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3-Bromofluoranthene (3-BrFlu) is the secondary metabolite of fluoranthene, which is classified as a polycyclic aromatic hydrocarbon, through bromination and exists in the fine particulate matter of air pollutants. Endothelial dysfunction plays a critical role in the pathogenesis of cardiovascular and vascular diseases. Little is known about the molecular mechanism of 3-BrFlu on endothelial dysfunction in vivo and in vitro assay. In the present study, 3-BrFlu included concentration-dependent changes in ectopic angiogenesis of the sub-intestinal vein and dilation of the dorsal aorta in zebrafish. Disruption of vascular endothelial integrity and up-regulation of vascular endothelial permeability were also induced by 3-BrFlu in a concentration-dependent manner through pro-inflammatory responses in vascular endothelial cells, namely, SVEC4-10 cells. Generation of pro-inflammatory mediator PGE2 was induced by 3-BrFlu through COX2 expression. Expression of COX2 and generation of pro-inflammatory cytokines, including TNFα and IL-6, were induced by 3-BrFlu through phosphorylation of NF-κB p65, which was mediated by phosphorylation of MAPK, including p38 MAPK, ERK and JNK. Furthermore, generation of intracellular ROS was induced by 3-BrFlu, which is associated with the down-regulated activities of the antioxidant enzyme (AOE), including SOD and catalase. We also found that 3-BrFlu up-regulated expression of the AOE and HO-1 induced by 3-BrFlu through Nrf-2 expression. However, the 3-BrFlu-induced upregulation of AOE and HO-1 expression could not be revised the responses of vascular endothelial dysfunction. In conclusion, 3-BrFlu is a hazardous substance that results in vascular endothelial dysfunction through the MAPK-mediated-NFκB pro-inflammatory pathway and intracellular ROS generation. [ABSTRACT FROM AUTHOR]

Augmentation cystoplasty (AC) is an effective surgical procedure for patients with neurogenic bladder whenever conservative treatments have failed. The present study aimed to determine the risks of metabolic complications, malignancy, long-term outcomes and histopathologic changes of native bladder and the augmented intestine after AC in children with neurogenic bladder. Pediatric patients < 18 years who underwent AC between 2000 and 2020 were enrolled. Early postoperative complications, long-term outcomes and histopathologic changes in mucosal biopsies of native bladder and the augmented intestine after AC were reviewed. Twenty-two patients with a mean age of 7.6 ± 4.4 years were included. The ileum was used in 19 patients and the sigmoid colon in 3 patients. The length of hospital stay was 14.8 ± 6.8 days. Post-operatively, the urinary continence rate improved from 22.7 to 81.8% (p < 0.001). Hydronephrosis resolved in 17 of 19 patients. Vesicoureteral reflux resolved in 16 (64.0%) of the refluxing ureter units and was downgraded in 7 (28.0%). Grades of hydronephrosis and reflux significantly improved following AC (p < 0.001). The estimated glomerular filtration rate also significantly increased (p = 0.012). Formation of urinary tract stones was the most frequent late complication (in 8 patients, 36.4%). Life-threatening spontaneous bladder perforation occurred in 1 patient. After a mean follow-up of 13.4 ± 5.9 years, there were no cases of mortality, new-onset symptomatic metabolic acidosis, or changes in serum electrolytes. Of the 17 patients who were followed for > 10 years, no cases of malignancy or metaplastic changes were identified in the native bladder or augmented bowel epithelium. AC is a safe and effective procedure with low surgical and metabolic complication rates. In addition, AC provides a satisfactory continence rate and long-term protection of renal function, increases functional capacity, and regresses reflux and hydronephrosis. Individualized surveillance is recommended for the early identification of urolithiasis and metabolic disturbances. [ABSTRACT FROM AUTHOR]

Background: Stereoelectroencephalography (SEEG) is an effective presurgical invasive evaluation for drug-resistant epilepsies. The introduction of robotic devices provides a simplified, accurate, and safe alternative to the conventional SEEG technique. We report our institutional experience with robot-assisted SEEG and compare its in vivo accuracy, operation efficiency, and safety with the more traditional SEEG workflow. Methods: All patients with medically refractory focal epilepsy who underwent SEEG depth electrode implantation between 2014 and 2022 were included in this study. Technical advancements of the robot-assisted technique are described. Analyses of patient demographics, electrode implantation accuracy, operation time, and procedure-related complications were performed. Results: One hundred and sixty-six patients underwent 167 SEEG procedures. The first 141 procedures were performed using a conventional approach involving a Leksell stereotactic system, and the last 26 procedures were robot-assisted. Among the 1726 depth electrodes that were inserted, the median entry point localization error was as follows: conventional (1.0 mm; range, 0.1–33.5 mm) and robot-assisted (1.1 mm; range, 0–4.8 mm) (P = 0.17). The median target point localization error was as follows: conventional (2.8 mm; range, 0.1–49 mm) and robot-assisted (1.8 mm; range, 0–30.3 mm) (P < 0.001). The median operation time was significantly reduced with the robot-assisted workflow (90 min vs. 77.5 min; P < 0.01). Total complication rates were as follows: conventional (17.7%) and robot-assisted (11.5%) (P = 0.57). Major complication rates were 3.5% and 7.7% (P = 0.77), respectively. Conclusions: SEEG is a safe and highly accurate method that provides essential guidance for epilepsy surgery. Implementing SEEG in conjunction with multimodal planning systems and robotic devices can further increase safety margin, surgical efficiency, and accuracy. [ABSTRACT FROM AUTHOR]

