Faculty Bibliography

BACKGROUND:Alveolar soft part sarcoma (ASPS) is an uncommon malignancy which may present in a manner similar to benign vascular tumors. METHODS:A six-year-old male with autism spectrum disorder was referred to the Johns Hopkins All Children's Hospital vascular anomalies clinic for the evaluation of a tongue mass. RESULTS:Prior to the presentation, at five years of age a neck computed tomography (CT) scan was performed. This study showed a well circumscribed, enhancing mass at the anterior aspect of the tongue. The radiologic impression concluded that this lesion was most likely a hemangioma. Two years later, the patient was evaluated in the vascular anomalies clinic. The examination demonstrated a 2-3 cm swelling on the dorsal aspect of the tongue. The mass was fleshy and firm with discrete borders. A handheld Doppler examination demonstrated a high flow lesion. The patient underwent an excisional biopsy. The lesion was identified as an alveolar soft part sarcoma based on pathologic characteristics. CONCLUSION/CONCLUSIONS:Familiarity with common vascular tumors and malformations allow providers to diagnose the majority of these lesions with the combination of clinical history and physical examination. Atypical and combined lesions do benefit from imaging studies to help characterize and aid in the differential diagnosis. Biopsy represent the definitive means of obtaining a diagnosis but are necessary in the minority of cases. When in doubt, referral to a specialized, multidisciplinary vascular anomalies clinic will ensure these patients receive management for this challenging collection of conditions. This article is protected by copyright. All rights reserved.

OBJECTIVES/OBJECTIVE:To determine the accuracy of a modern navigation system in temporal bone surgery. While routine in other specialties, navigation has had limited use in the temporal bone due to issues of accuracy, perceived impracticality, and value. STUDY DESIGN/METHODS:Prospective observational study. SETTING/METHODS:Temporal bone laboratory. SUBJECTS AND METHODS/METHODS:Eighteen cadaveric specimens were dissected after rigid fiducials were implanted and computed tomography scans were obtained. Target registration and target localization errors were then measured at various points. RESULTS:The mean overall target registration error was 0.48 ± 0.29 mm. The mean target localization error was 0.54 mm at the sinodural angle, 0.48 mm at the lateral semicircular canal, 0.55 mm at the round window, 0.39 mm at the oval window, and 0.52 mm at the second genu of the facial nerve. CONCLUSION/CONCLUSIONS:A modern navigation system demonstrated submillimeter accuracy for all points of interest. Its use in clinical as well as training settings has yet to be fully elucidated.

Acoustic coordinated reset (CR) therapy based on neuromodulation and neuroplasticity principles has been proposed for the treatment of tonal tinnitus. The original therapy involved periodic delivery of randomly ordered sequences of four low-level tones centered around the frequency of a tone that matched the tinnitus pitch, f_T, with fixed ratios relative to f_T and delivered several hours/day over several weeks. Here we transform the original CR tone selection method to a more perceptually-relevant equivalent rectangular bandwidth (ERB) frequency scale, the ERB_N-number scale. Specifically, we provide a mathematical model that enables calculation of CR tones that accounts for f_T- and hearing loss-related ERB widening and ERB overlaps and gaps of CR tone alignments. Further, the model ensures symmetric CR tone alignments based on modelling studies that indicate the effect is optimal if the CR stimuli are symmetrically spaced relative to the tinnitus-related population of abnormally synchronized cortical neurons to activate the adjacent sub-populations. We also present experimentally testable ERB-based CR tone alignment strategies and explain how to use the ERB-based model in experiments, clinical studies, other types of tinnitus sound treatment such as tailor-made notch music training and limitations of our approach.

BACKGROUND:Low socioeconomic status has been linked with numerous poor health outcomes, but data are limited regarding the impact of insurance status on inflammatory bowel disease (IBD) outcomes. We aimed to characterize utilization of healthcare resources by IBD patients based on health insurance status, using Medicaid enrollment as a proxy for low socioeconomic status. METHODS:We retrospectively identified adult patients with IBD engaged in a colorectal cancer surveillance colonoscopy program from July 2007 to June 2017. Our primary outcomes included emergency department (ED) visits, inpatient hospitalizations, biologic infusions, and steroid exposure, stratified by insurance status. We compared patients who had ever been enrolled in Medicaid with all other patients. RESULTS:Of 947 patients with IBD, 221 (23%) had been enrolled in Medicaid. Compared with patients with other insurance types, patients with Medicaid had higher rates of ever being admitted to the hospital (77.6% vs 42.6%, P < 0.0001) or visiting the ED (90.5% vs 38.4%, P < 0.0001). When adjusted for sex, age at first colonoscopy, and ethnicity, patients with Medicaid had a higher rate of inpatient hospitalizations (Rate ratio [RR] 2.95; 95% CI 2.59-3.36) and ED visits (RR 4.24; 95% CI 3.82-4.70) compared to patients with other insurance. Patients with Medicaid had significantly higher prevalence of requiring steroids (62.4% vs 37.7%, P < 0.0001), and after adjusting for the same factors, the odds of requiring steroids in the patients with Medicaid was increased (OR 3.77; 95% CI 2.53-5.62). CONCLUSIONS:Medicaid insurance was a significant predictor of IBD care and outcomes. Patients with Medicaid may have less engagement in IBD care and seek emergency care more often.

