Decreasing tracheostomy placement in hospitalized pediatric patients with subglottic stenosis - Kids' Inpatient Database (KID) analysis.

OBJECTIVES: We aimed to assess procedural trends for tracheostomy placement in the management of children with subglottic stenosis. We hypothesize that with increased use of less invasive airway interventions for subglottic stenosis, tracheostomy may be performed less frequently over time. METHODS: Data were collected from the Kids' Inpatient Database (KID) for pediatric discharges with an admission diagnosis of subglottic stenosis (Stenosis of larynx: ICD-9 code 478.74) from across the United States for years 2000, 2003, 2006, 2009, and 2012. The number of overall procedures and specific airway procedures including tracheostomy (ICD-9-CM codes 31.1, 31.29) were evaluated. RESULTS: A weighted estimate of 18,124 pediatric discharges with a diagnosis of subglottic stenosis were identified. Overall, there was an increase in the mean number of procedures performed during hospitalization from 2000 to 2012 (p < 0.001); however, there was a decrease in the proportion of children undergoing tracheostomy placement (p < 0.001). CONCLUSIONS: Trends over the past decade from a US national database have revealed decreasing tracheostomy placement in hospitalized pediatric patients with subglottic stenosis. This may be due to a change in paradigm with increased use of less invasive, possibly endoscopic, treatment in this patient population. Further investigations may be helpful to identify management paradigms to optimize care in order to help avoid tracheostomy placement.

Survival Outcomes in Sinonasal Poorly Differentiated Squamous Cell Carcinoma.

OBJECTIVE: Sinonasal squamous cell carcinoma (SCC) is rare with no consensus on treatment regimen. Our goal is to analyze treatment outcomes in poorly differentiated SCC (PDSCC) using a large national database. STUDY DESIGN: Retrospective database study. METHODS: The National Cancer Database was queried for sinonasal invasive SCC, grade 3 (poorly differentiated) from 2004 to 2014. Patient demographics and tumor and treatment characteristics were tabulated. Kaplan-Meier (KM) analysis was performed to compare overall survival (OS) between histology subtype and primary site. Multivariable Cox proportional hazards regression was performed for statistical analysis of treatment regimen on OS. RESULTS: A total of 1,074 patients were identified. The maxillary sinus was the most common site (45%). T4 tumors were observed in 50% of patients, with most patients treated at high-volume facilities (77%). In KM analysis, spindle cell SCC histological subtype, primary tumors of the maxillary sinus, and poorly differentiated grade had worse OS. In our Cox-PH model, higher T stage and age were associated with worse OS. Those treated at a high-volume facility and those who underwent surgical resection followed by adjuvant radiation had improved OS. Chemotherapy within the treatment regimen did not confer survival benefit except in surgical patients when positive margins were present, and surgery with adjuvant chemoradiation trended toward improved survival. CONCLUSIONS: Sinonasal PDSCC appears to be best treated at high-volume centers with surgical resection followed by adjuvant radiation. Poorly differentiated grade has worse OS compared to more differentiated tumors. Chemotherapy along with adjuvant radiation may have a role in patients with positive surgical margins. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1040-E1048, 2021.

Sinonasal Squamous Cell Carcinoma Outcomes: Does Treatment at a High-Volume Center Confer Survival Benefit?

