Clinical features and treatment strategy of paraspinal arteriovenous shunt (PAVS): a systematic review with individual participants data meta-analysis.

BACKGROUND: Because of the rarity of the disease, paraspinal arteriovenous shunt (PAVS) is not well recognized, and therapeutic options remain controversial. To introduce a rare disease of PAVS and demonstrate its etiology, clinical features, treatment options, and outcomes, we presented a case report and conducted a systematic review and individual participants data (IPD) meta-analysis. METHODS: Studies regarding on PAVS were integrated and IPD were obtained including patients' demographics, disease etiology, clinical and radiologic features, clinical courses and outcomes. Clinical manifestation and treatment outcomes were reviewed, and comparison analysis (cervical versus thoracolumbar) were performed. Further, logistic regression analyses were conducted to identify the poor prognostic factors (incomplete obliteration). RESULTS: Fifty-two articles were selected, and 88 patients enrolled. General and location-specific characteristics of PAVSs were identified: '3/4 of the isolated and 1/4 of the associated etiology', 'bruit, thrill, or murmur (cervical) and weakness (thoracolumbar) as common symptoms', '40% multiple feeders', and '22% intradural venous involvement'. Endovascular treatment was usually preferred (75%). Of 88 enrolled patients, 18 patients showed incomplete obliteration (20.5%). In multivariate analysis, 'etiologies of systematic genetic dysplasia (P = 0.031) and trauma (negatively, 0.038)' were significantly associated with incomplete obliteration. The parameters of 'multiple feeders (0.066)' and 'combined approach (negatively, 0.065)' are verified only in univariate analysis. CONCLUSION: General as well as location-specific characteristics of PAVS is successfully demonstrated. Approximately 20% of the incomplete obliteration is noted, and three potential poor prognostic factors are identified, namely, 'etiology of systematic genetic dysplasia (positive) and trauma (negative)', 'combined approach (negative), and 'multiple feeders'.

Long-Term Outcomes of Indirect Bypass for 629 Children With Moyamoya Disease: Longitudinal and Cross-Sectional Analysis.

Background and Purpose- In pediatric moyamoya disease, there are few reports on the efficacy of surgical intervention for stroke prevention. We evaluated the long-term outcomes of indirect bypass surgery on a relatively large number of children with moyamoya disease in a single center. Methods- From August 1988 to December 2012, 772 children underwent indirect bypass surgery. This study included 629 patients who were followed up for >5 years, excluding patients with moyamoya syndrome. The mean clinical follow-up duration was 12 years (range, 5-29 years). Cross-sectional analysis was performed based on either Karnofsky Performance Scale or Lansky Play Performance Scale to evaluate overall clinical outcomes and factors associated with unfavorable outcomes. To analyze the longitudinal effect of surgery, the annual risk of symptomatic infarction or hemorrhage on the operated hemisphere after indirect bypass surgery was calculated with a person-year method, and the event-free survival rate was evaluated using the Kaplan-Meier method. Results- The overall clinical outcome was favorable in 95% of the patients. The annual risks of symptomatic infarction and hemorrhage on the operated hemispheres were 0.08% and 0.04%, respectively. Furthermore, the 10-year event-free survival rates for symptomatic infarction and hemorrhage were 99.2% and 99.8%. Conclusions- Indirect bypass surgery could provide satisfactory long-term improvement in overall clinical outcome and prevention of recurrent stroke in children with moyamoya disease.

Neurocognitive profile in children with arachnoid cysts before and after surgical intervention.

