion parameters. However, changes in white matter hemodynamics are still underestimated. To date, only a few studies have examined brain perfusion changes within deep white matter after bypass surgery in patients with MMA.

Methods: Ten children with MMA were evaluated using the CT perfusion technique before and after revascularization surgery. Brain perfusion parameters within gray and white matter were compared before and after surgery. The correlations between the perfusion parameters before surgery and the Suzuki stage, as well as between the perfusion parameters and the cognitive scores, were also evaluated.

Results: Brain perfusion parameters improved significantly in both gray matter (predominantly due to cerebral blood flow within the anterior circulation, p < 0.01) and white matter (predominantly due to cerebral blood volume within the semiovale centrum, p < 0.001). We revealed that the pattern of improvement in perfusion in white matter differed from the pattern of improvement in perfusion in gray matter. Significant correlations were revealed between the Suzuki stage before surgery and the perfusion parameters within the posterior cerebral artery circulation (adjusted p < 0.05). There were also significant correlations between cognitive scores and brain perfusion parameters in gray matter and white matter (adjusted p < 0.05).

Conclusions: The perfusion parameters of gray matter and white matter in the brain improve differently after bypass surgery in patients with MMA. Different hemodynamics within these compartments could explain this.

Introduction: Human herpes virus-6 (HHV-6) is a ubiquitous virus but can lead to deleterious clinical manifestations due to its predilection for the pediatric central nervous system. Despite significant literature describing its common clinical course, it is rarely considered as a causative agent in CSF pleocytosis in the setting of craniotomy and external ventricular drainage device. Identification of a primary HHV-6 infection allowed for timely treatment with an antiviral agent along with earlier discontinuation of antibiotic regimen and expedited placement of a ventriculoperitoneal shunt.

Case presentation: A two-year-old girl presented with 3 months of progressive gait disturbance and intranuclear ophthalmoplegia. Following craniotomy for removal of 4th ventricular pilocytic astrocytoma and decompression of hydrocephalus, she suffered a prolonged clinical course due to persistent fevers and worsening CSF leukocytosis despite multiple antibiotic regimens. The patient was admitted to the hospital during the COVID-19 pandemic and isolated with her parents in the intensive care unit with strict infection control measures. FilmArray Meningitis/Encephalitis (FAME) panel ultimately detected HHV-6. Clinical confirmation of HHV-6-induced meningitis was proposed given improvement in CSF leukocytosis and fever reduction following the initiation of antiviral medications. Pathologic analysis of brain tumor tissue failed to show HHV-6 genome positivity, suggesting a primary peripheral etiology of infection.

Conclusion: Here, we present the first known case of HHV-6 infection detected by FAME following intracranial tumor resection. We propose a modified algorithm for persistent fever of unknown origin which may decrease symptomatic sequelae, minimize additional procedures, and shorten length of ICU stay.

Background: Periodic updates to the World Health Organization (WHO) classification system for central nervous system (CNS) tumors reflect advances in the pathological diagnosis, categorization, and molecular underpinnings of primary brain, spinal cord, and peripheral nerve tumors. The 5th edition of the WHO Classification of CNS Tumors was published in 2021. This review discusses the guiding principles of the revision, introduces the more common new diagnostic entities, and describes tumor classification and nomenclature changes that are relevant for pediatric neurological surgeons.

Summary: Revisions to the WHO CNS tumor classification system introduced new diagnostic entities, restructured and renamed other entities with particular impact in the diffuse gliomas and CNS embryonal tumors, and expanded the requirements for incorporating both molecular and histological features of CNS tumors into a unified integrated diagnosis. Many of the new diagnostic entities occur at least occasionally in pediatric patients and will thus be encountered by pediatric neurosurgeons. New nomenclature impacts the terminology that is applied in communication between pathologists, surgeons, clinicians, and patients. Requirements for molecular information in tumor diagnosis are expected to refine diagnostic categories while also introducing practical considerations for intraoperative consultation, preliminary histological evaluation, and triaging of neurosurgical tissue samples for histology, molecular testing, and clinical trial requirements.

Key messages: Pediatric brain tumor diagnosis and clinical management are a multidisciplinary effort that is rapidly advancing in the molecular era. Interdisciplinary collaboration is critical for providing the best care for pediatric CNS tumor patients. Pediatric neurosurgeons and their local neuropathologists and neuro-oncologists must work collaboratively to put the most current CNS tumor diagnostic guidelines into standard practice.

Introduction: This study aimed to identify factors affecting progression-free survival (PFS) in pediatric patients with giant supratentorial brain tumors (GSBTs) treated with surgical excision. The secondary aim was to analyze how these same factors affected the functional outcome in the long term.

