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Telemedicine can improve access to pediatric otolaryngology care by decreasing travel time and cost, and lowering the risk of viral transmission during the SARS-CoV-2 (COVID-19) pandemic. This study aims to identify the clinical role and outcomes of telemedicine for tracheostomy-dependent children before and during the COVID-19 pandemic.Retrospective chart review of 42 tracheostomy-dependent pediatric patients who utilized telemedicine between October 2013 and April 2020 (pre-COVID-19), and 111 patients who utilized telemedicine between May 2020 and July 2021 (during COVID-19) at a tertiary free-standing children's hospital outpatient clinic.The majority of pre-COVID-19 telecommunication solely addressed tracheostomy stomal concerns as compared with during COVID-19 (99% vs. 3%,The clinical role of telemedicine has evolved from problem-based evaluation to routine follow-up during the COVID-19 pandemic. Although telemedicine can decrease the need for in-person office evaluation of routine tracheostomy concerns, respiratory complications and tracheostomy bleeding still require urgent in-person ED evaluation.Level 4.

Competent speech requires closure of the velopharyngeal sphincter by dynamic apposition of the velum and posterior and lateral pharyngeal walls. An accurate estimation of lateral pharyngeal wall motion is an important determinant in the planning and the outcome of any operation to correct velopharyngeal insufficiency (VPI). The purpose was to compare the assessment of lateral pharyngeal wall movement by videofluoroscopy (VP) versus nasopharyngoscopy (NP).The authors retrospectively reviewed the charts of 269 consecutive patients in our cleft lip/palate clinic from 1982 to 2008 and culled those treated with a pharyngeal flap for VPI. The authors included patients who were evaluated preoperatively by both VP and NP, and had studies of suitable quality. Percentage of lateral pharyngeal wall motion was estimated with each technique and compared for each patient.The authors identified 25 patients who underwent both VP and NP at the same median age (4.7 years). The estimated percentage of lateral pharyngeal wall motion between the 2 techniques was significantly different ( P0.001). Average lateral pharyngeal wall motion was estimated to be 59±25% (range: 5%-90%) by VP and only 40%±25% (range: 0%-95%) during NP.VP and NP are complementary, but assessment of lateral pharyngeal wall motion can vary between the 2 methods. The surgeon should be aware of the difference in estimated lateral pharyngeal wall movement when planning a procedure to correct VPI.

The reported prevalence of voice disorders in the pediatric population varies widely between studies, ranging from 3.9% to 23%. Despite this, not all children with dysphonia are referred to a voice specialist for further evaluation. The objective of this study is to examine the relationship between dysphonia history, voice assessment, and laryngeal findings to help guide referrals of dysphonic children. A retrospective review was conducted of pediatric patients at a tertiary voice clinic between January 2014 and December 2017. Data including dates of presentation, demographics, co-morbidities, presenting symptoms, laryngeal exam findings, Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) scores, and Pediatric Voice Handicap Index (pVHI) scores were collected and analyzed. Of 475 patients initially identified, 459 met inclusion criteria and were reviewed. In all, 272 (59.3%) were male and 187 (40.7%) were female. Mean age at first presentation was 8.6 years old (range: 2-18). Males were more likely to present at a younger age than females. CAPE-V data were available for 439 patients, and pVHI data were available for 109 patients. The mean CAPE-V Overall Severity score was 38.2. The mean total pVHI score was 25.4. Males had higher CAPE-V Overall Severity (40.0 vs. 35.4), Roughness (32.2 vs. 27.6), and Strain scores (37.2 vs. 32.4) than females. Patient pVHI scores did not differ by gender. In all, 283 patients self-reported a length of symptoms prior to evaluation. Children with a longer duration of symptoms prior to evaluation had higher CAPE-V Overall Severity scores. Diagnoses of vocal fold movement impairment and benign vocal fold lesions that were not nodules were associated with higher average CAPE-V Overall Severity scores. Overall, 310 patients (67.5%) were recommended intervention for their dysphonia. These patients had higher CAPE-V Overall Severity scores than those who were solely recommended observation (42.8 vs. 28.0). Males were more likely than females to present with dysphonia and presented with more severe perceptual dysphonia scores on average. The length of symptoms and certain diagnoses correlated with higher CAPE-V Scores. Referrals to a pediatric voice clinic should be considered in patients with a dysphonia history lasting greater than 3 months and in patients with more severe symptoms.

Vocal fold nodules are the most common etiology of chronic dysphonia in the pediatric population. Voice therapy is an effective first line of treatment, with increasing evidence supporting the use of telepractice in speech pathology. Despite this, there is limited data on its effectiveness in the pediatric population. The aim of this retrospective study was to investigate the feasibility and efficacy of telepractice in delivering voice therapy to children diagnosed with vocal fold nodules.A retrospective review was conducted of patients treated with virtual voice therapy from April 2020 to June 2021. Patients were included if diagnosed with vocal fold nodules, 2-18 years of age, and completed therapy in a virtual format. Data includes demographics, Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) scores and pediatric Voice Handicap Index (pVHI) scores.Twenty-three children were included, 17 (74%) male and six (26%) female (with an age range of 2.4-9.9 years at the start of therapy). Prior to treatment, the average CAPE-V Overall Severity score was 37.9 (SD 13.8); the average posttreatment score was 22.4 (SD 10.2). The average pVHI total score prior to treatment was 26.3 (SD 12.1), with an average posttreatment score of 20.2 (SD 11.7). Patients who underwent virtual voice therapy had improved posttreatment CAPE-V severity scores than those prior to treatment (average difference = -15.5 points; 95% CI: -8.3 to -22.7; P0.001). An increased number of therapy sessions was associated with both higher initial CAPE-V severity scores (r = 0.72; P0.01) and a greater decrease in posttreatment CAPE-V scores (r = -0.55; P0.01).Virtual voice therapy may be feasible and efficacious in treating dysphonic children diagnosed with vocal fold nodules. Significant improvements were found in perceptual CAPE-V scores in overall severity; positive changes were also seen in parental measures of quality of life. Delivery of voice therapy in a telehealth format may increase access of care and should be considered as a treatment option.

Objectives/hypothesis Describe augmentative communication tools and strategies used by pediatric patients referred to inpatient speech-language pathologists prior to tracheostomy placement. Study design Retrospective review. Methods A review of patients who underwent initial tracheostomy placement from 2013-2016 was conducted at a tertiary pediatric center. Eligible patients were those who were referred to a specialized speech-language pathologist prior to the date of the tracheostomy placement to support communication abilities. Patients were identified by surgical procedural and billing codes. Data collected included patient demographics, speech and language disorders, and interventions performed. Chart review and cross analysis of billing data for types of assessment and intervention procedures were conducted by two speech-language pathologists for consensus agreement. Results Forty-six patients (aged 1 month-27 years, mean = 12.9 years) were included in the study. Average time between the bedside communication assessment and tracheostomy procedure date was 17 days. Baseline speech-language disorders were identified in 11 patients (24%). Thirty-eight (83%) patients were nonspeaking at the time of consultation. Thirty-two (70%) patients utilized an electronic communication tool, and 36 (78%) utilized low-technology communication strategies during the preoperative period. A total of 32 (70%) patients were documented as using no-technology or speech-enhancement strategies during the acute hospitalization. Conclusions Multidisciplinary tracheostomy teams should consider consultation to speech-language pathologists for patients prior to tracheostomy placement to assess for utility of high-technology, low-technology, and no-technology augmentative and alternative communication strategies. Level of evidence 4 Laryngoscope, 130:1817-1822, 2020.