We aimed to assess the efficacy of eplerenone, a steroidal mineralocorticoid receptor antagonist known to reduce blood pressure and mitigate cardiovascular disease (CVD) progression, in retarding the progression of chronic kidney disease (CKD) and CVD in a rat model of type 4 cardiorenal syndrome (CRS). We grouped rats into four experimental categories: sham surgery, sham treatment with eplerenone, nephrectomy without eplerenone (Nx), and nephrectomy with eplerenone (Nx + EP). For the Nx + EP group, rats received five-sixths nephrectomy, inducing CKD and CVD conditions such as renal hypertension and hyperglycemia, and were then treated with eplerenone (100 mg/kg/day, orally) over 4 weeks after an initial 4-week observation period. Heart rate, blood pressure, blood sugar levels, and sympathetic nerve excitation were monitored biweekly. In addition, assessments of renal and cardiac tissues, including evaluation of renal tubulointerstitial injury, glomerular injury, and cardiomyocyte hypertrophy, were conducted at week 8. Eplerenone administration mitigated CKD and CVD progression in the Nx + EP group, evident by improved blood pressure (217.3 ± 5.4 versus 175.3 ± 5.6), blood sugar (121.8 ± 1.3 versus 145.6 ± 6.0) level, reduced sympathetic nerve excitation, and cardiomyocyte hypertrophy compared to the Nx group. However, renal tubulointerstitial injury, glomerular injury, and cardiovascular dysfunction, which were increased in rats with type 4 CRS, did not show significant changes with eplerenone treatment. Our study demonstrated that eplerenone treatment did not exacerbate type 4 CRS but improved blood pressure, blood sugar levels, sympathetic nerve excitation, and cardiomyocyte hypertrophy in this model. [ABSTRACT FROM AUTHOR]

Background: Laparoscopic sleeve gastrectomy (LSG) is a relative safe procedure in bariatric surgery. However, relatively few studies had been assessed its comprehensive efficacies. In the current study, the efficacies of LSG were comprehensively explored on glycemic control and cardiovascular disease (CVD) risk reduction. Methods: A total of 95 obese patients, who owned body mass index (BMI) of more than 35, were recruited. All of them primarily underwent LSG from 2014 to 2016. Type 2 diabetes mellitus (T2DM) remission was defined as levels of glycated hemoglobin (A1C) and fasting blood glucose (FBG) of less than 6.4% and 125 mg/dL, respectively. The further efficacies of LSG on CVD and coronary heart disease (CHD) risks were explored by using original- and recalibrated Framingham 10-year CHD risk scores and the other 3 well-established CVD risk prediction models. Results: Systolic blood pressure (SBP), serum FBG, A1C, triglyceride (TG), BMI, and body weight showed significantly declined and high-density lipoprotein-cholesterol (HDL) displayed twice higher than beginning level after LSG. The 71 of 95 patients with obesity were T2DM; 62 of them exhibited persistent DM remission until 1 year after LSG. Cardiovascular age, general cardiovascular risk (GCVR), and atherosclerotic cardiovascular disease risk (ASCVD) also showed significant decrements after LSG. We also observed significant reductions in estimated CVD and CHD risks. Conclusion: LSG resulted in a persistent T2DM remission and corrected metabolic abnormalities. Subsequently, LSG also benefits declined risks of CVD and 10-year CHD developments. LSG may be helpful for primary CVD care in obese patients with BMI of more than 35. [ABSTRACT FROM AUTHOR]

Spinal metastasis from malignant primary brain tumors (MPBTs) in pediatric patients is rare and often appears as enhancing lesions on MRI. However, some indolent enhancing spinal lesions (IESLs) resulting from previous treatment mimic metastasis on MRI, leading to unnecessary investigation and treatment. In 2005–2020, we retrospectively enrolled 12 pediatric/young patients with clinical impression of spinal metastasis and pathological diagnosis of their spinal lesions. Three patients had MPBT with IESL, and 9 patients had malignant tumors with metastases. The histopathologic diagnosis of IESL was unremarkable marrow change. We evaluated their MRI, CT, and bone scan findings. The following imaging findings of IESL vs. spinal metastasis were noted: (1) IESLs appeared round/ovoid (3/3, 100%), whereas spinal metastasis appeared irregular (9/9, 100%) (P = 0.005); (2) target-shaped enhancement was noted in (3/3, 100%) vs. (0/9, 0%) of cases, respectively (P = 0.005); (3) pathologic fracture of the vertebral body was noted in (1/3, 33.3%) vs. (9/9, 100%) of cases, respectively (P = 0.045); (4) expansile vertebral shape was noted in (0/3, 0%) vs. (9/9, 100%) of cases, respectively (P = 0.005); (5) obliteration of the basivertebral vein was noted in (0/3, 0%) vs. (9/9, 100%) of cases, respectively (P = 0.005); and (6) osteoblastic change on CT was noted in (3/3, 100%) vs. (2/9, 22.2%) of cases, respectively (P = 0.034). IESL in pediatric patients with MPBT can be differentiated from metastasis based on their imaging characteristics. We suggest close follow-up rather than aggressive investigation and treatment for IESL. [ABSTRACT FROM AUTHOR]

