A comprehensive review of the diagnosis and management of congenital scoliosis.

PURPOSE: To provide the reader with a comprehensive but concise understanding of congenital scoliosis METHODS: We have undertaken to summarize available literature on the pathophysiology, epidemiology, and management of congenital scoliosis. RESULTS: Congenital scoliosis represents 10% of pediatric spine deformity and is a developmental error in segmentation, formation, or a combination of both leading to curvature of the spine. Treatment options are complicated by balancing growth potential with curve severity. Often associated abnormalities of cardiac, genitourinary, or intraspinal systems are concurrent and should be evaluated as part of the diagnostic work-up. Management balances the risk of progression, growth potential, lung development/function, and associated risks. Surgical treatment options involve growth-permitting systems or fusions. CONCLUSION: Congenital scoliosis is a complex spinal problem associated with many other anomalous findings. Treatment options are diverse but enable optimization of management and care of these children.

Evaluation and management of adolescent idiopathic scoliosis: a review.

Adolescent idiopathic scoliosis (AIS) is a 3D spinal deformity affecting children between the ages of 11 and 18, without an identifiable etiology. The authors here reviewed the available literature to provide spine surgeons with a summary and update on current management options. Smaller thoracic and thoracolumbar curves can be managed conservatively with observation or bracing, but corrective surgery may be indicated for rapidly growing or larger curves. The authors summarize the atypical features to look for in patients who may warrant further investigation with MRI during diagnosis and review the fundamental principles of the surgical management of AIS. Patients with AIS can be managed very well with a combination of conservative and surgical options. Outcomes for these children are excellent with sustained longer-term results.

Fully endoscopic microvascular decompression for trigeminal neuralgia: technique review and early outcomes.

OBJECT: Fully endoscopicmicrovascular decompression (E-MVD) of the trigeminal nerve was initially described more than 1 decade ago, but has not yet gained wide acceptance. The authors present the experience of their first 47 consecutive E-MVDs for trigeminal neuralgia (TN). METHODS: All surgeries were performed by a single surgeon (J.Y.K.L.) at the Pennsylvania Hospital at the University of Pennsylvania. Patients prospectively completed pain scales before and after surgery by using the Brief Pain Inventory-Facial outcomes tool. All patients were called on the telephone, and the same outcome tool was administered without reference to their preoperative pain status. RESULTS: Forty-seven patients (17 men) were identified and enrolled. Forty (85%) had Burchiel Type 1 TN. Vascular compression was observed at surgery in 42 patients (89%). No surgery was aborted or converted to microscope. One patient suffered permanent hearing loss, for a permanent neurological morbidity rate of 2%. Overall improvement in pain outcomes was excellent, with a median maximum pain intensity preoperatively of 10 and postoperatively of 0 (p< 0.0001). The mean interference with global function scores were 6.2 preoperatively and reduced to 1.0 at last follow-up (p < 0.0001). The mean interference with facial function was 7.3 preoperatively and reduced to 1.2 at last follow-up (p < 0.0001). The mean follow-up period after surgery was 15 ± 8 months. CONCLUSIONS: In experienced hands, E-MVD offers superb visualization and illumination and is both safe and effective, at least in the short term. Further longer-term study is needed to compare E-MVD to traditional microscopic MVD.

Quality of Life in Patients Undergoing Spine Surgery: Systematic Review and Meta-Analysis.

STUDY DESIGN: Meta-analysis. OBJECTIVE: Despite the increasing importance of tracking clinical outcomes using valid patient-reported outcome measures, most providers do not routinely obtain baseline preoperative health-related quality of life (HRQoL) data in patients undergoing spine surgery, precluding objective outcomes analysis in individual practices. We conducted a meta-analysis of pre- and postoperative HRQoL data obtained from the most commonly published instruments to use as reference values. METHODS: We searched PubMed, EMBASE, and an institutional registry for studies reporting EQ-5D, SF-6D, and Short Form-36 Physical Component Summary scores in patients undergoing surgery for degenerative cervical and lumbar spinal conditions published between 2000 and 2014. Observational data was pooled meta-analytically using an inverse variance-weighted, random-effects model, and statistical comparisons were performed. RESULTS: Ninety-nine articles were included in the final analysis. Baseline HRQoL scores varied by diagnosis for each of the 3 instruments. On average, postoperative HRQoL scores significantly improved following surgical intervention for each diagnosis using each instrument. There were statistically significant differences in baseline utility values between the EQ-5D and SF-6D instruments for all lumbar diagnoses. CONCLUSIONS: The pooled HRQoL values presented in this study may be used by practitioners who would otherwise be precluded from quantifying their surgical outcomes due to a lack of baseline data. The results highlight differences in HRQoL between different degenerative spinal diagnoses, as well as the discrepancy between 2 common utility-based instruments. These findings emphasize the need to be cognizant of the specific instruments used when comparing the results of outcome studies.

