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1.
J Pediatr Gastroenterol Nutr ; 77(4): 460-467, 2023 10 01.
Article in English | MEDLINE | ID: mdl-37438891

ABSTRACT

OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.


Subject(s)
Deglutition Disorders , Gastroenterology , Medicine , Humans , Child , Deglutition Disorders/diagnosis , Deglutition Disorders/etiology , Deglutition Disorders/therapy , Lung
2.
Paediatr Anaesth ; 33(11): 883-893, 2023 11.
Article in English | MEDLINE | ID: mdl-37408495

ABSTRACT

Laryngotracheal stenosis, congenital or acquired, is a common cause of pediatric airway obstruction. Acquired subglottic stenosis frequently results from prolonged neonatal intubation. The clinical presentation of subglottic stenosis is variable, ranging from biphasic stridor and frequent upper respiratory infections to acute airway compromise. Optimal patient care requires clinical coordination within a multidisciplinary subspecialty team. Medical management includes optimizing respiratory status, gastroesophageal reflux, speech, feeding, nutrition therapies, and providing psychosocial support. If surgical intervention is required, the otolaryngologist, anesthesiologist, and perioperative team must collaborate closely to ensure successful operative outcomes. This narrative review of laryngotracheal stenosis will discuss the pathophysiology, clinical evaluation, medical management, and surgical interventions, and focus on the perioperative anesthetic considerations for children undergoing laryngotracheal reconstruction.


Subject(s)
Anesthesia , Laryngostenosis , Plastic Surgery Procedures , Tracheal Stenosis , Infant, Newborn , Child , Humans , Constriction, Pathologic/complications , Constriction, Pathologic/surgery , Treatment Outcome , Laryngostenosis/surgery , Laryngostenosis/etiology , Tracheal Stenosis/surgery , Tracheal Stenosis/complications , Anesthesia/adverse effects , Retrospective Studies
3.
Am J Hum Genet ; 103(5): 679-690, 2018 11 01.
Article in English | MEDLINE | ID: mdl-30401457

ABSTRACT

Non-secretor status due to homozygosity for the common FUT2 variant c.461G>A (p.Trp154∗) is associated with either risk for autoimmune diseases or protection against viral diarrhea and HIV. We determined the role of FUT2 in otitis media susceptibility by obtaining DNA samples from 609 multi-ethnic families and simplex case subjects with otitis media. Exome and Sanger sequencing, linkage analysis, and Fisher exact and transmission disequilibrium tests (TDT) were performed. The common FUT2 c.604C>T (p.Arg202∗) variant co-segregates with otitis media in a Filipino pedigree (LOD = 4.0). Additionally, a rare variant, c.412C>T (p.Arg138Cys), is associated with recurrent/chronic otitis media in European-American children (p = 1.2 × 10-5) and US trios (TDT p = 0.01). The c.461G>A (p.Trp154∗) variant was also over-transmitted in US trios (TDT p = 0.01) and was associated with shifts in middle ear microbiota composition (PERMANOVA p < 10-7) and increased biodiversity. When all missense and nonsense variants identified in multi-ethnic US trios with CADD > 20 were combined, FUT2 variants were over-transmitted in trios (TDT p = 0.001). Fut2 is transiently upregulated in mouse middle ear after inoculation with non-typeable Haemophilus influenzae. Four FUT2 variants-namely p.Ala104Val, p.Arg138Cys, p.Trp154∗, and p.Arg202∗-reduced A antigen in mutant-transfected COS-7 cells, while the nonsense variants also reduced FUT2 protein levels. Common and rare FUT2 variants confer susceptibility to otitis media, likely by modifying the middle ear microbiome through regulation of A antigen levels in epithelial cells. Our families demonstrate marked intra-familial genetic heterogeneity, suggesting that multiple combinations of common and rare variants plus environmental factors influence the individual otitis media phenotype as a complex trait.