Medulloblastoma is the most common embryonic brain tumor in children. We investigated a cohort of 52 Asian medulloblastoma patients aged between 0 and 19 years old, who received surgical resections and post-resection treatments in the Taipei Medical University Hospital and the Taipei Veterans General Hospital. Genome-wide RNA sequencing was performed on fresh-frozen surgical tissues. These data were analyzed using the CIBERSORTx immune deconvolution software. Two external clinical and molecular datasets from United States (n = 62) and Canada (n = 763) were used to evaluate the transferability of the gene-signature scores across ethnic populations. The abundance of 13 genes, including DLL1, are significantly associated with overall survival (All Cox regression P < 0.001). A gene-signature score was derived from the deep transcriptome, capable of indicating patients' subsequent tumor recurrence (Hazard Ratio [HR] 1.645, confidence interval [CI] 1.337–2.025, P < 0.001) and mortality (HR 2.720, CI 1.798–4.112, P < 0.001). After the adjustment of baseline clinical factors, the score remains indicative of recurrence-free survival (HR 1.604, CI 1.292–1.992, P < 0.001) and overall survival (HR 2.781, CI 1.762–4.390, P < 0.001). Patients stratified by this score manifest not only distinct prognosis but also different molecular characteristics: Notch signaling ligands and receptors are comparatively overexpressed in patients with poorer prognosis, while tumor infiltrating natural killer cells are more abundant in patients with better prognosis. Additionally, immunohistochemical staining showed the DLL1 protein, a major ligand in the Notch signaling pathway, and the NCAM1 protein, a representative biomarker of natural killer cells, are present in the surgical tissues of patients of four molecular subgroups, WNT, SHH, Group 3 and Group 4. NCAM1 RNA level is also positively associated with the mutation burden in tumor (P = 0.023). The gene-signature score is validated successfully in the Canadian cohort (P = 0.009) as well as its three molecular subgroups (SHH, Group 3 and Group 4; P = 0.047, 0.018 and 0.040 respectively). In conclusion, pediatric medullablastoma patients can be stratified by gene-signature scores with distinct prognosis and molecular characteristics. Ligands and receptors of the Notch signaling pathway are overexpressed in the patient stratum with poorer prognosis. Tumor infiltrating natural killer cells are more abundant in the patient stratum with better prognosis. [ABSTRACT FROM AUTHOR]

Purpose: A basal ganglia (BG) germinoma is a rare tumor, and the optimal treatment remains unknown. We evaluated the clinical outcomes of treatment of BG germinoma in pediatric patients in Taiwan. Methods: We retrospectively reviewed the medical records of 34 children with BG germinoma who were treated with radiotherapy (RT) at Taipei Veterans General Hospital between 1989 and 2016. The median follow-up time is 8.3 years (1.8–25.2 years). Survival was analyzed using the Kaplan–Meier estimate. Univariate Cox proportional-hazards models were used to identify the potential risk factors. Results: Only four patients (11.8%) experienced recurrence and all successfully underwent salvage therapy. One patient (2.97%) died due to suspected radiotherapy (RT)-related sarcoma in the scalp. The 2-, 3-, and 5-year DFS rates were 91.2%, 88.2%, and 79.4%, respectively; the 2-, 3-, and 5-year OS rates were 97.1%, 94.1%, and 82.4%, respectively. Focal RT showed low DFS in the Kaplan–Meier survival curves (P =.028) compared with non-focal RT (whole ventricle, whole brain, or cranial spinal area). In the univariate Cox proportional-hazards model, there was a significant difference in DFS between focal and non-focal RT (P =.03). There is no difference in DFS and OS between BG germinoma patients and non-BG germinoma patients. Conclusions: We found an excellent DFS and OS in pediatric patients with BG germinoma treated with RT. Whole ventricle irradiation is recommended for good tumor control and low treatment-related toxicity. BG germinoma patients showed similar treatment results as germinoma patients in other common sites. [ABSTRACT FROM AUTHOR]

Purpose: Stereotactic radiosurgery (SRS) is a potential re-irradiation treatment for recurrent intracranial ependymoma after prior radiation therapy. The purpose of this study was to examine the efficacy and safety of repeated SRS in the treatment of recurrent intracranial ependymomas. Methods: This is a retrospective study of consecutive patients with residual or recurrent intracranial ependymomas who were treated with SRS between 1993 and 2018. Tumor progression was defined as a ≥ 10% increase in tumor volume. Tumor regression was defined as a ≥ 10% reduction in tumor volume. A tumor that remained within 10% of its original volume was defined as stable. Tumor control comprised tumor regression and stability. Time-dependent analyses were performed using two treatment failure endpoint definitions: (1) evidence of local tumor progression or distant metastasis (single SRS analysis), and (2) lack of tumor response to SRS (repeated SRS analysis). These analyses were adjusted for the competing risk of death. Results: The study comprised 37 patients (65 intracranial ependymomas) who underwent multiple SRS sessions (range: 1–7). Median age was 10.2 years (range: 0.8–53.8 years), and median tumor volume was 1.5 mL (range: 0.01–22.5 mL). The median radiation dose was 13.3 Gy (range: 7.9–22.0 Gy) at a median isodose line of 57% (range: 50–90%). Overall tumor control rates in the single SRS analysis adjusting for the competing risk of death were 53.6%, 30.5%, and 23.6% at 1, 3, and 5 years, respectively. Overall tumor control rates in the repeated SRS analysis adjusting for the competing risk of death were 70.6%, 50.4%, and 43.1% at 1, 3, and 5 years, respectively. Prior gross total resection was the only independent predictor of overall tumor control after SRS (aHR = 25.62 (1.55–422.1), p = 0.02). Conclusions: Repeated GKRS appeared to be an effective treatment strategy for recurrent or residual intracranial ependymomas, with acceptable complication rates. [ABSTRACT FROM AUTHOR]

