Treatment outcomes and risk factors of patients with intracranial germ cell tumour with choriocarcinoma element or ß-HCG level higher than 500 IU/L.

BACKGROUND: In previous studies, patients with intracranial germ cell tumour (iGCT) with pure choriocarcinoma or mixed germ cell tumours with choriocarcinoma element showed similar dismal prognoses, with median overall survival (OS) of 22 months and 1-year survival rate of approximately 60%. However, these conclusions need to be updated because radiotherapy, which is the mainstay for this disease, was not applied in a number of patients. Additionally, prognostic factors need to be explored in this population. METHODS: Clinical data of patients with iGCTs with histologically confirmed choriocarcinoma element or beta-human chorionic gonadotropin (ß-HCG) > 500 IU/L were collected from the archives of our institution and retrospectively studied. RESULTS: A total of 76 patients were eligible for this study. Except for two early deaths, all patients received radiotherapy (craniospinal irradiation [CSI], n = 23; non-CSI, n = 51). The median follow-up duration for the entire series was 63 months (range, 6-188 months). The 5-year event-free survival (EFS) and OS rates were 81.5% and 84.1%, respectively. Among patients who did not have early death or progressive disease after induction chemotherapy, multivariate analysis revealed that chemotherapy cycles (> 4 vs. ≤ 4) (hazard ratio [HR] for EFS 0.144, p = 0.020; HR for OS 0.111, p = 0.028) and ß-HCG levels (> 3000 IU/L vs. ≤ 3000 IU/L) (HR for EFS 4.342, p = 0.059; HR for OS 6.614, p = 0.033) were independent factors for survival. CONCLUSIONS: Patients with iGCTs with choriocarcinoma element or ß-HCG > 500 IU/L showed improved survival with radiotherapy-based treatments. Additional chemotherapy cycles could result in additional survival benefits. Patients with ß-HCG level > 3000 IU/L had poorer prognosis.

A nomogram for predicting post-operative hydrocephalus in children with medulloblastoma.

Post-operative hydrocephalus is common among children with medulloblastoma after initial tumor resection. This study aimed to establish a novel model for predicting the development of post-operative hydrocephalus in children with medulloblastoma. Only pediatric patients who received initial medulloblastoma resection at Beijing Tiantan Hospital between January 2018 and May 2021 were included in this study. The potential risk factors associated with post-operative hydrocephalus were identified based on multivariate logistic regression and the nomogram. Receiver operating characteristic (ROC) curve were used to evaluate the performance of the nomogram model based on an independent cohort of medulloblastoma patients who underwent surgery from June 2021 to March 2022. A total of 105 patients were included in the primary cohort. Superior invasion (P = 0.007), caudal invasion (P = 0.025), and intraventricular blood ≥ 5 mm (P = 0.045) were significantly related to the development of post-operative hydrocephalus and thus were assembled into the nomogram model. The model accurately predicted post-operative hydrocephalus based on the calibration curve. The area under the ROC curves for the primary and validation cohorts was 0.849 and 0.855, respectively. In total, the nomogram we developed may aid clinicians in assessing the potential risk of pediatric patients with MB developing post-operative hydrocephalus, especially those who would otherwise not have received a diversionary procedure at presentation.

Bilateral ultrasound-guided erector spinae plane block versus wound infiltration for postoperative analgesia in lumbar spinal fusion surgery: a randomized controlled trial.

