Measures of Health-Related Quality of Life Outcomes in Pediatric Neurosurgery: Literature Review

Background Improving value in healthcare means optimizing outcomes and minimizing costs. The emerging pay-for-performance era requires understanding of the effect of healthcare services on health-related quality of life (HRQoL). Pediatric and surgical subspecialties have yet to fully integrate HRQoL measures into practice. The present study reviewed and characterized the HRQoL outcome measures across various pediatric neurosurgical diagnoses. Methods A literature review was performed by searching PubMed and Google Scholar with search terms such as “health-related quality of life” and “pediatric neurosurgery” and then including the specific pathologies for which a HRQoL instrument was found (e.g., “health-related quality of life” plus “epilepsy”). Each measurement was evaluated by content and purpose, relative strengths and weaknesses, and validity. Results We reviewed 68 reports. Epilepsy, brain tumor, cerebral palsy, spina bifida, hydrocephalus, and scoliosis were diagnoses found in reported studies that had used disease-specific HRQoL instruments. Information using general HRQoL instruments was also reported. Internal, test–retest, and/or interrater reliability varied across the instruments, as did face, content, concurrent, and/or construct validity. Few instruments were tested enough for robust reliability and validity. Significant variability was found in the usage of these instruments in clinical studies within pediatric neurosurgery. Conclusions The HRQoL instruments used in pediatric neurosurgery are currently without standardized guidelines and thus exhibit high variability in use. Clinicians should support the development and application of these methods to optimize these instruments, promote standardization of research, improve performance measures to reflect clinically modifiable and meaningful outcomes, and, ultimately, lead the national discussion in healthcare quality and patient-centered care

Intracranial arachnoid cysts: Pediatric neurosurgery update

Background: With the greater worldwide availability of neuroimaging, more intracranial arachnoid cysts (IACs) are being found in all age groups. A subset of these lesions become symptomatic and requires neurosurgical management. The clinical presentations of IACs vary from asymptomatic to extremely symptomatic. Here, we reviewed the clinical presentation and treatment considerations for pediatric IACs. Case Description: Here, we presented three cases of IAC, focusing on different clinical and treatment considerations. Conclusion: IACs can be challenging to manage. There is no Class I Evidence to guide how these should be treated. We suggest clinical decision-making framework as to how to treat IACs based on our understanding of the natural history, risks/benefits of treatments, and outcomes in the future, require better patient selection for the surgical management of IACs will be warranted

Surgical Management of Trigeminal Neuralgia in Children

Background Trigeminal neuralgia (TN) is a well-recognized facial pain syndrome. Discrete forms with disparate pain symptoms include classic and atypical. However, atypical facial pain includes neuralgiform pain along a spectrum. Most cases of TN are diagnosed in the adult population. Case reports and series of children have presented TN as a similar entity, with treatment similar to that for adults. We reviewed the pertinent data and present 2 pediatric TN cases successfully treated with microvascular decompression (MVD). Case Description Two pediatric patients (age 12 and 15 years) with TN refractory to previous medical therapy were identified. Both patients were deemed appropriate surgical candidates and underwent MVD to manage their TN. TN compression was arterial in both cases and involved portions of the anterior inferior cerebellar artery. Patient 1 was pain free 6 months after the procedure. Patient 2 was pain free immediately after the procedure and had been weaned off preoperative symptomatic management at the latest follow-up visit. The most recent follow-up examination was 12 and 8 months for patients 1 and 2, respectively, with both experiencing continued freedom from pain. Conclusions Few studies have reported on the effectiveness of MVD in the pediatric population for the management of TN. The supporting data and our 2 cases have demonstrated that MVD is effective for pediatric patients to treat their TN. Furthermore, the side effects appear to be minimal, with excellent pain relief after MVD in this patient population

Application of Lean Principles to Neurosurgical Procedures: The Case of Lumbar Spinal Fusion Surgery, a Literature Review and Pilot Series

BACKGROUND Delivery of higher value healthcare is an ultimate government and public goal. Improving efficiency by standardization of surgical steps can improve patient outcomes, reduce costs, and lead to higher value healthcare. Lean principles and methodology have improved timeliness in perioperative medicine; however, process mapping of surgery itself has not been performed. OBJECTIVE To apply Plan/Do/Study/Act (PDSA) cycles methodology to lumbar posterior instrumented fusion (PIF) using lean principles to create a standard work flow, identify waste, remove intraoperative variability, and examine feasibility among pilot cases. METHODS Process maps for 5 PIF procedures were created by a PDSA cycle from 1 faculty neurosurgeon at 1 institution. Plan, modularize PIF into basic components; Do, map and time components; Study, analyze results; and Act, identify waste. Waste inventories, spaghetti diagrams, and chartings of time spent per step were created. Procedural steps were broadly defined in order to compare steps despite the variability in PIF and were analyzed with box and whisker plots to evaluate variability. RESULTS Temporal variabilities in duration of decompression vs closure and hardware vs closure were significantly different (P = .003). Variability in procedural step duration was smallest for closure and largest for exposure. Wastes including waiting and instrument defects accounted for 15% and 66% of all waste, respectively. CONCLUSION This pilot series demonstrates that lean principles can standardize surgical workflows and identify waste. Though time and labor intensive, lean principles and PDSA methodology can be applied to operative steps, not just the perioperative period