OBJECTIVE: To determine whether treatment of sinonasal squamous cell carcinoma (SCC) at a high-volume facility affects survival. STUDY DESIGN: Retrospective database analysis. SETTING: National Cancer Database (2004-2014). SUBJECTS AND METHODS: The National Cancer Database was queried for sinonasal SCC from 2004 to 2014. Patient demographics, tumor characteristics and classification, resection margins, treatment regimen, and facility case-specific volume-averaged per year and grouped in tertiles as low (0%-33%), medium (34%-66%), and high (67%-100%)-were compared. Overall survival was compared with Cox proportional hazards regression analysis. RESULTS: A total of 3835 patients treated for sinonasal SCC between 2004 and 2014 were identified. Therapeutic options included surgery alone (18.6%), radiotherapy (RT) alone (29.1%), definitive chemoradiation (15.4%), surgery with adjuvant RT (22.8%), and combinations (14.1%) of the aforementioned treatments. Patients who underwent surgery with adjuvant RT had better overall survival (hazard ratio [HR], 0.74; P < .001; 95% CI, 0.63-0.86). As for treatment volume per facility, 7.4% of patients were treated at a low-volume center, 17.5% at a medium-volume center, and 75.1% at a high-volume center. Univariate analysis showed that treatment at a high-volume facility conferred a significantly better overall survival (HR, 0.77; P = .002). Multivariable Cox proportional hazards regression analysis, adjusting for age, sex, tumor classification, and treatment regimen, demonstrated that patients who underwent treatment at a high-volume facility (HR, 0.81; P < .001) had significantly improved survival. CONCLUSION: This study shows a better overall survival for sinonasal SCC treated at high-volume centers. Further study may be needed to understand the effect of case volume on the paradigms of sinonasal SCC management.

Sinonasal Quality of Life Outcomes After Extended Endonasal Approaches to the Skull Base.

Introduction Endoscopic endonasal skull base surgery (EESBS) leads to significant alterations in sinonasal anatomy and physiology. However, there is limited data available on quality of life (QOL) outcomes following EESBS. Methods A retrospective review of patients undergoing EESBS from January 2014 to April 2017 was performed. Records were reviewed for clinical history, operative details, and 22-item Sinonasal Outcomes Test (SNOT-22) scores. Unadjusted and adjusted linear regression models were utilized to compare change in SNOT-22 scores from baseline in patients who underwent a simple sella approach (SA) or an extended beyond sella approach (BSA). Results A total of 108 patients were in the SA group, while 61 patients were in the BSA group. SNOT-22 scores were available at baseline and 3 months for 84 patients, while 6-month scores were available for 49 patients. SNOT-22 scores for all patients were not significantly different at 3 months ( p = 0.40) or at 6 months ( p = 0.58). Unadjusted linear regression model did not show an association between the type of approach and change in SNOT-22 score at 3 months ( p = 0.07) and 6 months ( p = 0.28). Adjusted regression model showed a significant decrease in SNOT-22 scores at 3 months ( p = 0.04) for the BSA group, but there was no significant change in SNOT-22 score at 6 months ( p = 0.22). Conclusion Patients undergoing EESBS had no significant change in outcomes at 3 and 6 months. A more extensive BSA was not associated with worse QOL outcomes as measured by SNOT-22.

Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1.

Myotonic dystrophy type I (DM1) is a multi-system, autosomal dominant disorder caused by expansion of a CTG repeat sequence in the 3'UTR of the DMPK gene. The size of the repeat sequence correlates with age at onset and disease severity, with large repeats leading to congenital forms of DM1 associated with hypotonia and intellectual disability. In models of adult DM1, expanded CUG repeats lead to an RNA toxic gain of function, mediated at least in part by sequestering specific RNA splicing proteins, most notably muscleblind-related (MBNL) proteins. However, the impact of CUG RNA repeat expression on early developmental processes is not well understood. To better understand early developmental processes in DM1, we utilized the zebrafish, Danio rerio, as a model system. Direct injection of (CUG)91 repeat-containing mRNA into single-cell embryos induces toxicity in the nervous system and muscle during early development. These effects manifest as abnormal morphology, behavioral abnormalities and broad transcriptional changes, as shown by cDNA microarray analysis. Co-injection of zebrafish mbnl2 RNA suppresses (CUG)91 RNA toxicity and reverses the associated behavioral and transcriptional abnormalities. Taken together, these findings suggest that early expression of exogenously transcribed CUG repeat RNA can disrupt normal muscle and nervous system development and provides a new model for DM1 research that is amenable to small-molecule therapeutic development.