OBJECTIVES: Treatment indications for arachnoid cysts are not clear. Some surgeons take improvement in neurocognitive function into account as a surgical indication for arachnoid cysts. However, only a few studies have evaluated the relationship between arachnoid cysts and neurocognitive function. Furthermore, studies that analyze neurocognitive function as an effect of arachnoid cyst surgery are even rarer. The purpose of this study was to analyze the neurocognitive function scores of children with arachnoid cysts before and after surgery and to examine whether surgical treatment led to improved neurocognitive function. METHODS: From June 2009 to August 2012, data for 24 children diagnosed with arachnoid cysts who underwent surgery at Seoul National University Children's Hospital were analyzed. Pre-operative and post-operative cyst volume was assessed and neurocognitive function was tested using the Korean version of the Wechsler Intelligence Scale for Children-Revised (WISC-R) and the Bender-Gestalt Test (BGT). Comparison of pre- and post-operative profiles by laterality of the arachnoid cyst was performed. RESULTS: Patients had age-appropriate full-scale intelligent quotients (FSIQ), verbal IQ (VIQ), and performance IQ (PIQ) pre-operatively, which were maintained after surgery. Of the subtests, Block Design showed significant improvement post-operatively (p = 0.021). This means that visuo-spatial integration and mental construction abilities were improved after surgery. Patients with left or right arachnoid cysts did not show statistically significant changes in FSIQ, VIQ, or PIQ after surgery (110.21 versus 113.95, p = 0.307; 108.92 versus 111.54, p = 0.368; 107.88 versus 111.04, p = 0.152, respectively). Subanalysis showed that the pre- and post-operation VIQ mean scores of the patients with right arachnoid cysts were significantly higher (p < 0.054) than those of the patients with left arachnoid cysts, and there was no significant change after the surgery. INTERPRETATION: There was no significant association among cyst volume reduction, laterality, and clinical neurocognitive function improvement. The present findings indicate a limited role for surgical intervention in improving the intellectual abilities of children with arachnoid cysts.

Syringomyelia caused by an arachnoid web in a patient with shunted Dandy-Walker malformation.

INTRODUCTION: Dandy-Walker malformation (DWM) is a congenital brain anomaly characterized by dysgenesis of the cerebellar vermis and the presence of a posterior fossa cyst. The association of syringomyelia with DWM is extremely rare. CASE REPORT: A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. He had been treated with cystoventriculoperitoneal shunting with a Y-connection during infancy, which was followed by a revision 6 years later. During the revision surgery, intraventricular bleeding occurred and was managed conservatively. Imaging studies for the current visit revealed syringomyelia along the cervicothoracic spinal cord and a membranous structure around the cervicomedullary junction. Phase-contrast cine magnetic resonance imaging (MRI) revealed disturbed cerebrospinal fluid (CSF) flow across the membrane. We excised the arachnoid web that was tethering the brainstem and blocking CSF flow. Postoperatively, the patient experienced symptom relief, and the follow-up imaging study demonstrated a dramatic decrease in the size of the syringomyelia. DISCUSSION: We suggest that syrinx formation in this patient was possibly caused by disturbed CSF flow and tethering of the brainstem. CONCLUSION: We experienced an unusual case of DWM with syringomyelia which was caused by an arachnoid web blocking CSF flow and tethering the brainstem. The arachnoid web seems to be formed by previous bleeding which occurred at the time of shunt revision. After excision of the arachnoid web, the patient showed good outcome.

Comparison of clinical and radiological manifestations and surgical outcomes of pediatric Chiari I malformations in different age groups.

PURPOSE: The aim of this study was to elucidate the differences in clinical and radiological manifestations and treatment outcomes of pediatric Chiari I malformation patients according to age. METHODS: We retrospectively reviewed the patients who received surgery in our institution for symptomatic Chiari I malformations between January 1991 and December 2012. Fifty-four patients were identified, and their medical records were reviewed for clinical presentation, radiological findings, surgical treatment, and outcomes including complications. We divided the patients into 3 groups: Group I (n = 4) younger than 3 years old; Group II (n = 9) between 3 and 5 years old; and Group III (n = 41) older than 5 years old. Surveyed data were compared among the groups. The mean follow-up period was 82.8 months. RESULTS: All of Group I patients presented with stem compression signs. Ventriculomegaly was common (3/4, 75%), but no syrinx or scoliosis was observed. In Group II, scoliosis was the most common presentation (5/9, 56%), and these patients rarely complained any other symptoms. Pain, such as headache (18/41, 44%), was the predominant presentation in Group III. The incidences of syrinx and scoliosis were similar in Groups II and III. There were no differences in the treatment outcomes among the groups. The spinal curve did not improve in 4 of 5 Group II patients despite their early FMD surgeries. The incidence of complications related to cerebrospinal fluid leakage was higher in the young age group. CONCLUSIONS: The clinical and radiological manifestations of pediatric Chiari I malformation appeared to be different according to age.

