STUDY DESIGN: A retrospective cohort study. PURPOSE: To examine the validity of prophylactic surgery for children with tethered cord syndrome (TCS). OVERVIEW OF LITERATURE: Prophylactic surgery for pediatric patients with TCS remains controversial. METHODS: We retrospectively analyzed the surgical outcomes of 14 children (nine boys and five girls) with asymptomatic TCS who were surgically treated at Hokkaido University Hospital between 1989 and 2015. RESULTS: The median age at the time of initial surgery for asymptomatic TCS was 28.6 months (range, 0–66 months). The median final follow-up period was 142 months (range, 7–232 months). Of the 14 children with asymptomatic TCS, 12 had lumbosacral lipoma and two had meningocele. According to the classification of spinal lipoma, two children had dorsal type, four had caudal type, two had transitional type, and four had filar type. There were no children with lipomyelomeningocele. All children were free of neurological symptoms until 94 months after the initial surgery. Subsequently, one child exhibited delayed neurological deficits and underwent a second surgery because of motor and sensory disturbances; slight sensory disturbance was noted at the final follow-up examination. Another child later showed bowel and bladder dysfunction. However, a second surgery was not performed for this child because his motor and sensory functions were normal; hence, we chose to avoid nerve injury in the case of dissecting adhesion. CONCLUSIONS: All 14 children with asymptomatic TCS were free of neurological symptoms until 94 months after the initial surgery. However, two children exhibited delayed neurological deficits at 94 months and 177 months. We believe that prophylactic surgery for asymptomatic TCS is effective for a certain period. However, because the natural history of TCS is poorly understood, strict follow-up after surgery is necessary.
Child ; Classification ; Cohort Studies ; Follow-Up Studies ; Humans ; Lipoma ; Meningocele ; Natural History ; Neural Tube Defects ; Retrospective Studies ; Sensation ; Spinal Dysraphism ; Urinary Bladder

STUDY DESIGN: Retrospective cohort study. PURPOSE: To compare long-term results of surgery with the outcomes of symptomatic and asymptomatic tethered cord syndrome (TCS) in children and adolescents and to assess the surgery duration for those with TCS. OVERVIEW OF LITERATURE: Pediatric patients with TCS continue to pose significant diagnostic and management challenges. METHODS: We retrospectively analyzed the outcomes of 31 patients (16 males, 15 females) with TCS, including 21 with lumbosacral lipoma. All were surgically treated between 1989 and 2015. They were divided into symptomatic and asymptomatic TCS groups. The results of the treatment were summarized and analyzed using a non-parametric Mann–Whitney U test. RESULTS: Nineteen patients had symptomatic TCS and 12 had asymptomatic TCS. Patients had a median age of 34 months (range, 0–201 months). The median follow-up period was 116 months (range, 7–223 months). Of the 19 symptomatic TCS patients, preoperative deficits improved after surgery in two (10.5%) and remained stable in 17 (89.5%) patients. One of the 12 asymptomatic TCS (8.3%) patients showed an exacerbated illness after surgery, and one in 11 patients remained stable (11.7%). There were significant differences in monthly age at surgery, preoperative bowel and bladder dysfunction, neurological function, presence/absence of clean intermittent catheterization introduction, and presence/absence of motor disorder at final follow-up (all p<0.05). In the univariate analysis, the presence/absence of preoperative bowel and bladder dysfunction, and symptoms were strongly associated with the risk of children and adolescents with TCS (p<0.05). CONCLUSIONS: Early accurate diagnosis and adequate surgical release might lead to successful outcomes in children and adolescents with TCS. Surgical untethering is a safe and effective method for treatment of children with TCS.
Adolescent* ; Child* ; Cohort Studies ; Diagnosis ; Follow-Up Studies ; Humans ; Intermittent Urethral Catheterization ; Lipoma ; Male ; Methods ; Neural Tube Defects* ; Retrospective Studies ; Spinal Dysraphism ; Urinary Bladder