Methods: We performed a retrospective, analytical, single-center cohort study. We included all pediatric patients with GSBT between January 2014 and June 2018. Patients were followed for a minimum of 24 months for the PFS and overall survival (OS) analysis. Functional status score (FSS) was used to assess the functional outcome.

Results: We included 27 patients with GSBT, the median age was six (range 2-12), and eleven patients had a grade IV tumor. The 24-month PFS and OS were 51.85% and 74.04%, respectively. A PFS-ending event or treatment failure occurred in 13 patients. We found that patients with postoperative FFS >16 have a worse PFS than patients with a postoperative FSS <15 (HR 4.51; p = 0.03). Patients with more than three surgeries had worse PFS than patients with one or two procedures (HR 11.39; p = 0.004). High-grade tumors were associated with worse PFS than low-grade tumors (HR 1.55; p = 0.04). Finally, patients with CNS infections had worse PFS than patients without that complication (HR 2.70; p = 0.04).

Conclusions: GSBTs in pediatric patients are complex lesions that require multidisciplinary management. Surgical management and quality of life should be considered when choosing the best treatment. Factors influencing long-term PFS were high-grade histopathology, the need for three or more surgeries, postoperative FSS >16, and CNS infections.

Introduction: Role of CT scan, MRI, ophthalmoscopy, direct monitoring by a transducer probe in identifying raised intracranial pressure (ICP) in emergency department is limited. There are few studies correlating elevated optic nerve sheath diameter (ONSD) measured by point of care ultrasound (POCUS) with raised ICP in pediatrics emergencies. We studied the diagnostic accuracy of ONSD, crescent sign, and optic disc elevation in identifying increased ICP in pediatrics.

Methods: Prospective observational study was done between April 2018 and August 2019 after ethics approval. Out of 125 subjects, 40 patients without clinical features of raised ICP were recruited as external controls and 85 with clinical features of raised ICP as study subjects. Their demographic profile, clinical examination, and ocular ultrasound findings were noted. This was followed by CT scan. Out of 85 patients, 43 had raised ICP (cases) and 42 had normal ICP (disease controls). Diagnostic accuracy of ONSD in identifying raised ICP was evaluated using STATA.

Results: The mean ONSD in case group was 5.5 ± 0.6 mm, 4.9 ± 0.5 mm in disease control group and external control group was 4.8 ± 0.3 mm. Cut-off of ONSD for raised ICP at ≥4.5 mm had a sensitivity and specificity of 97.67% and 10.98%, while ≥5.0 mm showed a sensitivity and specificity of 86.05% and 71.95%. Crescent sign and optic disc elevation had good correlation with increased ICP.

Conclusion: ONSD ≥5 mm by POCUS identified raised ICP in pediatric population. Crescent sign and optic disc elevation may function as additional POCUS signs in identifying raised ICP.

Introduction: Sagittal craniosynostosis (SC) is associated with scaphocephaly, an elongated narrow head shape. Assessment of regional severity in the scaphocephalic head is limited by the use of serial computed tomographic (CT) imaging or complex computer programing. Three-dimensional measurements of cranial surface morphology provide a radiation-free alternative for assessing cranial shape. This study describes the creation of an occipital bulleting index (OBI), a novel tool using surface morphology to assess the regional severity in patients with SC.

Methods: Surface imaging from CT scans or 3D photographs of 360 individuals with SC and 221 normocephalic individuals were compared to identify differences in morphology. Cartesian grids were created on each individual's surface mesh using equidistant axial and sagittal planes. Area under the curve (AUC) analyses were performed to identify trends in regional morphology and create measures capturing population differences.

Results: The largest differences were located in the medial regions posteriorly. Using these population trends, a measure was created to maximize AUC. The OBI has an AUC of 0.72 with a sensitivity of 74% and a specificity of 61%. When the frontal bossing index is applied in tandem, the two have a sensitivity of 94.7% and a specificity of 93.1%. Correlation between the two scores in individuals with SC was found to be negligible with an intraclass correlation coefficient of 0.018. Severity was found to be independent of age under 24 months, sex, and imaging modality.

Conclusions: This index creates a tool for differentiating control head shapes from those with SC and has the potential to allow for objective evaluation of the regional severity, outcomes of different surgical techniques, and tracking shape changes in individuals over time, without the need for radiation.

Introduction: Gram-negative rod (GNR) bacterial ventriculitis is a rare complication of shunt-dependent hydrocephalus, often requiring an extended and invasive treatment course. Accumulation of purulent material, as well as empyema and septation formation, limits circulation of antibiotics and infection clearance. Supplementation of standard care with neuroendoscopic-guided intraventricular lavage with lactated Ringer solution and fenestration of septations may facilitate infection clearance and simplify the eventual shunt construct required. Here, the utility of serial lavage for ventriculitis is described in a population of shunt-dependent neonates and infants at high risk for morbidity and mortality.