Purpose: The optimal treatment strategy for pediatric atypical teratoid rhabdoid tumor (ATRT) is inconclusive. This study evaluated the prognostic value of early radiotherapy (RT) and high-dose chemotherapy with autologous stem cell rescue (HDC/ASCR) in pediatric ATRT. Methods: This pooled analysis included ATRT patients treated at our institution and from other studies who were identified by a search of the PubMed electronic database. The effect of patient demographics and treatment profiles on progression-free survival (PFS) and overall survival (OS) were analyzed using Cox regression. Results: Overall, 34 patients from our institution and 436 patients from 35 published studies were included. In multivariable analysis, patients with gross total resection (GTR), early RT (time to RT interval < 2 months), and HDC/ASCR had both better PFS [hazard ratio (HR) 0.46, p < 0.001; HR 0.64, p = 0.011; and HR 0.51, p = 0.005, respectively] and OS (HR 0.55, p = 0.002; HR 0.48, p = 0.004; and HR 0.42, p < 0.001, respectively). For patients aged < 3 years, both RT and HDC/ASCR were significant favorable factors for PFS (HR 0.32 and 0.46, respectively) and OS (HR 0.40 and 0.36, respectively), while early RT was not prognostic. For patients aged ≥ 3 years, early RT was significantly associated with better PFS (HR 0.51) and HDC/ASCR did not affect PFS, and neither was related to OS. Conclusion: Both early RT initiation and HDC/ASCR were important components in the treatment of pediatric ATRT. However, the optimal treatment strategies might differ by age. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the study is to evaluate possible prognostic factors and optimal management for pediatric atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS). Methods: Twenty-eight pediatric patients with CNS AT/RT who were treated with radiation therapy (RT) as part of multimodality treatment regimens at a single institution (1996–2015) were reviewed. Survival outcomes were analyzed in relation to possible prognostic factors. Results: The 28 patients analyzed were followed up for a median 48-month period. Median progression-free survival (PFS) was 11 months, and overall survival (OS) was 57 months. Patients < 3 years old had RT delayed for a longer period after surgery (p = 0.04), and the mean RT dose to tumor bed was lower (p < 0.01) than in patients ≥ 3 years old. In multivariate analysis, a higher primary tumor bed RT dose was identified as a favorable prognostic factor for both PFS (hazard ratio [HR] = 0.85 per gray, p < 0.01) and OS (HR = 0.92 per gray, p = 0.02). In addition, an interval between surgery and RT initiation > 2 months, with disease progression observed before RT, as compared with an interval ≤ 2 months without disease progression prior to RT, was associated with worse PFS (HR = 8.50, p < 0.01) and OS (HR = 5.27, p < 0.01). Conclusions: Early and aggressive RT after surgery is critical for successful disease control in AT/RT patients. Conversely, a delay in RT until disease progression is observed that leads to unfavorable outcomes. [ABSTRACT FROM AUTHOR]

Purpose: Medulloblastoma (MB) is the most commonly occurring malignant pediatric brain tumor worldwide. However, a recent study found that the treatment outcomes in those with high-risk disease receiving conventional treatment were suboptimal. This study aimed to assess outcomes and treatment strategies for specific histologic subtypes of pediatric MB. Methods: A total of 114 pediatric patients (age < 20 years) diagnosed with MB between March 1998 and August 2011 were retrospectively reviewed; 52 that were treated with surgery followed by adjuvant radiotherapy (RT) and chemotherapy (CHT) were included. Results: The 5-year overall survival (OS) and relapse-free survival (RFS) rates were 73 and 69%, respectively. Median time to relapse was 17 months with a median survival time of 6 months after relapse. Patients of average risk had a better 5-year OS rate compared with high-risk patients ( p = 0.027). The 5-year RFS of high-risk patients was lower compared with average risk ( p = 0.038). A greater proportion of patients with large cell/anaplastic (LC/A) MB had recurrence than classic MB with 5-year RFS rate of 34 and 76%, respectively ( p = 0.001), and OS rate of 56 and 76%, respectively ( p = 0.04). Conclusion: High-risk group and histology of LC/A were the most significant factors associated with worse OS and RFS. Patients with LC/A-MB had higher relapse rates and worse survival than those with classic MB. LC/A-MB carries a high risk for recurrence and should be treated with the more aggressive strategies. [ABSTRACT FROM AUTHOR]

Background: This study was a robust examination of the clinical outcomes and technical feasibility of sequential microvascular reconstruction for recurrent or second primary oral cancer. Methods: A retrospective, cross-sectional analysis of adult patients undergoing microvascular reconstruction of head and neck oncologic defects was performed at Chang Gung Memorial Hospital, spanning 10 years. The patients were divided into three groups as follows: first episode, second episode, and third or more episodes of reconstruction. Demographics, operative details, and clinical outcomes were compared. Results: The study cohort included 3186, 319, and 62 patients who respectively received first, second, and third or more episodes of reconstruction. The most common tumor sites were the tongue (36.9 %) and the buccal region (36.8 %), with squamous cell carcinoma as the prevalent histology. The anterior lateral thigh was the most popular donor site used (76.1 %). The third or more episodes of microvascular reconstruction were associated with an increased incidence of flap failure (8.1 %) relative to the first (1.9 %; p = 0.003) and the second (1.6 %; p = 0.01) episodes. Re-exploration of venous occlusion (9.7 vs. 3.2 %), neck wound infections (53.2 vs. 35.5 %), fistula (17.7 vs. 8.1 %), and hospital stay (28.9 ± 14.6 vs. 25.3 ± 10.1) also showed significantly higher values for the third or more episodes group than for the first episode group. Conclusions: Sequential microvascular reconstruction for recurrent or second primary oral cancer is associated with an increased incidence of postoperative complications. However, in appropriate candidates for repeat ablation, microvascular reconstruction remains the gold standard for attaining functional and cosmetic outcomes. [ABSTRACT FROM AUTHOR]