Down syndrome is the most common chromosomal abnormality and is associated with a higher incidence of congenital heart defects, which often require surgery within the first year of life. Previous studies have found that children with Down syndrome are at higher risk for subglottic stenosis, vocal fold paralysis, and laryngomalacia. The goal of this study is to review children with Down syndrome presenting with dysphonia and to characterize their laryngeal pathologies.A retrospective review was performed of patients with Down syndrome seen at a tertiary pediatric hospital's department of otolaryngology from Jan. 2007-Jul. 2021 for voice-related concerns. Inclusion criteria included age less than 18 years, diagnosis of Trisomy 21, and complaint of dysphonia. The data extracted included history of dysphonia, co-morbidities, demographic information, age at presentation, perceptual voice assessments, voice quality of life scores, acoustic data, laryngoscopic and/or videostroboscopic exams, and surgical procedures.Twenty-three total patients met the study criteria. Of these children, 13 (57%) were male and 10 (43%) were female. The mean age at first presentation was 4.08 years (range 12 days-16.3 years). Eleven of the 23 patients presented within the first 12 months of life. Sixteen patients were diagnosed with vocal fold immobility, 13 of which were left-sided unilateral immobility and the remaining 3 were bilateral immobility. 5 patients were diagnosed with vocal fold nodules. 12 children in the immobility group had a history of cardiothoracic surgery at our institution. Only 3 patients had Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) assessments, though all three showed overall dysphonia ratings of severely deviant, with roughness and strain scores being the most severe.The most common etiology of dysphonia in our Down syndrome patient population was vocal fold immobility and hypomobility, as opposed to vocal fold nodules (which is the most common in the general pediatric population). The higher likelihood of cardiac surgery in patients with Trisomy 21 may result in the increased incidence of vocal fold immobility. There should be a low threshold to refer dysphonic patients with Down syndrome for laryngoscopic evaluation, as treatment options may be available.

Objective To provide guidance for home care tracheostomy management in the pediatric population. The mission of the IPOG is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. Methods Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results Survey results provide guidance for caregiver teaching, the reuse of tracheostomies and suction catheters while inpatient and following discharge, acceptable sterilization practices for tracheostomies, tracheitis workup and management, and outpatient follow-up practices. Conclusion This presentation of common home tracheostomy care practices are aimed at improving patient-centered care in the pediatric population.

The most common etiologies of dysphonia in the pediatric population are vocal fold nodules and muscle tension dysphonia. Vocal therapy is the first line treatment for these disorders in children. Despite this, not all children undergo therapy. The goal of this study is to examine how factors such as patient demographics and parental perceptions differ between children that choose to undergo or not to undergo voice therapy.A retrospective review was conducted of all pediatric patients seen at a tertiary voice clinic between January 2014 and December 2017. Patients were included if diagnosed with vocal fold nodules and/or muscle tension dysphonia. Patients were divided into groups of children that received voice therapy at our institution and those that did not. Data include demographics, Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) scores and pediatric Voice Handicap Index (pVHI) scores. Distance to therapy site was approximated using patient zip codes.Three hundred and forty-six children were included, 224 (65%) boys and 122 (35%) girls. In the 2 years following initial diagnosis, 74 (21%) children participated in voice therapy at our institution. Patients who underwent voice therapy were older than those who did not (mean age: 9.1 [SD 3.5] vs 7.6 [SD 3.8] years; P = 0.004). Patients who received voice therapy were more likely to live closer to the therapy site (mean distance: 15.5 [SD 13.0] vs 24.3 [SD 23.9] miles; P0.001). Likelihood of receiving voice therapy did not differ by gender or health insurance status (private vs public). Patients who underwent voice therapy had significantly greater CAPE-V Overall Severity scores than those who did not (mean score: 44.6 [SD 19.4] vs 37.4 [SD 18.0]; P = 0.003). Higher CAPE-V Strain scores were associated with increased likelihood of voice therapy. pVHI scores did not differ between the two groups.Older age, shorter distance to therapy site, and increased CAPE-V Overall Severity and Strain scores were associated with higher likelihood of receiving voice therapy. Gender, insurance status, and pVHI scores did not affect likelihood of receiving voice therapy. Patients may primarily consider ease of access and necessity of treatment when considering voice therapy.

Background Sialorrhea is a common comorbidity among children with neurologic disorders. Botulinum toxin injections and surgical procedures are recommended for the management of pathological sialorrhea in patients who fail conservative management or with concerns for salivary aspiration. The following review evaluates outcomes following botulinum toxin injections and surgical interventions for sialorrhea over a 10-year period with a focus on treatment options and outcomes for patients with anterior and posterior drooling. Methods The study included all patients less than 25 years of age who underwent a procedure for drooling (Current Procedural Terminology (CPT) codes 42440, 42450, 42509, 42510, 64611 matched with the International Classification of Diseases (ICD)-9 and ICD-10 codes 527.7 and K11.7) from January 1, 2006 to December 31, 2015. A chart review collected demographics, drooling medication use, and type of drooling (anterior, posterior, both). Outcome variables included pre- and post-procedure number of bibs, parent-reported outcomes, post-intervention drooling medication requirement, post-procedure length of stay, and complications. Results Seventy-one patients were included in our analysis, with 88 total procedures performed. The average age at first intervention was 8.9 years; 43 patients were male and 40 patients had cerebral palsy. Thirty-one patients experienced posterior drooling or anterior/posterior drooling. These patients were more likely to undergo surgery as the first invasive intervention. The most commonly performed interventions were botulinum toxin injections (28 patients, 39%) and sublingual gland excision (SLGE) with submandibular duct ligation (SMDL) (36 patients, 51%). Improvement following injections was noted in 56% of patients versus 73% of patients following any surgical intervention. Conclusion Management of drooling is complex with 18 different procedures performed over 10 years. Surgical interventions, specifically SLGE with SMDL and submandibular gland excision (SMGE), result in substantial improvement; these are commonly performed as the first intervention in patients with posterior drooling. By reviewing our experience, we hope to guide management decisions and help manage patient and caregiver expectations.

Objectives/hypothesis To describe the prevalence of aspiration in children with unilateral vocal fold paralysis who underwent objective assessment of swallow function. Study design Retrospective chart review. Methods A study of patients presenting to our institution with unilateral vocal fold paralysis in 2015 was conducted. All patients were diagnosed using flexible laryngoscopy. Patients were included if they underwent at least one modified barium swallow (MBS) study for evaluation of their swallowing function due to recurrent respiratory issues and/or feeding difficulty. Results Twenty-eight patients diagnosed with unilateral vocal fold paralysis underwent an MBS study at our institution in 2015. Median age at the time of MBS study was 1.7 years (interquartile range: 0.4-4.3). Twenty-six patients (92.9%) had dysphagia. Sixteen patients were found to aspirate on MBS study. All patients who aspirated did so without overt signs (silent aspiration). Eighteen patients had congenital heart disease (64.3%) and nine had a history of prematurity (32.1%). Eight patients (28.6%) presented with developmental delays. Conclusions Patients who present with unilateral vocal fold paralysis and recurrent respiratory and/or feeding issues may be affected by prominent issues such as swallowing dysfunction and silent aspiration. Clinicians should be aware of this risk and evaluate patients for any signs of feeding or swallowing difficulties. Level of evidence 4 Laryngoscope, 129:569-573, 2019.

