Publications by authors named "Jeffrey P Simons"

58 Publications

Unique Molecular Signatures are Associated with Aggressive Histology in Pediatric Differentiated Thyroid Cancer.

Thyroid 2021 Dec 16. Epub 2021 Dec 16.

Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 6619, Pediatric Otolaryngology, 4401 Penn Avenue, Pittsburgh, Pennsylvania, United States, 15224;

Background: Molecular testing (MT) enhances the diagnostic accuracy of thyroid fine needle aspiration (FNA) biopsy, reducing the need for diagnostic lobectomy in adult patients with indeterminate nodules (Bethesda class III/IV). However, little is known about genetic alterations in pediatric thyroid carcinoma (TC). Our aim was to analyze MT results in pediatric differentiated TC (DTC) to determine associations with histologic and clinical features.

Methods: A retrospective review identified all patients (age <19 years) diagnosed with DTC from 2001-2017 at the University of Pittsburgh Medical Center. Histology was re-reviewed to confirm diagnosis and identify tissue for MT using next-generation sequencing (ThyroSeq version 3, TSv3). Correlation with histologic and clinical features was analyzed using regression analysis.

Results: Of 71 patients with MT results, 62 (87%) of patients had papillary TC. All patients were alive at a median follow-up of 6 years (range 18 days-18 years). Genetic alterations were identified in 65 (92%) patients. These alterations were grouped as BRAF-like point mutations or fusions (39, 55%), RAS-like mutations or fusions (21, 30%), or copy number alterations (5, 7%). On multiple regression analysis accounting for patient sex and tumor size in patients with papillary TC, increased tumor stage (β: 0.234, p<0.001), multifocal disease (OR: 3.60, p=0.042), and lymph node metastases (OR: 6.13, p=0.044) were associated with BRAF-like gene fusions. When considering individual mutations, ETV6/NTRK3 fusions were associated with increased tumor stage (β: 2.07, p=0.023) and BRAF-like point mutations were associated with increased likelihood of undergoing surgery for recurrence over time (HR: 19.5, p=0.004).

Conclusions: Among our cohort of pediatric TC patients with comprehensive MT, >90% had an identifiable genetic alteration. Aggressive features were primarily associated with BRAF-like gene fusions. Preoperative MT results may be useful in guiding the extent of initial operation in pediatric patients (age<19 years) with TC.
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http://dx.doi.org/10.1089/thy.2021.0317DOI Listing
December 2021

Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child.

Pediatr Pulmonol 2022 01 28;57(1):330-332. Epub 2021 Oct 28.

Pediatric Pulmonology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Isolated endobronchial inflammatory myofibroblastic tumors (IMT) are rare, accounting for about 1% of primary endobronchial tumors in children. The mainstay of treatment for this tumor has been surgical resection. Recently, the identification of anaplastic lymphoma kinase (ALK) gene mutations in half of IMTs and promising results of treatment with ALK inhibitors in other ALK-positive tumors have opened the possibility of alternative approaches. We present a 4-year-old child with an ALK-positive endobronchial IMT, treated with endoscopic resection and neoadjuvant therapy with crizotinib, without evidence of tumor recurrence 2 years after the initial resection.
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http://dx.doi.org/10.1002/ppul.25742DOI Listing
January 2022

Chronic Lymphocytic Thyroiditis and Aggressiveness of Pediatric Differentiated Thyroid Cancer.

Laryngoscope 2021 Oct 23. Epub 2021 Oct 23.

Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

Objectives/hypothesis: Hashimoto's Thyroiditis (HT) is a common cause of hypothyroidism. Among adults with differentiated thyroid cancer (DTC), HT appears to be associated with less severe disease burden. In the absence of information regarding HT and disease burden among children with DTC, we assessed the relationship between pediatric DTC severity and HT.

Study Design: Retrospective cohort.

Methods: Charts from 90 pediatric patients who underwent surgical removal of DTC from 2002 to 2017 at tertiary-care children's hospital were reviewed. Demographic, clinical, surgical, pathology, and outcome details were compared between patients with and without HT. Consistency among diagnostic modalities of HT was also evaluated.

Results: Median age at presentation was 16.0 years (range 4.2-18.9 years). Twenty-two patients were male (24%). Forty-five patients (50%) had HT based on presence of thyroid autoantibodies and/or surgical pathology findings and 45 patients did not have HT. Patients with HT had increased odds of microcalcifications (odds ratio [OR]: 3.01, P = .031) and decreased odds of palpable nodules (OR: 0.212, P = .024) and T2 lesions (vs. T1) (OR: 0.261, P = .015) compared with non-HT. No significant differences in demographics and the incidence of multifocality, extrathyroidal extension, lymphovascular invasion, lymph node or pulmonary metastases, disease recurrence, or radioactive iodine treatment were found between the two groups. Thyroglobulin/thyroid peroxidase autoantibodies and surgical pathology indicative of HT were concordant in 82.4% (κ = 0.635, P < .001).

Conclusion: HT was present in 50% of children with DTC. Patients with DTC and HT presented with smaller tumors compared to non-HT patients. No significant differences in other markers of disease aggressiveness were found between the two groups.

Level Of Evidence: 3 Laryngoscope, 2021.
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http://dx.doi.org/10.1002/lary.29908DOI Listing
October 2021

Dysphagia in Pediatric Patients with Tracheostomy.

Ann Otol Rhinol Laryngol 2021 Jun 18:34894211025179. Epub 2021 Jun 18.

Department of Otolaryngology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

Objective: Post-tracheotomy swallowing function has not been well described in the pediatric population. This study aims to (1) determine differences in swallowing functioning pre- and post-tracheotomy and (2) examine the association between postoperative dysphagia and indication for tracheotomy, age at the time of tracheotomy, and time between tracheotomy and modified barium swallow (MBS).

Methods: A retrospective chart review was performed on 752 patients who underwent a tracheotomy from 2003 to 2018 and had adequate documentation for review. Patients were included if they received a post-operative MBS. Descriptive statistics, logistic regression, and Fisher's exact test were used to analyze the data.

Results: The cohort included 233 patients. The mean age at the time of tracheotomy was 25 months (±50.5). The indications for the tracheotomy were upper airway obstruction (110/233, 47.2%), chronic respiratory failure (104/233, 44.6%), and neurologic disease (19/233, 8.2%). The mean time from tracheotomy to post-operative MBS was 224 days (±297.7). Of the patients who had documented pre- and post-tracheotomy diets, nearly half of patients had improvement in their swallowing function after tracheotomy placement (82/195; 42.1%). Post-tracheotomy MBS recommended thickened liquids in 30.9% of the patients (72/233) and 42.5% (99/233) were recommended thin liquids. The remainder (62/233, 26.6%) remained nothing by mouth (NPO). Patients with neurological disease as the indication for the tracheotomy were more likely to remain NPO ( = .039).

Conclusion: A tracheotomy can functionally and anatomically affect swallowing in pediatric patients. The majority of our studied cohort was able to resume some form of an oral diet postoperatively based on MBS. This study highlights the need for objective measurements of swallowing in the postoperative tracheotomy patient to allow for safe and timely commencement of an oral diet.

