Ewing Sarcoma of the Ethmoid Sinus in an Adult.

Extraskeletal Ewing sarcoma (EES) is a relatively rare primary tumor of the soft tissues predominantly affecting men in the second and third decades of life. They are a less common form of the cancerous growth known as an Ewing sarcoma, which occurs in bones or soft tissue such as cartilage. Head and neck ESS can require intervention including endoscopic sinus surgery, septoplasty, inferior turbinectomy, and left internal nasal valve repairs with septal cartilage This is a case report on an unusual presentation of ESS in the sinonasal region.

Evaluating the Efficacy and Trend of Sinus Surgery

Abstract Introduction From April 2009 to December 2016, 661 consecutive patients undergoing sinus surgery completed a quality of life (QOL) questionnaire (SNOT-22) preoperatively and at 3, 6, and 12 months postoperatively. Objective (1) To evaluate the long-term efficacy of sinus surgery using QOL instruments. (2) To determine the optimal evaluation time for surgical efficacy. (3) To determine if surgical results improve with yearly experience. Methods The prospective study patients were split into two groups: Group A, those who completed the initial preoperative evaluation and all postoperative evaluations, and Group B, who completed the preoperative questionnaire and at least one but not all of the postoperative questionnaires. Group A included 93 patients. Group B included 240 patients at 3 months, 180 at 6 months, and 121 at 12 months postoperatively. Results Group A efficacy reported at 3 months was 82.8%, 80.6% at 6 months, and 84.9% at 12 months postoperatively. Group B efficacy reported at 3 months was 71.3%, 78.3% at 6 months, and 84.3% at 12 months postoperatively. An 8-year trend analysis of year-to-year 12 months postoperative data illustrates a significant improvement with an analysis of variance (ANOVA) linear rate of 1.594 (p≤0.12). Conclusion The 8-year trend at 12 months postoperatively shows a positive improvement in surgical results. Patients undergoing sinus surgery at tertiary medical center showed 84.9% improvement in sinus disease symptoms by 12 months postoperatively. Long-term improvement analysis showed no difference between 6 months postoperatively and 12 months, signifying 6 months as an effective evaluation for surgical efficacy.

Evaluating the Efficacy and Trend of Sinus Surgery.

Introduction From April 2009 to December 2016, 661 consecutive patients undergoing sinus surgery completed a quality of life (QOL) questionnaire (SNOT-22) preoperatively and at 3, 6, and 12 months postoperatively. Objective (1) To evaluate the long-term efficacy of sinus surgery using QOL instruments. (2) To determine the optimal evaluation time for surgical efficacy. (3) To determine if surgical results improve with yearly experience. Methods The prospective study patients were split into two groups: Group A, those who completed the initial preoperative evaluation and all postoperative evaluations, and Group B, who completed the preoperative questionnaire and at least one but not all of the postoperative questionnaires. Group A included 93 patients. Group B included 240 patients at 3 months, 180 at 6 months, and 121 at 12 months postoperatively. Results Group A efficacy reported at 3 months was 82.8%, 80.6% at 6 months, and 84.9% at 12 months postoperatively. Group B efficacy reported at 3 months was 71.3%, 78.3% at 6 months, and 84.3% at 12 months postoperatively. An 8-year trend analysis of year-to-year 12 months postoperative data illustrates a significant improvement with an analysis of variance (ANOVA) linear rate of 1.594 ( p ≤0.12). Conclusion The 8-year trend at 12 months postoperatively shows a positive improvement in surgical results. Patients undergoing sinus surgery at tertiary medical center showed 84.9% improvement in sinus disease symptoms by 12 months postoperatively. Long-term improvement analysis showed no difference between 6 months postoperatively and 12 months, signifying 6 months as an effective evaluation for surgical efficacy.

Voice-Related Quality of Life in Patients with Chronic Rhinosinusitis.