Introduction: The prevalence of voice abnormalities in children born prematurely has been reported to be as high as 58%. Few studies have examined these abnormalities with laryngoscopic or videostroboscopic findings and characterized their laryngeal pathologies. Objective: To review voice abnormalities in patients with a history of prematurity and characterize the etiology of their voice problems. A secondary objective is to see if there is a correlation between the findings and the patient’s intubation and surgical history. Methods: A retrospective chart review was conducted of all preterm patients seen in voice clinic at a tertiary pediatric hospital. Demographic data, diagnoses, and office laryngoscopies were reviewed as well as any speech therapy evaluations and/or medical and surgical treatments. Results: Fifty-seven patients were included. Mean age at presentation was 5.1 (±4.3) years. Mean gestational age was 27.8 (±3.7) weeks. Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) perceptual evaluations included a mean overall dysphonia severity of 46.6 (±24.2). Patients who had undergone prolonged intubation (⩾28 days) in the NICU or had prolonged NICU stays (>12 weeks) had significantly higher overall dysphonia severity scores. Thirty-three patients with vocal fold hypo- or immobility had significantly greater voice deviance in breathiness, loudness, and overall severity compared to those without vocal fold immobility. Of all patients, 35% were recommended surgical intervention and 49% voice therapy. Conclusion: Intubation greater than 28 days and prolonged NICU stays are associated with more severe dysphonia in premature patients. There should be a low threshold for clinical evaluation of dysphonia in this unique patient population.

OBJECTIVES/HYPOTHESIS Advancement in neonatal and pediatric intensive care has increased the need for chronic-care interventions, including tracheostomy. It is well established that children with a tracheostomy are at a high risk for adverse events, many of which are preventable. Despite this, there is no standardized method of monitoring tracheostomy-related adverse events (TRAEs). Our objective was to describe and assess a standardized, closed-loop system for monitoring TRAEs. STUDY DESIGN Prospective Study. METHODS A specific tracheostomy-related category was established within the adverse event reporting system in January 2015. Monthly TRAE reports were supplied to the multidisciplinary tracheostomy team (MDT) with descriptions of event type, severity, and preventability. The MDT reviewed events and discussed necessary follow-up. The frequency of events was standardized by inpatient tracheostomy days (ITDs) using an automated monthly list. Adverse events were tracked using a control chart. Aggregated data were divided into biannual reports for analysis. RESULTS Eighty-five TRAEs were reported between January 2015 and June 2017, averaging 5.75 per 1,000 ITDs. Most common events include unplanned decannulation (50%) and improper use of tracheostomy supplies (21%). The frequency of all preventable events has decreased by 76% since the second half of 2015. During this timeframe, minor events have decreased, moderate events have maintained a frequency of less than one per 1,000 ITDs, and only one severe event occurred. CONCLUSIONS This standardized, closed-loop reporting method, modeled after other successful intensive care unit reporting systems, accurately tracks TRAEs. We have observed a decrease in preventable TRAEs without a negative impact on rates of severe events. Results suggest improved quality of care for patients with tracheostomy. LEVEL OF EVIDENCE 4. Laryngoscope, 128:2419-2424, 2018.

Objectives To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. Results Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. Conclusions Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

To discuss the presentation, evaluation, and management of pediatric laryngeal web.Retrospective case series.Single tertiary care center.All patients with laryngeal web at Boston Children's Hospital in the past 22 years.No exclusion criteria. Charts mined for age at presentation, presenting symptoms, degree/location of web, associated syndromes, number/type of surgical procedures, and postoperative outcomes.Thirty-seven patients were included (13 male, 24 female). Average age at diagnosis was 3.7 years (0-19.5 years). Mean follow-up was 4.4 years (range, 0-16.4 years). There were 26 congenital webs (70.2%) and 11 acquired webs (29.8%). Presenting symptoms were vocal (29 patients, 78.4%) and respiratory (22 patients, 60%). Underlying syndromes or synchronous airway lesions included the following: premature (n = 5), congenital heart disease (n = 18), subglottic stenosis (n = 5), 22q11.2 deletion syndrome (n = 10), and recurrent respiratory papillomatosis (n = 4). There were 20 type 1 webs, 6 type 2 webs, 8 type 3 webs, and 3 type 4 webs; 10 had subglottic extension of the laryngeal web. Twelve patients were managed conservatively with observation. Eighty-four interventions were performed: 18 open and 66 endoscopic (sharp division, 32; dilation, 33; mitomycin C, 14; laser, 5; keel, 6; triamcinolone injection, 8; stent, 15; removal of granulation tissue, 5). Tracheotomy was required in 11 patients, and 5 patients were decannulated. Voice improved in 12 patients, with respiratory symptoms in 12 patients. Web recurred in 17 patients. One patient died due to airway complications.Pediatric laryngeal web is an uncommon but challenging lesion. Patients need to be evaluated for comorbid syndromes and synchronous airway lesions. Management includes open and endoscopic procedures. Procedures should be tailored to the child's presentation.

Vocal pitch discrimination abilities were compared in sixteen children with vocal fold nodules (CwVN) and sixteen matched controls with typical voices (CwTV). Vocal pitch discrimination was also evaluated in thirty-five vocally healthy children and twenty adults to examine potential changes as a function of maturation. CwTV were categorized as either younger (N = 15, 5.6&ndash, 7.7 years) or older (N = 20, 8.2&ndash, 11.7 years). Participants completed two-alternative, forced choice listening tasks in which they judged whether pairs of sustained /ɑ/ tokens were different in pitch. Each pair consisted of a base token with a fundamental frequency (fo) of 216.2 Hz and a test token with a fo that was adaptively modified, according to the participant&rsquo, s prior judgments. There were no significant differences in pitch discrimination abilities between CwVN and CwTV. Pitch discrimination abilities were significantly poorer in younger and older CwTV as compared to adults. Additionally, younger CwTV had significantly poorer discrimination abilities than older CwTV. Findings from this study suggest that CwVN do not have differences in pitch discrimination abilities, yet, therapies designed for CwVN should consider this developmental trend in perceptual abilities.