Surgical outcome of Chiari I malformation in children: clinico-radiological factors and technical aspects.

PURPOSE: The optimal treatment for Chiari I malformation in children is still under debate. The aim of this study was to evaluate the surgical outcome of the pediatric Chiari I malformation,focusing on clinico-radiological factors and technical aspects. METHODS: Fifty-six patients with Chiari I malformation who received surgery at Seoul National University Children's Hospital were included. The mean age was 7.9 years. The patients were divided into three groups: group I (n =8) with hydrocephalus, group II (n =11) without syrinx, and group III (n =37) with syrinx. Group I received shunting operation initially,and others received foramen magnum decompression (FMD). Group III was further subdivided: group IIIa (n =9), minimal intradural manipulation, and group IIIb (n =27), active intradural manipulation. The outcomes were compared between the groups. The mean follow-up period was 75.9 months. RESULTS: In group I, symptoms were resolved or had improved in most patients, with only one patient received additional FMD. Symptoms resolved or improved in 10 (91 %) and 25 cases (84 %) in groups II and III, respectively. Syrinx was markedly decreased in 31 cases (86 %) in group III. FMD was less effective for scoliosis (improved or stabilized in 57 %).The persistence of syrinx was related with an aggravation of scoliosis. The outcomes between group IIIa and IIIb showed no significant difference. CONCLUSIONS: In most pediatric Chiari I patients with hydrocephalus,a shunting operation was sufficient. FMD showed high efficacy in treating patients without hydrocephalus. The extent of the intradural procedure did not have a significant effect on the clinical outcome.

Electroencephalography in pediatric moyamoya disease: reappraisal of clinical value.

PURPOSE: The clinical value of electroencephalography (EEG) in pediatric moyamoya disease has been underestimated, though the characteristic patterns are well known. We undertook this study to evaluate the clinical value of EEG as a diagnostic and postoperative follow-up modality in pediatric moyamoya disease. METHODS: We retrospectively reviewed the pre and postoperative EEG with effective hyperventilation in 127 pediatric moyamoya patients and compared their patterns with hemodynamic images. RESULTS: One hundred and two patients (80.3 %) among 127 showed abnormal EEG findings before revascularization surgery. The typical rebuild-up phenomenon was observed in 82 (64.6 %) and localized build-up in 32 (25.2 %) without any significant clinical ischemic events during and after hyperventilation. The rebuild-up was observed more frequently in younger age groups (less than 13 years) and Suzuki stages III. The location of the rebuild-up distribution and asymmetric build-up was consistent with the area showing hemodynamic abnormalities on single photon emission computed tomography and/or perfusion magnetic resonance imaging. Postoperative follow-up EEGs were performed in 41 patients. Six patients with remaining rebuild-up in postoperative follow-up EEG showed poorer postoperative clinical outcomes. CONCLUSIONS: This study may reappraise EEG as an easy, safe, and adjunctive diagnostic and postoperative follow-up modality for evaluation of hemodynamic status and clinical outcome, especially in children with moyamoya disease.

Secondary neurulation of human embryos: morphological changes and the expression of neuronal antigens.