OBJECTIVE: Chronic rhinosinusitis (CRS) has long been associated with vocal dysfunction. However, studies quantifying the presence of voice dysfunction in CRS patients or the effects of functional endoscopic sinus surgery (FESS) are sparse. The goal of this study was to determine the voice-related quality of life in patients undergoing FESS for CRS using the validated Voice Related Quality of Life Survey (VRQL). We correlated the preoperative VRQL scores to the Sino-Nasal Outcome Test (SNOT-22) scores, and we determined the effect of FESS on postoperative VRQL scores. METHODS: Consecutive patients undergoing FESS were preoperatively administered both the VRQL and the SNOT-22 surveys. Spearman (ρ) and Pearson (r) correlation coefficients were calculated. The VRQL was mailed to patients postoperatively between 3 and 6 months. The paired t-test was used to compare pre- and post-FESS scores. RESULTS: A total of 102 patients were enrolled, and 81 patients completed the two surveys. A total of 51 (62.9%) patients had raw VRQL score ≥ 10, signifying presence of significant vocal symptoms. The mean ± standard deviation (SD) raw VRQL score of the entire study population was 12.4 ± 4.6, and the mean SNOT-22 score was 37.8 ± 19.2. The Spearman correlation coefficient between VRQL and the total SNOT-22 score was 0.34 (P =.002), and the Pearson correlation coefficient was 0.36 (P = .001). Both correlations were similar, demonstrating that increasing severity of CRS symptoms correlates with decreasing voice-related quality of life (QOL). Seventy patients completed the postoperative survey for an 86% retention rate. Thirty-six of these patients had abnormal preoperative VRQL scores, and these patients improved significantly after FESS. The mean preoperative versus postoperative raw scores were 15.2 ± 5.6 versus 12.5 ± 4.1, respectively (P = .003). CONCLUSION: This study demonstrates the increasing presence of vocal complaints with increasing severity of CRS. It also demonstrates that VRQL scores improve after FESS in those patients with preoperative vocal complaints. LEVEL OF EVIDENCE: IV.

Eustachian Tube Quality of Life and Severity of Disease in Patients With Chronic Rhinosinusitis.

BACKGROUND: Chronic rhinosinusitis (CRS) has long been a suspected risk factor for Eustachian tube dysfunction (ETD). However, there have been few studies quantifying the presence of ETD in CRS patients. We sought to determine the prevalence of ETD symptoms in patients undergoing functional endoscopic sinus surgery (FESS) for CRS using the validated 7-item Eustachian Tube Dysfunction Questionnaire (ETDQ-7) and to correlate the ETDQ-7 scores with scores of CRS symptom severity based on the 22-item Sino-Nasal Outcome Test (SNOT-22). METHODS: Patients for FESS were preoperatively administered both the ETDQ-7 and the SNOT-22 validated quality of life instruments. Pearson and Spearman correlation coefficients were calculated. Changes in ETDQ-7 were measured at 3 months and differences were compared via paired t test. RESULTS: A total of 82 patients completed the surveys. Thirty-nine (47.6%) patients had ETDQ-7 score ≥14.5, signifying clinically significant ETD symptoms. The mean ETDQ-7 score of the study population was 15.8 ± 8.8, and the mean SNOT-22 score was 37.5 ± 19.7. The Pearson and Spearman correlation coefficients between ETDQ-7 and the total SNOT-22 score were 0.52 (P > .0001) and 0.51 (P < .0001), respectively. There was significant improvement in ETDQ-7 scores postoperatively. CONCLUSION: While the association between ETD and CRS has long been known, this is one of the few prospective patient studies evaluating otologic symptoms in a CRS population. We found that a significant percentage of CRS patients suffer from ETD symptoms based on patient-reported subjective outcome measures. This study demonstrates that otologic symptoms increase with CRS severity and improve after FESS.

Clinical Decision Support for Hyperbilirubinemia Risk Assessment in the Electronic Health Record.