Background: Tumors with epicenter in the thalamus occur in about 4 % of pediatric brain tumors. The histological diagnosis is mainly gliomas. Among them, low-grade glioma (LGG) constituted of a significant entity of the tumors (Cuccia et al., Childs Nerv Syst 13:514-521, 1997; Puget et al., J Neurosurg 106:354-362, 2007; Bernstein et al., J Neurosurg 61:649-656, 1984; Bilginer et al., Childs Nerv Syst 30:1493-1498, 2014). Since Kelly's report in 1989, >90 % resection of thalamic tumors were achieved in reported series (Ozek and Ture, Childs Nerv Syst 18:450-6, 2002; Villarejo et al., Childs Nerv Syst 10:111-114, 1994; Moshel et al., Neurosurgery 61:66-75, 2007; Albright, J Neurosurg 100(5 Suppl Pediatrics): 468-472, 2004; Kelly, Neurosurgery 25:185-195, 1989; Drake et al., Neurosurgery 29: 27-33, 1991). Materials and methods: Sixty-nine cases of thalamic tumors in children were retrospectively reviewed. There were 25 cases of LGGs. We analyzed our experience and correlated it with reported series. Results: Summing up of 4 reported series and the present series, there were 267 cases of thalamic tumors in children. Among these tumors, 107 (40.1 %) were LGGs and 91 (34.1 %) were low-grade astrocytomas (LGAs). In the present series, all of the 25 LGGs were LGAs that consisted of 11 pilocytic astrocytomas (PAs) and 14 diffuse astrocytomas (DAs). Six cases received biopsy sampling only. The remaining 19 cases received different degrees of surgical resection via several approaches. Radical (>90 %) resection was achieved better in PAs comparing with DAs. There was no operative mortality. Two patients had increased neurological deficits. In a mean follow-up period of 11.9 years, three patients died of tumor progression and one patient died of anaplastic change. The 5- and 10-year overall survival (OS) was 87.1 and 87.1 %, respectively. Conclusion: Thalamic LGGs are mainly LGAs and are indolent. The rate of >90 % resection was relatively low in the present series. By applying contemporary diagnostic MRI studies, surgical facilities, and appropriate approaches in selective cases, we may try maximum neuroprotective radical (>90 %) resection. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to investigate the incidence, clinical profiles, latency, and outcomes of radiotherapy (RT)-related intracranial aneurysms, rare but often fatal complications of cranial irradiation. Methods: We reviewed all published individual patient data regardless of language, using survival analysis to make statistical inferences. Results: We examined a total of 58 patients with RT-related intracranial aneurysms, including one unpublished case presented here, of whom 74.1 % presented with rupture. In the study, 29.3 % were younger than 18 years. The mean age at which patients received the first course of RT was 34.8 ± 22.8 years old. The mean latency between initiating RT and presenting with aneurysm was 10.4 ± 8.5 years. Rapid death ensured in 24 % shortly after presentation. The only significant predictor of death was rupture. In those with a single aneurysm, 43.1 % were located at the internal carotid artery, while 15.5 % of patients had multiple aneurysms. A male-to-female ratio of 1.87, 0.5, and 1.32 was found in patients younger than age 52, 52 years of age or older, and all 58 patients, respectively. Older age when receiving RT and presentation with ruptured aneurysm were significantly associated with shorter latency. Conclusions: RT-related intracranial aneurysms presented differently from classical ones based on age, sex, site, multiplicity, and type. Sex ratios differed with age. The younger age group showed a longer latency of occurrence of an aneurysm. Older patients and those who develop ruptured aneurysms presented earlier. Since rupture may affect outcome, early detection of aneurysms before rupture may save lives. [ABSTRACT FROM AUTHOR]

Dear Editor: We have read with interest the case reported by Basilotta Marquez et al. [[1]], in which a previously asymptomatic choroid plexus cyst (CPC) caused acute obstructive hydrocephalus in a 2-year-old boy. 1 The evolution of a choroid plexus cyst in an infant. a-f Brain sonography demonstrating the cystic lesion at 6 (a and d), 9 (b and e), and 13 (c and f) months of age, respectively. A subsequent magnetic resonance imaging (MRI) of the brain similarly showed a cystic lesion, with the signal of the content roughly isointense to that of cerebrospinal fluid (CSF) on T1- (Fig. [Extracted from the article]

Medulloblastoma (MB) is a type of malignant tumor arising only in the cerebellum that was first defined by Cushing and Bailey in 1920s. In this review paper, we trace the evolution of risk stratification and the correlated changing concept of management in the past years. Outcome analysis of the hospital series of the Taipei Veterans General Hospital, Cheng Hsin General Hospital, and Taipei Medical University Hospital was performed to correlate prognostic indicators with reported studies. The purpose is to provide clues for age-specific and risk-adjusted optimal, effective, but beneficial and protective treatment strategies of these tumors in children. [ABSTRACT FROM AUTHOR]