Subject(s)
Fucosyltransferases/genetics , Genetic Variation/genetics , Otitis Media/genetics , Animals , COS Cells , Cell Line , Chlorocebus aethiops , Ear, Middle/microbiology , Exome/genetics , Female , HEK293 Cells , Humans , Male , Mice , Mice, Inbred C57BL , Microbiota/physiology , Otitis Media/microbiology , Pedigree , Galactoside 2-alpha-L-fucosyltransferase
4.
Hum Mutat ; 40(8): 1156-1171, 2019 08.
Article in English | MEDLINE | ID: mdl-31009165

ABSTRACT

A genetic basis for otitis media is established, however, the role of rare variants in disease etiology is largely unknown. Previously a duplication variant within A2ML1 was identified as a significant risk factor for otitis media in an indigenous Filipino population and in US children. In this report exome and Sanger sequencing was performed using DNA samples from the indigenous Filipino population, Filipino cochlear implantees, US probands, Finnish, and Pakistani families with otitis media. Sixteen novel, damaging A2ML1 variants identified in otitis media patients were rare or low-frequency in population-matched controls. In the indigenous population, both gingivitis and A2ML1 variants including the known duplication variant and the novel splice variant c.4061 + 1 G>C were independently associated with otitis media. Sequencing of salivary RNA samples from indigenous Filipinos demonstrated lower A2ML1 expression according to the carriage of A2ML1 variants. Sequencing of additional salivary RNA samples from US patients with otitis media revealed differentially expressed genes that are highly correlated with A2ML1 expression levels. In particular, RND3 is upregulated in both A2ML1 variant carriers and high-A2ML1 expressors. These findings support a role for A2ML1 in keratinocyte differentiation within the middle ear as part of otitis media pathology and the potential application of ROCK inhibition in otitis media.


Subject(s)
Down-Regulation , Gene Expression Profiling/methods , Mutation , Otitis Media/genetics , Sequence Analysis, DNA/methods , alpha-Macroglobulins/genetics , Adolescent , Adult , Child , Child, Preschool , Female , Finland , Gene Expression Regulation , Genetic Predisposition to Disease , Humans , Infant , Male , Middle Aged , Pakistan , Pedigree , Philippines , Sequence Analysis, RNA , Signal Transduction , United States , Young Adult
5.
Clin Gastroenterol Hepatol ; 17(12): 2455-2462, 2019 11.
Article in English | MEDLINE | ID: mdl-30708107

ABSTRACT

BACKGROUND & AIMS: Evaluation and treatment of children with eosinophilic esophagitis (EoE) requires serial endoscopic, visual, and histologic assessment by sedated esophagogastroduodenoscopy (EGD). Unsedated transnasal endoscopy (TNE) was reported to be successful in a pilot study of children. We evaluated video goggle and virtual reality-based unsedated TNE in children with EoE, collecting data on rates of completion, adverse events, and adequacy of visual and histologic findings. METHODS: We performed a retrospective study of 190 children and young adults (age, 3-22 y) who underwent video goggle or virtual reality-based unsedated TNE from January 2015 through February 2018. We analyzed data on patient demographics, procedure completion, endoscope type, adverse events, visual and histologic findings, estimated costs, and duration in the facility. Esophageal biopsies from the first 173 subjects who underwent TNE were compared with those from previous EGD evaluations. RESULTS: During 300 attempts, 294 TNEs were performed (98% rate of success). Fifty-four patients (age, 6-18 y) underwent multiple TNEs for dietary or medical management of EoE. There were no significant adverse events. Visual and histologic findings were adequate for assessment of EoE. TNE reduced costs by 53.4% compared with EGD (TNE $4393.00 vs EGD $9444.33). TNE was used increasingly from 2015 through 2017, comprising 31.8% of endoscopies performed for EoE. The total time spent in the clinic (front desk check-in to check-out) in 2018 was 71 minutes. CONCLUSIONS: In a retrospective study of 190 children and young adults (age, 3-22 y) who underwent video goggle or virtual reality-based unsedated TNE, TNE was safe and effective and reduced costs of EoE monitoring. Advantages of TNE include reduced risk and cost associated with anesthesia as well as decreased in-office time, which is of particular relevance for patients with EoE, who require serial EGDs.