PURPOSE: Both erector spinae plane block and wound infiltration are used to improve analgesia following spinal fusion surgery. Herein, we compared the analgesic effect of bilateral erector spinae plane block with wound infiltration in this patient population. METHODS: In this randomized trial, 60 patients scheduled for elective open posterior lumbar interbody fusion surgery were randomized to receive either ultrasound-guided bilateral erector spinae plane block before incision (n = 30) or wound infiltration at the end of surgery (n = 30). Both groups received standardized general anesthesia and postoperative analgesia, including patient-controlled analgesia with sufentanil and no background infusion. Opioid consumption and pain intensity were assessed at 2, 6, 12, 24, and 48 h after surgery. The primary outcome was cumulative opioid consumption within 24 h after surgery. RESULTS: All 60 patients were included in the intention-to-treat analysis. The equivalent dose of sufentanil consumption within 24 h was significantly lower in patients given erector spinae plane block (median 11 µg, interquartile range 5-16) than in those given wound infiltration (20 µg, 10 to 43; median difference - 10 µg, 95% CI - 18 to - 3, P = 0.007). The cumulative number of demanded PCA boluses was significantly lower with erector spinae plane block at 6 h (median difference - 2, 95% CI - 3 to 0, P = 0.006), 12 h (- 3, 95% CI - 6 to - 1, P = 0.002), and 24 h (- 5, 95% CI - 8 to - 2, P = 0.005) postoperatively. The proportion given rescue analgesia was also significantly lower in patients given erector spinae plane block group within 48 h (relative risk 0.27, 95% CI 0.07 to 0.96, P = 0.037). There were no statistical differences in pain intensity at any timepoints between groups. No procedure-related adverse events occurred. CONCLUSIONS: Compared with wound infiltration, bilateral ultrasound-guided erector spinae plane block decreases short-term opioid consumption while providing similar analgesia in patients following lumbar spinal fusion surgery. Chinese Clinical Trial Registry: ChiCTR2100053008.

Treatment and outcome of pediatric intracranial ependymoma after first relapse.

INTRODUCTION: Almost 50% of children with intracranial ependymoma experience disease relapse, and their outcomes are extremely poor. The aim of this study was to investigate optimal salvage treatment for pediatric intracranial ependymoma after the first relapse and to identify prognostic factors affecting survival. METHODS: We conducted a retrospective analysis of 159 children who underwent initial treatment for intracranial ependymoma at Beijing Tiantan Hospital from 2013 to 2017. RESULTS: Relapse was observed in 73 patients (73/159; 45.9%), with a median age of 7.2 ± 3.5 years old. Molecular subgrouping analysis identified H3K27me3-negative PF-EPNs in 74% of patients, ST-RELA EPNs in 21% of patients, and H3K27me3-positive PF-EPNs in 5% of patients. The 5-year event-free survival (EFS) and overall survival (OS) rates after first relapse were 21.1% (95% CI 16.0-26.2) and 30.5% (95% CI 19.8-30.8), respectively. Patients with GTR at first relapse had higher 5-year EFS and 5-year OS than those with STR (P = 0.031 and P = 0.003) or no surgery (P = 0.007 and P = 0.001). Radiotherapy or re-radiotherapy at first relapse significantly prolonged 5-year EFS and OS (both P < 0.001). Patients with H3K27me3-negative PF-EPN had worse 5-year EFS and OS than those with ST-RELA EPN (P = 0.001 and P = 0.002). Multivariate analysis showed that both tumor resection and radiotherapy at first relapse had independent prognostic significance for survival (all P < 0.05). CONCLUSION: Children with recurrent intracranial EPN have poor outcomes, and surgery and radiotherapy at first relapse should be encouraged to improve their prognosis.

Two-stage treatment for severe spinal kyphotic deformity secondary to tuberculosis: halo-pelvic traction followed by a posterior-only approach correction.