Comparing Utility Scores in Common Spinal Radiculopathies: Results of a Prospective Valuation Study.

Study Design Prospective observational study. Objective To determine whether preference-based health utility scores for common spinal radiculopathies vary by specific spinal level. Methods We employed a standard gamble study using the general public to calculate individual preference-based quality of life for four common radiculopathies: C6, C7, L5, and S1. We compared utility scores obtained for each level of radiculopathy with analysis of variance and t test. Multivariable regression was used to test the effects of the covariates age, sex, and years of education. We also reviewed the literature for publications reporting EuroQol-5 Dimensions (EQ-5D) scores for patients with radiculopathy. Results Two hundred participants were included in the study. Average utility for the four spinal levels fell within a narrow range (0.748 to 0.796). There were no statistically significant differences between lumbar and cervical radiculopathies, nor were there significant differences among the different spinal levels (F = 0.0850, p = 0.086). Age and sex had no significant effect on utility scores. There was a significant correlation between years of education and utility values for S1 radiculopathy (p = 0.037). On review of the literature, no study separated utility values by specific spinal level. EQ-5D utilities for both cervical and lumbar radiculopathy were considerably lower than the results of our study. Conclusions Utility values associated with the most common levels of cervical and lumbar radiculopathy do not significantly differ from each other, validating the current practice of grouping utility by spinal segment rather than by specific root levels. The discrepancy in average utility values between our study and the EQ-5D highlights the need to be mindful of the underlying instruments used when assessing outcomes studies from different sources.

Microsurgical treatment of sacral perineural (Tarlov) cysts: case series and review of the literature.

OBJECTIVE Tarlov cysts (TCs) occur most commonly on extradural components of the sacral and coccygeal nerve roots. These lesions are often found incidentally, with an estimated prevalence of 4%-9%. Given the low estimated rates of symptomatic TC and the fact that symptoms can overlap with other common causes of low-back pain, optimal management of this entity is a matter of ongoing debate. Here, the authors investigate the effects of surgical intervention on symptomatic TCs and aim to solidify the surgical criteria for this disease process. METHODS The authors performed a retrospective review of data from consecutive patients who were surgically treated for symptomatic TCs from September 2011 to March 2013. Clinical evaluations and results from surveying pain and overall health were used. Univariate statistical analyses were performed. RESULTS Twenty-three adults (4 males, 19 females) who had been symptomatic for a mean of 47.4 months were treated with laminectomy, microsurgical exposure and/or imbrication, and paraspinous muscle flap closure. Eighteen patients (78.3%) had undergone prior interventions without sustained improvement. Thirteen patients (56.5%) underwent lumbar drainage for an average of 8.7 days following surgery. The mean follow-up was 14.4 months. Univariate analyses demonstrated that an advanced age (p = 0.045), the number of noted perineural cysts on preoperative imaging (p = 0.02), and the duration of preoperative symptoms (p = 0.03) were associated with a poor postoperative outcome. Although 47.8% of the patients were able to return to normal activities, 93.8% of those surveyed reported that they would undergo the operation again if given the choice. CONCLUSIONS This is one of the largest published studies on patients with TCs treated microsurgically. The data suggest that patients with symptomatic TCs may benefit from open microsurgical treatment. Although outcomes seem related to patient age, duration of symptoms, and extent of disease demonstrated on imaging, further study is warranted and underway.

ASA grade and Charlson Comorbidity Index of spinal surgery patients: correlation with complications and societal costs.

BACKGROUND CONTEXT: The Charlson Comorbidity Index (CCI) and the American Society of Anesthesiologists (ASA) Physical Status Classification System (ASA grade) are useful for predicting morbidity and mortality for a variety of disease processes. PURPOSE: To evaluate CCI and ASA grade as predictors of complications after spinal surgery and examine the correlation between these comorbidity indices and the cost of care. STUDY DESIGN/SETTING: Prospective observational study. PATIENT SAMPLE: All patients undergoing any spine surgery at a single academic tertiary center over a 6-month period. OUTCOME MEASURES: Direct health-care costs estimated from diagnosis related group and Current Procedural Terminology (CPT) codes. METHODS: Demographic data, including all patient comorbidities, procedural data, and all complications, occurring within 30 days of the index procedure were prospectively recorded. Charlson Comorbidity Index was calculated from International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes and ASA grades determined from the operative record. Diagnosis related group and CPT codes were captured for each patient. Direct costs were estimated from a societal perspective using Medicare rates of reimbursement. A multivariable analysis was performed to assess the association of the CCI and ASA grade to the rate of complication and direct health-care costs. RESULTS: Two hundred twenty-six cases were analyzed. The average CCI score for the patient cohort was 0.92, and the average ASA grade was 2.65. The CCI and ASA grade were significantly correlated, with Spearman ρ of 0.458 (p<.001). Both CCI and ASA grade were associated with increasing body mass index (p<.01) and increasing patient age (p<.0001). Increasing CCI was associated with an increasing likelihood of occurrence of any complication (p=.0093) and of minor complications (p=.0032). Increasing ASA grade was significantly associated with an increasing likelihood of occurrence of a major complication (p=.0035). Increasing ASA grade showed a significant association with increasing direct costs (p=.0062). CONCLUSIONS: American Society of Anesthesiologists and CCI scores are useful comorbidity indices for the spine patient population, although neither was completely predictive of complication occurrence. A spine-specific comorbidity index, based on ICD-9-CM coding that could be easily captured from patient records, and which is predictive of patient likelihood of complications and mortality, would be beneficial in patient counseling and choice of operative intervention.