Subject(s)
Eosinophilic Esophagitis/pathology , Esophagoscopy/methods , Smart Glasses , Virtual Reality , Adolescent , Biopsy , Child , Child, Preschool , Disease Management , Endoscopy, Digestive System/economics , Esophagoscopy/economics , Esophagus/pathology , Female , Humans , Male , Retrospective Studies , Time Factors , Young Adult
7.
J Pediatr Gastroenterol Nutr ; 66(1): 33-36, 2018 01.
Article in English | MEDLINE | ID: mdl-28505050

ABSTRACT

Monitoring therapy in esophageal inflammatory disorders such as eosinophilic esophagitis and reflux esophagitis often requires frequent endoscopic evaluation. We recently reported the effective use of unsedated in-office transnasal esophagoscopy that significantly decreased costs and anesthetic exposure associated with pediatric esophagoscopy in eosinophilic esophagitis. Here we report a series of pediatric patients with esophagitis with gastrostomy tubes who underwent unsedated transgastrostomy esophagoscopy (TGE) in an office setting. Nine patients (ages 16 months-21 years) tolerated TGE without significant adverse events. Biopsy specimens were adequate for evaluation. This series confirms that unsedated in-office TGE can be used to successfully obtain mucosal biopsies to monitor esophageal inflammatory conditions in children without the use of sedation.


Subject(s)
Ambulatory Care/methods , Eosinophilic Esophagitis/diagnostic imaging , Esophageal Mucosa/diagnostic imaging , Esophagoscopy/methods , Gastrostomy , Adolescent , Child , Child, Preschool , Eosinophilic Esophagitis/pathology , Eosinophilic Esophagitis/surgery , Esophageal Mucosa/pathology , Female , Humans , Infant , Male , Outcome Assessment, Health Care , Retrospective Studies , Young Adult
8.
Paediatr Anaesth ; 28(9): 768-773, 2018 09.
Article in English | MEDLINE | ID: mdl-29962064

ABSTRACT

Tracheomalacia is a broad term used to describe an abnormally compliant trachea that can lead to exaggerated collapse and obstruction with expiration. We describe the perioperative management of a complex pediatric patient undergoing a posterior tracheopexy which is a relatively new surgical treatment, with a novel surgical approach-thoracoscopy. This procedure has competing surgical and anesthetic needs and presents unique challenges to the physicians involved in caring for these patients. We also review the current literature on pediatric tracheomalacia and examine the newest treatment options to highlight the potential anesthetic challenges and pitfalls associated with management.


Subject(s)
Anesthesia/methods , Bronchoscopy/methods , Thoracoscopy/methods , Trachea/physiopathology , Trachea/surgery , Tracheomalacia/diagnosis , Tracheomalacia/surgery , Anesthetics/administration & dosage , Child, Preschool , Female , Humans , Perioperative Care/methods , Tracheomalacia/classification , Tracheomalacia/physiopathology
9.
Gastrointest Endosc ; 83(2): 299-306.e1, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26142551

ABSTRACT

BACKGROUND AND AIMS: Unsedated transnasal endoscopy (TNE) is safer and less costly than sedated EGD. The aim of this study was to evaluate the performance of TNE with biopsies in monitoring the esophageal mucosa of pediatric patients with eosinophilic esophagitis. METHODS: Patients between 8 and 17 years of age with eosinophilic esophagitis and their parents were enrolled. Unsedated TNE was performed. A 2.8-mm (1.2-mm channel) or a 4-mm flexible bronchoscope (2-mm channel) was used, and esophageal biopsy specimens were obtained. Biopsy specimen analysis, duration, adverse events, and billing charges of TNE were assessed. Immediately after TNE and a minimum of 2 weeks later, a modified Group Health Association of America 9 survey and a preference questionnaire were completed, respectively. RESULTS: Twenty-one of 22 enrolled patients underwent TNE. TNE was performed with no serious adverse events. Histopathological analysis revealed 0 eosinophils per high-power field (n = 12), fewer than 15 eosinophils per high-power field (n = 4), and more than 15 eosinophils per high-power field (n = 5). The total epithelial surface area of mucosal biopsy samples from either TNE Forceps (1.2 mm or 2 mm biopsy channel forceps) compared with those obtained during the subject's previous EGD by using standard endoscopic forceps was not statistically different (P = .308 [1.2 mm]/P = .492 [2 mm]). All parents and 76.2% of subjects would undergo the TNE again. TNE was preferred over EGD by 85.7% of parents and 52.4% of subjects. The modified Group Health Association of America 9 survey revealed a high degree of satisfaction (average, 43.19 ± 2.6; maximum score, 45). Charges associated with TNE were 60.1% lower than for previous EGDs. CONCLUSIONS: Unsedated TNE is an effective, lower-cost procedure for monitoring the esophageal mucosa of children with eosinophilic esophagitis.