PURPOSE: The morphological changes and expression patterns of neuronal antigens of human embryos, obtained from the therapeutic termination of pregnancy or from surgical procedures, were analyzed in order to characterize the secondary neurulation. METHODS: A total of 21 human embryos from Carnegie stages 12 to 23 and two fetuses in early stages were studied. The markers used for immunohistochemical study were neural cell adhesion molecule (N-CAM), neuronal nuclear antigen (NeuN), neurofilament-associated protein (3A10), synaptophysin, and glial fibrillary acidic protein (GFAP). RESULTS: The formation of the caudal neural tube to the tip of the caudal portion of the embryo was finished at stage 17. The postcloacal gut had completely disappeared at stage 18, and multiple cavities of the caudal neural tube were clearly visible. The caudal portion of the neural tube showed findings suggestive of involution at stage 19. The expression patterns of neuronal antigens were as follows: N-CAM and NeuN showed immunoreactivity at the germinal layer of the spinal cord at stages 17 and 18. Neurofilament-associated protein (3A10) showed persistent immunoreactivity at the caudal cell mass and notochord during the observation period, along with the spinal cord, and the positive reactions were mainly located at the dorsal white matter at stage 17. Synaptophysin showed a weak positive reaction at the caudal cell mass and notochord at stages 13 and 14, evident by staining observed at the spinal cord at stages 15 and 16. There was no definite positive reaction for GFAP. CONCLUSIONS: These characteristic patterns might be helpful for the understanding of human congenital anomalies involving secondary neurulation processes.

Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute.

BACKGROUND: Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC). METHODS: The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed. RESULTS: The median age at diagnosis was 18 months--55 months for choroid plexus papilloma (CPP), 8 months for ACPP, and 15 months for CPC. Gross total resections were achieved in seven of eight patients with CPP, seven of seven with ACPP, and three of eight with CPC. Seven patients with CPC received chemotherapy. Four patients received high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (aPBSCT), and three among them have survived. Four patients with CPC received radiotherapy. One CPP patient and one CPC patient underwent radiosurgery. All CPP and ACPP patients have survived. The overall survival rate of the CPC patients was 62.5% in the first year and 42.9% in the second year. The progression-free survival rate of the CPC patients was 50% in the first year and 0% in the second year. Seven patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion. CONCLUSION: CPP and ACPP were surgically curable. Multi-modal treatments are necessary in the management of CPC with poor prognosis. HDCT and aPBSCT may be important to treat infants for whom radiotherapy is limited. Hydrocephalus and subdural effusion should be resolved with appropriate management.

Comparison of the transcranial approach and transsphenoidal approach based on the anatomical location of origin in pediatric craniopharyngiomas.

BACKGROUND: Various clinical classifications of craniopharyngiomas (CRPs) have been proposed to suggest optimal surgical planning. We aimed to evaluate the clinical outcomes of pediatric CRPs and the clinical significance of anatomical classification in relation to the diaphragm sellae. METHODS: A retrospective review was conducted on patients under 18 years of age who underwent surgery for CRPs from July 1998 to August 2022. The patients were divided into transcranial approach (TCA), and transsphenoidal approach (TSA) groups, which included microscopic TSA and endoscopic endonasal approach (EEA) groups. EEA has been adopted at our institute since 2011. CRPs were classified by their origin and relationship with the diaphragm sellae. RESULTS: A total of 132 pediatric CRP patients were included in this study, 117 of whom underwent surgery for primary CRP and 15 for recurrent CRP. Among them, 89 (67.4%) underwent TCA, 9 (6.8%) had microscopic TSA, and 34 (25.8%) had EEA. In subdiaphragmatic CRPs with competent diaphragm sellae, TSA tended to yield better outcomes than did TCA in terms of stalk preservation and ophthalmological outcomes. After the introduction of EEA, the proportion of supradiaphragmatic CRPs treated via the TSA increased from 0% to 50% (p < 0.001). Gross total resection (HR=0.194; 95% CI=0.102-0.367, p < 0.001) and adjuvant therapy (HR=0.208; 95% CI=0.048-0.897, p = 0.035) were found to be positive prognostic factors for long-term tumor control. CONCLUSIONS: Over time, with the adoption of EEA at our institute, the impact of anatomical classification on the surgical apprpoach has decreased. Nevertheless, an individualized surgical approach should be employed to improve long-term outcomes and minimize complications for pediatric CRPs.

