945 research outputs found
Syndromic Hearing Loss: A Brief Review of Common Presentations and Genetics
Congenital hearing loss is one of the most common birth defects worldwide, with around 1 in 500 people experiencing some form of severe hearing loss. While over 400 different syndromes involving hearing loss have been described, it is important to be familiar with a wide range of syndromes involving hearing loss so an early diagnosis can be made and early intervention can be pursued to maximize functional hearing and speech-language development in the setting of verbal communication. This review aims to describe the presentation and genetics for some of the most frequently occurring syndromes involving hearing loss, including neurofibromatosis type 2, branchio-oto-renal syndrome, Treacher Collins syndrome, Stickler syndrome, Waardenburg syndrome, Pendred syndrome, Jervell and Lange-Nielsen syndrome, Usher syndromes, Refsum disease, Alport syndrome, MELAS, and MERRF
Millimeter Wave Localization: Slow Light and Enhanced Absorption
We exploit millimeter wave technology to measure the reflection and
transmission response of random dielectric media. Our samples are easily
constructed from random stacks of identical, sub-wavelength quartz and Teflon
wafers. The measurement allows us to observe the characteristic transmission
resonances associated with localization. We show that these resonances give
rise to enhanced attenuation even though the attenuation of homogeneous quartz
and Teflon is quite low. We provide experimental evidence of disorder-induced
slow light and superluminal group velocities, which, in contrast to photonic
crystals, are not associated with any periodicity in the system. Furthermore,
we observe localization even though the sample is only about four times the
localization length, interpreting our data in terms of an effective cavity
model. An algorithm for the retrieval of the internal parameters of random
samples (localization length and average absorption rate) from the external
measurements of the reflection and transmission coefficients is presented and
applied to a particular random sample. The retrieved value of the absorption is
in agreement with the directly measured value within the accuracy of the
experiment.Comment: revised and expande
A Systematic Review Including an Additional Pediatric Case Report: Pediatric Cases of Mammary Analogue Secretory Carcinoma
Importance
Mammary Analogue Secretory Carcinoma (MASC) is a newly characterized salivary gland carcinoma resembling secretory carcinoma of the breast. Prior to being described, MASC was most commonly misdiagnosed as Acinic Cell Carcinoma. Though MASC is predominantly an adult neoplasm, cases have been reported in the pediatric population. Reporting and summarizing of known cases is imperative to understand the prognosis and clinical behavior of MASC.
Objective
(1)
Report a rare case of pediatric MASC.
(2)
Review and consolidate the existing literature on MASC in the pediatric population.
Evidence review
Web of Science, Medline, EMBASE, and The Cochrane Library were searched for studies that included pediatric cases of MASC. Data on clinical presentation, diagnosis and management, and pathology were collected from all pediatric cases.
Findings
Case Report
14 year old with left-sided parotid mass diagnosed as MASC based on histology and immunohistochemistry. He was managed surgically with left superficial parotidectomy with selective neck dissection.
Literature review
The majority of MASC cases have been identified via retrospective reclassification of previously misclassified salivary gland tumors. Of all the pediatric cases (N=11) of MASC, the female-to-male ratio is 1:1.2 with an age range of 10-17 years old. The most common clinical presentation was a slowly growing, fixed, and painless mass of the parotid gland, often detected incidentally on physical examination. Common pathological features include eosinophilic vacuolated cytoplasm within cystic, tubular, and/or papillary architecture. Immunohistochemistry showed positivity for S100, mammaglobin, cytokeratin 19, and vimentin. The diagnosis was confirmed by the detection of the characteristic ETV6-NTRK6 fusion gene via fluorescent in-situ hybridization (FISH). Only 4 cases discussed treatment. Each of these underwent successful surgical resection alone with or without lymph node dissection.
Conclusions and relevance
Since the first case of MASC in the pediatric population was described in 2011, only 12 cases, including this one, have been described in the literature. With this paucity of information, much remains unknown regarding this new pathologic diagnosis. The collection of clinical outcomes data of children with MASC is needed to better understand the behavior of this malignancy as well as determine optimal treatment regimens
Endoscopic Posterior Cricoid Split with Costal Cartilage Graft: A FifteenâYear Experience
Objective
To evaluate outcomes of the endoscopic posterior cricoid split with rib graft (EPCS/RG) procedure in the treatment of subglottic stenosis (SGS), posterior glottic stenosis (PGS), and bilateral vocal fold immobility (BVFI).
Study Design
Retrospective chart review.
Methods
Chart review of all patients who underwent EPCS/RG at a single tertiary-care facility between 1999 and 2014. Patients were grouped based on the primary indication for the procedure. Decannulation was the primary endpoint. Secondary endpoints were the number of subsequent airway procedures and length of hospitalization.