Level Of Evidence: Level 3.
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http://dx.doi.org/10.1177/00034894211025179DOI Listing
June 2021

Predictive Utility of the Penetration-Aspiration Scale in Inter-Arytenoid Injection Augmentation.

Laryngoscope 2021 05 3;131(5):E1707-E1713. Epub 2020 Oct 3.

Department of Otolaryngology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, U.S.A.

Objectives/hypothesis: To determine the utility of preoperative penetration-aspiration scale (PAS) scores and clinical findings on modified barium swallow (MBS) in predicting advancement of diet after interarytenoid injection augmentation (IAIA).

Study Design: Retrospective review.

Methods: In this retrospective cohort study, 372 consecutive patients who underwent IAIA for pharyngeal dysphagia between 2009 and 2019 were initially identified. Patients were excluded from the study if they had insufficient preop MBS, no postop MBS within 3 months of injection, supraglottoplasty, or underlying neurological condition. Ninety-three patients were included in the study. Pre- and postoperative PAS scores were recorded, as were pre and postop diets. PAS scores were calculated by a single pediatric speech and language pathologist.

Results: Average PAS score on MBS was 5.87 (standard deviation [SD] 2.74); median (range) = 8 (1-8). Postop average was 4.29 (SD 3.02); median (range) = 2 (1-8), P < .001. Those with worse preop PAS scores had increased odds of improvement in diet (odds ratio 1.24, 95% confidence interval [CI] 1.02-1.49, P = .029). An improvement in PAS score of 3.0 or greater predicted an improvement in diet with a sensitivity of 76.7% and a specificity of 85.7%.

Conclusions: PAS score on MBS can be a useful tool when assessing pediatric patients who may be candidates for IAIA. Prospectively comparing PAS score in patients post-IAIA to patients solely undergoing diet modification can help to better objectively assess differences in outcomes and understand the full utility of PAS score.

Level Of Evidence: Level III (Individual Cohort Study) Laryngoscope, 131:E1707-E1713, 2021.
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http://dx.doi.org/10.1002/lary.29142DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8063001PMC
May 2021

Heterotopic gastric mucosa of the proximal esophageal (HGMPE) and its potential role in pediatric dysphonia and dysphagia.

Int J Pediatr Otorhinolaryngol 2020 Nov 3;138:110271. Epub 2020 Aug 3.

Department of Otolaryngology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Objective: Despite a reported incidence of HGMPE of 10%, proof of acid production, and an increased incidence of respiratory symptoms, the pediatric otolaryngology, swallowing and voice care literature is silent on this entity. This case series describes pediatric patients confirmed to have HGMPE with dysphonia and/or dysphagia.

Methods: Retrospective case series of Pediatric Voice, Resonance, and Swallowing Center patients at a tertiary Children's Hospital in 2019.

Setting: Tertiary academic medical center.

Summary Of Results: Three patients who underwent triple endoscopy for dysphonia or dysphagia were histologically diagnosed with HGMPE. Esophageal biopsies were otherwise normal. Two of the three patients resolved their primary aerodigestive symptoms following treatment with acid suppression and a protectant (sucralfate). The third patient reported significant improvement in symptoms by phone. The significance of this case series cannot be understated: 1) A need for increased awareness among pediatric otolaryngologists, voice care and swallowing professionals of this entity given its relatively common incidence of 10% offset by a dearth of presentations & scientific publications in our literature clearly exists. 2) Otolaryngologists have abandoned operative upper aerodigestive tract endoscopy in lieu of office-based less comprehensive videolaryngostroboscopy and fiberoptic endoscopic evaluation of swallowing. HGMPE & other esophageal disorders (i.e. eosinophilic esophagitis) support revisiting triple endoscopy in select patients where office endoscopy has failed to diagnose and successfully treat such patients. 3) Both acid suppression therapy and a protectant (sucralfate) may be useful in these patients. 4) Modification of rigid esophagoscopy technique to carefully assess the introitus and superior esophageal segment is paramount 5) Otolaryngologists over-diagnose & over-treat laryngopharyngeal reflux. The pediatric & adult literature is replete with significant safety warnings associated with acid suppression therapy and guidelines admonish their indiscriminate use, raising the liability bar of empiric treatment. Large scale prospective, randomized and controlled studies are needed to confirm the pathophysiologic role of this entity in pediatric aerodigestive disorders.

Conclusion: HGMPE is a clinical entity that can be easily missed upon swift entry into the esophagus with rigid endoscopy. Careful scrutiny and visualization of the proximal esophagus is critical in order to identify HGMPE, as there is a higher rate of laryngospasm, stricture, and potentially neoplasm in this population.
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http://dx.doi.org/10.1016/j.ijporl.2020.110271DOI Listing
November 2020

Clinical Consensus Statement: Ankyloglossia in Children.

Otolaryngol Head Neck Surg 2020 May 14;162(5):597-611. Epub 2020 Apr 14.

American Academy of Otolaryngology-Head and Neck Surgery Foundation, Alexandria, Virginia, USA.

Objective: To identify and seek consensus on issues and controversies related to ankyloglossia and upper lip tie in children by using established methodology for American Academy of Otolaryngology-Head and Neck Surgery clinical consensus statements.

Methods: An expert panel of pediatric otolaryngologists was assembled with nominated representatives of otolaryngology organizations. The target population was children aged 0 to 18 years, including breastfeeding infants. A modified Delphi method was used to distill expert opinion into clinical statements that met a standardized definition of consensus, per established methodology published by the American Academy of Otolaryngology-Head and Neck Surgery.

Results: After 3 iterative Delphi method surveys of 89 total statements, 41 met the predefined criteria for consensus, 17 were near consensus, and 28 did not reach consensus. The clinical statements were grouped into several categories for the purposes of presentation and discussion: ankyloglossia (general), buccal tie, ankyloglossia and sleep apnea, ankyloglossia and breastfeeding, frenotomy indications and informed consent, frenotomy procedure, ankyloglossia in older children, and maxillary labial frenulum.

Conclusion: This expert panel reached consensus on several statements that clarify the diagnosis, management, and treatment of ankyloglossia in children 0 to 18 years of age. Lack of consensus on other statements likely reflects knowledge gaps and lack of evidence regarding the diagnosis, management, and treatment of ankyloglossia. Expert panel consensus may provide helpful information for otolaryngologists treating patients with ankyloglossia.
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http://dx.doi.org/10.1177/0194599820915457DOI Listing
May 2020

Management and Outcomes of Button Batteries in the Aerodigestive Tract: A Multi-institutional Study.

Laryngoscope 2021 01 18;131(1):E298-E306. Epub 2020 Feb 18.

Division of Pediatric Otolaryngology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

Objectives/hypothesis: To describe the clinical presentation, management, and complications associated with button battery impaction in the aerodigestive tract in children.

Study Design: Retrospective case series.