Tumors in the cerebellopontine angle in children: warning of a high probability of malignancy.

Cerebellopontine angle (CPA) tumors are uncommon in children, and the pathological spectrum is different from that of adults. In this study, we reviewed the pathological diagnosis of pediatric patients with a CPA tumor to determine the pattern in this age group. In a cohort of 267 patients with posterior fossa tumor, tumor locations were determined with preoperative magnetic resonance imaging (MRI). The pathological diagnosis, imaging characteristic, and treatment outcomes of patients with CPA tumors was reviewed and analyzed. Twenty-six patients (9.7 %) had a tumor in the CPA. The pathological spectrum was wide, from malignant intrinsic brain tumors to benign extra-axial tumors and sarcomatous lesions. Eighteen patients (69 %) had malignant tumors. The pathological nature was strongly linked to patient age. The mean age of malignant tumor group was significantly younger than that of benign tumor group. MRI findings that favored malignant histology included a plastic feature of the tumor, multiple signal voids, encasement of major arteries, widening of lateral recess, focal cerebellar edema, and hydrocephalus. The presence of seeding in the neuraxis also indicated malignant pathology. Especially, increased density on precontrast computed tomography was a strong predictor of malignant pathology. Malignant CPA tumors showed high surgical morbidity rate and grim long-term prognosis. Patient age and tumor location are the two most important clues for the diagnosis of any brain tumor. Unlike in adult patients, clinicians should expect a high probability of malignant histology for pediatric CPA tumors, especially in infants and young children.

Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery.

PURPOSE: Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease, and progressive involvement of the posterior cerebral artery (PCA) has been reported. However, majority of MMD articles are presenting classic anterior circulation related issues. This study investigates the preoperative factors related to the long-term outcome of posterior circulation in MMD. METHODS: Retrospective review of 88 MMD patients (166 PCAs in either hemisphere) without symptomatic disease involvement of PCA at initial diagnosis was done. Data at initial diagnosis regarding age, presence of infarction, status of the PCA, type of posterior communicating artery, and the angle between PCA and basilar artery were reviewed. Progressive stenosis of PCA was evaluated by symptom or radiological imaging during follow up. RESULTS: During an average follow up of 8.3 years, 29 out of 166 (18 %) evaluated PCAs showed progressive disease involvement. The average time of progression from the initial operation was 4.9 years, with the latest onset at 10.8 years. The patients who showed progressive stenosis of the PCA tended to be younger, present with infarction, have smaller angle between PCA and basilar artery, and have asymptomatic stenosis of the PCA at initial presentation. However, multivariate analysis confirmed only the presence of initial infarction and a smaller angle between PCA and basilar artery to be significantly associated with progressive stenosis of PCA. CONCLUSIONS: Involvement of PCA in MMD may occur in a delayed fashion, years after the completion of revascularization of anterior circulation. Persistent long-term follow-up regarding the posterior circulation is recommended.

Hydration-induced rapid growth and regression after indirect revascularization of an anterior choroidal artery aneurysm associated with Moyamoya disease: A case report.

The prevalence of aneurysm formation in adults with Moyamoya disease (MMD) is higher than that in the general population. The treatment strategy is often individualized based on the patient's disease characteristics. A 22-year-old man was diagnosed with MMD after presenting a small thalamic intracerebral and subarachnoid hemorrhage in the quadrigeminal cistern. Cerebral angiography revealed a small aneurysm (2.42 mm) in the left anterior choroidal artery. Since the hemodynamics in the left hemisphere was compromised, an indirect bypass surgery was performed. The patient's condition deteriorated postoperatively because of poor perfusion of the internal carotid artery, and massive hydration was required. During neurocritical care, the aneurysm increased in size (5.33 mm). An observation strategy was adopted because of the distal aneurysmal location and the high risk involved. Subsequently, the patient recovered, and newly developed collateral flow appeared from the external carotid artery. Additionally, a dramatic size reduction of the aneurysm (1.51 mm) was noticed. Our case suggests that MMD-related dissecting aneurysms on a distal cerebral artery, which present a high risk of embolization, could be managed by indirectly reducing the hemodynamic burden. Massive hydration in such cases should be avoided or balanced to avoid the risk of rapid growth and aneurysm rupture.