STUDY DESIGN: A retrospective study. PURPOSE: The purpose of this study was to obtain useful information for establishing the guidelines for treating high-grade spinal cord gliomas. OVERVIEW OF LITERATURE: The optimal management of high-grade spinal cord gliomas remains controversial. We report the outcomes of the surgical management of 14 high-grade spinal glioma. METHODS: We analyzed the outcomes of 14 patients with high-grade spinal cord gliomas who were surgically treated between 1989 and 2012. Survival was charted with the Kaplan-Meier plots and comparisons were made with the log-rank test. RESULTS: None of the patients with high-grade spinal cord gliomas underwent total resection. Subtotal resection was performed in two patients, partial resection was performed in nine patients, and open biopsy was performed in three patients. All patients underwent postoperative radiotherapy and six patients further underwent radiation cordotomy. The median survival time for patients with high-grade spinal cord gliomas was 15 months, with a 5-year survival rate of 22.2%. The median survival time for patients with World Health Organization grade III tumors was 25.5 months, whereas the median survival time for patients with glioblastoma multiforme was 12.5 months. Both univariate and multivariate Cox proportional hazards models demonstrated a significant effect only in the group that did not include cervical cord lesion as a factor associated with survival (p=0.04 and 0.03). CONCLUSIONS: The surgical outcome of patients diagnosed with high-grade spinal cord gliomas remains poor. Notably, only the model which excluded cervical cord lesions as a factor significantly predicted survival.
Biopsy ; Cordotomy ; Glioblastoma ; Glioma* ; Humans ; Proportional Hazards Models ; Radiotherapy ; Retrospective Studies ; Spinal Cord* ; Survival Rate ; World Health Organization

STUDY DESIGN: Retrospective study. PURPOSE: To obtain information useful in establishing treatment guidelines by evaluating baseline clinical features and treatment outcomes of patients with spinal cord astrocytoma (SCA). OVERVIEW OF LITERATURE: The optimal management of SCA remains controversial, and there are no standard guidelines. METHODS: The study included 20 patients with low-grade and 13 with high-grade SCA surgically treated between 1989 and 2014. Patients were classified according to the extent of surgical resection. Survival was assessed using Kaplan-Meier plots and compared between groups by log-rank tests. Neurological status was defined by the modified McCormick scale and compared between groups by Mann-Whitney U tests. RESULTS: Surgical resection was performed for 19 of 20 low-grade (95%) and 10 of 13 high-grade (76.9%) SCA patients. Only nine patients (27.3%) underwent gross total resection, all of whom had low-grade SCA. Of all patients, 51.5% showed deteriorated neurological status compared to preoperative baseline. Median overall survival was significantly longer for low-grade SCA than that (91 months, 78% at 5 years vs. 15 months, 31% at 5 years; p=0.007). Low-grade SCA patients benefited from more aggressive resection, whereas high-grade SCA patients did not. Multivariate analysis revealed histology status (hazard ratio [HR], 0.30; 95% confidence interval [CI], 0.09-0.98; p<0.05) and postoperative neurological status (HR, 0.12; CI, 0.02-0.95; p<0.05) as independent predictors of longer overall survival. Adjuvant radiotherapy had no significant impact on survival rate. However, a trend for increased survival was observed with radiation cordotomy (RCT) in high-grade SCA patients. CONCLUSIONS: Aggressive resection for low-grade and RCT may prolong survival. Preservation of neurological status is an important treatment goal. Given the low incidence of SCA, establishing strong collaborative, interdisciplinary, and multi-institutional study groups is necessary to define optimal treatments.
Astrocytoma* ; Cordotomy ; Humans ; Incidence ; Multivariate Analysis ; Prognosis* ; Radiotherapy ; Radiotherapy, Adjuvant ; Retrospective Studies ; Spinal Cord* ; Survival Rate