Methods: This multi-institutional study, endorsed by the American Society of Pediatric Otolaryngology research consortium, is a retrospective medical record review, including all children at five tertiary-care institutions presenting with button batteries impacted in the aerodigestive tract between January 2002 and December 2014. Battery type/size, duration and location of impaction, presenting symptoms, treatment, complications, and outcomes were examined.

Results: Eighty-one patients were included (64.2% male), with ingestion witnessed in 20 (24.7%). Median age at presentation was 3 years (range, 1 week-14 years). Median time from diagnosis to removal was 2.5 hours (range, 0.4-72 hours). Locations included the esophagus (n = 48), hypopharynx (n = 1), stomach (n = 6), nasal cavity (n = 22), and ear canal (n = 4). Most common symptoms for esophageal/hypopharyngeal impactions included dysphagia (26.5%), nausea/vomiting (26.5%), drooling (24.5%), cough (18.4%), and fever (18.4%). Most common symptoms for nasal impactions included epistaxis (54.6%), rhinorrhea (40.9%), nasal pain (27.3%), and fever (22.7%). Almost all esophageal impactions were from 3-V (89.5%), 20-mm (81.8%) lithium batteries. Severe esophageal complications included stricture (28.6%), perforation (24.5%), tracheoesophageal fistula formation (8.2%), pneumothorax (4.1%), and bilateral true vocal fold paresis (4.1%). Nasal complications included necrosis (59.1%), septal perforation (27.3%), and saddle nose deformity (4.5%). Duration of impaction correlated with an increased likelihood of persistent symptoms only for nasal batteries (P = .049).

Conclusions: Button batteries in the upper pediatric aerodigestive tract or ear canal should be considered a surgical emergency, requiring urgent removal and careful vigilance for complications.

Level Of Evidence: 4 Laryngoscope, 131:E298-E306, 2021.
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http://dx.doi.org/10.1002/lary.28568DOI Listing
January 2021

Airway anomalies in patients with craniosynostosis.

Laryngoscope 2019 Nov 19;129(11):2594-2602. Epub 2018 Nov 19.

Department of Otolaryngology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, U.S.A.

Objectives: 1) Characterize the spectrum of airway anomalies in patients with craniosynostosis, and 2) identify clinical characteristics of these patients that may be associated with the development of airway anomalies.

Methods: This study is a retrospective case series assessing the type and frequency of airway anomalies in all patients with craniosynostosis seen at a tertiary-care children's hospital between 2000 and 2016. Cohort analyses were then performed to identify differences in airway anomalies dependent on syndromic associations, multisutural fusion, and location of suture fusion. Clinical characteristics examined included demographics and additional neurologic and craniofacial abnormalities.

Results: Four hundred and ninety-six patients with craniosynostosis (83.5% white, 64.5% male; 33.9% sagittal, 28.8% metopic, 11.5% coronal, 1.2% lambdoid, and 24.6% multisutural) were included. Notable airway anomalies included the following: 13.3% adenotonsillar hypertrophy, 8.9% laryngomalacia, 7.3% tracheomalacia, 7.1% subglottic stenosis, 4.0% bronchomalacia, 3.8% laryngeal cleft, and 1.2% vocal fold paresis. Multisutural craniosynostosis patients (n = 122) were more likely to have obstructive sleep apnea (P = 0.005), adenotonsillar hypertrophy (P = 0.014), tracheomalacia (P = 0.011), subglottic stenosis (P < 0.001), and epiglottic/base of tongue collapse (P = 0.003) and require tracheostomy (P = 0.001) and mechanical ventilation (P = 0.017) compared with single suture craniosynostosis. Syndromic craniosynostosis patients (n = 33) were more likely to have obstructive sleep apnea (P < 0.001), laryngomalacia (P = 0.047), and subglottic stenosis (P = 0.009) compared with nonsyndromic patients.

Conclusion: Airway anomalies are prevalent in patients with craniosynostosis; patients with multisutural or syndromic types have an increased risk of developing certain abnormalities. There should be a lower threshold for referral for airway evaluation in these populations.

Level Of Evidence: 4. Laryngoscope, 129:2594-2602, 2019.
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http://dx.doi.org/10.1002/lary.27589DOI Listing
November 2019

Structured care to improve outcomes in primary single stage laryngotracheal reconstruction.

Int J Pediatr Otorhinolaryngol 2018 Nov 23;114:71-75. Epub 2018 Aug 23.

Children's Hospital of Pittsburgh of UPMC, Department of Pediatric Otolaryngology, Head and Neck Surgery, Pittsburgh, PA, USA; Texas Children's Hospital, Department of Pediatric Otolaryngology, Head and Neck Surgery, Houston, TX, USA.

Purpose: To examine single stage laryngotracheal reconstruction (SSLTR) care to reduce complication and failure rate.

Methods: Forty-five patients that underwent primary SSLTR were examined retrospectively. All had pre-operative direct laryngoscopy and bronchoscopy, esophagoscopy with biopsy and MRSA screening. Pre-operative subglottic stenosis (SGS) grade and associated comorbidities were recorded. Intraoperative graft location and type was documented. Hospital course and results were evaluated and compared to cited literature.

Results: The median age at reconstruction was 2 years (0-15 years). 42.2% were male. 66.7% had gastroesophageal disease and 24.4% a MRSA history. Grade 2 SGS was noted pre-operatively in 37.8% and grade 3 or 4 in 57.7% of patients. Post-surgical hospital course was examined. 77.8% of patients were extubated on planned date. 95.6% of patients had operation specific successful decannulation. Graft type and variations of graft placement as well as MRSA and GERD status didn't affect procedure success rate. Active GERD was related to failure of extubation on planned day (p = 0.02). An abnormal pre-operative swallowing examination was associated with higher complication rates (p = 0.03).

Conclusion: Utilizing a more structured approach to SSLTR work-up and addressing potential SSLTR pitfalls may result in higher operation specific decannulation rates. Pre-operative GERD and swallowing dysfunction were associated with higher rates of adverse events.
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http://dx.doi.org/10.1016/j.ijporl.2018.08.023DOI Listing
November 2018

Accuracy of chest X-Ray measurements of pediatric esophageal coins.

Int J Pediatr Otorhinolaryngol 2018 Oct 10;113:1-3. Epub 2018 Jul 10.

Department of Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

Objective: To determine the accuracy of chest x-ray measurements in children using ingested radiopaque foreign bodies of known size.

Methods: A database of foreign body ingestions at a tertiary care children's hospital was queried from 2013 to 2016 for children who had ingested a US coin, had a pre-operative chest x-ray and documentation of coin type at the time of endoscopic removal. Four blinded research subjects measured the coin diameter on chest x-ray using iSite PACS software and based on the measurement, predicted the coin type. Measurements were compared to the known coin diameters published by the US Mint.