Subject(s)
Eosinophilic Esophagitis/surgery , Esophagoscopy/methods , Intestinal Mucosa/pathology , Natural Orifice Endoscopic Surgery/methods , Patient Satisfaction , Adolescent , Biopsy/methods , Child , Eosinophilic Esophagitis/diagnosis , Female , Humans , Intestinal Mucosa/surgery , Male , Mouth
10.
Paediatr Anaesth ; 26(2): 132-8, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26530711

ABSTRACT

Pediatric laryngotracheal injuries from blunt neck trauma are extremely rare, but can be potentially catastrophic. Early diagnosis and skillful airway management is critical in avoiding significant morbidity and mortality associated with these cases. We present a case of a patient who suffered a complete tracheal transection and cervical spine fracture following a clothesline injury to the anterior neck. A review of the mechanisms of injury, clinical presentation, initial airway management, and anesthetic considerations in laryngotracheal injuries from blunt neck trauma in children are presented.


Subject(s)
Intubation, Intratracheal/methods , Larynx/injuries , Spinal Fractures/surgery , Trachea/injuries , Tracheostomy/methods , Wounds, Nonpenetrating/surgery , Androstanols , Anesthetics, Intravenous , Child , Fentanyl , Humans , Laryngoscopy/methods , Larynx/diagnostic imaging , Larynx/surgery , Male , Midazolam , Neuromuscular Nondepolarizing Agents , Radiography , Rocuronium , Spinal Fractures/diagnostic imaging , Spinal Fractures/etiology , Trachea/diagnostic imaging , Trachea/surgery , Wounds, Nonpenetrating/complications , Wounds, Nonpenetrating/diagnostic imaging
11.
Fetal Diagn Ther ; 35(2): 137-40, 2014.
Article in English | MEDLINE | ID: mdl-24281263

ABSTRACT

We present a case report of a 28-year-old primigravida with a singleton pregnancy complicated by a fetal bronchogenic cyst compressing the left mainstem bronchus with resultant hyperinflation of the entire left lung and rightward mediastinal shift. An ex utero intrapartum treatment to resection of the fetal bronchogenic cyst via a fetal thoracotomy was performed at 36 weeks' gestational age, circumventing a potentially complicated neonatal airway emergency at birth.


Subject(s)
Airway Obstruction/diagnostic imaging , Bronchogenic Cyst/diagnostic imaging , Adult , Airway Obstruction/surgery , Bronchogenic Cyst/surgery , Female , Fetal Therapies , Humans , Magnetic Resonance Imaging , Pregnancy , Thoracotomy/methods , Ultrasonography, Prenatal
12.
Int J Pediatr Otorhinolaryngol ; 183: 112044, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39029310