BACKGROUND AND PURPOSE: Several surgical procedures are used to treat tuberculous kyphosis. However, the treatment of extreme spinal kyphosis is challenging, and associated with various complications. Halo traction has been used as an adjunctive method in the treatment of severe spinal deformities. However, there are few reports about the effectiveness of halo-pelvic traction (HPT) for the treatment of extreme spinal kyphosis secondary to tuberculosis. This study evaluated the effectiveness of halo-pelvic traction followed by a posterior-only approach correction in the treatment of severe spinal kyphosis secondary to tuberculosis. METHODS: The records of 19 patients with severe spinal kyphosis secondary to tuberculosis were retrospectively reviewed. All 19 patients were treated with a two-stage approach: HPT combined with posterior fusion surgery by a posterior-only approach. Radiographic parameters were measured and evaluated. America Spinal Injury Association grade (ASIA), Scoliosis Research Society outcome (SRS-22) score, and complications were also evaluated. RESULTS: There were 9 males and 10 females, with an average age of 29.7 years at the time of surgery. The average HPT duration was 10.4 weeks. The mean kyphosis angle decreased from 131.40 ± 10.7° pre-traction to 77.1 ± 7.4° post-traction (P < 0.01). The traction correction rate was 41.3%. The mean postoperative kyphosis angle was 65.7 ± 8.5°, and the surgical correction rate was 8.7%. Of the total correction, 82.6% was the result of HPT. At a mean follow-up of 26.5 months, the average kyphosis correction loss was 2.9°. The mean sagittal balance was 11.1 ± 45.2 mm before traction, - 25.0 ± 37.4 mm after traction, 7.0 ± 13 mm after surgery, and 2.8 ± 9.6 mm at the final follow-up. The mean preoperative SRS-22 score was 3.0 and postoperative was 4.2 (P < 0.01). The neurological status of most patients was improved. The total complication rate was 15.7%, including 1 neurological and 2 non-neurological complications. CONCLUSIONS: HPT is effective in the management of severe spinal kyphotic deformity secondary to tuberculosis. Preoperative HPT can greatly reduce global kyphosis, and the need for corpectomy.

Hybrid surgery with PEEK rods for lumbar degenerative diseases: a 2-year follow-up study.

BACKGROUND: Finite element analyses and biomechanical tests have shown that PEEK rods promote fusion and prevent adjacent segment degeneration. The purpose of this study was to evaluate the effects and complications of hybrid surgery with PEEK rods in lumbar degenerative diseases. METHODS: From January 2015-December 2017, 28 patients who underwent lumbar posterior hybrid surgery with PEEK rods were included in the study. The patients were diagnosed with lumbar disc herniation, lumbar spinal stenosis, or degenerative grade I spondylolisthesis. Before the operation and at the last follow-up, the patients completed lumbar anteroposterior and lateral X-ray, dynamic X-ray, MRI examinations. In addition, at the last follow-up the patients also completed lumbar CT examinations. The radiographic parameters, clinical visual analog scale (VAS) score and Oswestry disability index (ODI) score were compared. RESULTS: The average age of the patients was 44.8 ± 12.6 years, and the average follow-up duration was 26.4 ± 3.6 months. The VAS score improved from 6.3 ± 1.6 to 1.0 ± 0.9, and the ODI score decreased from 38.4 ± 10.8 to 6.8 ± 4.6. The fusion rate of the fused segment was 100%. There were no significant changes in the modified Pfirrmann classifications or disc height index for the nonfused segments and the upper adjacent segments from pre- to postoperatively. No cases of screw loosening, broken screws, broken rods or other mechanical complications were found. CONCLUSION: Hybrid surgery with PEEK rods for lumbar degenerative diseases can yield good clinical results and effectively reduce the incidence of complications such as adjacent segment diseases.

Halo-pelvic traction for thoracic spine dislocation in neurofibromatosis type 1: a case series.

PURPOSE: To present a case series of 6 patients with Neurofibromatosis type 1 (NF-1) who had severe kyphoscoliosis with vertebral dislocation, and were treated with halo-pelvic traction (HPT) and posterior fusion. To discuss the pathological characteristics of this rare entity, and report our experience using HPT to manage the condition. METHODS: From March, 2016 to September, 2017, 6 patients with severe kyphoscoliosis with vertebral dislocation were admitted for HPT and posterior surgery, and were followed for 2 years. RESULTS: The 6 patients received HPT for an average of 42 days. The average scoliosis Cobb angle was 131.2° before HPT, 69.9° after HPT, 52.7° after posterior surgery, and 51.7° at the 2-year follow-up. The average 2-year correction rate was 61.7%. The average kyphosis Cobb angle was 125.2° before HPT, 62.9° after HPT, 62.2° after posterior surgery, and 64.9° at the 2-year follow-up. The average 2-year correction rate was 46.5%. Proximal screw pull-out was found in one patient at the 3-month follow-up visit, but revision surgery was not needed. No patients experienced permanent neurological deficits or death. No screw malposition occurred in any patient. CONCLUSION: HPT is a viable treatment option for vertebral dislocation in NF-1. After 6 weeks of traction, the vertebral dislocation, scoliosis, and kyphosis can be dramatically reduced, and thus the conditions for corrective surgery can be improved.