Results: A total of 51 patients with sixteen esophageal quarters (diameter 24.26 mm), fourteen nickels (21.21 mm), fourteen pennies (19.05 mm) and seven dimes (17.91 mm) were included in the study. The four subjects had a mean accuracy of 60.3% (range 49.0%-72.5%) in predicting the correct coin type. Across all raters, there was poor agreement for pennies (kappa = 0.161) and dimes (kappa = 0.131), fair agreement for nickels (kappa = 0.259), good agreement for quarters (kappa = 0.687), and fair agreement overall (kappa = 0.371). The study measurements overestimated the coin size in 203 of the 204 measurements by a mean of 1.84 mm (range -0.31-3.85 mm). The mean size discrepancy was larger (2.40 vs. 1.30 mm, p < 0.001) and accuracy of coin type identification was worse (44.6% vs. 74.1%, p = 0.001) in children <4 years old.

Conclusions: Measurement of esophageal coins on chest x-ray is relatively inaccurate and overestimates the size in the majority of cases. Clinicians should use caution when performing fine measurements on chest x-rays, especially in children younger than 4 years old.
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http://dx.doi.org/10.1016/j.ijporl.2018.07.011DOI Listing
October 2018

Laryngomalacia in Patients With Craniosynostosis.

Ann Otol Rhinol Laryngol 2018 Aug 20;127(8):543-550. Epub 2018 Jun 20.

1 Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.

Objective: To characterize differences in the clinical presentation and treatment outcomes of laryngomalacia in patients with and without craniosynostosis.

Methods: A retrospective cohort study was performed comparing all patients with concomitant laryngomalacia and craniosynostosis seen at a tertiary care children's hospital between 2000 and 2016 with a control group of patients with isolated laryngomalacia. Thirty-two patients with craniosynostosis (59% male) and 68 control patients (56% male) were included. There were no significant differences in age of diagnosis or incidence of prematurity. Symptom presentation, disease severity, swallowing function, comorbidities, treatment modalities, and outcomes were examined using logistic regression.

Results: Patients with craniosynostosis had increased odds of presenting with stertor (odds ratio [OR] = 3.41, P = .022), increased work of breathing (OR = 18.8, P = .007), obstructive sleep apnea (OR = 8.48, P = .003), dysphagia (OR = 3.40, P = .008), and aspiration (OR = 40.2, P < .001) and decreased odds of presenting with stridor (OR = 0.0804, P < .001) compared with controls. Patients with craniosynostosis had increased odds of severe laryngomalacia (OR = 5.00, P = .031) and other airway anomalies such as tracheomalacia (OR = 5.73, P = .004), bronchomalacia (OR = 15.5, P = .013), and subglottic stenosis (OR = 2.75, P = .028). Treatment of patients with craniosynostosis was more likely to include tracheostomy (OR = 24.8, P < .001) and gastrostomy tube (OR = 88.4, P < .001). There were no significant differences in rates of supraglottoplasty.

Conclusion: Clinical presentations, comorbidities, and treatments of laryngomalacia are significantly different in the context of craniosynostosis.
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http://dx.doi.org/10.1177/0003489418779413DOI Listing
August 2018

Association between Eosinophilic Esophagitis and Esophageal Food Impaction in the Pediatric Population.

Otolaryngol Head Neck Surg 2018 10 29;159(4):750-754. Epub 2018 May 29.

3 Division of Pediatric Otolaryngology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.

Objectives (1) To describe the presentation, management, and outcomes associated with pediatric esophageal food impaction (EFI) at a single tertiary care institution. (2) To identify the key clinical features of pediatric EFI that are associated with a diagnosis of eosinophilic esophagitis (EoE). Study Design Case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods Thirty-five children <18 years of age presenting with EFI between November 1, 2006, and October 31, 2013, were included. Presenting symptoms, medical history, biopsy results, endoscopic findings, and underlying etiology were examined. Fisher exact test, t tests, and logistic regression were used to compare between patients with and without EoE. Results Thirty-five patients had isolated EFI and were included in the study. EoE accounted for 74% (n = 26) of pediatric EFI, with the remaining cases being attributed to neurologic impairment (n = 5, 15%), prior surgeries (n = 1, 3%), reflux esophagitis (n = 1, 3%), or unknown etiologies (n = 2, 6%). EFI was the initial manifestation of EoE in 81% (n = 21) of patients. The most common presenting symptoms were dysphagia (n = 34), choking (n = 26), and vomiting (n = 23). Linear furrowing was the only endoscopic finding that was significantly associated with EoE ( P < .001). Conclusion Most esophageal food impactions in the pediatric population are associated with an underlying diagnosis of EoE and are often the initial manifestation of the disease. EoE must be considered in all pediatric patients with EFI; esophageal biopsies should be strongly considered in these patients at the time of endoscopic management of the EFI.
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http://dx.doi.org/10.1177/0194599818779049DOI Listing
October 2018

Quality, Readability, and Trends for Websites on Ankyloglossia.

Ann Otol Rhinol Laryngol 2018 Jul 18;127(7):439-444. Epub 2018 May 18.

2 Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.

Objective: This study evaluates the quality and readability of websites on ankyloglossia, tongue tie, and frenulectomy.

Methods: Google was queried with six search terms: tongue tie, tongue tie and breastfeeding, tongue tie and frenulectomy, ankyloglossia, ankyloglossia and breastfeeding, and ankyloglossia and frenulectomy. Website quality was assessed using the DISCERN instrument. Readability was evaluated using the Flesch-Kincaid Reading Grade Level, Flesch Reading Ease Score, and Fry readability formula. Correlations were calculated. Search terms were analyzed for frequency using Google Trends and the NCBI database.

Results: Of the maximum of 80, average DISCERN score for the websites was 65.7 (SD = 9.1, median = 65). Mean score for the Flesch-Kincaid Reading Grade Level was 11.6 (SD = 3.0, median = 10.7). Two websites (10%) were in the optimal range of 6 to 8. Google Trends shows tongue tie searches increasing in frequency, although the NCBI database showed a decreased in tongue tie articles.

Conclusions: Most of the websites on ankyloglossia were of good quality; however, a majority were above the recommended reading level for public health information. Parents increasingly seek information on ankyloglossia online, while fewer investigators are publishing articles on this topic.
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http://dx.doi.org/10.1177/0003489418776343DOI Listing
July 2018

What parents are reading about laryngomalacia: Quality and readability of internet resources on laryngomalacia.

Int J Pediatr Otorhinolaryngol 2018 May 28;108:175-179. Epub 2018 Feb 28.

Children's Hospital of Pittsburgh of UPMC, Division of Otolaryngology, 4401 Penn Avenue, Faculty Pavilion, 7th Floor, Pittsburgh, PA 15224, United States. Electronic address:

Objective: The goal of this study is to measure the quality and readability of websites related to laryngomalacia, and to compare the quality and readability scores for the sites accessed through the most popular search engines.

Introduction: Laryngomalacia is a common diagnosis in children but is often difficult for parents to comprehend. As information available on the internet is unregulated, the quality and readability of this information may vary.