Importance Incidence of tracheostomy placement in children is increasing, and these children continue to have high incidences of morbidity and mortality. A multidisciplinary tracheostomy program may help improve the quality of care received by these patients. Objective To determine whether implementation of a multidisciplinary tracheostomy program can improve the care of children who received a tracheostomy through reduction in tracheostomy-related adverse events (TRAEs), improved tracheostomy education, and caregiver preparedness. Design, Setting, and Participants A prospective cohort study was conducted from January 2015 to June 2018 at a pediatric tertiary referral center in Boston, Massachusetts. The participants included 700 children who had received a tracheostomy, most of whom were aged birth to 18 years, but some patients with congenital disorders were much older. Exposures Institution of a multidisciplinary tracheostomy team (MDT) whose activities included conducting staff meetings, organizing outpatient clinics, conducting inpatient tracheostomy ward rounds, and conducting inpatient tracheostomy rounds at a local rehabilitation hospital. Quality improvement initiatives included monitoring standardized TRAEs and distributing standardized tracheostomy “go-bags.” Main Outcomes and Measures Reduction of TRAEs and improved caregiver preparedness through distribution of tracheostomy go-bags were assessed following the establishment of a multidisciplinary tracheostomy program. Results In total, 700 children who had received a tracheostomy during the study period were actively followed up by the MDT. Of these children, 378 (54.0%) were males and 322 (46.0%) were females; mean (SD) age was 4.1 (6.1) years. More than 60 new pediatric tracheostomies were performed annually at the referral center. Reported TRAEs were reduced by 43.0% from the first to the third year after the implementation of a standardized, closed-loop monitoring system (from a mean [SD] of 6.1 [5.2] TRAEs per 1000 inpatient tracheostomy-days in 2015 to a mean [SD] of 4.0 [2.5] in 2018). The most common TRAE was unplanned decannulation, which occurred 64 times during the study period. On average, 10 patients were seen in each monthly multidisciplinary tracheostomy clinic. Clinic interventions included continuing care (146 [52.5%]), communication enhancement (67 [23.6%]), plans for decannulation (52 [18.6%]), and referrals for comorbidities (13 [4.6%]). Approximately 19 inpatients were seen during biweekly rounds and 8 during monthly rounds at a local rehabilitation hospital. A total of 297 patients received standardized tracheostomy go-bags, and more than 70 positive bag checks were performed in the monthly MDT clinics. A positive bag check refers to the incidence when a family is given a go-bag and also uses it. In contrast, a negative bag check refers to when a family is given a go-bag but neither brings it to the clinic nor acknowledges that they use it. Conclusions and Relevance This study’s findings suggest that a multidisciplinary tracheostomy program may be a powerful tool for enhancing patient safety and quality improvement. Ongoing studies will develop measurable pediatric tracheostomy outcome metrics and assess long-term outcomes.

Introduction Although dyspnea with exercise in the pediatric population can be multifactorial, the diagnosis of paradoxical vocal fold motion disorder (PVFMD) in this group is not well characterized. The objective of this study is to review the multiple causes of dyspnea with exercise in children, including the prevalence of PVFMD within this study population. Methods A retrospective review was conducted of patients seen at a tertiary pediatric hospital for exercise-induced dyspnea suspected to be related to PVFMD between January 2007 and July 2015. Inclusion criteria included assessment in a specialty exercise clinic and evaluation by a pediatric otolaryngologist and pulmonologist. Pre- and post-exercise pulmonary function tests and laryngoscopic examinations were performed. Data including co-morbidities, presenting symptoms, prior diagnoses and treatments, final diagnoses, prescribed treatments and outcomes were collected and analyzed. Results 294 patients were evaluated at our institution during the study period. 4 patients were excluded for insufficient data, which left 290 for analysis. 75 were male, 215 female. All patients underwent treadmill testing with monitoring to simulate strenuous exercise. Average patient age was 14.6 years; average BMI was 21.53. The most common sports to elicit symptoms were running and soccer. Patients most frequently complained of stridor or wheezing in addition to dyspnea. Throat tightness was also a common complaint. After evaluation, 86 patients were given the primary diagnosis of exercise-induced PVFMD. 54 patients were diagnosed with physiologic dyspnea and 30 with exercise-induced asthma. Conclusion Pediatric patients presenting with exercise-induced dyspnea can have multiple etiologies for their symptoms including PVFMD. Other causes of dyspnea with exercise should not be underestimated.

Introduction Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation. Objective Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy. Methods Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method. Results Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: • on a standard definition of Suprastomal Collapse • that there is clinical importance between significant and insignificant collapse • that the gold standard diagnostic modality is endoscopic examination under anesthesia • that the severity of collapse is a major determinant in the surgical approach • that surgical intervention is the first line management in combined anterior and lateral Suprastomal Collapse. Conclusion This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.

Introduction Children with tracheostomy are a heterogeneous population requiring care from multiple specialties. Multidisciplinary approaches to treating such patients helps to improve the quality of care they receive. Our institution established a Multidisciplinary Tracheostomy Clinics (MDTC) to address outpatient care coordination for tracheostomy patients by providing care from multiple disciplines at a single visit. We report patient/caregivers’ experiences of our MDTC. Methods Patients with tracheostomy or their caregivers were prospectively recruited between Dec 2017–Oct 2019 to complete surveys assessing their experience at the MDTC. Demographic and satisfaction questionnaires were sent electronically by a REDCap survey distribution tool. Demographic data were collected, such as patient's residence and education level. Medical care variables assessed included history of MDTC attendance, commute time, medical specialties seen, tracheostomy “Go-Bag” use, home-care nursing, and MDTC satisfaction ratings. Results Twenty-nine patients/caregivers completed the satisfaction survey and 22 completed both the satisfaction survey and demographics questionnaire. Patient ages ranged from 11 months to 36 years. Twenty-three (79%) participants commuted for up to 2 h to the MDTC, and 6 (21%) commuted for more than 2 h. The median number of medical specialties seen at the MDTC was 3. All participants were satisfied that they saw all requested specialties. Tracheostomy supplies were checked for 25 of 28 patients. Twenty-three of 28 subjects rated staff teamwork as “excellent.” Twenty-four of 28 patients were “highly likely” to recommend the MDTC. Twenty-three of 28 participants were “highly likely” to return, and 4 were “somewhat likely” to return. Conclusion This study demonstrates that patients with tracheostomy and caregivers were satisfied with the improved coordination and facilitation of care through a Multidisciplinary Tracheostomy Clinic.

Objectives: The aims of this study were to describe the impact of laryngoplasty in pediatric unilateral vocal fold immobility (UVFI) and to determine the impact of etiology and technique on voice and swallowing. Methods: A retrospective review was conducted of all children with UVFI undergoing medialization laryngoplasty at a pediatric hospital (2010-2017). Data including demographics, etiology, subjective voice quality, and swallowing function were collected. Results: The median age at first surgery among 25 patients with UVFI was 11 years (range, 1.2-25 years). The causes of UVFI were iatrogenic (76%), congenital (16%), and idiopathic (8%). A total of 38 laryngoplasties (24 injections, 11 Silastic implants, 3 Gore-Tex) were performed. Postoperatively, 78% of patients reported improvements in voice and 81% in swallowing. The median duration of voice improvement was 1.0 years (range, 0.1-10 years), with no significant difference by etiology or laryngoplasty technique. Patients who were ⩾10 years of age at surgery reported voice improvement significantly more often than patients Conclusions: UVFI has a significant impact on health and quality of life. In this study we found that laryngoplasty is an effective way to address both voice and swallowing in pediatric UVFI. A greater proportion of children with improved voice quality were older at injection. Surprisingly, there was no difference in duration of voice improvement between permanent and absorbable materials. Although this duration would be considered acceptable for many injectable materials, the limited duration in permanent implantation techniques may represent the challenges of managing UVFI in the growing larynx of the pediatric population. Injection laryngoplasty with absorbable materials may serve as an adequate method of addressing UVFI in this population.