Stereoelectroencephalography-guided radiofrequency thermocoagulation for hypothalamic hamartoma: Electroclinical patterns and the relationship with surgical prognosis.

BACKGROUND: Radiofrequency thermocoagulation (RFTC) guided by stereo-electroencephalography (SEEG) has been proved to be a safe method to reduce seizure frequency in patients with drug-resistant epilepsy. However, there are few reports addressing the value and safety of this procedure in hypothalamic hamartoma (HH). OBJECTIVE: To present the results of our experience using SEEG-guided RFTC in HH patients with drug-resistant epilepsy, and identify outcome predictors. METHODS: We retrospectively reviewed the clinical and surgical characteristics of 27 HH-related patients with epilepsy in our center between 2015 and 2019. All patients underwent invasive recordings with SEEG before RFTC was performed. We reported surgical outcome predictors and postoperative follow-up concerning safety and efficacy (mean follow-up, 27.3 months; range, 12-63). Surgical strategy was also analyzed. RESULTS: Nineteen patients (70.4%) achieved Engel's class I outcome, while 4 patients (14.8%) did not show significant improvement. Of all observed seizures, two different onset patterns of intracranial electrophysiology recorded by SEEG were observed. Patients presented with focal low-voltage fast activity were more likely to obtain seizure freedom (p = 0.045), while classification (p = 0.478), volume (p = 0.546), history of resection (p = 0.713), seizure types (p = 0.859), or seizure duration (p = 0.415) showed no significant effect on the outcome. Weight gain was the most common long-term complication (18.5%). CONCLUSION: The SEEG can guide the ablation of HH and serve as an important factor to predict favorable seizure outcomes. Radiofrequency thermocoagulation guided by SEEG can offer a minimally invasive and low-risk surgical approach with excellent outcomes. Disconnecting the attachment of HH should be the appropriate strategy to obtain the best seizure outcome.

Factors impacting time to diagnosis in pediatric CNS tumors in Chinese children.

OBJECTIVE: This study aimed to investigate the factors impacting time to diagnosis in pediatric central nervous system tumors. METHODS: A descriptive, cross-sectional design was used in this study. A self-developed questionnaire for health-seeking behavior and influencing factors was used in children with intracranial tumors. The factors related to time to diagnosis and the long-term prognosis of children were analyzed. RESULTS: A total of 433 families replied to the questionnaire. The median parental interval was 50 days (range 0 ~ 884), the median diagnostic interval was 97 days (range 4 ~ 1646), and the median prediagnostic symptomatic interval (PSI) was 123 days (range 8 ~ 1844). Higher education was associated with a shorter parental interval (mother: P = 0.048; father: P = 0.035). The diagnostic interval was shortened in patients with dizziness (P = 0.022), abnormal eye movement (P = 0.034), or drowsiness (P = 0.021). A shorter PSI was observed in patients who presented with high intracranial pressure such as headache (P = 0.016), dizziness (P = 0.009), or drowsiness (P = 0.023) and those who went to a higher-level health institution or patients who went to neurology or neurosurgery department as the first medical consultation. No statistically significant difference was found in the interval time (parental interval, diagnostic interval, and PSI) regarding patients' outcomes. CONCLUSION: Different time intervals showed different factors influencing the long delay in diagnosing central nervous system tumors, highlighting the need for increased awareness to improve the treatment efficacy.

Retrospective investigation of hereditary syndromes in patients with medulloblastoma in a single institution.