ABSTRACT

OBJECTIVES: In the pediatric population, nasal septal perforations (NSP) are rare, and management is not well described. The use of various techniques has been reported in adults, including the use of interposition grafts, however this technique has not been described in the pediatric population. Our hypothesis is that the closure rate using absorbable d-lactide and l-lactide (each 50 %), polymer (PDLLA) plates as interposition grafts with temporalis fascia for NSP repair in the pediatric population will be an effective method compared to previous techniques. METHODS: Chart review was performed on patients who underwent NSP repair before June 2021, results were compared to a prospective evaluation of an interposition plate graft for repair at a tertiary care children's hospital. RESULTS: Fifteen patients via previous techniques and 5 patients via PDLLA and fascia graft were reviewed. Etiology of perforations included 45 % idiopathic, 25 % traumatic, and 15 % iatrogenic. In the previous techniques group, 10 were male, mean (median) age 14.4 years (15.2). Average size of NSP was 12.6 mm ± 6.6 mm (SD). 14/15 (93 %) patients had resolution of symptoms at 10-week follow-up, and 2/15 (13 %) required repeat repair. Five prospective patients were repaired with a PDLLA and fascia interposition graft, 4 were female, mean (median) age 14.6 years (Nassif and Scott, 2021 Feb 1) [14]. Average size of NSP was 11 mm ± 2.2 mm (SD). 100 % had resolution of symptoms at 10-week follow-up, 0 needed repeat surgery. No significant difference was found in size of NSP or in need for repeat procedure (p > 0.05) between the groups. CONCLUSION: Use of absorbable PDLLA interposition grafts with temporalis fascia for NSP repair in the pediatric population is effective at closing and resolving symptoms associated with NSP.


Subject(s)
Fascia , Nasal Septal Perforation , Humans , Female , Male , Adolescent , Prospective Studies , Fascia/transplantation , Nasal Septal Perforation/surgery , Treatment Outcome , Child , Absorbable Implants , Polyesters , Temporal Muscle/transplantation
13.
Int J Pediatr Otorhinolaryngol ; 182: 112016, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38943832

ABSTRACT

OBJECTIVE: To define the essential elements of the intake questionnaire that will be a part of a larger multicenter registry for aerodigestive patients. METHODS: A modified Delphi method was utilized to obtain consensus on the data elements that should warrant inclusion in the final research database. Patient questionnaires from the eight participating institutions were reviewed and individual elements were aggregated into 14 categories. RESULTS: A total of 198 initial elements were voted on for inclusion. The categories included demographics, respiratory symptoms, gastrointestinal symptoms, ear nose and throat symptoms, feeding, birth history, medical history, surgical history, family history, social history, medications prior to evaluation, devices used prior to evaluation, prior diagnostic evaluations, and prior evaluation by aerodigestive team members. 83 of the 198 elements met consensus for inclusion in the final registry for an inclusion rate of 41.9 %. Three separate rounds of ranking were required to obtain consensus. CONCLUSION: The aerodigestive registry is an important initiative that will help foster research and help guide future management. The intake questionnaire of the registry is a critical component of this project, and the consensus obtained during this study should help create a streamlined and efficient registry that will help all aerodigestive patients on a national level.


Subject(s)
Delphi Technique , Registries , Humans , Surveys and Questionnaires , Consensus , Female , Male
14.
Int J Pediatr Otorhinolaryngol ; 170: 111602, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37224738

ABSTRACT

OBJECTIVES: Burnout among healthcare workers is a public health crisis. Burnout is associated with elevated cynicism, emotional exhaustion, and low job satisfaction. Methods to combat burnout have been challenging to identify. Based on positive experiences of pediatric aerodigestive team members, we hypothesized that social support in multidisciplinary aerodigestive teams moderates the effects of burnout on job satisfaction. METHODS: Using a survey of the Aerodigestive Society, members of Aerodigestive teams (N = 119) completed demographics, the Maslach Burnout Inventory, and measures of job satisfaction, emotional, and instrumental social support. In addition to assessing relationships between components of burnout and job satisfaction, six tests were conducted using PROCESS to ascertain the degree to which social support moderated these relationships. RESULTS: Similar to US healthcare base rates, burnout scores in this sample suggest that a third-to-half felt Emotionally Exhausted and Burned Out from work "A few times a month"-to-"Every Day." Simultaneously, however, the majority in sample (60.6%) noted feeling that they "positively impact others' lives" with 33.3% endorsing "Every Day." Job satisfaction was strikingly high at 89%, with most reporting Aerodigestive team affiliation related to higher job satisfaction. Both Emotional and Instrumental social support moderated the effect of Cynicism and Emotional Exhaustion on Job Satisfaction, with higher Job Satisfaction scores in conditions of high support. CONCLUSIONS: These results support the hypothesis that social support from a multidisciplinary aerodigestive team moderates the effect of burnout in its team members. Further work is needed to understand if membership in other interprofessional healthcare teams can help combat the negative effects of burnout.


