Socioeconomic and racial disparities in revisits, indication, and readmission or reoperation in pediatric tonsillectomy.

INTRODUCTION: Pediatric tonsillectomy is a frequent otolaryngologic procedure. This study aimed to characterize disparities in post-tonsillectomy revisits, including emergency department evaluation, readmission, or reoperation as well as indication for revisit. METHODS: Cases of inpatient and ambulatory pediatric tonsillectomy in New York and Florida in 2016 constituted the analytic sample. Patients were extracted from the State Ambulatory Surgery Databases (SASD) and State Inpatient Databases (SID) and linked to the SID and State Emergency Department Database (SEDD) and SASD. Outcomes include 3 types of revisits within 30 days: ED visits, hospital readmissions, and reoperation. Indication for revisit was also analyzed. Multivariable analysis determined the association of each outcome with gender, age, race/ethnicity, primary payer, urbanicity, and zip code median household income quartile. The Holm Bonferroni test was used to correct for multiple hypothesis testing. RESULTS: 15,264 pediatric tonsillectomies were included. The revisit rate was 6.77% (N = 1,034, 49.1% female; 6 years median age [interquartile range: 5]). The 30-day ED revisit rate was 4.85%, readmission rate was 1.27%, and reoperation rate was 0.65%. On multivariate analysis, Latinx patients (OR = 3.042, 95% CI = 1.393-6.803) and those who identify as other race/ethnicity (OR = 6.116, 95% CI = 1.989-19.245) have greater odds of requiring inpatient care for indications including pain, dehydration, nausea, and vomiting compared to white patients. No significant differences in tier of care for the management of post-tonsillectomy hemorrhage were identified. CONCLUSION: Disparities in pediatric post-tonsillectomy ED presentation, readmission and reoperation demonstrate opportunities to improve patient safety and equity.

Pediatric intraoperative nerve monitoring during thyroid surgery: A review from the American Head and Neck Society Endocrine Surgery Section and the International Neural Monitoring Study Group.

Children are more likely to experience recurrent laryngeal nerve (RLN) injury during thyroid surgery. Intraoperative nerve monitoring (IONM) may assist in nerve identification and surgical decision making. A literature review of pediatric IONM was performed and used to inform a monitoring technique guide and expert opinion statements. Pediatric IONM is achieved using a variety of methods. When age-appropriate endotracheal tubes with integrated surface electrodes are not available, an alternative method should be used. Patient age and surgeon experience with laryngoscopy influence technique selection; four techniques are described in detail. Surgeons must be familiar with the nuances of monitoring technique and interpretation; opinion statements address optimizing this technology in children. Adult IONM guidelines may offer strategies for surgical decision making in children. In some cases, delay of second-sided surgery may reduce bilateral RLN injury risk.

Outcomes in Pediatric Thyroidectomy: Results From a Multinational, Multi-institutional Database.

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.

The Effect of the COVID-19 Pandemic on Pediatric Tympanostomy Tube Placement.

OBJECTIVE: To evaluate how the coronavirus disease 2019 (COVID-19) pandemic has affected tympanostomy tube placement and practice patterns. STUDY DESIGN: A retrospective review of billing data. SETTING: A large-volume practice with both community and tertiary care providers. METHODS: As part of a quality initiative, billing data were queried to identify children <18 years of age who underwent tympanostomy tube placement between January 2019 and December 2020. Patient age, practice location, and case numbers were gathered. RESULTS: The study included data from 2652 patients. Prior to state-mandated clinic and operating room restrictions, there were no significant differences in the number of tympanostomy tubes placed (P = .64), including month-to-month comparisons, the distribution of patients being cared for at community vs tertiary care sites (P = .63), or patient age at the time of surgery (P = .97) between 2019 and 2020. After resumption of outpatient clinical and elective surgical activities, the number of tympanostomy tubes placed decreased significantly between 2019 and 2020 (831 vs 303 cases, P = .003), with a persistent month-to-month difference. In addition, patients undergoing tube placement were older (4.5 vs 3.2 years, P < .001). The distribution of cases performed in the community setting decreased during this time period as well (P < .001). CONCLUSION: During the COVID-19 pandemic, the rate of pediatric tympanostomy tube placement has significantly decreased. The age of patients undergoing surgery has increased, and more children are being cared for in a tertiary setting. These findings may reflect changes in the prevalence of acute and chronic otitis media as the result of the pandemic.

Otoendoscopes to Enhance Telemedicine During the COVID-19 Pandemic.