Factors Affecting Cage Obliquity and the Relationship between Cage Obliquity and Radiological Outcomes in Oblique Lateral Interbody Fusion at the L4-L5 Level.

OBJECTIVE: This retrospective study investigated the factors that affect cage obliquity angle despite orthogonal maneuvers performed during oblique lateral interbody fusion (OLIF) and assessed the relationship between cage obliquity angle and radiological outcomes post-surgery. METHODS: Twenty-nine males who underwent L4-L5 OLIF for lumbar degenerative disease between 2019 and 2021 with a followup duration greater than 12 months were analyzed. Radiological parameters were measured including psoas muscle volume, total psoas area index (total psoas muscle area [cm2]/height squared [m2]), distance from the iliac artery to the origin of the psoas muscle (DIAPM), angle between the origin of the psoas muscle and the center of the vertebral disc (APCVD), iliac crest height, disc height, lumbar flexibility (lumbar flexion angle minus extension angle), cage location ratio, cage-induced segmental lumbar lordosis (LL) (postoperative index level segmental LL minus used cage angle), foraminal height changes, fusion grade. RESULTS: DIAPM, APCVD, iliac crest height, postoperative index level segmental LL, and cage-induced segmental LL were significantly correlated with OLIF cage obliquity angle. However, other radiological parameters did not correlate with cage obliquity. Based on multiple regression analysis, the predictive equation for the OLIF cage obliquity angle was 13.062-0.318×DIAPM+0.325×A PCVD+0.174×iliac crest height. The greater the cage obliquity, the smaller the segmental LL compared to the cage angle used. CONCLUSION: At the L4-L5 level, OLIF cage obliquity was affected by DIAPM, APCVD, and iliac crest height, and as the cage obliquity angle increases, LL agnle achievable by the used cage could not be obtained.

Long-Term Surgical Outcome of Indirect Bypass Surgery in Young Children With Moyamoya Disease.

BACKGROUND: The prognosis of moyamoya disease (MMD) in young children (younger than 4 years) is worse than that of older adults. The effectiveness of surgery is still inconclusive. OBJECTIVE: To evaluate long-term outcomes after indirect bypass in young children with MMD. METHODS: A total of 1417 MMD children underwent indirect bypass from August 1988 to October 2020. This study included 135 patients who were younger than 4 years at the time of surgery. The clinical features and surgical outcomes of these patients were assessed. We analyzed the long-term outcome of 102 children who were followed up for more than 5 years (mean: 18.8 years, range: 5-27.3 years). Cross-sectional analysis was performed to evaluate overall outcomes based on the Lansky Play Performance Scale (LPS). The annual risk of symptomatic stroke after surgery was calculated with a person-year method, and the event-free survival rate was evaluated using the Kaplan-Meier method. RESULTS: The overall clinical outcome was favorable (LPS ≥ 80) in 88% of the patients. The overall postoperative adverse event rate was 15%, including 1 death. At the last follow-up, 86% of patients who had seizures at diagnosis were seizure-free. During the follow-up, there were 3 symptomatic infarctions on the operated hemisphere (postoperative 3, 3, and 10 months each). There was no hemorrhagic event. The annual infarction rate was 0.16% per person-year. The 20-year event-free survival rates for symptomatic infarction were 97%. CONCLUSION: Indirect bypass could provide a satisfactory long-term outcome and prevent recurrent stroke in young children with MMD.

Growth-inhibitory effect of neurotrophin-3-secreting adipose tissue-derived mesenchymal stem cells on the D283-MED human medulloblastoma cell line.