PURPOSE: To investigate the incidence rate of hereditary disease in patients with medulloblastoma. METHODS: The genetic reports of 129 patients with medulloblastoma from January 2016 to December 2019 were retrospectively analyzed. A panel sequence of 39 genes (Genetron Health) were used for all patients to evaluate the tumor subgroup. Four genes (TP53, APC, PTCH1, SUFU) were screened to routinely rule out germline mutation. RESULTS: Five patients (3.9%) were found with hereditary disease, and all belonged to the sonic hedgehog (SHH) subgroup. Two patients were retrospectively diagnosed with Gorlin-Goltz disease with germline PTCH1 and SUFU mutations. One patient (PTCH1 mutation) accepted whole craniospinal irradiation and had scalp nevoid basal cell carcinoma 5 years later. The other patient (SUFU mutation) accepted chemotherapy and had local tumor relapse 1 year later. Three patients were diagnosed with Li-Fraumeni syndrome and carried the TP53 mutation; all three patients died. One of the patients had bone osteosarcoma, while all three had early tumor relapse. CONCLUSION: Patients with SHH medulloblastoma should routinely undergo genetic testing. We propose that whole genome, whole exome sequence, or custom-designed panel-targeted exome sequencing should be performed.

Minimally invasive surgery versus standard posterior approach for Lenke Type 1-4 adolescent idiopathic scoliosis: a multicenter, retrospective study.

PURPOSE: To compare the safety and efficacy of posterior minimally invasive surgery (MIS) to standard posterior spinal fusion (PSF) surgery for Lenke Type 1-4 adolescent idiopathic scoliosis (AIS). METHODS: This multicenter retrospective study enrolled 112 patients with Lenke Type 1-4 AIS who treated with MIS (n = 64) or PSF (n = 48) between March 2007 and January 2015. Coronal and sagittal parameters were evaluated before surgery, immediately after surgery, and at the last follow-up. Operative time, level of fusion, intraoperative blood loss, blood transfusion, and intraoperative radiation exposure were recorded. 22-item Scoliosis Research Society questionnaire (SRS-22) was applied for assessment of life quality. The accuracy of pedicle screw placement was assessed according to postoperative computed tomography images, and the complications were collected in follow-up period. RESULTS: The baseline characteristics of 2 groups were matched. There was no significant difference between 2 groups in terms of radiographic parameters immediately after surgery and at the last follow-up. The MIS group had significantly longer operative time, more level of fusion, less intraoperative blood loss, and lower blood transfusion rate (p < 0.001). The evaluation of pain using SRS-22 showed significantly lower score in MIS group (p < 0.05). No significant difference was found between 2 groups in terms of accuracy of pedicle screw placement and complications. CONCLUSION: Posterior MIS is a safe and effective alternative to standard open approach for Lenke Type 1-4 AIS patients with curves < 70° and reasonable flexibility. Mid-term results showed MIS had the advantages of less blood loss and pain with more fusion segments.

One-level mini-open pedicle subtraction osteotomy for treating spinal kyphosis in patients with ankylosing spondylitis.

BACKGROUND: To report a mini-open pedicle subtraction osteotomy (PSO) technique, to summarize the 2-year follow-up results of 25 patients, and to describe a modified operating table which allows the osteotomy to be closed in a more controllable manner. METHODS: We retrospectively reviewed the records of patients with AS who received one-level mini-open PSO between July 2015 and January 2018. The 25 patients with complete medical records and 2-year radiographic follow-up were included in the analysis. Estimated blood loss, operation time, incision length, complications, bed rest period, and length of hospitalization were extracted from the medical records and recorded. RESULTS: The mean age of the 25 patients (22 males and 3 females) was 39.5 years. The average global kyphosis(GK) decreased from 70.2° before surgery to 22.3° after surgery; the average C7- sagittal vertical axis (C7-SVA) decreased from 15.5 cm before surgery to 5.1 cm after surgery; the average pelvic incidence(PT) decreased from 37.8° before surgery to 22.5° after surgery. The average length of the incision was 10.2 cm. The average surgical time was 263.0 min, the average estimated blood loss was 840.0 ml, and the average time to mobilization was 4.1 days. CONCLUSIONS: The current report shows that one-level PSO can be performed through an incision of about 10 cm. The one-level mini-open PSO could be superior to traditional PSO surgery with respect to cosmetic outcomes. Further comparative studies are necessary to evaluate the current and conventional techniques.

