Extent of Tumor Resection and Survival in Pediatric Patients With High-Grade Gliomas: A Systematic Review and Meta-analysis.

Importance: Pediatric patients with high-grade gliomas have a poor prognosis. The association among the extent of resection, tumor location, and survival in these patients remains unclear. Objective: To ascertain whether gross total resection (GTR) in hemispheric, midline, or infratentorial pediatric high-grade gliomas (pHGGs) is independently associated with survival differences compared with subtotal resection (STR) and biopsy at 1 year and 2 years after tumor resection. Data Sources: PubMed, EBMR, Embase, and MEDLINE were systematically reviewed from inception to June 3, 2022, using the keywords high-grade glioma, pediatric, and surgery. No period or language restrictions were applied. Study Selection: Randomized clinical trials and cohort studies of pHGGs that stratified patients by extent of resection and reported postoperative survival were included for study-level and individual patient data meta-analyses. Data Extraction and Synthesis: Study characteristics and mortality rates were extracted from each article. Relative risk ratios (RRs) were pooled using random-effects models. Individual patient data were evaluated using multivariate mixed-effects Cox proportional hazards regression modeling. The PRISMA reporting guideline was followed, and the study was registered a priori. Main Outcomes and Measures: Hazard ratios (HRs) and RRs were extracted to indicate associations among extent of resection, 1-year and 2-year postoperative mortality, and overall survival. Results: A total of 37 studies with 1387 unique patients with pHGGs were included. In study-level meta-analysis, GTR had a lower mortality risk than STR at 1 year (RR, 0.69; 95% CI, 0.56-0.83; P < .001) and 2 years (RR, 0.74; 95% CI, 0.67-0.83; P < .001) after tumor resection. Subtotal resection was not associated with differential survival compared with biopsy at 1 year (RR, 0.82; 95% CI, 0.66-1.01; P = .07) but had decreased mortality risk at 2 years (RR, 0.89; 95% CI, 0.82-0.97; P = .01). The individual patient data meta-analysis of 27 articles included 427 patients (mean [SD] age at diagnosis, 9.3 [5.9] years), most of whom were boys (169 of 317 [53.3%]), had grade IV tumors (246 of 427 [57.7%]), and/or had tumors that were localized to either the cerebral hemispheres (133 of 349 [38.1%]) or midline structures (132 of 349 [37.8%]). In the multivariate Cox proportional hazards regression model, STR (HR, 1.91; 95% CI, 1.34-2.74; P < .001) and biopsy (HR, 2.10; 95% CI, 1.43-3.07; P < .001) had shortened overall survival compared with GTR but no survival differences between them (HR, 0.91; 95% CI, 0.67-1.24; P = .56). Gross total resection was associated with prolonged survival compared with STR for hemispheric (HR, 0.29; 95% CI, 0.15-0.54; P < .001) and infratentorial (HR, 0.44; 95% CI, 0.24-0.83; P = .01) tumors but not midline tumors (HR, 0.63; 95% CI, 0.34-1.19; P = .16). Conclusions and Relevance: Results of this study show that, among patients with pHGG, GTR is independently associated with better overall survival compared with STR and biopsy, especially among patients with hemispheric and infratentorial tumors, and support the pursuit of maximal safe resection in the treatment of pHGGs.

Comparison of intrathecal baclofen pump insertion and selective dorsal rhizotomy for nonambulatory children with predominantly spastic cerebral palsy.

OBJECTIVE: In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options. METHODS: The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes. RESULTS: Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified. CONCLUSIONS: Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.

Transient involvement of the cerebral cortex in a flare of Behçet's syndrome.

We describe a flare of Behçet's syndrome in a 42-year-old man who presented with florid mucocutaneous manifestations, headache and vomiting, but without meningeal or neurologic signs. A single, non-enhancing cortical lesion was found in the frontal lobe by computed tomography and magnetic resonance (MR) imaging studies. Diffusion-weighted analysis and MR spectroscopy were consistent with focal inflammation. The patient improved with colchicine, and after 5 months, a repeat MR scan demonstrated resolution of the inflammatory changes suggesting that involvement of the cerebral cortex may be a self-limiting phenomenon, distinct from other more frequent and destructive parenchymal manifestations of neuro-Behçet's disease.