Medulloblastoma (MBL), the most common malignant pediatric brain tumor, is incurable in about one-third of patients and can lead to long-term disabilities despite current multimodal treatments. The purpose of this study was to demonstrate in vitro biological effects of neurotrophins-3 (NT-3) on MBL cells and to evaluate the growth-inhibitory effect of neurotrophin-3 (NT-3)-secreting stem cells on tumor cells. We confirmed by western blotting that D283-MED cells express tyrosine kinase C, a specific receptor for NT-3. Analyzing the biological effects of NT-3 on MBL cells, we evaluated autophagy, apoptosis, senescence, and differentiation of tumor cells with NT-3. The NT-3 induced a concentration-dependent increase in apoptosis in the tumor cell line (P < 0.001). The high concentrations of NT-3 increased the expression of class III ß-tubulin (P < 0.001) and decreased the expression of Nestin (P < 0.05). NT-3-secreting stem cells were produced by nucleofecting pIRES2.EGFP-NT3 into human adipose tissue-derived mesenchymal stem cells (hAT-MSCs) and their tropic property toward MBL cells was confirmed by migration assay. Double-layered co-culture experiments with the NT-3-secreting hAT-MSCs and D283-MED MBL cells were performed, and NT-3-induced cell death was studied by 3-(4,5-dimethylathiazol-2-yl)-2,5-dephenyl-tetrazolium bromide (MTT) assay. Consequently, the high concentrations of NT-3-secreting hAT-MSCs significantly (P < 0.05) increased the death of D283-MED cells in vitro. The present study demonstrated that both apoptotic cell death and neuronal differentiation of tumor cells were the mechanisms of growth-inhibitory effect of NT-3-secreting hAT-MSCs on MBL cell line.

Atypical teratoid/rhabdoid tumors: the need for more active therapeutic measures in younger patients.

Despite active and aggressive therapeutic measures, atypical teratoid/rhabdoid tumors (AT/RTs) are without doubt one of the most malignant brain tumors that occur during childhood. These tumors are more prevalent in children less than 3 years of age, and the prognosis is worse in these young children. Medical records of 14 patients diagnosed at our institution were reviewed. Patients diagnosed with AT/RT at our institution between 1997 and 2009 were identified. Clinical data for those patients with pathologic confirmation of AT/RTs were reviewed regarding surgery, adjuvant treatment, and survival. A total of 14 patients were identified. One patient who died 3 days after initial surgery was excluded, and 13 patients were recruited. Of the 13 patients, 7 were less than 1 year of age, and only 2 were over 3 years of age. Nine patients showed dissemination at diagnosis. Four patients were treated with radiation therapy (RTx), and 2 of these were less than 3 years of age. Ten patients had chemotherapy (CTx), and 3 of these underwent high dose chemotherapy and autologous peripheral blood stem cell transplantation. The median progression-free survival (PFS) was 4 months, and the median overall survival (OS) was 9 months. Univariable analysis revealed age more than 1 year, CTx, and RTx to be significant factors for better PFS and OS. None of the variables were significant in multivariable analysis. Young patients with AT/RT have a bad prognosis. An aggressive therapeutic strategy needs to be profiled for this age group.

Enlarging arachnoid cyst: a false alarm for infants.

PURPOSE: The natural history of change in size of arachnoid cysts (ACs) is an unsettled issue. This leads to confusion in deciding treatment strategies of incidental, asymptomatic AC in children, since the risks and complications of surgery are not negligible. The aim of the present study was to elucidate the clinical significance of change in size of AC in young children. METHODS: From 2000 to 2008, 86 patients younger than 5 years with AC documented on brain imaging were included. They were divided into four different age groups (group I: 0-0.5 year, group II: 0.5-1 year, group III: 1-3 years, group IV: 3-5 years). The temporal changes in size of the AC on consecutive ultrasonography, computed tomography (CT), or magnetic resonance imaging (MRI) of each patient were measured and plotted as individual graphs. Natural progression of AC size was assessed by evaluation of the pattern of each graph. RESULTS: Seventeen patients showed enlargement of AC during follow-up; 14 patients were younger than 1 year. None of the patients in group IV showed enlargement during follow-up. Overall, 11 of the 17 patients with demonstrated AC enlargement were not operated on. In six patients, AC enlargement ceased, and spontaneous regression was observed in three patients. The remaining two untreated patients had shown asymptomatic increase in size at last follow-up. Statistical analysis revealed age at diagnosis to be the only significant factor affecting cyst enlargement. CONCLUSION: Asymptomatic enlargement of AC in infants may not be an indication for surgical intervention. Close follow-up is a viable option for such children.