OBJECTIVES: As telemedicine has become increasingly utilized during the COVID-19 pandemic, portable otoendoscopy offers a method to perform an ear examination at home. The objective of this pilot study was to assess the quality of otoendoscopic images obtained by non-medical individuals and to determine the effect of a simple training protocol on image quality. METHODS: Non-medical participants were recruited and asked to capture images of the tympanic membrane before and after completion of a training module, as well as complete a survey about their experience using the otoendoscope. Images were de-identified, randomized, and evaluated by 6 otolaryngologists who were blinded as to whether training had been performed prior to the image capture. Images were rated using a 5-point Likert scale. RESULTS: Completion of a training module resulted in a significantly higher percentage of tympanic membrane visible on otoendoscopic images, as well as increased physician confidence in identifying middle ear effusion/infection, cholesteatoma, and deferring an in-person otoscopy (P < .0001). However, even with improved image quality, in most cases, physicians reported that they would not feel comfortable using the images to for diagnosis or to defer an in-person examination. Most participants reported that the otoendoscope was simple to use and that they would feel comfortable paying for the device. CONCLUSIONS: At-home otoendoscopes can offer a sufficient view of the tympanic membrane in select cases. The use of a simple training tool can significantly improve image quality, though often not enough to replace an in-person otoscopic exam.

Hybrid laryngotracheal reconstruction vs single and double stage: Indications and outcomes.

OBJECTIVES: To describe outcomes from laryngotracheal reconstruction and decannulation rates for patients undergoing single stage, double stage and hybrid staged procedures at a single tertiary care institution and evaluate if the 1.5LTR is a viable reconstructive option for patients with subglottic stenosis. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care otolaryngology specialty hospital and internationally. SUBJECTS: All patients who underwent LTR by a single pediatric otolaryngology surgeon from 2008 to 2018. METHODS: Charts were assessed for age, gender, etiology, type of reconstruction, comorbidities, length of stay, tracheostomy status and socioeconomic status. Analysis was performed using Microsoft Excel and multivariate logistic regression models. RESULTS: 96 patients underwent laryngotracheal reconstruction at MEEI. Internationally, 36 patients underwent laryngotracheal reconstruction with the primary surgeon. Overall decannulation rates for ssLTR, dsLTR, and 1.5LTR were 95.6%, 77.8%, and 91.2% respectively. Our Operation Specific Decannulation Rates (one open airway procedure only) for ssLTR, dsLTR, and 1.5LTR were 87.5%, 33%, and 88% respectively. Adjusted odds of decannulation were not significantly different between males and females, white and non-white patients, or socioeconomic status. Neurological comorbidity was statistically significant for a decreased rate of decannulation (p = 0.0216). CONCLUSION: The 1.5LTR is a viable option for airway reconstruction with strengths derived from both the ssLTR and dsLTR. At our institution we have seen decannulation rates and operation specific decannulation rates with the 1.5LTR approaching our ssLTR. It has replaced the bulk of our dsLTRs, which we reserve for patients that have significant neurological deficits and cannot tolerate extended sedation. LEVEL OF EVIDENCE: 4.

Consensus Statement by the American Association of Clinical Endocrinology (AACE) and the American Head and Neck Society Endocrine Surgery Section (AHNS) on Pediatric Benign and Malignant Thyroid Surgery.

OBJECTIVES: To provide a clinical disease state review of recent relevant literature and to generate expert consensus statements regarding the breadth of pediatric thyroid cancer diagnosis and care, with an emphasis on thyroid surgery. To generate expert statements to educate pediatric practitioners on the state-of-the-art practices and the value of surgical experience in the management of this unusual and challenging disease in children. METHODS: A literature search was conducted and statements were constructed and subjected to a modified Delphi process to measure the consensus of the expert author panel. The wording of statements, voting tabulation, and statistical analysis were overseen by a Delphi expert (J.J.S.). RESULTS: Twenty-five consensus statements were created and subjected to a modified Delphi analysis to measure the strength of consensus of the expert author panel. All statements reached a level of consensus, and the majority of statements reached the highest level of consensus. CONCLUSION: Pediatric thyroid cancer has many unique nuances, such as bulky cervical adenopathy on presentation, an increased incidence of diffuse sclerosing variant, and a longer potential lifespan to endure potential complications from treatment. Complications can be a burden to parents and patients alike. We suggest that optimal outcomes and decreased morbidity will come from the use of advanced imaging, diagnostic testing, and neural monitoring of patients treated at high-volume centers by high-volume surgeons.