Methods: An advanced search on Google, Yahoo, and Bing was conducted using the terms "laryngomalacia" OR "soft larynx" OR "floppy voice box." The first ten websites meeting inclusion and exclusion criteria were evaluated, for each search engine. Quality and readability were assessed using the DISCERN criteria and the Flesch reading ease scoring (FRES) and Flesch-Kincaid grade level (FKGL) tests, respectively.

Results: The top 10 hits on each search engine yielded 15 unique web pages. The median DISCERN score (out of a possible high-score of 80) was 48.5 (SD 12.6). The median USA grade-level estimated by the FKGL was 11.3 (SD 1.4). Only one website (6.7%), had a readability score in the optimal range of 6th to 8th grade reading level. DISCERN scores did not correlate with FKGL scores (r = 0.10).

Conclusion: Online information discussing laryngomalacia often varies in quality and may not be easily comprehensible to the public. It is important for healthcare professionals to understand the quality of health information accessible to patients as it may influence medical decision-making by patient families.
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http://dx.doi.org/10.1016/j.ijporl.2018.02.036DOI Listing
May 2018

Hassall's corpuscles in the fine-needle aspiration cytology of pediatric ectopic thymic tissue.

Diagn Cytopathol 2017 Aug 28;45(8):735-737. Epub 2017 May 28.

Department of Otolaryngology, Children's Hospital of Pittsburgh of UPMC and University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

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http://dx.doi.org/10.1002/dc.23736DOI Listing
August 2017

Utilization of Flow Cytometry in Pediatric Fine-Needle Aspiration Biopsy Specimens.

Acta Cytol 2016 3;60(4):344-353. Epub 2016 Sep 3.

Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pa., USA.

Background: Fine-needle aspiration (FNA) coupled with flow cytometry (FC) can be helpful in suspicious pediatric lymph nodes or masses to exclude a lymphoproliferative disorder. The aim of this study was to analyze FC findings in a series of pediatric FNAs and to correlate abnormal findings with follow-up information.

Materials And Methods: All pediatric FNAs performed at a tertiary-care children's hospital over a 46-month period that had FC performed were retrospectively analyzed and correlated with follow-up.

Results: A total of 163 FNA procedures were performed in children (age ≤21 years), and 47 (28.8%) of these cases had FC performed. Specimens were mostly obtained from the head and neck (72.3% of cases). Nine cases (19.1%) had abnormal FC findings, including double-negative T cells (n = 3; 33.3%), double-positive T cells (n = 3; 33.3%), excess λ light chains (n = 1; 11.1%), weak CD34 positivity (n = 1; 11.1%), and T-lymphoblastic lymphoma (n = 1; 11.1%).

Conclusion: Unusual FC results that are not diagnostic of malignancy can be seen in lymph node FNA in a minority of young patients. In our series, these findings were seen mainly in small populations of T cells and occurred primarily in the setting of reactive lymphoid hyperplasia or ectopic thymic tissue. Cytopathologists performing FNA on children should be aware of these abnormalities and, although they may warrant further investigation and follow-up, they are unlikely to be associated with malignancy.
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http://dx.doi.org/10.1159/000448510DOI Listing
January 2017

Predictors of Foreign Body Aspiration in Children.

Otolaryngol Head Neck Surg 2016 09 12;155(3):501-7. Epub 2016 Apr 12.

Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA

Objectives: To examine the sensitivity and specificity of history, physical examination, and radiologic studies as predictors of foreign body aspiration in children.

Study Design: Case series with chart review.

Setting: Tertiary care children's hospital.

Subjects And Methods: Medical records were reviewed for 102 children who presented to our institution from 2006 to 2013 with suspected foreign body aspiration and who underwent endoscopy. Data included symptoms, physical examination, radiologic, and endoscopy findings. Descriptive statistics, sensitivity and specificity, and univariate and multivariable analyses were performed.

Results: A total of 102 patients were included (62% male). The mean age was 3.3 years (SD, 3.7). A foreign body was identified on endoscopy in 69 cases (68%). The most common presenting symptoms were cough (88%), choking/gagging (67%), and wheezing (57%). Decreased breath sounds and wheezing on examination were independently associated with increased odds of foreign body. The most common abnormal radiographic finding was air trapping (33%). The most frequent items retrieved were fragments of seeds and nuts (49%). There were no serious complications related to endoscopy. The sensitivity and specificity of any finding on history, physical examination, and imaging were 100% and 3%, 90% and 33%, 61% and 77%, respectively. Having a positive history, examination, and chest radiograph combined was 46% sensitive and 79% specific.

Conclusions: Patients with airway foreign bodies have varied presentations. The presence of any radiologic finding suggests that endoscopy should be performed, as a foreign body is probable. The absence of any history or physical examination finding was associated with a low likelihood of a foreign body.
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http://dx.doi.org/10.1177/0194599816644410DOI Listing
September 2016

Utility of Fine-Needle Aspiration Biopsy in the Evaluation of Pediatric Head and Neck Masses.

Otolaryngol Head Neck Surg 2016 05 8;154(5):928-35. Epub 2016 Mar 8.

Department of Otolaryngology, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

Objectives: Fine-needle aspiration biopsy (FNAB) has a well-established role in the evaluation of an adult head and neck mass (HNM) but remains underused in children. The objectives of this study were to assess the diagnostic accuracy, safety profile, use of anesthesia, and influence on surgical decision making of FNAB of HNM in the pediatric population.

Study Design: Case series with chart review.

Setting: Tertiary care children's hospital.

Subjects And Methods: In total, 257 consecutive patients with HNM who underwent 338 FNABs from July 2007 to July 2014 were reviewed. Patients ranged in age from 0 to 21 years (mean, 9.3 years); lesions ranged in size from 0.3 to 12.5 cm (mean, 2.4cm). Fine-needle aspiration biopsies were performed in the interventional radiology suite, operating room, clinic, or ward.

Results: The most common patient final diagnoses included reactive lymphadenopathy (n = 99, 38.5%), benign thyroid colloid nodule (n = 31, 12.1%), malignancies (n = 21, 8.2%), and atypical mycobacterial infection (n = 15, 5.8%). On surgical histopathologic and clinical follow-up, overall sensitivity of FNAB was 94.6% and specificity was 97.7%. The complication rate was 2.1%, and general anesthesia or sedation was used for 73% of FNAB. Surgery occurred only 9 times following the 191 patients with negative FNAB results, indicating that 95.3% of unnecessary surgeries were avoided with the assistance of the FNAB result.

Conclusions: Fine-needle aspiration biopsy is an accurate and safe diagnostic tool for guiding management of persistent lymphadenopathy, thyroid nodules, and other HNM in pediatric patients. Negative FNABs can often obviate the need for surgical intervention.
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http://dx.doi.org/10.1177/0194599816631728DOI Listing
May 2016

Button battery injuries in the pediatric aerodigestive tract.

Ear Nose Throat J 2015 Dec;94(12):486-93

Division of Pediatric Otolaryngology, Children's Hospital of Pittsburgh of UPMC, 4401 Penn Ave., Pittsburgh, PA 15224, USA.