Methods: Five infants with shunt-dependent hydrocephalus and subsequent GNR ventriculitis were treated with standard care measures with the addition of serial neuroendoscopic lavage. A retrospective chart review was performed to collect patient characteristics, shunt dependency, and shunt revisions within a year of ventriculitis resolution.

Results: Patients demonstrated a mean 74% decrease in cerebrospinal fluid (CSF) protein following each neuroendoscopic lavage and trended toward a shorter time to infection clearance in comparison to previously published literature. Patients required 0-2 shunt revisions at 1-year follow-up following hospitalization for shunt-related ventriculitis (mean 0.8 +/- 0.8).

Conclusions: Serial neuroendoscopic lavage is an effective technique, used alone or in combination with fenestration of septations, to reduce the CSF protein and bacterial load in the treatment of ventriculitis, decreasing time until eradication of infection. Serial lavage may reduce the risk of future shunt malfunction, simplify the future shunt construct, and decrease duration of infection.

Introduction: Peri-insular hemispherotomy (PIH) is a hemispheric separation technique under the broader hemispherotomy group, a surgical treatment for patients with intractable epilepsy. Hemispherotomy techniques such as the PIH, vertical parasagittal hemispherotomy (VPH), and modified-lateral hemispherotomy are commonly assessed together, despite significant differences in anatomical approach and patient selection. We aim to describe patient selection, outcomes, and complications of PIH in its own right.

Methods: A systematic review of the literature, in accordance with the Preferred Reporting Items of Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was conducted, with searches of the PubMed and Embase databases. A local series including patients receiving PIH and followed up at the Queensland Children's Hospital between 2014 and 2020 was included.

Results: Systematic review of the literature identified 393 patients from 13 eligible studies. Engel class 1 outcomes occurred in 82.4% of patients, while 8.6% developed post-operative hydrocephalus. Hydrocephalus was most common in the youngest patient cohorts. Developmental pathology was present in 114 (40.8%) patients, who had fewer Engel 1 outcomes compared to those with acquired pathology (69.1% vs. 83.7%, p = 0.0167). The local series included 13 patients, 11/13 (84.6%) had Engel class 1 seizure outcomes. Post-operative hydrocephalus occurred in 2 patients (15.4%), and 10/13 (76.9%) patients had worsened neurological deficit.

Conclusion: PIH delivers Engel 1 outcomes for over 4 in 5 patients selected for this procedure, greater than described in combined hemispherectomy analyses. It is an effective technique in patients with developmental and acquired pathologies, despite general preference of VPH in this patient group. Finally, very young patients may have significant seizure and cognitive benefits from PIH; however, hydrocephalus is most common in this group warranting careful risk-benefit assessment. This review delivers a dedicated PIH outcomes analysis to inform clinical and patient decision-making.

Introduction: Hydrocephalus is a common pediatric neurosurgical pathology, typically treated with a ventricular shunt, yet approximately 30% of patients experience shunt failure within the first year after surgery. As a result, the objective of the present study was to validate a predictive model of pediatric shunt complications with data retrieved from the Healthcare Cost and Utilization Project (HCUP) National Readmissions Database (NRD).

Methods: The HCUP NRD was queried from 2016 to 2017 for pediatric patients undergoing shunt placement using ICD-10 codes. Comorbidities present upon initial admission resulting in shunt placement, Johns Hopkins Adjusted Clinical Groups (JHACG) frailty-defining criteria, and Major Diagnostic Category (MDC) at admission classifications were obtained. The database was divided into training (n = 19,948), validation (n = 6,650), and testing (n = 6,650) datasets. Multivariable analysis was performed to identify significant predictors of shunt complications which were used to develop logistic regression models. Post hoc receiver operating characteristic (ROC) curves were created.

Results: A total of 33,248 pediatric patients aged 6.9 ± 5.7 years were included. Number of diagnoses during primary admission (OR: 1.05, 95% CI: 1.04-1.07) and initial neurological admission diagnoses (OR: 3.83, 95% CI: 3.33-4.42) positively correlated with shunt complications. Female sex (OR: 0.87, 95% CI: 0.76-0.99) and elective admissions (OR: 0.62, 95% CI: 0.53-0.72) negatively correlated with shunt complications. ROC curve for the regression model utilizing all significant predictors of readmission demonstrated area under the curve of 0.733, suggesting these factors are possible predictors of shunt complications in pediatric hydrocephalus.

Conclusion: Efficacious and safe treatment of pediatric hydrocephalus is of paramount importance. Our machine learning algorithm delineated possible variables predictive of shunt complications with good predictive value.