Subject(s)
Burnout, Professional , Job Satisfaction , Humans , Child , Burnout, Professional/epidemiology , Burnout, Professional/psychology , Social Support , Emotions , Surveys and Questionnaires
15.
ERJ Open Res ; 9(2)2023 Mar.
Article in English | MEDLINE | ID: mdl-37057093

ABSTRACT

Background: Antagonists to the P2X purinergic receptors on airway sensory nerves relieve refractory or unexplained chronic cough (RCC/UCC) but can evoke unwanted dysgeusias because the gustatory nerves innervating taste buds express this same family of receptors. However, the subunit composition of the P2X receptors in these systems may differ, with implications for pharmacological intervention of RCC/UCC. In most species, the extrapulmonary airway nerves involved in cough predominantly express P2X3 subunits that form homotrimeric P2X3 receptors. In contrast, most sensory nerves innervating taste buds in mice express both P2X2 and P2X3 subunits, so the majority of receptors in that system are likely P2X2/P2X3 heteromers. Methods: Since neural P2X subunit composition can differ across species, we used immunohistochemistry to test whether taste nerves in humans and rhesus macaque monkeys express both P2X2 and P2X3 as in mice. Results: In taste bud samples of fungiform papillae and larynx from humans and monkeys, all taste bud samples exhibited P2X3+ nerve fibres, but the majority lacked substantial P2X2+. Of the 35 human subjects, only four (one laryngeal and three fungiform) showed strong P2X2 immunoreactivity in taste nerves; none of the rhesus monkey samples showed immunoreactivity for P2X2. Conclusions: These findings suggest that for most humans, unlike mice, taste buds are innervated by nerve fibres predominantly expressing only P2X3 homomeric receptors and not P2X2/P2X3 heteromers. Thus, antagonists specific for P2X3 homomeric receptors might not be spared from affecting taste function in RCC/UCC patients.

16.
Laryngoscope ; 133(4): 970-976, 2023 04.
Article in English | MEDLINE | ID: mdl-35730686

ABSTRACT

OBJECTIVE: To explore patient-reported outcome measures of pediatric paradoxical vocal fold motion through a multi-institutional study of geographically diverse United States medical facilities to assess long-term management and outcomes. METHODS: Eligible participants >8 years of age diagnosed with PVFM over a 10-year period from 7 tertiary pediatric hospitals were invited to complete a survey addressing study objectives. RESULTS: 65 participants completed the survey, of whom 80% were female, 75% reported a 3.5 grade point average or better, and 75% identified as competitive athletes or extremely athletic individuals. Participants rated their perceived efficacy of 13 specific treatments. Only five treatments were considered effective by a majority of the participants who tried them. The treatments that participants tried most often were breathing exercises (89.2%), bronchodilator treatments (45%), and allergy medications (35.4%). 78.8% of participants reported receiving more than one treatment and 25% reported receiving a combination of bronchodilators, anticholinergics, and steroids. At the time of PVFM diagnosis, 38% of participants had no idea when their symptoms would completely resolve. 23.3% of participants did not experience symptom resolution until greater than 1 year after diagnosis. CONCLUSIONS: Traditional management tools such as breathing exercises and biofeedback treatments may not provide the long-term benefit that providers anticipate. In addition to these commonly used management strategies, highly efficacious techniques such as counseling and lifestyle management should be incorporated into the long-term management of patients whose symptoms are refractory to traditional care. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:970-976, 2023.