Children with a button battery impaction present with nonspecific symptoms that may account for a delay in medical care. We conducted a retrospective study of the clinical presentation, management, and complications associated with button battery ingestion in the pediatric aerodigestive tract and to evaluate the associated long-term morbidity. We reviewed the medical records of 23 patients who were treated for button battery impaction at our tertiary care children's hospital from Jan. 1, 2000, through July 31, 2013. This population was made up of 14 boys and 9 girls, aged 7 days to 12 years (mean: 4 yr). Patients were divided into three groups based on the site of impaction; there were 9 impactions in the esophagus and 7 each in the nasal cavity and stomach. We compiled information on the type and size of each battery, the duration of the impaction, presenting symptoms, treatment, and outcomes. The mean duration of battery impaction was 40.6, 30.7, and 21.0 hours in the esophagus, nasal cavity, and stomach, respectively. We were able to identify the specific type of battery in 13 cases; 11 of these cases (85%) involved a 3-V 20-mm lithium ion battery, including all cases of esophageal impaction in which the type of battery was identified. The most common presenting signs and symptoms were vomiting (n = 7 [30%]), difficulty feeding (n = 5 [22%]), cough (n = 5), and bloody nasal discharge (n = 5); none of the presenting signs and symptoms predicted the severity of the injury or the outcome. The median length of hospital stay was far greater in the esophageal group (12 days) than in the nasal and stomach groups (1 day each; p = 0.006). Battery impaction in the esophagus for more than 15 hours was associated with a significantly longer postoperative hospital stay than impaction for less than 15 hours (p = 0.04). Esophageal complications included strictures (n = 5), perforation (n = 3), and tracheoesophageal fistula formation (n = 2). Clinicians should consider battery impaction in the upper aerodigestive tract as an emergency that can lead to significant long-term morbidity, and therefore immediate surgical intervention is required.
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December 2015

Objective endoscopic findings in patients with recurrent croup: 10-year retrospective analysis.

Int J Pediatr Otorhinolaryngol 2015 Dec 30;79(12):2343-7. Epub 2015 Oct 30.

Department of Otolaryngology, University of Pittsburgh Medical Center, Children's Hospital of Pittsburgh-UPMC, Suite 7121, 4401 Penn Avenue, Faculty Pavilion, 7th Floor, Pittsburgh, PA 15224, United States. Electronic address:

Objective: (1) To determine the incidence and severity of subglottic stenosis on endoscopic evaluation in a pediatric population of patients with recurrent croup. (2) To determine the incidence of abnormal findings on bronchoalveolar lavage and esophageal biopsy in a pediatric population with recurrent croup.

Methods: Case series with historical chart review of clinical data for pediatric patients (age ≤18 years) at a tertiary care children's hospital who underwent endoscopic evaluation of the upper aerodigestive tract with a diagnosis of recurrent croup over a ten-year period (2002-2012). Subglottic stenosis was graded on Myer-Cotton scale. Lipid-laden macrophages on bronchoalveolar lavage were noted as none/small/moderate/large with evidence of reflux noted as moderate or large. Esophageal biopsy specimens were evaluated for evidence of esophagitis. Data is expressed as mean±SEM.

Results: 1825 charts were reviewed of which 197 met inclusion criteria. Mean age at endoscopy was 53±3 months. Subglottic stenosis was noted in 41 patients (20.8%) with 95.1% being mild or Grade I. Abnormal findings on bronchoalveolar lavage were noted on 9.5% of bronchoalveolar lavage specimens. Abnormal esophageal biopsies were noted on 19.9% of specimens. Esophagitis was noted on 8.8% of biopsy specimens.

Conclusions: Subglottic stenosis is a risk factor for recurrent croup. Evidence suggestive of reflux may be noted on bronchoalveolar lavage or esophageal biopsy, but these findings may not correlate with subglottic stenosis in recurrent croup patients.
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http://dx.doi.org/10.1016/j.ijporl.2015.10.039DOI Listing
December 2015

Diagnosis of Pediatric Foreign Body Ingestion: Clinical Presentation, Physical Examination, and Radiologic Findings.

Ann Otol Rhinol Laryngol 2016 Apr 16;125(4):342-50. Epub 2015 Oct 16.

Department of Otolaryngology; Children's Hospital of Pittsburgh of UPMC; University of Pittsburgh School of Medicine; Pittsburgh, Pennsylvania, USA

Objectives: (1) To describe clinical and radiologic findings in patients with esophageal foreign bodies. (2) To examine the sensitivity and specificity of history, physical examination, and radiologic studies in children with suspected foreign body ingestion.

Methods: A retrospective cohort study was performed evaluating all children who underwent esophagoscopy for suspected foreign body ingestion at our institution from 2006 to 2013.

Results: Five hundred forty-three patients were included (54% male). Average age was 4.7 years (SD = 4.1 years). Foreign bodies were identified on esophagoscopy in 497 cases (92%). Ingestion was witnessed in 23% of cases. Most common presenting symptoms were choking/gagging (49%), vomiting (47%), and dysphagia/odynophagia (42%). Most patients with foreign bodies had a normal exam (76%). Most foreign bodies were radiopaque (83%). In 59% of patients with normal chest radiographs, a foreign body was present. Sensitivity and specificity of 1 or more findings on history, physical examination, and imaging were 99% and 0%, 21% and 76%, and 83% and 100%, respectively.

Conclusions: Most patients with esophageal foreign bodies are symptomatic. Although many patients will have a normal physical examination, an abnormal exam should increase suspicion for a foreign body. Most esophageal foreign bodies are radiopaque, but a normal chest radiograph cannot rule out a foreign body.
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http://dx.doi.org/10.1177/0003489415611128DOI Listing
April 2016

Molecular Characterization of Sporadic Pediatric Thyroid Carcinoma with the DNA/RNA ThyroSeq v2 Next-Generation Sequencing Assay.

Pediatr Dev Pathol 2016 Mar-Apr;19(2):115-22. Epub 2015 Sep 14.

1 Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.

The aim of this study was to test the hypothesis that our 60-gene DNA/RNA ThyroSeq v2 next-generation sequence (NGS) assay would identify additional genetic markers, including gene fusions in sporadic pediatric differentiated thyroid carcinomas (DTC) that had no known molecular alterations. Sporadic pediatric DTCs with informative molecular testing (n=18) were studied. We previously tested 15 cases by our standard 7-gene (BRAF, NRAS, HRAS, KRAS, RET/PTC1, RET/PTC3, PAX8/PPARg) mutation panel. Three cases were not tested previously. The standard 7-gene panel identified molecular alterations in 9 of 15 tumors (60%). Cases analyzed by ThyroSeq v2 NGS included the six previously negative cases by the standard 7-gene panel and three cases not previously tested. The NGS assay revealed new gene fusions in four of six previously negative cases (67%). These gene fusions included ETV6/NTRK3 (n=3) and TPR/NTRK1 (n=1). A point mutation (BRAF-V600E) was detected in one of three untested cases. While standard testing could identify only molecular alterations in 60% of cases, with the addition of the ThyroSeq v2 NGS, this increased to 87% (n=13/15). Some cases with chromosomal rearrangements, including ETV6/NTRK3, appear to be associated with an aggressive histopathologic phenotype, but had no documented history of radiation exposure. Additional work is needed to investigate if pediatric DTCs could benefit from a reclassification based on molecular subtypes, which may better reflect their underlying biologic potential. Our data support the use of broad gene panels for the molecular diagnostics of pediatric thyroid nodules to aid future classification, treatment, and clinical management recommendations.
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http://dx.doi.org/10.2350/15-07-1667-OA.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5894824PMC
June 2016

Clinical relevance of quality of life in laryngomalacia.