Prognostic value of H3K27me3 in children with ependymoma.

OBJECTIVE: To investigate the expression of H3K27me3 in different anatomical sites and analyze its prognostic value in children with ependymoma. METHODS: A total of 188 children diagnosed with ependymoma were admitted to the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, between 2012 and 2017, and regular follow-up was conducted. Expression of H3K27me3 was analyzed by immunohistochemistry and scored semiquantitatively. The prognostic correlation was analyzed by Kaplan-Meier and Cox regression survival analyses. RESULTS: Of the 188 children with ependymoma, 61.7% were male, and the median and average age was five years (0-17 years) and 6.26 years, respectively. There were 65 cases of supratentorial ependymoma, 115 cases of infratentorial ependymoma, and 8 cases of spinal cord ependymoma. The median follow-up time was 39.95 months (0.3-90.19 months). Five-year progression-free survival (PFS) and overall survival (OS) were 48.5% and 61.4%, respectively. Kaplan-Meier univariate survival analysis showed that H3K27me3 expression had significant effects on PFS (P = 0.0003) and OS (P < 0.0001) in infratentorial ependymoma, but only affected OS (P = 0.03) in supratentorial ependymoma. CONCLUSION: In Chinese children, infratentorial ependymoma with incomplete resection and no adjuvant radiotherapy is associated with poor OS. On the other hand, low expression of H3K27me3 indicates poor prognosis of infratentorial ependymoma, but it has no significant prognostic value for supratentorial ependymoma. In addition, high expression of H3K27me3 in spinal ependymoma may indicate a better prognosis.

Clinical efficacy of short-term pre-operative halo-pelvic traction in the treatment of severe spinal deformities complicated with respiratory dysfunction.

BACKGROUND: Halo traction has been used as an adjunctive method in the treatment of severe spinal deformities. But there are few reports on the clinical efficacy of halo-pelvic traction (HPT) in the treatment of severe spinal deformities complicated with respiratory dysfunction. This study was to evaluate the clinical efficacy and complications associated with pre-operative HPT in the treatment of severe spinal deformities with respiratory dysfunction. METHODS: Thirty patients with severe spinal deformities complicated with respiratory dysfunction treated with short-term pre-operative HPT were retrospectively reviewed. Inclusion criteria were: (1) patients with severe kyphoscoliosis (coronal Cobb angle or kyphosis angle ≥100°) and respiratory failure, (2) patients undergoing HPT until posterior fusion surgery. All patients underwent general anesthesia for HPT application, which the pelvic ring used in this study was a half-ring, and the rods were all placed on the anterolateral side of the truck. RESULTS: The major coronal curve scoliosis averaged 116.00 ± 16.70° and was reduced to 63.23 ± 14.00° after HPT, 46.33 ± 10.70° after surgery. The major kyphosis was 102.40 ± 27.67° and was reduced to 52.23 ± 14.16° after HPT, 42.0 ± 11.92° after surgery. A significantly increased FVC was observed after HPT (p < 0.001), with a significantly improved FVC% (p < 0.001). Similarly, a significantly increased FEV1 was also observed (p < 0.001), with a significantly improved FEV1% (p < 0.001). CONCLUSION: This study indicated that the modified HPT could be used to help patients with severe spinal deformities complicated with respiratory dysfunction achieve significant correction in both the coronal and sagittal deformities during the pre-operative treatment period along with improved respiratory function and in the absence of severe complications.

[Clinical effect of surgery combined with chemotherapy and radiotherapy in children with central primitive neuroectodermal tumor and prognostic analysis].