Objective To report on the prevalence of voice disturbances in pediatric airway patients. Methods Consecutive patients seen in a specialized Center for Airway Disorders at a tertiary children's hospital from February 2017 to September 2017 were included. Patients' families were invited to complete a pediatric voice health handicap index (pVHI) questionnaire. Patients underwent evaluation including flexible laryngoscopy and/or direct laryngoscopy and bronchoscopy. Results 146 patients were included. Of these children, 73 patients (50.3%) presented with swallowing difficulty and 44 patients (30.3%) presented with respiratory complaints. Only 9 patients (6.2%) reported hoarseness initially. The median age at referral was 2.7 years of age (interquartile range: 1.4-4.3). The mean total pVHI score was 9.5 (± 12.9). Sixty-seven patients (45.9%) had abnormal pVHI findings of score > 4. Six patients (4.1%) had pVHI > 40. The mean pVHI score was 26.0 (± 21.1) among 12 patients with a history of tracheostomy, 12.0 (± 14.3) among 30 patients with laryngeal cleft, and 9.0 (± 9.9) among 19 patients with laryngomalacia. Conclusion Voice disturbances are not uncommon in pediatric patients evaluated for airway disorders. Although patients may present with primary concerns for breathing or swallowing difficulties, many of these patients may need further work-up and treatment for dysphonia.

Objective: To examine the prevalence of upper airway anomalies in patients diagnosed with congenital tracheoesophageal fistula and esophageal atresia (TEF/EA). Methods: A retrospective review was conducted of all TEF/EA patients seen at a tertiary pediatric hospital between January 2008 and December 2013. Inclusion criteria included evaluation by the otolaryngology service. Exclusion criteria included age >18 years, acquired TEF/EA, subsequent rule out of TEF/EA, and otolaryngology evaluation for reasons not pertaining to the airway. Data collected and analyzed included demographics, comorbidities, presenting symptoms, surgical interventions, laryngoscopic and bronchoscopic examinations, and subsequent medical and surgical management. Results: Four hundred and thirty patients were diagnosed with TEF/EA at our institution. In all, 32.3%, or 139 children, were included in the analysis; 56.1% (n = 78) male, 43.9% (n = 61) female. Of the analyzed patients, 4.3% (n = 6) were diagnosed with laryngomalacia. Eighteen patients (12.9%) were diagnosed with subglottic stenosis. Thirty (21.6%) had vocal fold paresis or immobility. Laryngeal cleft was diagnosed in 25.9% (n = 36). Tracheomalacia was the most common airway finding, diagnosed in 37.4% (n = 52) patients. Conclusion: Patients diagnosed with congenital TEF/EA have a high rate of secondary upper airway anomalies. Consideration should be given to perform a complete airway evaluation in all of these patients.

Objective The presentation, etiology, and treatment of nasal septal perforation have been described in the adult literature; however, reports in the pediatric population are limited. In this study, we review our experience with pediatric nasal septal perforations with a focus on presentation, pathogenesis, management, and outcomes of surgical repair. Methods A retrospective chart review was performed on pediatric patients diagnosed with nasal septal perforations from 1998 to 2015. Data regarding patient demographics, perforation characteristics, and treatment were extracted and analyzed. Results Twenty-seven patients met inclusion criteria. Mean age was 10.8 years (range 2 months–17 years). Nasal crusting (n = 19, 73%) and epistaxis (n = 15, 58%) were the most common complaints at presentation. The most common etiologies were trauma (n = 9, 33%), iatrogenic sources (n = 5, 19%), and neoplasm (n = 3, 11%). Septal perforations were primarily located in the anterior septum (n = 17, 81%) and the average size was 0.9 cm (±0.37) in diameter. Four patients were managed with a nasal septal button. Successful closure was achieved in four out of six patients (66.7%) who underwent surgical repair. Conclusions In our series, septal perforations in children occurred most frequently due to digital nasal trauma, and crusting was the most common symptom. Factors to consider prior to repair include symptomatology, the etiology of the perforation, co-morbidities, ability to comply with post-operative care/restrictions, availability of adjacent tissue/grafts, and potential effects on nasal growth. Even with careful consideration of these factors, successful closure was limited to two-thirds of patients who were offered repair.

Objectives To develop consensus recommendations for peri-operative tracheotomy care in pediatric patients. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. Results Consensus recommendations including pre-operative, intra-operative, and post-operative considerations, as well as sedation and nutrition management are described. These recommendations are based on the collective opinion of the IPOG members and are targeted to (i) otolaryngologists who perform tracheotomies on pediatric patients, (ii) intensivists who are involved in the shared-care of these patients, and (iii) allied health professionals. Conclusion Pediatric peri-operative tracheotomy care consensus recommendations are aimed at improving patient-centered care in this patient population.

Purpose Approximately 25% to 40% of patients with cleft lip/palate develop maxillary retrusion that requires Le Fort I osteotomy. Maxillary advancement brings the soft palate forward, and this may cause velopharyngeal insufficiency (VPI). The goal of this study was to identify predictors that place patients with repaired cleft palate at risk of developing VPI after Le Fort I advancement. Materials and Methods This was a retrospective study of nonsyndromic patients with cleft lip/palate who had a Le Fort I osteotomy between 2000 and 2008. Charts were reviewed and data were collected on patient characteristics, preoperative speech assessments, and nasopharyngoscopic reports. Pre- and postoperative cephalometric radiographs were used to measure maxillary advancement and to assess the structure of the velopharynx. Simple logistic regression analysis examined the association between each predictive variable and postoperative VPI, as indicated by need for pharyngeal flap. Predictors with P ≤ .10 were included in the multivariate regression model. In both the univariate and the multivariate analyses, P ≤ .05 was considered statistically significant. Results Univariate analysis showed a significant association between preoperative soft palatal length and need for a pharyngeal flap (P = .005). By multivariate analysis, both preoperative soft palatal length and postoperative pharyngeal depth were associated with need for pharyngeal flap (P = .003 and P = .030). Conclusion This study shows that a short soft palate is associated with VPI after Le Fort I osteotomy. Assessment of palatal length and pharyngeal depth on cephalometric radiographs is helpful in predicting postoperative VPI and need for a pharyngeal flap in patients with cleft palate after maxillary advancement.