Rapid Improvement Project: Improving Caregivers’ Understanding of Safety Recommendations for Neurosurgical Devices

There has been a proliferation in the development of indwelling neuromodulatory devices with varied safety recommendations, making it difficult for providers to remain up-to-date. This deficit presents an opportunity for significant improvement in patient safety. Methods: We performed a search for monopolar electrocautery and magnetic resonance imaging safety recommendations for several indwelling neuromodulatory devices. We developed a questionnaire followed by an educational compendium and a posttest for 50 care providers. Results: Overall, there was a poor performance on the pretest (mean 39%, SD 19%) but significant improvement on the posttest (mean 71%, SD 16%), P < 0.0001. We placed the educational compendium that included all manufacturer recommendations in the operating room for easy reference. A 2.4 times decrease in the case start times of vagus nerve stimulator cases is evidence of its effectiveness. Conclusions: The authors highlight the lack of knowledge about manufacturer safety recommendations for indwelling neurosurgical devices, which led to the creation of operating room supplements and educational devices

Giant Actinomyces brain abscess in an immunocompetent child: A management strategy

Background: Intraparenchymal brain abscess is a collection of microbes caused by inoculation through direct extension or hematogenous spread. Although rare, intraparenchymal abscesses are potentially fatal and can be detected when patients are symptomatic due to local mass effect on adjacent neural tissue. Brain abscess treatment includes medical management with appropriate antibiotics alone or medical management in combination with surgical debridement. Treatment strategies depend on the size and location of disease, as well as the virulence of the microorganism. Similar to medical management strategies, surgical strategies among providers are not uniform, with variation in approaches from complete extirpation of the abscess, including the abscess wall, to minimally invasive stereotactic needle aspiration. In particular, for children, there are no guidelines for therapy. Case description: We report a case of giant Actinomycosis right frontal brain abscess in an immunocompetent child without risk factors. A review of the literature for the treatment of brain abscess caused very rarely by Actinomyces in children is performed. Conclusion: Successful treatment of brain access depends on organism and location. The even more uncommon giant intraparenchymal abscesses can be managed with minimal access and prolonged antibiosis, especially when slow-growing organisms are identified. Long-term follow-up should be employed to mitigate missed late failures

Drug development in Alzheimer’s disease: The path to 2025

The global impact of Alzheimer’s disease (AD) continues to increase, and focused efforts are needed to address this immense public health challenge. National leaders have set a goal to prevent or effectively treat AD by 2025. In this paper, we discuss the path to 2025, and what is feasible in this time frame given the realities and challenges of AD drug development, with a focus on disease-modifying therapies (DMTs). Under the current conditions, only drugs currently in late Phase 1 or later will have a chance of being approved by 2025. If pipeline attrition rates remain high, only a few compounds at best will meet this time frame. There is an opportunity to reduce the time and risk of AD drug development through an improvement in trial design; better trial infrastructure; disease registries of well-characterized participant cohorts to help with more rapid enrollment of appropriate study populations; validated biomarkers to better detect disease, determine risk and monitor disease progression as well as predict disease response; more sensitive clinical assessment tools; and faster regulatory review. To implement change requires efforts to build awareness, educate and foster engagement; increase funding for both basic and clinical research; reduce fragmented environments and systems; increase learning from successes and failures; promote data standardization and increase wider data sharing; understand AD at the basic biology level; and rapidly translate new knowledge into clinical development. Improved mechanistic understanding of disease onset and progression is central to more efficient AD drug development and will lead to improved therapeutic approaches and targets. The opportunity for more than a few new therapies by 2025 is small. Accelerating research and clinical development efforts and bringing DMTs to market sooner would have a significant impact on the future societal burden of AD. As these steps are put in place and plans come to fruition, e.g., approval of a DMT, it can be predicted that momentum will build, the process will be self-sustaining, and the path to 2025, and beyond, becomes clearer

Trigeminal schwannoma presenting with malocclusion: A case report and review of the literature