OBJCTIVE: To study the clinical effect of surgery combined with chemotherapy and radiotherapy in children with central primitive neuroectodermal tumor (cPNET), as well as the risks factors for poor prognosis. METHODS: A retrospective analysis was performed for the clinical data of 42 children who were diagnosed with cPNET from June 2012 to September 2018. RESULTS: The 42 children had a median overall survival (OS) time of 2.0 years and a median event-free survival (EFS) time of 1.3 years; the 1-, 3-, and 5-year OS rates were 76.2%±6.6%, 41.4%±8.7%, 37.3%±8.8% respectively, and the 1-, 3-, and 5-year EFS rates were 64.3%±7.4%, 32.7%±8.0%, 28.0%±8.1% respectively. The univariate analysis showed that there were significant differences in the OS and EFS rates among the children with different patterns of surgical resection, chemotherapy cycles, and risk grades (P<0.05), and there was also a significant difference in the OS rate between the children receiving radiotherapy and those not receiving radiotherapy (P<0.05). The multivariate Cox regression analysis showed that chemotherapy cycles and risk grade were independent influencing factors for EFS and OS rates (P<0.05). The EFS and OS rates increased with the increase in chemotherapy cycles and the reduction in risk grade. CONCLUSIONS: Multimodality therapy with surgery, chemotherapy, and radiotherapy is an effective method for the treatment of cPNET in children. Early diagnosis and treatment and adherence to chemotherapy for as long as possible may improve EFS and OS rates.

Surgical consideration in Hunter syndrome: a case of hydrocephalus and a case of epidural hematoma.

INTRODUCTION: Hunter syndrome (HS) is a rare X-linked lysosomal storage disorder which affects multiple organ systems. Surgical intervention and general anesthesia should be used with caution because of significant airway complications. CASE REPORT: Two HS patients underwent surgery with different prognosis are presented below. In the first case, symptoms of progressive disabilities on motor function, language, intelligence, and development last for 1 year in a 6-year-old boy; magnetic resonance imaging (MRI) showed severe hydrocephalus. Third ventriculostomy was performed in this patient to relieve the hydrocephalus. Unfortunately, this patient died postoperatively due to postsurgical tracheal collapse. In the second case, an 8-year-old girl was referred to our hospital with epidural hematoma because of a falling accident. Trephination surgery was performed under local anesthesia to remove the hematoma. Three days postsurgical, the patient was discharged uneventfully. CONCLUSION: General anesthesia in HS patients was associated with poor prognosis due to respiratory complications. Local anesthesia and less intensified treatment should be recommended.

Establishing reproducible predictors of cerebellar mutism syndrome based on pre-operative imaging.

PURPOSE: To establish some explicit, feasible, and reproducible predictors for CMS. MATERIALS AND METHODS: This study was a retrospective case study. Data were obtained from 82 patients with medulloblastoma at a single center, Beijing Tiantan Hospital. Based on medical records, we created two independent samples: the CMS group comprising 23 patients and the non-CMS group comprising 23 patients. Pre-operative imaging was studied by performing quantitative assessments of specific indicators. RESULTS: The CMS group showed greater differences in pre-operative imaging data with the non-CMS group. The Aaxi/daxi ratio in pre-operative MR imaging captured in the axial plane was used to quantify the compression of the cerebellum and brainstem, and significant differences were observed between the CMS group and non-CMS group (p = 0.0002). In the sagittal plane, Dsag*dsag was used to quantify the area of the tumor that invaded the brainstem, and significant differences were observed between the two groups (p = 0.0003). In the coronal plane, Acor/dcor was used to quantify the compression of the upper functional brain region, and significant differences were noted between the two groups (p = 0.0219). Additionally, Evans' index was introduced to quantify the degree of hydrocephalus. The CMS group tended to show an increased Evans' index (p = 0.0027). CONCLUSION: Based on pre-operative imaging data, some reproducible predictors, such as Aaxi/daxi, Dsag*dsag, Acor/dcor, and Evans' index, were established.

Clinical Characteristics and Prognostic Factors of Treatment in Pediatric Posterior Cranial Fossa Ependymoma.