Methods: We retrospectively reviewed patients diagnosed with spinal ependymomas who underwent tumor resection in our institution. We obtained data regarding patients’ clinical characteristics, tumoral radiological characteristics, and surgical factors and compared them to clear prognostic factors. Two-stage surgery was performed in 11 patients (36.7%), and single surgery was performed in 19 patients (63.3%). Results: Thirty patients were included in the analyses and divided into two groups: single surgery and two-stage surgery groups. In the single surgery group, high tumor–cord ratio (TCR) and intraoperative motor evoked potential (MEP) reduction were significantly correlated with unfavorable outcomes, which were defined as deterioration of the modified McCormick scale grades 2 months and 1 year postoperatively. Alternatively, these factors were not significantly correlated with postoperative unfavorable outcomes in the two-stage surgery group. Receiver operating characteristic curves indicated that TCR of 0.866 yielded 85.7% sensitivity and 83.3% specificity 2 months postoperatively. Conclusions The results suggested that high TCR might be an indication of two-stage surgery and that its cutoff value is 0.866. Moreover, switching from single surgery to two-stage surgery may prevent postoperative neurological deterioration when intraoperative MEP is decreasing.

STUDY DESIGN: Retrospective cohort study. PURPOSE: To investigate the correlation between the syrinx morphology and neuropathic pain caused by syringomyelia associated with Chiari I malformation. OVERVIEW OF LITERATURE: Neuropathic pain caused by syringomyelia is refractory and markedly impairs the patient. METHODS: We examined 24 patients with neuropathic pain caused by syringomyelia associated with Chiari I malformation. We statistically analyzed the illness duration and age at surgery between patients with and without neuropathic pain. Additionally, we classified the morphology of the syringes into deviated (D), enlarged (E), central (C), and bulkhead (B) types using T2-weighted axial imaging. Moreover, we investigated the correlation between syrinx morphology and neuropathic pain. A Mann–Whitney U-test was performed to compare between the presence or absence of neuropathic pain and the presence or absence of type D syringes. RESULTS: The median age at surgery was 27.5 years, and the median illness duration was 24 months. Among the 24 patients, 11 had preoperative neuropathic pain, one of which was free of neuropathic pain during the final follow-up period. Among patients with neuropathic pain, the syringes’ preoperative morphology was type D in nine patients and types E and C in one patient each. No patient exhibited type B morphology. Among patients without neuropathic pain, the preoperative morphology of the syringes was type D in three patients, type E in seven patients, and types C and B in two patients each. For types D and E, a correlation between neuropathic pain and syrinx morphology was observed. Moreover, type D was associated with significant neuropathic pain in both preoperative and postoperative states. CONCLUSIONS: This study showed a correlation between the morphological features of the syringes and the occurrence of neuropathic pain in patients with syringomyelia associated with Chiari I malformation.
Cohort Studies ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Neuralgia ; Retrospective Studies ; Syringes ; Syringomyelia

Background/Aims: The Lyon Consensus defined parameters based on upper endoscopy and 24-hour combined multichannel intraluminal impedancepH (MII-pH), that conclusively establish the presence of gastroesophageal reflux disease (GERD). However, the true role of upper endoscopy and MII-pH to evaluate patients with extraesophageal symptoms (EES) has not been well established. Hypopharyngeal MII (HMII), which directly measures laryngopharyngeal reflux (LPR) events, has been utilized to evaluate patients with EES suggestive of LPR. Methods: This was a retrospective study involving patients with EES for > 12 weeks despite proton pump inhibitor therapy, and had no endoscopic confirmatory evidence for GERD and negative MII-pH. All patients were subsequently referred for further evaluation of EES with “unknown” etiology and underwent laryngoscopy and HMII. Based on HMII, abnormal proximal exposure (APE) was defined as LPR ≥ 1/day and/or full column reflux (reflux 2 cm distal to the upper esophageal sphincter) > 4/day. Patients with APE were offered antireflux surgery (ARS) and the outcome of ARS was objectively assessed using Reflux Symptom Index. Results: Of 21 patients with EES which was thought to be GERD-unrelated based on endoscopy and MII-pH, 17 patients (81%) had APE. Eight patients with APE who had undergone ARS had significant symptomatic improvement in the Reflux Symptom Index score (19.6 ± 4.9 pre-ARS to 5.8 ± 1.4 post-ARS, P = 0.008). Conclusions A conventional diagnostic approach using endoscopy and MII-pH may not be sufficient to evaluate patients with EES suggestive of LPR. HMII is essential to evaluate patients with EES, and APE could be a reliable indicator for successful treatment outcomes.