Results
Thirty-three patients were identified; 32 had tracheotomy. Overall decannulation rate was 65.6%. Subgroup analysis demonstrated the following decannulation rates: 53.8% for SGS, 100% for PGS, and 28.6% for BVFI. Fisher exact test found a significant difference in overall decannulation rates between groups (P = 0.002). Operation-specific decannulation rates for patients who never required an open procedure were 23% for SGS, 91.6% for PGS, and 28.6% for BVFI. This difference was also statistically significant (P = 0.001). Multivariate logistic regression analysis found prematurity had a positive correlation with decannulation that approached statistical significance (P < 0.051; odds ratio 6.1; 95% confidence interval 0.99, 37.6). The percentage of patients who underwent repeat airway procedures for the groups was 61.5% for SGS, 16.6 % for PGS, and 14.3% for BVFI. The median length of hospitalization after EPCS/RG was 3 days.
Conclusion
This represents the largest series of patients who have undergone EPCS/RG and demonstrates that the majority of patients can be decannulated after this procedure. Patients with PGS had the highest operation-specific decannulation rates
A clinical pathway for the postoperative management of hypocalcemia after pediatric thyroidectomy reduces blood draws
Objectives
Postoperative calcium management is challenging following pediatric thyroidectomy given potential limitations in self-reporting symptoms and compliance with phlebotomy. A protocol was created at our tertiary children's institution utilizing intraoperative parathyroid hormone (PTH) levels to guide electrolyte management during hospitalization.
The objective of this study was to determine the effect of a new thyroidectomy postoperative management protocol on two primary outcomes: (1) the number of postoperative calcium blood draws and (2) the length of hospital stay.
Study design
Institutional review board approved retrospective study (2010â2016).
Methods
Consecutive pediatric total thyroidectomy and completion thyroidectomyâŻÂ±âŻneck dissection cases from 1/1/2010 through 8/5/2016âŻat a single tertiary children's institution were retrospectively reviewed before and after initiation of a new management protocol. All cases after 2/1/2014 comprised the experimental group (post-protocol implementation). The pre-protocol control group consisted of cases prior to 2/1/2014. Multivariable linear and Poisson regression models were used to compare the control and experimental groups for outcome measure of number of calcium lab draws and hospital length of stay.
Results
53 patients were included (nâŻ=âŻ23, control group; nâŻ=âŻ30 experimental group). The median age was 15 years. 41 patients (77.4%) were female. Postoperative calcium draws decreased from a mean of 5.2 to 3.6 per day post-protocol implementation (Rate RatioâŻ=âŻ0.70, pâŻ<âŻ.001), adjusting for covariates. The mean number of total inpatient calcium draws before protocol initiation was 13.3 (±13.20) compared to 7.2 (±4.25) in the post-protocol implementation group. Length of stay was 2.1 days in the control group and 1.8 days post-protocol implementation (pâŻ=âŻ.29). Patients who underwent concurrent neck dissection had a longer mean length of stay of 2.32 days compared to 1.66 days in those patients who did not undergo a neck dissection (pâŻ=âŻ.02). Hypocalcemia was also associated with a longer mean length of stay of 2.41 days compared to 1.60 days in patients who did not develop hypocalcemia (pâŻ<âŻ.01).
Conclusions
The number of calcium blood draws was significantly reduced after introduction of a standardized protocol based on intraoperative PTH levels. The hospital length of stay did not change. Adoption of a standardized postoperative protocol based on intraoperative PTH levels may reduce the number of blood draws in children undergoing thyroidectomy
Clinically small tonsils are typically not obstructive in children during drug-induced sleep endoscopy
OBJECTIVE:
To determine whether the degree of lateral pharyngeal wall (LPW) obstruction on pediatric drug-induced sleep endoscopy (DISE) correlates with preprocedure tonsillar hypertrophy score on physical examination, and to determine if clinically small tonsils are obstructive.
METHODS:
Retrospective review of 154 patients who underwent DISE at a single pediatric tertiary care center over a 4-year period. Inclusion criteria were documentation of Brodsky tonsil score on preoperative physical examination. Exclusion criteria were previous tonsillectomy, adenoidectomy, or adenotonsillectomy. Lateral pharyngeal wall obstruction was graded for each patient from 0 (no obstruction) to 3 (severe obstruction) using a validated pediatric DISE scoring system known as the Chan-Parikh scoring system (C-P). Data were analyzed using multivariate linear regression controlling for age at time of DISE and presence of comorbid conditions.
RESULTS:
One hundred fifteen patients met criteria for analysis. Median age at DISE was 5.1 years. A moderate positive correlation was calculated between Brodsky score and DISE score, Spearman correlation coefficient 0.55, P = 50% obstruction); and six (9%) had a score of 3 (100% obstruction).
CONCLUSION:
There is a positive correlation between Brodsky Score and DISE LPW score. The majority of children with sleep-disordered breathing with a Brodsky score of 1 did not demonstrate LPW obstruction. These children may benefit from DISE for identification of alternative sites of upper airway obstructio
A Comparison of Favored Learning Modalities
Presented as a poster at Indiana Society of Anesthesiologists Annual Meeting 2020
Lessons Learned from Interdisciplinary Simulation with Pediatric Anesthesia Fellows and Otolaryngology Residents
Presented as a poster at Indiana Society of Anesthesiologists Annual Meeting 2021
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