Subject(s)
Laryngoscopes , Vocal Cord Dysfunction , Humans , Female , Child , Male , Vocal Cord Dysfunction/diagnosis , Vocal Cord Dysfunction/therapy , Biofeedback, Psychology , Breathing Exercises , Patient Reported Outcome Measures , Vocal Cords
17.
Int J Pediatr Otorhinolaryngol ; 172: 111631, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37567085

ABSTRACT

INTRODUCTION: Comorbidities such as chronic lung disease and gastroesophageal reflux (GERD), prematurity, and numerous other conditions may impact the success of LTR. Single-center studies are limited in terms of patient numbers and may be underpowered. OBJECTIVES: To analyze the impact of specific comorbidities on the operation-specific and overall surgical success of LTR in a large multicenter cohort and validate a predictive model for surgical success. METHODS: A large retrospective multicenter 10-year review was undertaken to validate the data of a previous single-center study (Wertz et al. Laryngoscope 2020) which identified specific predictive comorbidities which impacted LTR outcomes. A Monte Carlo simulation based on the previous data set suggested that 300-400 cases would be needed to optimize the statistical power of a Bayesian model developed from the single-center data to predict surgical success. An IRB-approved data-sharing agreement was executed for 4 large U.S. CENTERS: A virtual REDCap® data entry form inquired about patient characteristics that best predicted surgical success in the single-center model. These included demographics, surgical approaches, cardiac, airway, genetic, endocrine, musculoskeletal, gastrointestinal, and pulmonary comorbidities; details of the surgical procedures, and results of esophagogastroduodenoscopy (EGD), esophageal pH/impedance and flexible bronchoscopy with bronchioalveolar lavage (BAL) were included. Surgical success defined as successful decannulation or resolution of airway symptoms was recorded as single surgery success and overall success following open surgical revision surgery. Multivariate Bayesian analysis, logistical regression, and Kaplan-Meier analysis were performed. RESULTS: 542 patients were identified, including 165 from the single-center study and an additional 377 patients from the multicenter group. The median age was 36 months at the time of the most recent surgery. 70.9% of the LTRs were double-staged procedures. The overall success rate was 86.4% and operation-specific success rate was 69.2%. The specific comorbidities and aerodigestive test results that impacted success based on univariate analysis included staging, bronchiectasis, pulmonary hypertension, GERD, ASD, PDA, grade of stenosis, advanced levels of stenosis, Trisomy 21, MRSA, prior open surgery at another hospital, and gross appearance on EGD. Bayesian model averaging with backward selection was used to validate and refine a predictive model for surgical success with favorable receiver operating curve characteristics - AUC values of 0.827 for single surgery success and 0.797 for overall success. DISCUSSION: With over 500 patients reviewed, this was the largest multicenter study of LTR to date, which elucidated the impact of comorbidities on success with LTR and was able to improve upon the predictive modeling based on single-center data. Patient factors are most critical in the outcome of LTR. Stage and levels of stenosis, as well as pulmonary and GI conditions most strongly impact the likelihood of success. Future prospective case-control studies will be performed to further optimize the current model for outcome prediction and patient management.


Subject(s)
Gastroesophageal Reflux , Laryngostenosis , Tracheal Stenosis , Humans , Child, Preschool , Laryngostenosis/complications , Laryngostenosis/epidemiology , Laryngostenosis/surgery , Tracheal Stenosis/complications , Tracheal Stenosis/epidemiology , Tracheal Stenosis/surgery , Constriction, Pathologic , Bayes Theorem , Retrospective Studies , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/diagnosis , Gastroesophageal Reflux/epidemiology , Treatment Outcome
18.
Int J Pediatr Otorhinolaryngol ; 170: 111600, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37201337