Laryngoscope 2016 05 30;126(5):1232-5. Epub 2015 Jul 30.

Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, U.S.A.

Objectives/hypothesis: To examine aspects of laryngomalacia and correlate findings with quality of life (QOL).

Study Design: Prospective cross-sectional study.

Methods: Seventy-two patients with laryngomalacia were examined; the mean age was 8.8 weeks. Parents answered questions from the Infant and Toddler Quality of Life Questionnaire-47 (ITQOL-SF47). Fiberoptic laryngoscopy and endoscopic examination of swallowing (FEES) were performed. The presence of laryngomalacia-associated characteristics and swallowing status were recorded. Patient age, sex, presence of reflux, clinical severity, anatomical findings, and swallowing results were evaluated through logistic regression. Independent sample t tests were used to compare responses on the ITQOL-SF47. Overall laryngomalacia ITQOL-SF47 scores were compared to the scores of a large healthy sample population.

Results: Forty-three (60%) patients had mild laryngomalacia, and 61 (85%) patients had findings suggesting gastroesophageal reflux disease. The most common abnormality was shortened aryepiglottic folds. Ten patients failed FEES. Patients with moderate laryngomalacia (χ = 7.62; P = .006) or prolapsing cuneiforms (χ = 4.79; P = .029) were more likely to fail FEES. Laryngomalacia severity impacted parental perception of their child's health (P < .05). Parents of children who demonstrated aspiration or penetration reported significant emotional impact (mean = 56.9; t = 2.74; P = .008). The mean ITQOL-SF47 scores of patients were significantly lower in certain sections than the reported general sample population.

Conclusions: Epiglottal prolapse correlated with severity of laryngomalacia and cuneiform prolapse with swallowing dysfunction. Perceptions of worsening health and physical ability were related to severity of disease. Swallowing dysfunction had a significant emotional impact on parental daily life. Infants with laryngomalacia have a lower QOL LEVEL OF EVIDENCE: 3 Laryngoscope, 126:1232-1235, 2016.
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http://dx.doi.org/10.1002/lary.25491DOI Listing
May 2016

Laryngomalacia and swallowing function in children.

Laryngoscope 2016 Feb 7;126(2):478-84. Epub 2015 Jul 7.

Center for Pediatric ENT-Head and Neck Surgery, Boynton Beach, Florida, U.S.A.

Objectives/hypothesis: 1) To determine the prevalence of dysphagia in children with laryngomalacia, 2) To ascertain whether severity of laryngomalacia influences the presence of swallowing dysfunction, and 3) To examine whether patients with medical comorbidities and laryngomalacia have a higher prevalence of swallowing dysfunction.

Study Design: Retrospective cohort study.

Methods: All patients seen in the aerodigestive center at our institution between January 2007 and December 2012 with the diagnosis of laryngomalacia were included. Swallowing function was assessed by symptoms, clinical swallowing evaluations (CSE) performed by speech pathologists, modified barium swallow (MBS) studies, and fiberoptic endoscopic evaluations of swallowing (FEES).

Results: There were 324 patients with laryngomalacia identified (41.4% female, 58.6% male). Severity of laryngomalacia was categorized in 279 patients, with 62.7% mild, 28.7% moderate, and 8.6% severe. Gastroesophageal reflux disease (GERD) was diagnosed in 69.8% of patients. Other medical comorbidities included Down syndrome (3.1%), neurological impairment (6.5%), and congenital heart disease (0.9%). Symptoms of dysphagia or feeding difficulty were present in 163/324 (50.3%), and failure to thrive was present in 31/324 patients (9.6%). At least one abnormal swallowing assessment was present in 97/120 (80.8%) patients presenting with subjective dysphagia and 43/65 (66.2%) patients without subjective dysphagia. A total of 140/185 (75.7%) patients had at least one abnormal baseline swallowing assessment. There was no significant relationship between severity of laryngomalacia and presence of abnormal swallowing function based on symptoms, CSE, MBS, or FEES. However, patients with greater severity were more likely to have failure to thrive. There was not a significant association between the presence of swallowing dysfunction or disease severity and medical comorbidities such as Down syndrome, neurological impairment, or congenital heart disease. However, GERD was more likely to be present in patients with moderate and severe laryngomalacia than in patients with mild disease.

Conclusions: Swallowing dysfunction is common in children with laryngomalacia regardless of disease severity or other medical comorbidities. Swallowing studies are frequently abnormal in laryngomalacia patients presenting both with and without subjective symptoms of dysphagia. Dysphagia assessment should be considered as part of the evaluation of infants with laryngomalacia.

Level Of Evidence: 4.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5064436PMC
http://dx.doi.org/10.1002/lary.25440DOI Listing
February 2016

Predictors of Intensive Care Unit Stay After Pediatric Supraglottoplasty.

JAMA Otolaryngol Head Neck Surg 2015 Aug;141(8):704-9

Department of Otolaryngology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

Importance: Supraglottoplasty is a common procedure performed without evidence-based postoperative management plans. Patients are routinely admitted to the intensive care unit (ICU) postoperatively, but this may not be necessary in all cases.

Objective: To determine (1) whether routine admission to the ICU after supraglottoplasty is warranted in all patients who undergo this procedure and (2) which factors predict requirement for ICU-level care.

Design, Setting, And Participants: Retrospective case series and analysis of immediate postoperative outcomes of all children aged 1 month to 18 years who underwent supraglottoplasty at 1 tertiary-care children's hospital from January 1, 2008, through January 31, 2014. Exclusion criteria included preoperative admission to the ICU, preoperative need for positive-pressure ventilation, history of major airway reconstruction, or any concomitant other major procedure.

Exposure: Supraglottoplasty.

Main Outcomes And Measures: Need for ICU-level care as defined by need for intubation, positive-pressure ventilation, multiple doses of racemic epinephrine, or oxygen via nasal cannula at greater than 4 L/min within the first 24 hours.

Results: Of 223 patients identified, 25 (11.2%) met our criteria for ICU-level care. Nine patients required intubation. Twenty of the 25 patients met ICU criteria within 4 hours of surgery. Univariate analysis was performed on 38 risk factors. Risk factors for ICU requirement that remained statistically significant on multivariable analysis (P < .05) included surgical duration longer than 30 minutes (odds ratio [OR], 4.48 [95% CI, 1.51-13.19]; P = .007), nonwhite race (OR, 4.42 [95% CI, 1.54-12.66]; P = .006), and a preoperative diagnosis of gastroesophageal reflux disease (OR, 0.10 [95% CI, 0.09-0.36]; P < .001).