Objective To assess the efficacy and safety of endoscopic management of subglottic stenosis both as a primary and as an adjunctive treatment in the pediatric population. Methods Retrospective review of pediatric patients with subglottic stenosis undergoing endoscopic airway procedures at a tertiary care pediatric medical center. Outcomes were assessed by systematic review to determine the success and failure of the endoscopic approach. Results Forty patients (22 male, 18 female) underwent endoscopic interventions for a diagnosis of subglottic airway stenosis between 2003 and 2006. Age ranged from 22 days old to 20 years old. Recorded degree of subglottic stenosis ranged from 10% to 99%. Fifty-three percent (21/40) had a history of prematurity, and 40% (16/40) had secondary airway diagnoses. Twenty-four patients underwent an endoscopic intervention initially (including laser or dilation, with or without topical mitomycin treatment), including four patients who underwent tracheostomy prior to the first endoscopic intervention. Sixteen underwent laryngotracheoplasty initially, including ten patients who underwent tracheostomy prior to the laryngotracheoplasty. Endoscopic treatment resulted in resolution of symptoms, and/or decannulation, and no further need for an open procedure in 58% of patients. Of the 24 patients undergoing endoscopic interventions initially, 14 patients underwent two or more endoscopic interventions, and 10 patients subsequently required tracheostomy or laryngotracheoplasty. When endoscopic procedures were used as an adjunct to laryngotracheoplasty, 60% (12/20) had resolution of symptoms, underwent decannulation, and did not require tracheostomy or revision laryngotracheoplasty. Conclusions The endoscopic approach can be successful in the management of properly selected patients with subglottic stenosis, either as the initial treatment modality or as an adjunctive treatment in cases of re-stenosis after open airway surgery. The likelihood of success with a minimally invasive procedure as the primary treatment decreases with worsening initial grade of subglottic stenosis.

Many children with dysphonia present with benign vocal fold lesions, including bilateral vocal fold nodules, cysts, vocal fold varices, and scarring. Evaluation and treatment of these children are best undertaken in a thoughtful and coordinated manner involving both the speech-language pathologist and the otolaryngologist. The goals of this article are (a) to describe the team evaluation process based on a “whole system” approach; (b) to discuss etiological factors and diagnosis; and (c) to review current medical, behavioral, and surgical treatments for children with different types of dysphonia.

To describe the voice characteristics of pediatric unilateral vocal fold paralysis (UVFP).Retrospective series from a children's hospital, 1996 to present.Forty-two patients with a mean age of 7.1 years were diagnosed with UVFP in our voice clinic (prevalence = 5.4%). Paralysis was left sided in 88%, and was most commonly seen after cardiac surgery (28.5%) and prolonged intubation/prematurity (16.7%). Voice analysis showed a moderate degree of breathiness, mild-to-moderate hoarseness and straining, mild muscle tension, and soft loudness. Twenty-six percent of patients required surgical intervention, including injection into the paralyzed fold (7 patients) and medialization thyroplasty (4 patients). Pre-operatively, breathiness was worse (P.05) in patients undergoing surgical intervention.Voice characteristics of pediatric UVFP include increased breathiness, hoarseness, straining, muscle tension, and soft loudness. One-fourth of patients underwent surgical intervention; breathiness was the predominant abnormal voice characteristic in the operative cohort.The voice characteristics of pediatric patients with UVFP are described.C-4.

Recent studies suggest that rates of intracranial hemorrhage (ICH) increased concomitant with the HIV epidemic among the hemophilia population, but no studies have directly examined factors associated with ICH. To determine ICH rates and identify factors associated with ICH, we performed a nested case-control study of a cohort composed of all hemophilic males identified by a surveillance system. Data were obtained from medical records of care received during 1993-1997. Patients with ICH listed in hospital records or on death certificates during the 5-year period were compared to the remainder of the cohort to examine associations between ICH and patients' demographic and clinical factors including the presence of HIV infection. Among the 3,269 males in the cohort, 88 (2.7%) had an ICH during follow-up, an average incidence rate of 0.0054 case/year. Hemorrhage sites were intracerebral for 37.5%, subdural for 34.1%, unspecified for 19.3%, subarachnoid for 12.5%, and epidural for 8% of cases. For 22% of cases, the ICH was trauma-related, and, overall, 16 patients (18.2%) died. Several factors were independently associated with ICH (odds ratio, P value): severe disease (2.0, 0.05); age 51+ years compared to 6-10 year olds (3.7, 0.02); presence of an inhibitor (3.5

We hypothesized that magnetic resonance imaging (MRI) scans taken prior to radiosynoviorthesis may be predictive of response to the procedure in persons with haemophilia. Specifically, response would be inversely related to the severity of synovial hyperplasia. Radiosynoviorthesis was administered to 21 joints with recurrent haemorrhage (target joints). A detailed self-report of haemorrhage history, joint evaluation with scoring according to the World Federation of Haemophilia orthopaedic joint and pain scales, plain radiographs, and MRI studies of the joints were performed pre- and post-radiosynoviorthesis. To augment comparison of the MRI findings to those assessed using the Arnold-Hilgartner and Pettersson scales, a provisional MRI scale for evaluation of haemophilic arthropathy was designed. We found the MRI findings prior to the procedure were not predictive of clinical response; independent of the severity of synovial hyperplasia, most joints bled less and showed improvement by the WFH orthopaedic score. There was generally no change in the severity of synovial hyperplasia after the procedure. We conclude that MRI evaluation is not routinely indicated prior to radiosynoviorthesis.

It is disheartening to make the diagnosis of recurrent respiratory papillomatosis in a young child. This disease presents in the infant or young toddler with symptoms of worsening hoarseness, stridor, and sometimes respiratory distress. Such children will undergo multiple procedures in their lifetimes to clear their airways of papillomas; it is not uncommon to see a child return as many as 60 to 100 times. Otolaryngologists have successfully developed equipment and techniques to maintain an adequate airway in almost all cases of laryngeal papilloma. Unfortunately, achieving a satisfactory voice outcome both in the short term as well as in the long run is a much more difficult problem.

Importance To our knowledge, the rate of change in the size of pediatric vocal fold nodules (VFNs) has not been investigated. Improved understanding of the factors that affect change in VFN size may help to better guide treatment decisions and counselling of families. Objective To characterize the rate of change in the size of pediatric VFNs over time and to identify which factors affect increased rates of improvement. Design, Setting, and Participants Retrospective review of 67 children evaluated in a voice clinic between 2002 and 2011 with a primary diagnosis of VFNs. Exposure No treatment or behavioral modification only (n = 19) vs targeted voice therapy with or without the treatment of associated conditions (gastroesophageal reflux and allergic rhinitis) (n = 45) vs surgical intervention (n = 3). Main Outcomes and Measures Change in VFN grade (graded according to a previously validated scale based on size) over time. Results Sixty-seven patients with a median (range) age of 6.0 (3.8-20.6) years were analyzed. Median (range) follow-up was 25 (1-119) months. The rate of change in VFN grade over time was significantly associated with large baseline VFN size ( P P = .01); the association with postpubescent age was not significant ( P = .09). The rate of change in VFN grade was not significantly different at 1 and 3 years postbaseline ( P = .33). Conclusions and Relevance Baseline VFN size, treatment, and patient age are important in predicting the rate of improvement in nodule size over time. Rate of change in VFN size is a gradual decrease that is steady over time. This information can be used to help guide treatment decisions and counsel families of children with VFNs regarding expectations for improvement. Additional study is needed to evaluate whether the same factors that influence nodule size similarly influence parental perception of voice and expert perceptual voice analysis.