ABSTRACT

OBJECTIVES: Croup is characterized by a barky cough, inspiratory stridor, hoarseness and varying degrees of respiratory distress. Acute croup episodes are often treated with oral, inhaled, or intravenous corticosteroids. Recurrent croup, defined as more than 2-3 episodes of acute croup in the same patient, can mimic asthma. We hypothesized that inhaled corticosteroids (ICS) given at the first sign of a respiratory viral prodrome can be a safe treatment to reduce the frequency of recurrent croup episodes in children without fixed airway lesions. METHODS: A retrospective chart review of patients being treated over an 18-month period was performed at a large tertiary care pediatric hospital following Institutional Review Board (IRB) approval. Patients under 21 years old referred to Pediatric Pulmonology, Otolaryngology, or Gastroenterology for recurrent croup were analyzed for their demographics, medical history, evaluation, treatment and clinical improvement. A Fisher's two-tailed exact test was used to compare the number of croup episodes before and after interventions. RESULTS: 124 patients were included in our analysis: 87 male and 34 female with a mean age of 54 months. Of these, 78 had >5 episodes of croup, 45 had 3-5, and 3 had 2 episodes prior to their first visit for recurrent croup. Operative direct laryngoscopy/bronchoscopy was performed in 35 patients (27.8%), with 60% showing a normal exam without fixed lesions. Ninety-two patients (74.2%) were treated with ICS, 24 were lost to follow up. Of the remaining 68 treated patients, 59 (86.7%) saw improvement with reduced severity and overall number of episodes of croup. Additionally, patients with >5 episodes of croup (47) as compared to <5 (12) were more likely to improve with ICS, (p = 0.003). There were no adverse reactions reported with ICS treatment. CONCLUSION: The novel initiation of ICS at the earliest sign of a viral upper respiratory infection shows promise as a safe preventative treatment to mitigate the frequency of recurrent croup episodes.


Subject(s)
Asthma , Croup , Child , Humans , Male , Female , Child, Preschool , Young Adult , Adult , Croup/diagnosis , Retrospective Studies , Adrenal Cortex Hormones/therapeutic use , Cough , Asthma/diagnosis , Asthma/drug therapy
19.
Genet Test Mol Biomarkers ; 27(7): 221-228, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37522794

ABSTRACT

Background: Otitis media (OM) is defined as middle ear (ME) inflammation that is usually due to infection. Globally, OM is a leading cause of hearing loss and is the most frequently diagnosed disease in young children. For OM, pediatric patients with Down syndrome (DS) demonstrate higher incidence rates, greater severity, and poorer outcomes. However, to date, no studies have investigated the bacterial profiles of children with DS and OM. Method: We aimed to determine if there are differences in composition of bacterial profiles or the relative abundance of individual taxa within the ME and nasopharyngeal (NP) microbiotas of pediatric OM patients with DS (n = 11) compared with those without DS (n = 84). We sequenced the 16S rRNA genes and analyzed the sequence data for diversity indices and relative abundance of individual taxa. Results: Individuals with DS demonstrated increased biodiversity in their ME and NP microbiotas. In children with OM, DS was associated with increased biodiversity and higher relative abundance of specific taxa in the ME. Conclusion: Our findings suggest that dysbioses in the NP of DS children contributes to their increased susceptibility to OM compared with controls. These findings suggest that DS influences regulation of the mucosal microbiota and contributes to OM pathology.


Subject(s)
Down Syndrome , Microbiota , Otitis Media , Child , Humans , Child, Preschool , RNA, Ribosomal, 16S/genetics , Down Syndrome/genetics , Otitis Media/genetics , Ear, Middle/microbiology , Ear, Middle/pathology , Microbiota/genetics
20.
Pediatr Pulmonol ; 57(9): 2279-2281, 2022 09.
Article in English | MEDLINE | ID: mdl-35666077

ABSTRACT

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.


Subject(s)
Bronchopulmonary Dysplasia , Tracheobronchomalacia , Tracheoesophageal Fistula , Tracheomalacia , Bronchopulmonary Dysplasia/complications , Bronchopulmonary Dysplasia/surgery , Humans , Infant , Infant, Newborn , Infant, Premature , Tracheobronchomalacia/complications , Tracheobronchomalacia/surgery , Tracheoesophageal Fistula/surgery , Tracheomalacia/complications , Tracheomalacia/surgery
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