Background: This study aimed to identify the risk factors for postoperative plate exposure in head and neck cancer patients with composite mandibular defects undergoing tumor ablation followed by bridging plate and anterolateral thigh (ALT) flap transfer. Methods: Between January 2007 and June 2012, 1,452 patients who underwent free tissue transfer after head and neck cancer ablation were retrospectively reviewed. Only ALT flap coverage with a bridging plate for segmental mandibular defects was included. The Jewer's classification was used to define the type of mandibular defect. Results: The study enrolled 123 men and 7 women. The incidence of plate exposure was 37.7 % (49/130). The follow-up time ranged from 0.5 to 5.4 years (mean, 2.4 years). The 5-year probability of a plate exposure-free rate was 32.8 % for the patients with postoperative radiotherapy (RT) ( n = 33) and 64.3 % for the patients without it ( n = 97). Patients reconstructed with a fasciocutaneous or chimeric type of ALT flap had higher rates of plate exposure than those reconstructed with a musculocutaneous type of ALT flap ( p = 0.002). As shown by logistic regression, the significant predictive risk factors for postoperative plate exposure still were postoperative RT [adjusted odds ratio (OR) 2.76, 95 % confidence interval (CI) 1.09-6.99, p = 0.031] and intraoperative blood loss (adjusted OR 2.37, 95 % CI 1.13-4.99, p = 0.022). Conclusions: The type of ALT flap, postoperative RT, and intraoperative blood loss were the predisposing factors for increased risk of postoperative plate exposure in the specific disease group. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the treatment of intracranial nongerminomatous germ cell tumors (NGGCT) and to identify the prognostic factors for survival. Methods: Thirty-nine patients with nondisseminated NGGCTs, excluding those with pure mature teratomas, were treated between January 1985 and December 2010. Twenty-four patients received gross total or partial removal, 11 had excision biopsies, and 4 had no surgery. Radiotherapy was given postoperatively or definitively with a median tumor bed dose of 54 Gy (range 30-54) with or without craniospinal irradiation. All patients received ten cycles of adjuvant chemotherapy, vinblastine, bleomycin, etoposide, and cisplatin after radiotherapy, except for one with mixed anaplastic astrocytoma component who received oral temozolomide. Survival and prognostic factors were estimated by the Kaplan-Meier method and log-rank tests, respectively. Results: After a median follow-up of 77.7 months (range 14-336), the 6-year overall survival (OS) and progression-free survival (PFS) were 74.4 and 79.5 %, respectively. Inferior PFS was associated with lesions in the suprasellar region ( p = 0.017), poor pathological features ( p = 0.048), and with poor image ( p < 0.0001) and tumor marker (TM) response ( p = 0.003) to irradiation. Decreased OS was associated with lesions in the suprasellar region ( p = 0.026) and with poor image ( p < 0.0001) and TM response ( p = 0.027) to irradiation. Neither the extent of surgery nor the radiation field was found to significantly influence survival. Conclusions: By our multimodality approach, patients achieved comparable outcomes. Other than poor pathological features, patients with poor responses to radiotherapy are prone to early recurrence and inferior survival. These patients should be focused for more intensive adjuvant treatment. [ABSTRACT FROM AUTHOR]

Background: Focal cortical dysplasia (FCD) is a specific malformation of cortical development harboring intrinsic epileptogenicity, and most of the patients develop drug-resistant epilepsy in early childhood. The detrimental effects of early and frequent seizures on cognitive function in children are significant clinical issues. In this study, we evaluate the effects of early surgical intervention of FCD on epilepsy outcome and cognitive development. Methods: From 2006 to 2013, 30 children younger than 18 years old underwent resective surgery for FCDs at Taipei Veterans General Hospital. The mean age at surgery was 10.0 years (range 1.7 to 17.6 years). There were 21 boys and 9 girls. In this retrospective clinical study, seizure outcome, cognitive function, and quality of life were evaluated. To evaluate the effects to outcomes on early interventions, the patients were categorized into four groups according to age of seizure onset, duration of seizure before surgery, and severity of cognitive deficits. Results: Eleven of 22 (50 %) patients demonstrated developmental delay preoperatively. The Engel seizure outcome achievements were class I in 21 (70 %), class II in 2 (7 %), class III in 6 (20 %), and class IV in 1 (3 %) patients. The locations of FCDs resected were in the frontal lobe in 18 cases, temporal lobe in 7, parietal lobe in 2, and in bilobes including frontoparietal lobe in 2 and parieto-occipital lobes in 1. Eight cases that had FCDs involved in the rolandic cortex presented hemiparesis before surgical resection. Motor function in four of them improved after operation. The histopathological types of FCDs were type Ia in 1, type Ib in 7, type IIa in 7, type IIb in 12, and type III in 3 patients. FCDs were completely resected in 20 patients. Eighteen (90 %) of them were seizure free ( p < 0.001) with three patients that received more than one surgery to accomplish complete resection. The patients who had early seizure onset, no significant cognitive function deficit, and early surgical intervention with complete resection in less than 2 years of seizure duration showed best outcomes on seizure control, cognitive function, and quality of life. Conclusion: Delay in cognitive development and poor quality of life is common in children treated for FCDs. Early surgical intervention and complete resection of the lesion help for a better seizure control, cognitive function development, and quality of life. FCDs involved eloquent cortex may not prohibit complete resection for better outcomes. [ABSTRACT FROM AUTHOR]

The article presents case study of a 7-year-old boy diagnosed with hyponatremia and hypokalemia and showed body hair growth, laryngeal prominence and rapid height increases. It mentions physical examinations showing an increase in the bone age, and serologic hormone survey revealing high levels of human chorionic gonadotropin. It also mentions that craniotomy condusted for a total tumor removal.