BACKGROUND: Physiologic neonatal hyperbilirubinemia (jaundice) is common and affects most newborn infants. However, there is a risk for permanent neurological damage if the bilirubin levels rise above a certain threshold. The management of neonatal jaundice includes the assessment of bilirubin laboratory values, consideration of patient-specific risk factors, and plotting on a bilirubin nomogram reference to determine risk and guide therapy. When performed manually, the process can be time consuming and error-prone. Therefore, web-based calculators such as BiliTool have been developed to assist in risk assessment. METHODS: To streamline the risk assessment calculation process further within our electronic health record (EHR), we created a "BiliReport" to display patient bilirubin-related data and automate transmission of deidentified patient data to the BiliTool website (https://bilitool.org). After implementation, we evaluated usage data, provider satisfaction, and accuracy of documentation. RESULTS: We demonstrated high provider use of the BiliReport and satisfaction with the workflow. We found a significant improvement in the accuracy of bilirubin risk level documentation, with a reduction in erroneous risk stratification from 4% (15/232) to 0.4% (1/243), P < 0.001. We did not find significant a difference in erroneous documentation of the bilirubin lab value (P = 0.07). CONCLUSIONS: Integrating the neonatal hyperbilirubinemia risk assessment process into the EHR may reduce errors and improve provider documentation and adherence to recommended guidelines.

A drug use survey among clients of harm reduction sites across British Columbia, Canada, 2012.

BACKGROUND: In British Columbia (BC), understanding of high-risk drug use trends is largely based on survey and cohort study data from two major cities, which may not be representative of persons who use drugs in other regions. Harm reduction stakeholders, representing each of the five geographic health regions in BC, identified a need for data on drug use to inform local and regional harm reduction activities across the province. The aims of this project were to (1) develop a drug use survey that could be feasibly administered at harm reduction (HR) sites across all health regions and (2) assess the data for differences in reported drug use frequencies by region. METHODS: A pilot survey focusing on current drug use was developed with stakeholders and administered among clients at 28 HR supply distribution sites across the province by existing staff and peers. Data were collated and analysed using univariate and bivariate descriptive statistics to assess differences in reported drug use frequencies by geography. A post-survey evaluation was conducted to assess acceptability and feasibility of the survey process for participating sites. RESULTS: Crack cocaine, heroin, and morphine were the most frequently reported drugs with notable regional differences. Polysubstance use was common among respondents (70%) with one region having 81% polysubstance use. Respondents surveyed in or near their region's major centre were more likely to report having used crack cocaine (p < 0.0001) and heroin (p < 0.0001) in the past week as compared to those residing >50 km from the major centre. Participants accessing services >50 km from the regional centre were more likely to have used morphine (p < 0.0001). There was no difference in powder cocaine use by client/site proximity to the regional centre. Participating sites found the survey process acceptable, feasible to administer annually, and useful for responding to client needs. CONCLUSIONS: The survey was a feasible way for harm reduction sites across BC to obtain drug use data from clients who actively use drugs. Drug use frequencies differed substantially by region and community proximity to the regional centre, underlining the need for locally collected data to inform service planning.

Integrin-mediated adhesion maintains sarcomeric integrity.

Integrin-mediated adhesion to the ECM is essential for normal development of animal tissues. During muscle development, integrins provide the structural stability required to construct such a highly tensile, force generating tissue. Mutations that disrupt integrin-mediated adhesion in skeletal muscles give rise to a myopathy in humans and mice. To determine if this is due to defects in formation or defects in maintenance of muscle tissue, we used an inducible, targeted RNAi based approach to disrupt integrin-mediated adhesion in fully formed adult fly muscles. A decrease in integrin-mediated adhesion in adult muscles led to a progressive loss of muscle function due to a failure to maintain normal sarcomeric cytoarchitecture. This defect was due to a gradual, age dependent disorganization of the sarcomeric actin, Z-line, and M-line. Electron microscopic analysis showed that reduction in integrin-mediated adhesion resulted in detachment of actin filaments from the Z-lines, separation of the Z-lines from the membrane, and eventually to disintegration of the Z-lines. Our results show that integrin-mediated adhesion is essential for maintaining sarcomeric integrity and illustrate that the seemingly stable adhesive contacts underlying sarcomeric architecture are inherently dynamic.