OBJECTIVE: The purpose of this study was to explore the clinical features and risk factors of outcomes in pediatric posterior cranial fossa ependymoma. We aim to provide evidence-based recommendations for the improvement of prognoses. PATIENTS AND METHODS: The clinical data, treatment modalities, approaches performed, recurrence rates and times, as well as the outcomes of 94 cases were analyzed retrospectively. The characters of neuroimaging were further studied. RESULTS: In data from the most recent follow-up, 27 cases had tumor recurrence. The time for tumor recurrence was 13.7 ± 7.7 months. The estimated overall survival and progression-free survival, based on Kaplan-Meier analysis, was 42.2 ± 2.9 months and 38.7 ± 3.4 months, respectively. Univariate analysis showed that being free of recurrence is closely related to the high tumor sphericity (p = 0.018), homogeneity of tumor texture (p = 0.001), and gross total resection (GTR; p < 0.001). Mortality is linked to low sphericity (p = 0.017) and brain stem edema (p = 0.005). Cerebellar mutism is correlated with posterosuperior compression of the 4th ventricle roof by the tumor. The incidence rate of cerebellar ataxia, cerebellar mutism, and cerebellar dysarthria is related to the rostral extension of the tumor within the 4th ventricle. The recurrence rate is higher in subtotal resection (STR) than in GTR, and the difference is significant (p < 0.001). Although there is no significant difference between the recurrence rates in the three types, an earlier recurrence is prone with tumors located in the paramidline-lateral compared to the midline (p = 0.021) and paramidline-medial areas (p = 0.042). CONCLUSIONS: Based on our data, GTR is indicated as the most optimal choice. Recurrence is linked to lower tumor sphericity, inhomogeneous tumor texture, and STR/partial resection. Tumor located on the lateral side might be prone for an early recurrence.

[Effect of bevacizumab in treatment of children with optic pathway glioma].

OBJECTIVE: To investigate the effect of bevacizumab in the treatment of children with optic pathway glioma (OPG). METHODS: A retrospective analysis was performed for the clinical data of 30 children with OPG who underwent chemotherapy. According to whether bevacizumab was used, they were divided into conventional chemotherapy (carboplatin, vincristine and etoposide) group with 12 children and combined chemotherapy (bevacizumab, carboplatin, vincristine and etoposide) group with 18 children. The children were followed up to 6 months after chemotherapy, and the two groups were compared in terms of visual acuity and tumor size before and after chemotherapy and adverse reactions during chemotherapy. RESULTS: The combined chemotherapy group had a significantly higher proportion of children achieving tumor regression than the conventional chemotherapy group (P<0.05), while there were no significant differences between the two groups in the proportion of children with improved visual acuity or adverse reactions (P>0.05). No chemotherapy-related death was observed in either group. CONCLUSIONS: Bevacizumab combined with conventional chemotherapy can effectively reduce tumor size. Compared with conventional chemotherapy, such combination does not increase adverse reactions and can thus become a new direction for the treatment of OPG in children.

[Risk factors for recurrence within 2 years in children with medulloblastoma].

OBJECTIVE: To investigate the risk factors for recurrence of medulloblastoma (MB) within 2 years and their influence on progression-free survival (PFS). METHODS: A retrospective analysis was performed for the clinical data of 123 children with MB who were admitted from January to December, 2017. According to the presence or absence of recurrence, they were divided into recurrence group with 30 children and non-recurrence group with 93 children. The risk factors for recurrence within 2 years were analyzed, and PFS was compared between the children with different risk factors. RESULTS: Large-cell/anaplastic type and M stage were risk factors for MB recurrence within 2 years. The risk of recurrence in the children with M+ MB was 3.525 times that in those with M0 MB, and the risk of recurrence in the children with large-cell/anaplastic MB was 3.358 times that in those with classic MB (P<0.05). The survival analysis showed that the median PFS time was 20 months in the children with M+ MB, and the 20-month PFS rate was 50% ±â€…11% in the children with M+ MB and 81% ±â€…5% in those with M0 MB (P<0.05). The 20-month PFS rate was 80% ±â€…5% in the children with classic MB, 65% ±â€…10% in those with desmoplastic/nodular MB, 86% ±â€…13% in those with MB with extensible nodularity, and 36% ±â€…20% in those with large-cell/anaplastic MB (P<0.05). CONCLUSIONS: Recurrence is an important influencing factor for the prognosis of MB, and M+ stage and large-cell/anaplastic MB are risk factors for recurrence. Children with such risk factors tend to have a low PFS rate.