Conclusions And Relevance: Our study suggests that most children undergoing supraglottoplasty do not require ICU-level care postoperatively. Those who require ICU-level care are likely to be identified within the first 4 hours after surgery. Consideration for routine ICU admission should be given to those with longer surgical duration and those of nonwhite race.
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http://dx.doi.org/10.1001/jamaoto.2015.1033DOI Listing
August 2015

Effect of Adenotonsillectomy on Central and Obstructive Sleep Apnea in Children with Down Syndrome.

Otolaryngol Head Neck Surg 2015 Oct 4;153(4):644-8. Epub 2015 Jun 4.

Pediatric Otolaryngology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA Department of Otolaryngology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Objectives: To determine and quantify changes in both central and obstructive sleep apnea in patients with Down syndrome (DS) after adenotonsillectomy (AT).

Study Design: Case series with chart review.

Setting: Tertiary care children's hospital.

Subjects And Methods: The records of all patients with DS who underwent AT for sleep-disordered breathing between November 2008 and December 2014 were examined. In total, 113 patients were identified, and 36 of these patients had pre- and postoperative polysomnograms (PSGs) that were analyzed for obstructive and central components. Wilcoxon signed-rank test, paired t test, and McNemar test were used to examine pre- and postoperative PSG differences. Logistic regression and multivariate analysis of variance of patient characteristics (between subjects) and PSG results (within subjects) were conducted.

Results: The mean (SD) patient age was 5.5 (4.0) years (range, 0.9-15 years); 50.0% were male. After AT, significant reductions were identified in both obstructive apnea-hypopnea index (AHI) (P < .001) and overall AHI (P < .001). Among the 15 patients with severe obstructive sleep apnea, 86.7% experienced a significant AHI reduction to moderate or mild disease (P < .001). In addition, of the 15 patients with central sleep apnea (central apnea index [CAI] >1), 66.7% had resolution of central sleep apnea postoperatively (P = .004). There was also a significant interaction identified between CAI reduction, preoperative CO2 retention, and adenoid size, F(2, 20) = 6.87, P = .05.

Conclusion: Children with DS who underwent AT demonstrated significant reductions in both obstructive and central apneic indices on PSG. A significant number of patients with central sleep apnea demonstrated resolution postoperatively. Additional analysis demonstrated a significant interaction between CO2 retention, adenoid size, and postoperative CAI reduction.
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http://dx.doi.org/10.1177/0194599815587877DOI Listing
October 2015

Identification of Unique, Heterozygous Germline Mutation, STK11 (p.F354L), in a Child with an Encapsulated Follicular Variant of Papillary Thyroid Carcinoma within Six Months of Completing Treatment for Neuroblastoma.

Pediatr Dev Pathol 2015 Jul-Aug;18(4):318-23. Epub 2015 Mar 9.

1 Division of Endocrinology, Department of Pediatrics, Children's Hospital of Pittsburgh, UPMC, 4401 Penn Ave, Pittsburgh, PA, USA.

Papillary thyroid carcinoma (PTC) is rare in children, although it is a known secondary malignancy after treatment for neuroblastoma (NB). The interval between NB treatment completion and PTC is usually more than 5 years. A 4-year-old, female patient with a high risk adrenal NB was found to have a 2.9-cm, right thyroid nodule on surveillance chest computed tomography (CT) 6 months after completion of her NB treatment (induction chemotherapy, tumor resection, autologous stem cell transplantation, external beam radiation to the abdominal tumor site, immunotherapy, and retinoic acid). Posttreatment surveillance included iodine-123-metaiodobenzylguanidine scans and CT scans. Fine-needle aspiration of the thyroid nodule diagnosed a follicular neoplasm, which was negative for BRAF, NRAS, KRAS, HRAS, PAX8/PPARg, and RET/PTC mutations, without evidence of metastatic NB. Nodule histology demonstrated an encapsulated follicular variant of PTC (FVPTC). Next-generation sequence analysis for a 46 cancer-gene profile was performed on both tumors with subsequent peripheral blood DNA testing. A heterozygous missense mutation in STK11 (F354L) was identified in both the NB and FVPTC. This mutation was also detected in peripheral blood mononuclear cells. Two additional heterozygous somatic missense mutations of uncertain significance were identified: KDR/VEGF receptor 2 (Q472H) on chromosome 4 and MET (N375S) on chromosome 7. To our knowledge, this is the shortest reported duration from completion of NB treatment to detection of thyroid cancer. The association of the STK11 gene with Peutz-Jeghers syndrome, lung adenocarcinomas, and medullary thyroid cancer leads to a possible association between this genetic variant and our patient's tumors.
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http://dx.doi.org/10.2350/15-01-1597-CR.1DOI Listing
September 2015

Can malignant thyroid nodules be distinguished from benign thyroid nodules in children and adolescents by clinical characteristics? A review of 89 pediatric patients with thyroid nodules.

Thyroid 2015 Apr 3;25(4):392-400. Epub 2015 Mar 3.

1 Division of Endocrinology, Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC , Pittsburgh, Pennsylvania.

Background: Thyroid nodules are less common in children than adults, but the risk of malignancy in thyroid nodules is much higher in children. The ability to characterize pediatric thyroid nodules has improved with the use of ultrasound-guided fine-needle aspiration, the Bethesda System for Reporting Thyroid Cytopathology (TBSRTC) classification system, and expanded molecular testing. Nevertheless, stratification criteria to predict thyroid malignancy in children are poorly defined. Our objective was to determine if clinical presentation and molecular genetics could predict malignancy in pediatric thyroid nodules.

Methods: Retrospective chart review of patients ≤18 years of age at the Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center with the diagnosis of a thyroid nodule from January 2007 to January 2012 was conducted. Eighty-nine subjects fulfilled the inclusion criteria: 1) thyroid nodule ≥0.8 cm and biopsy (n=76), or 2) thyroid nodule ≥0.8 cm, no biopsy, and ultrasound follow-up for at least 2 years (n=13).

Results: Twenty-four (27%) of 89 patients were diagnosed with thyroid cancer (50% papillary thyroid carcinoma [PTC], 50% follicular variant of papillary thyroid carcinoma [FVPTC]). Features associated with malignancy included larger nodule size, palpable nodule, or palpable lymphadenopathy. There were no differences in presenting features between patients with PTC and those with FVPTC. Thyroid malignancy was diagnosed in all nine patients with a molecular abnormality (BRAF, RAS, RET/PTC, PAX8/PPARγ).

Conclusions: Clinical features, FNA cytology, and molecular genetics are valuable tools to discriminate benign from malignant nodules in pediatric patients. This information is important to direct subsequent clinical management.
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http://dx.doi.org/10.1089/thy.2014.0312DOI Listing
April 2015
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