Background: Trigeminal schwannomas are rare tumors of the trigeminal nerve. Depending on the location, from which they arise along the trigeminal nerve, these tumors can present with a variety of symptoms that include, but are not limited to, changes in facial sensation, weakness of the masticatory muscles, and facial pain. Case Description: We present a case of a 16-year-old boy with an atypical presentation of a large trigeminal schwannoma: painless malocclusion and unilateral masticatory weakness. This case is the first documented instance; to the best of our knowledge, in which a trigeminal schwannoma has led to underbite malocclusion; it is the 19th documented case of unilateral trigeminal motor neuropathy of any etiology. We discuss this case as a unique presentation of this pathology, and the relevant anatomy implicated in clinical examination aid in further understanding trigeminal nerve pathology. Conclusion: We believe our patient’s underbite malocclusion occurred secondary to his trigeminal schwannoma, resulting in associated atrophy and weakness of the muscles innervated by the mandibular branch of the trigeminal nerve. Furthermore, understanding the trigeminal nerve anatomy is crucial in localizing lesions of the trigeminal nerve

Network-Targeted Approach and Postoperative Resting-State Functional Magnetic Resonance Imaging Are Associated with Seizure Outcome

Objective Postoperative resting‐state functional magnetic resonance imaging (MRI) in children with intractable epilepsy has not been quantified in relation to seizure outcome. Therefore, its value as a biomarker for epileptogenic pathology is not well understood. Methods In a sample of children with intractable epilepsy who underwent prospective resting‐state seizure onset zone (SOZ)‐targeted epilepsy surgery, postoperative resting‐state functional MRI (rs‐fMRI) was performed 6 to 12 months later. Graded normalization of the postoperative resting‐state SOZ was compared to seizure outcomes, patient, surgery, and anatomical MRI characteristics. Results A total of 64 cases were evaluated. Network‐targeted surgery, followed by postoperative rs‐fMRI normalization was significantly (p < 0.001) correlated with seizure reduction, with a Spearman rank correlation coefficient of 0.83. Of 39 cases with postoperative rs‐fMRI SOZ normalization, 38 (97%) became completely seizure free. In contrast, of the 25 cases without complete rs‐fMRI SOZ normalization, only 3 (5%) became seizure free. The accuracy of rs‐fMRI as a biomarker predicting seizure freedom is 94%, with 96% sensitivity and 93% specificity. Interpretation Among seizure localization techniques in pediatric epilepsy, network‐targeted surgery, followed by postoperative rs‐fMRI normalization, has high correlation with seizure freedom. This study shows that rs‐fMRI SOZ can be used as a biomarker of the epileptogenic zone, and postoperative rs‐fMRI normalization is a biomarker for SOZ quiescence

The Type Ia Supernova Rate in Redshift 0.5--0.9 Galaxy Clusters

Supernova (SN) rates are potentially powerful diagnostics of metal enrichment and SN physics, particularly in galaxy clusters with their deep, metal-retaining potentials and relatively simple star-formation histories. We have carried out a survey for supernovae (SNe) in galaxy clusters, at a redshift range 0.5<z<0.9, using the Advanced Camera for Surveys (ACS) on the Hubble Space Telescope. We reimaged a sample of 15 clusters that were previously imaged by ACS, thus obtaining two to three epochs per cluster, in which we discovered five likely cluster SNe, six possible cluster SNe Ia, two hostless SN candidates, and several background and foreground events. Keck spectra of the host galaxies were obtained to establish cluster membership. We conducted detailed efficiency simulations, and measured the stellar luminosities of the clusters using Subaru images. We derive a cluster SN rate of 0.35 SNuB +0.17/-0.12 (statistical) \pm0.13 (classification) \pm0.01 (systematic) [where SNuB = SNe (100 yr 10^10 L_B_sun)^-1] and 0.112 SNuM +0.055/-0.039 (statistical) \pm0.042 (classification) \pm0.005 (systematic) [where SNuM = SNe (100 yr 10^10 M_sun)^-1]. As in previous measurements of cluster SN rates, the uncertainties are dominated by small-number statistics. The SN rate in this redshift bin is consistent with the SN rate in clusters at lower redshifts (to within the uncertainties), and shows that there is, at most, only a slight increase of cluster SN rate with increasing redshift. The low and fairly constant SN Ia rate out to z~1 implies that the bulk of the iron mass in clusters was already in place by z~1. The recently observed doubling of iron abundances in the intracluster medium between z=1 and 0, if real, is likely the result of redistribution of existing iron, rather than new production of iron.Comment: Accepted to ApJ. Full resolution version available at http://kicp.uchicago.edu/~kerens/HSTclusterSNe