Purpose: The purpose of the study is to determine corticospinal organization using intraoperative neurophysiologic monitoring (IONM) during resective epilepsy surgery for patients with congenital hemiparesis and intractable epilepsy. Methods: Ten patients, aged 3-17, with intractable epilepsy underwent resective surgery. Transcranial stimulation (TCS) was achieved using a pair of cork screws at Cz and C3/C4, respectively. A 1 × 4 stimulating electrode strip was placed on the presumed motor cortex of the affected hemisphere for direct cortical stimulation (DCS) after craniotomy. Multipulse TCS and DCS train stimulation was delivered, with simultaneous recordings from bilateral abductor pollicis brevis and abductor halluces, to determine the corticospinal projection pattern of the paretic limbs. Results: The above mapping techniques revealed ipsilateral corticospinal projections from the contralesional hemisphere to target muscles in the paretic limbs in three patients, projections from both hemispheres to target muscles in three, and preserved crossed projections from the affected hemisphere in four. Nine patients were seizure free after surgery. Five had unchanged postoperative functional status, and three showed minimally improved use of the paretic hand. Two developed new motor deficits after surgery, which may have been due to a premotor syndrome in one patient, since it completely resolved within 2 weeks. The other experienced increased weakness of the paretic lower limb because a small part of the eloquent cortex was removed for better seizure control. Conclusions: Using IONM to define the corticospinal projection pattern is a valuable technique that can potentially replace preoperative fMRI and transcranial magnetic stimulation in resective epilepsy surgery, particularly for younger patients. [ABSTRACT FROM AUTHOR]

This study evaluated whether people with ankylosing spondylitis (AS) and spondyloarthritis are at higher risk of type 2 diabetes mellitus (T2DM). We used a sub-dataset of the National Health Insurance Research Database from 1996 to 2010 to established a AS cohort consisting new patients with AS or spondyloarthritis ( N = 7,778) and a non-AS cohort without the diseases ( N = 31,112). Incidences of T2DM in the two cohorts, hazard ratios (HRs) of risk of T2DM in association with AS, and cumulative probability of having T2DM were estimated by the end of 2010. The incidence of T2DM was 1.17-fold higher in the AS cohort than in the non-AS cohort (13.5 vs. 11.5, per 1,000 person-years), with an adjusted HR of 1.16 (95 % CI = 1.05-1.29). The T2DM incidence was higher for women than for men; while the Cox model measured sex-specific adjusted HR of T2DM was higher for men than for women. The incidence rate of T2DM increased with age in both cohorts, while the age-specific measures showed that the adjusted HR of T2DM was higher in young AS patients (≤50 years of age) than older ones, compared to their peers of non-AS group. The plot of Kaplan-Meier analysis showed that the overall probability of having T2DM was 2 % higher in the AS cohort than in the non-AS cohort (log-rank test: p < 0.0001). Patients with AS and spondyloarthritis have an increased risk of developing T2DM. [ABSTRACT FROM AUTHOR]

Introduction: Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida. Case report: Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant. [ABSTRACT FROM AUTHOR]

Purpose: This study was designed to investigate health-related quality of life (HRQL) in children and adolescents with spinal dysraphism and to determine the differences between the myelomeningocele (MMC) and lipomyelomeningocele (LMMC) groups. Methods: Thirty-two patients (mean age, 13.2 years) with MMC and 28 patients (mean age, 11.3 years) with LMMC participated in this study. HRQL was measured using the Child Health Questionnaire-Parent Form 50 (CHQ-PF50). The CHQ-PF50 scores for the study participants were compared with those of a reference sample of healthy Taiwanese children. Outcomes for children with MMC were compared with those for children with LMMC. We also explored the relationships of the lesion level and associated medical problems with HRQL in these subjects. Results: Patients with MMC had significantly lower scores on the physical and psychosocial aspects of CHQ-PF50 than did healthy children. Patients with LMMC had lower scores on some domains but had comparable psychosocial summary scores. Significant differences were noted between the MMC and LMMC groups for all subscales and both summary scores. Children with a higher lesion level had poor physical health ( r = −0.52, P < 0.001) but not poor psychosocial health. The number of associated medical problems, indicating their severity, was related to both physical ( r = −0.77, P < 0.001) and psychosocial health ( r = −0.57, P < 0.001). Conclusions: Children and adolescents with MMC have reduced HRQL compared with those with LMMC. Children and adolescents with more associated medical problems have the greatest risk of diminished physical and psychosocial well-being. [ABSTRACT FROM AUTHOR]

Background: Rhabdoid glioblastoma is a rare type of recently described malignant brain tumor. It is characterized by a glioblastoma associated with rhabdoid components. Methods: Here we report two cases of rhabdoid glioblastoma and a brief literature review. The first patient was a 19-year-old boy who initially presented with a foul-smelling odor and progressive right-side weakness. The second case was a 29-year-old male patient who presented only with a severe headache. Results: Both of these patients were young, and the disease progression was quick despite optimal treatment. Conclusion: The diagnosis of rhabdoid glioblastoma was confirmed after microscopic and immunohistochemical findings. [ABSTRACT FROM AUTHOR]

The article presents a case study of six patients of Langerhans' cell histiocytosis (LCH) in the pediatric spine who underwent focal low-dose radiotherapy. The diagnosis in four patients was established by pathological report, and the other two were diagnosed through clinical course, radiological findings and response to radiotherapy. The patients underwent definitive or postoperative low-dose radiotherapy which involved four to six megavolt (MV) linear accelerators.