Neutrophil elastase is a serine protease that has been implicated in the pathogenesis of inflammatory bowel disease. Due to post-translational control of its activation and high expression of its inhibitors in the gut, measurements of total expression poorly reflect the pool of active, functional neutrophil elastase. Fluorogenic substrate probes have been used to measure neutrophil elastase activity, though these tools lack specificity and traceability. PK105 is a recently described fluorescent activity-based probe, which binds to neutrophil elastase in an activity-dependent manner. The irreversible nature of this probe allows for accurate identification of its targets in complex protein mixtures. We describe the reactivity profile of PK105b, a new analogue of PK105, against recombinant serine proteases and in tissue extracts from healthy mice and from models of inflammation induced by oral cancer and Legionella pneumophila infection. We apply PK105b to measure neutrophil elastase activation in an acute model of experimental colitis. Neutrophil elastase activity is detected in inflamed, but not healthy, colons. We corroborate this finding in mucosal biopsies from patients with ulcerative colitis. Thus, PK105b facilitates detection of neutrophil elastase activity in tissue lysates, and we have applied it to demonstrate that this protease is unequivocally activated during colitis.

BACKGROUND:The eighth edition of the American Joint Committee on Cancer staging manual (AJCC8) added depth of invasion to the definition of pathologic T stage (pT). In the current study, the authors assess pT stage migration and the prognostic performance of the updated pT stage and compare it with other clinicopathologic variables in patients with early squamous cell carcinoma of the oral tongue (OTSCC; tumors measuring ≤4 cm) with histologically benign lymph nodes (pN0). METHODS:A multi-institutional cohort of patients with early OTSCC was restaged as per AJCC8. Primary endpoints were local recurrence (LR) and locoregional recurrence (LRR). Influential variables were identified and an LR/LRR prediction model was developed. RESULTS:There were a total of 494 patients, with 49 LR and 73 LRR. AJCC8 pT criteria resulted in upstaging of 37.9% of patients (187 of 494 patients), including 34.5% (64 of 185 patients) from pT2 to pT3, without improving the prognostication for LR or LRR. Both LR and LRR were found to be similar for patients with AJCC8 pT2 and pT3 disease. On multivariate analysis, LR was only found to be associated with distance to the closest margin (hazard ratio, 0.36; 95% CI, 0.20-0.64 [P = .0007]) and perineural invasion (hazard ratio, 1.92; 95% CI, 1.10-0.64 [P = .046]). Based on these 2 predictors, a final proportional hazards regression model (which may be used similar to a nomogram) was developed. The proposed model appeared to be superior to AJCC pT stage for estimating the probability of LR and LRR for individual patients with early OTSCC. CONCLUSIONS:AJCC8 pT criteria resulted in pT upstaging of patients with pN0 disease without improved LR or LRR prognostication. The proposed model based on distance to the closest margin and perineural invasion, status outperformed pT as a predictor of LR and LRR in patients with early OTSCC.

OBJECTIVE:To compare closure rate, reduction in air-bone-gap, and operative time of butterfly tympanoplasty (BT) to underlay tympanoplasty (UT). METHODS:Retrospective cohort study of children (age <18y) undergoing Type I tympanoplasty between 2009 and 2017. Patients were excluded if they had <6 months of follow up, mastoidectomy, fat graft or cholesteatoma. RESULTS:Twenty-one patients (mean age 13.4) underwent BT while forty-one patients (mean age 13.5) underwent UT. The mean size of perforation in 30.6% in BT patients and 43.6% in UT patients (p = 0.01). Preoperative audiogram showed a similar air-bone-gap between the two groups of 31.7, 22.7, and 17.9 dB in BT vs 29.6, 24.8, and 17.6 dB in UT at 500, 1000, and 2000 Hz, respectively (p = 0.65, 0.63, and 0.94). Operative time was reduced in BT as compared to UT (94.0 min vs. 150.9, p = 0.01). Closure rate was similar at 85.7% in BT vs 75.6% in UT patients (p = 0.40). Average reductions in air-bone gap were similar with 19.2, 11.7, and 13.2 dB for BT vs 16.6, 12.1, and 10.3 dB for UT at 500, 1000, and 2000 hz, respectively (p = 0.66, 0.93, 0.40). CONCLUSION/CONCLUSIONS:BT has become a reliable tool for the pediatric otolaryngologist. This retrospective study shows that pediatric BT results in similar outcomes with reduced operative time.