Hemorrhagic moyamoya disease in children: clinical features and surgical outcome.

OBJECTIVE: The clinical presentation of moyamoya disease (MMD) typically includes cerebral ischemia in children and intracranial hemorrhage in adults. Because of its rarity, the benefit of surgery in the hemorrhagic type of pediatric MMD has not been clearly established. The purpose of this study was to delineate the clinical features and surgical outcome of hemorrhagic MMD in children. METHODS: Thirteen patients (six boys and seven girls with a combined mean age of 11.1 years and a range of 4-18 years) were retrospectively confirmed as having MMD with intracranial hemorrhage between 1988 and 2009. All of the patients underwent indirect bypass surgery that followed a relatively uniform scheme at a single institution. We retrospectively reviewed their clinical features and surgical outcome. The mean time of follow-up was 50.3 months (with a range of 3-99 months). RESULTS: Six of the patients presented with intracerebral hemorrhage (ICH), and six others presented with intraventricular hemorrhage (IVH); the remaining patient presented with both ICH and IVH. Preoperative angiography demonstrated that the patients were in various stages of the disease, but the majority of the patients (i.e., 16 of the 26 hemispheres) were in stage 3 with abundant moyamoya vessels (MMV). Three of four patients who presented with a history of prior hemorrhage or infarct exhibited a poor clinical outcome. Five patients with good revascularization had a decreased degree of MMV in their follow-up angiogram and showed good clinical outcome. Recurrent bleeding occurred after surgery in one patient. CONCLUSIONS: Revascularization surgery may have a role for prevention of rebleeding and cerebral infarction in pediatric MMD patients who present with intracranial hemorrhage.

Progression of Ossification of Posterior Longitudinal Ligament After Anterior Cervical Discectomy and Fusion in Military Patients Exposed to Minor Trauma.

Objective: Ossification of the posterior longitudinal ligament (OPLL) can progress even after cervical spine surgery and may cause neurological injury as a result of minor trauma. The purpose of this study was to investigate the preventive factors associated with OPLL progression after anterior cervical discectomy and fusion (ACDF), a procedure commonly performed in clinical practice. Methods: We retrospectively investigated 295 male soldiers who underwent ACDF surgery between 2012 and 2017. Patients who were followed up for >12 months using dynamic radiography and computed tomography (CT) were included in the study. Radiological parameters investigated included OPLL progression, C2-C7 angles on dynamic radiography, segmental angles, C2-C7 cervical sagittal vertical axis (C2-C7 SVA), and the T1 slope. These parameters were measured preoperatively and 1 year postoperatively. Results: A total of 49 patients were enrolled, and 10 patients were confirmed to have OPLL progression. Comparison between the OPLL progression and non-progression groups showed no statistically significant differences in pre- and postoperative cervical range of motion. However, statistically significant differences were observed in the postoperative neutral C2-C7 angle (progression -3.9°±6.4° vs. non-progression -13.4°±7.9°, p=0.001) and the SVA change (progression 5.8±7.9 mm vs. non-progression -3.7±6.3 mm, p=0.00). The cutoff values were -8.01° for the postoperative neutral C2-C7 angle and 1.4 mm for SVA changes. Conclusion: Increased SVA (>1.4 mm) and a small postoperative neutral C2-C7 angle (>-8.01°) 1 year after ACDF were associated with OPLL progression. It is important to be mindful of these factors during follow-up after ACDF, because additional surgical treatment may be necessary for OPLL progression due to neurological injury caused by minor trauma.