Objectives: We sought to create a validated scale of vocal fold nodules in children, based on digital video clips obtained during diagnostic fiberoptic laryngoscopy. Methods: We developed a 4-point grading scale of vocal fold nodules in children, based upon short digital video clips. A tutorial for use of the scale, including schematic drawings of nodules, static images, and 10-second video clips, was presented to 36 clinicians with various levels of experience. The clinicians then reviewed 40 short digital video samples from pediatric patients evaluated in a voice clinic and rated the nodule size. Statistical analysis of the ratings provided inter-rater reliability scores. Results: Thirty-six clinicians with various levels of experience rated a total of 40 short video clips. The ratings of experienced raters (14 pediatric otolaryngology attending physicians and pediatric otolaryngology fellows) were compared with those of inexperienced raters (22 nurses, medical students, otolaryngology residents, physician assistants, and pediatric speech-language pathologists). The overall intraclass correlation coefficient for the ratings of nodule size was quite good (0.62; 95% confidence interval, 0.52 to 0.74). The p value for experienced raters versus inexperienced raters was 0.1345, indicating no statistically significant difference in the ratings by these two groups. The intraclass correlation coefficient for intra-rater reliability was very high (0.89). Conclusions: The use of a dynamic scale of pediatric vocal fold nodule size most realistically represents the clinical assessment of nodules during an office visit. The results of this study show a high level of agreement between experienced and inexperienced raters. This scale can be used with a high level of reliability by clinicians with various levels of experience. A validated grading scale will help to assess long-term outcomes of pediatric patients with vocal fold nodules.

Objectives We examined the relationship between the size of vocal fold nodules and perceptual rating of voice quality in children. Methods We carried out an Institutional Review Board-approved retrospective study in a voice clinic within a tertiary-care pediatric medical center. We studied children seen between 2000 and 2009 with a primary diagnosis of vocal fold nodules as the cause of their voice disturbance. Pediatric vocal fold nodule size was rated with a published validated scale, and voice quality was rated on the Consensus Auditory-Perceptual Evaluation of Voice scale. Results One hundred forty-five patients met the inclusion criteria. Small nodules were noted in 23% of patients, medium nodules in 39%, and large nodules in 37%. Univariate and multivariate analyses demonstrated a statistically significant relationship (p < 0.05) between vocal fold nodule size and rated perceptual qualities of overall severity of voice disturbance, roughness, strain, pitch, and loudness. With the exception of loudness, as vocal fold nodule size increased, the mean value of perceptual characteristics became larger. The age of the patient was a significant factor associated with the overall severity of the voice disturbance and roughness. Conclusions The overall severity of a child's voice disturbance and qualities of roughness, strain, pitch, and loudness have a strong correlational relationship with pediatric vocal fold nodule size, which is suggestive of causality.

Objective To review the presentation and associated congenital abnormalities of laryngeal cleft and present guidelines for its evaluation and management. Design A 10-year retrospective study (1994-2004) with institutional review board approval. Setting Two pediatric tertiary care medical centers. Patients Twenty-two pediatric patients (mean age, 21 months) with laryngeal cleft. Intervention Surgical repair of laryngeal cleft. Main Outcome Measures Sex, age, symptoms, other associated abnormalities, method of evaluation, type of laryngeal cleft, method of surgical repair, treatment outcome, complications, and long-term follow-up. Results All 22 patients underwent surgical repair for laryngeal cleft. Airway endoscopy confirmed the following types of laryngeal clefts: type 1 (n = 3), type 2 (n = 10), and type 3 (n = 9). Surgical repair techniques included an open approach with or without interposition graft (n = 16) and an endoscopic approach (n = 6). Conclusions Early diagnosis and proper repair of laryngeal cleft are essential to prevent pulmonary damage and associated morbidity. Each patient should be assessed properly, and the surgical approach should be individualized based on the symptoms, other associated findings on airway endoscopy, and type of cleft.

Objective To design a grading scale for vocal fold nodules in pediatric patients. Study design and setting We conducted a prospective study in which a grading scale for vocal nodule size and contour based on static fiberoptic images of pediatric larynges was developed to achieve the scale presented here. Results Twenty-eight health care professionals each rated 28 images of pediatric vocal fold nodules. The intraclass correlation for nodule size was strong (0.77; 95% confidence interval, 0.67-0.87). The κ statistic for nodule contour was mild (0.35; 95% confidence interval, 0.33-0.37). Agreement between experienced and other raters found no significant difference for the nodule size or contour grade of a given image. Conclusions A grading scale for pediatric vocal fold nodules is presented. Interrater reliability for nodule size is high and can be reliably used by health care professionals with varying levels of experience. Significance A validated grading scale facilitates objective analysis of outcomes when studying and following patients with vocal nodules.

Effective treatment of haemophilic arthropathy requires a detailed evaluation of joint integrity. Methodological assessment of magnetic resonance imaging (MRI) scores are needed to assure reproducibility of measurements when comparing results of clinical trials conducted in different centres. We compared the reliability of two MRI scoring systems for assessment of haemophilic arthropathy: one progressive system that displays the most severe change and one additive system that depicts osteochondral and soft tissue-related changes. A total of 47 1.5 T MRI examinations of knees (n = 21) and ankles (n = 26) of 42 haemophilic boys, age range, 22 months to 18 years, performed at different centres (Toronto, n = 20, Europe, n = 12 and Denver, n = 15) were independently reviewed by four radiologists at two occasions. Twenty-two examinations were from children < 9 years and 25 from children G 9. Sagittal and coronal gradient-echo (MPGR, 3D FLASH with fat saturation, GRASS) images were obtained. The MRI examinations of the ankle and knee studies presented with osteochondral abnormalities in 38.5% and 23.8% of the cases respectively. The two scoring systems demonstrated an excellent inter-reader [progressive, 0.88; additive (A, e, s and h components), 0.86] and intra-reader [progressive, 0.92; additive (A, e, s and h components), 0.93] reliability using intraclass correlation coefficients (ICCs). Although ICCs were slightly higher for knees when compared with ankles, and for older children when compared with younger children, all values fell within excellent inter- and intra-reader reliability categories. The two MRI scoring systems demonstrated a comparable reliability. This result constitutes the basis for further development of a combined MRI scoring system for assessment of haemophilic arthropathy, which incorporates progressive and additive components. (Less)

Summary Objective: To present the epidemiology and correlation with perceptual analysis of vocal nodules in pediatric patients. Methods: Retrospective review of patients seen in a tertiary care pediatric hospital's voice center from 1996 to 2003. Six hundred and forty-six patients were evaluated with videostroboscopic examinations and perceptual analysis of voice characteristics by speech pathologists. Appropriate treatment was based on the pathology identified. Results: Two hundred and fifty-four patients (40%) with an average age of 7.7 years (range 0.1–19.3 years) were identified as having vocal nodules. Of these, 72% were male. Six patients (2%) were under the age of 7 months. Nodules were most commonly found in males, aged 3–10 years old. Evidence of gastro-esophageal reflux disease was found in one-quarter of patients; hyperfunction of the larynx was seen in three-fourths. Hyperfunction of the larynx correlates with the size of vocal nodules. Distortion of the vocal fold mucosal wave was not present. Perceptual analysis revealed positive correlation of the severity of hoarseness, breathiness, straining and aphonia with the size of vocal nodules. Conclusion: The epidemiology and correlation with perceptual voice analysis in pediatric patients with vocal nodules is presented. Hyperfunction of the larynx correlates with nodule size, while the presence of reflux disease does not. The severity of hoarseness, breathiness, straining and aphonia correlates with the size of vocal nodules.