Deep brain stimulation compared with methadone maintenance for the treatment of heroin dependence: a threshold and cost-effectiveness analysis.

AIMS: To determine the success threshold at which a theoretical course of deep brain stimulation (DBS) would provide the same quality of life (QoL) and cost-effectiveness for heroin dependence as methadone maintenance treatment (MMT). DESIGN: We constructed a decision analysis model to calculate QoL after 6 months of MMT and compared it to a theoretical course of DBS. We also performed a cost-effectiveness analysis using societal costs of heroin dependence, MMT and DBS. SETTING: Systematic literature review and meta-analysis. PARTICIPANTS: Patients (n = 1191) from 15 trials administering 6 months of MMT and patients (n = 2937) from 45 trials of DBS for movement disorders. MEASUREMENTS: Data on QoL before and after MMT, retention in MMT at 6 months, as well as complications of DBS and their impact on QoL in movement disorders. FINDINGS: We found a QoL of 0.633 (perfect health = 1) in heroin addicts initiating MMT. Sixty-six per cent of patients completed MMT, but only 47% of them had opiate-free urine samples, resulting in an average QoL of 0.7148 (0.3574 quality-adjusted life years (QALYs) over 6 months). A trial of DBS is less expensive ($81,000) than untreated (or relapsed) heroin dependence ($100,000), but more expensive than MMT ($58,000). A theoretical course of DBS would need a success rate of 36.5% to match MMT, but a success rate of 49% to be cost-effective. CONCLUSIONS: The success rate, defined as the percentage of patients remaining heroin-free after 6 months of treatment, at which deep brain stimulation would be similarly cost-effective in treating opiate addiction to methadone maintenance treatment, is estimated at 49%.

Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases.

OBJECT: Primary spinal cord ependymomas (EPNs) are rare in children, comprising classical WHO Grade II and III tumors and Grade I myxopapillary ependymomas (MEPNs). Despite their benign histology, recurrences and neural-axis dissemination have been reported in up to 33% MEPNs in the pediatric population. Treatment options beyond resection are limited, and little is known about their tumorigenesis. The purpose of this study was to explore the tumor biology and outcomes in a consecutive series of pediatric patients treated at a single institution. METHODS: The authors performed a retrospective clinicopathological review of 19 patients at a tertiary referral children's hospital for resection of a spinal cord ependymoma. The population included 8 patients with a pathological diagnosis of MEPN and 11 patients with a pathological diagnosis of spinal EPN (10 cases were Grade II and 1 case was Grade III). The upregulation of the following genes HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was studied by immunohistochemistry from archived paraffin-embedded tumor samples of the entire cohort to compare the expression in MEPN versus EPN. RESULTS: Gross-total resection was achieved in 75% of patients presenting with MEPNs and in 100% of those with EPNs. The average follow-up period was 79 months for the MEPN subset and 53 months for Grade II/III EPNs. Overall survival for both subsets was 100%. However, event-free survival was only 50% for patients with MEPNs. Of note, in all cases involving MEPNs that recurred, the patients had undergone gross-total resection on initial surgery. In contrast, there were no tumor recurrences in patients with EPNs. Immunohistochemistry revealed no significant differences in protein expression between the two tumor types with the exception of EPHB3, which demonstrates a tendency to be positive in MEPNs (6 reactive tumors of 9) rather than in EPN (2 reactive tumors of 10). CONCLUSIONS: The authors' experience shows that, following a gross-total resection, MEPNs are more likely to recur than their higher-grade counterpart, EPNs. This supports the recommendation for close long-term radiological follow-up of pediatric patients with MEPNs to monitor for recurrence, despite the tumor's low-grade histological feature. No significant difference in the protein expression of HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was present in this selected cohort of pediatric patients.