Introduction: Sciatic neuropathy is a rare but serious complication following surgery performed in the sitting position. Methods: We report a case of a 12-year-old boy who developed bilateral sciatic neuropathy after a 9-h-long craniotomy while in the sitting position. Results: Magnetic resonance imaging for the lower extremities revealed bilateral swelling of the sciatic nerves near the ischial tuberosities. Conclusion: A heightened awareness of sciatic nerve injury during prolonged surgery in the sitting position may make early diagnosis possible and allow prevention of further neurologic injury. [ABSTRACT FROM AUTHOR]

Background: Significant variability in weight loss and glycemic control has been observed in obese patients receiving bariatric surgery. Genetic factors may play a role in the different outcomes. Methods: Five hundred and twenty severely obese patients with body mass index (BMI) ≥35 were recruited. Among them, 149 and 371 subjects received laparoscopic adjustable gastric banding (LAGB) and laparoscopic mini-gastric bypass (LMGB), respectively. All individuals were genotyped for five obesity-related single nucleotide polymorphisms on ESR1, FTO, PPARγ, and UCP2 genes to explore how these genes affect weight loss and glycemic control after bariatric surgery at the 6th month. Results: Obese patients with risk genotypes on rs660339-UCP2 had greater decrease in BMI after LAGB compared to patients with non-risk genotypes (−7.5 vs. −6 U, p = 0.02). In contrast, after LMGB, obese patients with risk genotypes on either rs712221-ESR1 or rs9939609-FTO had significant decreases in BMI (risk vs. non-risk genotype, −12.5 vs. −10.0 U on rs712221, p = 0.02 and −12.1 vs. −10.6 U on rs9939609, p = 0.04) and a significant amelioration in HbA1c levels ( p = 0.038 for rs712221 and p < 0.0001 for rs9939609). The synergic effect of ESR1 and FTO genes on HbA1c amelioration was greater (−1.54%, p for trend <0.001) than any of these genes alone in obese patients receiving LMGB. Conclusions: The genetic variants in the ESR, FTO, and UCP2 genes may be considered as a screening tool prior to bariatric surgery to help clinicians predict weight loss or glycemic control outcomes for severely obese patients. [ABSTRACT FROM AUTHOR]

Background: Tumor-associated hydrocephalus is common in primary pediatric brain tumors. The managements involve radical tumor resection, temporary external ventricular drainage, and different definite shunting procedures. The purpose of this study is to sum up our experience of definite shunting procedures for tumoral hydrocephalus in children and correlate with reported literatures. Methods: This is a retrospective review of a series of 1,250 cases of primary pediatric brain tumors in patients <18 years of age collected in Taipei Veterans General Hospital from 1971 to 2008. Cases with questionable records about hydrocephalus were excluded. Results: A total of 56.7% of cases presented hydrocephalus, including hydrocephalus that occurred at tumor diagnosis (51.5%), and hydrocephalus developed after tumor diagnosis (5.1%). At tumor diagnosis, the hydrocephalus was mainly obstructive type (98%) and rarely communicating type (1.9%). Definite shunting procedures in this series comprised of ventriculoperitoneal (VP) shunt in 54.4%, endoscopic third ventriculostomy (ETV) in 10.9%, subduroperitoneal (SP) shunt in 4.8%, septostomy in 0.7%, lumboperitoneal shunt in 0.6%, and ventriculoatrial shunt in 0.1% of patients with hydrocephalus. There was a tendency of decreasing requirement and changing timing for VP shunt implantation. There was a gradual increase in usage of ETV for hydrocephalus in specific types and locations of tumors. Conclusion: In the past two decades, we tended to use the VP shunt more cautiously for obstructive tumoral hydrocephalus. We try to perform initial radical resection of tumors as indicated and the more frequent use of ETV in selective cases that help to decrease the requirement of VP shunt implantation. [ABSTRACT FROM AUTHOR]

Hypertensive rats with chronic kidney disease (CKD) exhibit enhanced gamma-aminobutyric acid (GABA)B receptor function and regulation within the nucleus tractus solitarii (NTS). For CKD with hypertension, renal denervation (RD) interrupts the afferent renal sympathetic nerves, which are connecting to the NTS. The objective of the present study was to investigate how RD improves CKD-induced hypertension. Rats underwent 5/6 nephrectomy for 8 weeks, which induced CKD and hypertension. RD was induced by applying phenol to surround the renal artery in CKD. RD improved blood pressure (BP) by lowering sympathetic nerve activity and markedly restored the baroreflex response in CKD. The GABAB receptor expression was increased in the NTS of CKD; moreover, the central GABA levels were reduced in the cerebrospinal fluid, and the peripheral GABA levels were increased in the serum. RD restored the glutamic acid decarboxylase activity in the NTS in CKD, similar to the effect observed for central treatment with baclofen, and the systemic administration of gabapentin reduced BP. RD slightly improved renal function and cardiac load in CKD. RD may improve CKD-induced hypertension by modulating the baroreflex response, improving GABA system dysfunction and preventing the development and reducing the severity of cardiorenal syndrome type 4 in CKD rats. [ABSTRACT FROM AUTHOR]

A correction to the article "ESR1, FTO, and UCP2 Genes Interact With Bariatric Surgery Affecting Weight Loss and Glycemic Control in Severely Obese Patients," by Tsan-Hon Liou and colleagues that was published online on December 7, 2011 is presented.