Pediatric vocal cord paralysis represents a relatively common cause of airway obstruction presenting to the pediatric otolaryngologist. Depending on whether the paralysis is unilateral or bilateral, the child may present with symptoms of stridor, vocal dysphonia or aphonia, and/or aspiration. A thorough search for a possible underlying disorder is critical. Exact timing of intervention and the method of intervention vary according to symptoms and remain controversial. This seminar will consist of talks from established pediatric otolaryngologists with a wide scope of experience with these disorders. Diagnostic evaluation including flexible fiberoptic evaluation of swallowing (FEES) as well as flexible digital stroboscopy will be discussed. Surgical treatment options including vocal fold injection, thyroplasty, laser cordotomy/arytenoidectomy, vocal fold pexy, posterior costal cartilage graft, and nerve reinervation procedures will be discussed as treatment options. Digital video will be used to demonstrate the various procedures. Timing of such interventions will be discussed as well as this remains a key controversy.

To evaluate the treatment, perioperative management, and outcome of infants who underwent repair of congenital tracheal stenosis. We hypothesized that early resection and tracheoplasty with early weaning of ventilatory support results in less mucosal injury, and thus better outcome.Retrospective study from 1986 to 1996.Tertiary care children's hospital.Seventeen consecutive infants with congenital tracheal stenosis, aged from birth to 16 months. Fifteen patients had complete tracheal rings, 6 of whom also had a left pulmonary artery sling. Fourteen patients underwent either tracheoplasty or resection and reanastomosis of the trachea, both facilitated by cardiopulmonary bypass.Six patients underwent resection and reanastomosis; 4 patients were extubated within 2 to 5 days without sequelae. There was 1 unrelated perioperative death. Two patients required reintubation. Eight patients required tracheoplasty due to severe tracheal stenosis and had variable postoperative courses. Seven of 14 patients required 0 to 1 postoperative bronchoscopies. Seven of 14 patients required 2 to 7 bronchoscopies for granulation tissue formation, cicatrix, graft collapse, and tracheitis. One patient required numerous procedures and revision tracheoplasty for cicatrix and stenosis.Correction of short-segment (5 rings) tracheal stenosis by resection and reanastomosis of the trachea with the aid of cardiopulmonary bypass and early weaning of ventilatory support is recommended. Tracheoplasty using either the castellation technique or slide tracheoplasty is recommended in the treatment of infants with severe (long segment) tracheal stenosis.

The term "endolymphatic sac tumor" (ELST) was coined to identify the likely origin of aggressive papillary tumors of the temporal bone. To evaluate the validity of this designation, the temporal bone collection at the Massachusetts Eye and Ear Infirmary was accessed in an effort to determine the pathologic relationship between these tumors and the endolymphatic sac. The search resulted in the identification of a de-novo papillary epithelial lesion arising within the confines of the endolymphatic sac in a patient with von Hippel-Lindau (VHL) disease who harbored a large, destructive ELST in the opposite temporal bone. This finding provides the most substantial evidence to date regarding the origin of the ELST and the accuracy of its nomenclature. Seven additional clinical cases of ELST were identified and analyzed in order to define the natural history of these tumors. All patients had a history of sensorineural hearing loss diagnosed an average of 10.6 years prior to tumor discovery. The presence of a polypoid external auditory canal mass, facial paralysis, and evidence of a destructive mass arising on the posterior fossa surface of the temporal bone were common physical and radiographic findings. The management of these patients, as well as those who are probably prone to such tumors (i.e., VHL patients), is discussed.

Objectives To review the presentation of laryngeal neurofibroma, including its association with neurofibromatosis types 1 and 2, and present guidelines for its management. Design Retrospective study. Patients Five pediatric patients with laryngeal neurofibroma, 4 girls (80%) and 1 boy (20%), were treated at a tertiary pediatric medical center from 1973 through 2003. Recorded data included age at initial presentation, sex, symptoms, significant medical and family history, preoperative evaluation, location of the tumor, surgical procedure, complications, outcome, and recurrence. Results The 5 patients presented with stridor and cafe-au-lait spots at or shortly after birth. All patients were diagnosed as having neurofibromatosis type 1 by the established criteria. Studies evaluating the disease processes included plain radiography, computerized tomography, magnetic resonance imaging, barium swallow, and laryngoscopy and bronchoscopy under anesthesia. Pathologic examination of biopsy specimens from all patients showed neurofibromas with plexiform and/or diffuse features. Treatments included tracheotomy (n = 4), carbon dioxide laser excision (n = 4), modified neck dissection (n = 3), partial pharyngectomy (n = 1), supraglottic laryngectomy (n = 1), and endoscopic hemilaryngectomy (n = 1). Three patients were successfully decannulated. Follow-up ranged from 1 to 15 years. One patient was lost to follow-up. No evidence of malignant degeneration was noted. Conclusions Neurofibroma of the larynx is a rare condition that should be considered in the differential diagnosis of children with a submucosal laryngeal mass. In our series, all patients had associated neurofibromatosis type 1. Complete surgical excision is the treatment of choice in cases of localized small lesions. To prevent debilitating outcomes due to aggressive surgery, minimally invasive procedures (partial excision via endoscopic approach) may be preferable for larger lesions that infiltrate the surrounding vital structures. Long-term follow up of these patients is essential owing to the possibility of malignant transformation.

Objective To evaluate the role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract. Design Prospective study; institutional review board–approved clinical trial. Setting Tertiary care pediatric medical center. Patients Fifteen patients; choanal atresia in 5 patients, airway stenosis in 8 patients, hypopharyngeal stenosis in 1 patient, and esophageal stenosis in 1 patient. Outcome The efficacy and safety of mitomycin in the prevention of scar formation. Intervention All patients underwent surgical repair of the stenotic area, followed by topical application of mitomycin (1 mL of 0.4 mg/mL) for 4 minutes. Results Ten patients (67%) showed major improvement, 4 patients (27%) showed minor improvement, and 1 patient (7%) showed no improvement. Conclusion Topical application of mitomycin can play an effective role in the prevention and treatment of scar formation in the aerodigestive tract.

Objective To establish normative electroglottography (EGG) data in the pediatric population. Design Clinical study with EGG data gathered on children with normal voices. Setting Major children's hospital and specialty eye and ear hospital. Patients A total of 164 children, 79 girls and 85 boys, aged 3 to 16 years. Methods Children with normal voices, determined through subjective evaluation and a voice use history questionnaire, underwent EGG recording. The EGG data were analyzed with commercially available software for fundamental frequency, jitter, open quotient, closing quotient, and opening quotient. Results Normative EGG data were established for children aged 3 to 16 years. Jitter, open quotient, closing quotient, and opening quotient were all found to have no significant dependence on age. Conclusions Children as young as 3 years can easily tolerate EGG, making it possible to establish this initial set of normative pediatric EGG data. These preliminary results suggest that EGG may have potential to assist clinicians with noninvasive documentation of vocal function in the pediatric population. This may be particularly important for tracking treatment-related changes in the vocal function of children who are difficult to examine endoscopically.