Consensus statement by the American Association of Clinical Endocrinology (AACE) and the American Head and Neck Society Endocrine Surgery Section (AHNS-ES) on Pediatric Benign and Malignant Thyroid Surgery.

OBJECTIVES: To provide a clinical disease state review of recent relevant literature and to generate expert consensus statements regarding the breadth of pediatric thyroid cancer diagnosis and care, with an emphasis on thyroid surgery. To generate expert statements to educate pediatric practitioners on the state-of-the-art practices and the value of surgical experience in the management of this unusual and challenging disease in children. METHODS: A literature search was conducted and statements were constructed and subjected to a modified Delphi process to measure the consensus of the expert author panel. The wording of statements, voting tabulation, and statistical analysis were overseen by a Delphi expert (J.J.S.). RESULTS: Twenty-five consensus statements were created and subjected to a modified Delphi analysis to measure the strength of consensus of the expert author panel. All statements reached a level of consensus, and the majority of statements reached the highest level of consensus. CONCLUSION: Pediatric thyroid cancer has many unique nuances, such as bulky cervical adenopathy on presentation, an increased incidence of diffuse sclerosing variant, and a longer potential lifespan to endure potential complications from treatment. Complications can be a burden to parents and patients alike. We suggest that optimal outcomes and decreased morbidity will come from the use of advanced imaging, diagnostic testing, and neural monitoring of patients treated at high-volume centers by high-volume surgeons.

Association of perioperative ibuprofen exposure with post-tonsillectomy bleeding requiring operative management.

INTRODUCTION: Pediatric tonsillectomy is one of the most common surgical procedures performed in the United States. The safety of ibuprofen use after surgery is debated given concern for increased bleeding. The primary objective of this study was to compare the rate of post-tonsillectomy hemorrhage requiring operative management in patients who received ibuprofen perioperatively vs. patients who did not. METHODS: Retrospective cohort study of patients 0-18 years old who underwent tonsillectomy with or without adenoidectomy (T&A) with recorded inpatient medication administration data at a single tertiary care institution from 1/2005-1/2019. The association between perioperative medication administration and return to operating room (OR) for control was evaluated using multivariable logistic regression adjusted for patient demographics and operative indication. Secondary outcomes evaluated included the time to operative bleed when it occurred. RESULTS: A total of 4098 patients with a median age of 6 years old (IQR 4-10) underwent T&A over the study period. The overall rate of post-tonsillectomy hemorrhage requiring OR was 3.37% (n = 138/4098). After adjustment for confounders, the odds of bleeding requiring OR did not differ significantly between the ibuprofen (OR 1.16, 95% CI (0.76, 1.74), 3.55%, n = 41/1,156, p = 0.47) and non-ibuprofen groups (3.30%, n = 97/2942). The median time to bleeding requiring OR was postoperative day 6.5 (IQR6-8) in the ibuprofen group and day 6 (IQR 3-8) in the non-ibuprofen group. CONCLUSIONS: No difference in post-tonsillectomy hemorrhage requiring OR was observed between patients receiving perioperative ibuprofen versus those patients not receiving this medication. Additional research is required to definitively determine a safe dose and interval for ibuprofen administration following tonsillectomy.

Update on hypoglossal nerve stimulation in children with down syndrome and obstructive sleep apnea.

OBJECTIVES/HYPOTHESIS: Hypoglossal nerve (HGN) stimulation is a novel therapy for obstructive sleep apnea (OSA) in adults. Its efficacy and safety in children with Down syndrome (DS) was previously reported in a preliminary case series of six adolescents. STUDY DESIGN: Case series. METHODS: Twenty nonobese children and adolescents (aged 10-21 years) with DS and severe OSA (apnea-hypopnea index [AHI] >10 and <50 events/hr) despite prior adenotonsillectomy were enrolled. Participants had failed a trial of continuous positive airway pressure therapy and underwent sleep endoscopy confirming surgical candidacy. The primary outcome was to assess safety and monitor for adverse events. Secondary outcomes included efficacy in reducing AHI (% reduction in AHI), adherence to therapy, and change in a validated quality-of-life instrument, the OSA-18 survey. RESULTS: All 20 children (median age = 16.0 years [interquartile range = 13-17 years], 13 male) were implanted with no long-term complications. We report two interval adverse events, both of which were corrected with revision surgery. Twenty participants completed the 2-month polysomnogram, with median percent reduction in titration AHI of 85% (interquartile range = 75%-92%). The median nightly usage for these children was 9.21 hours/night. There was a median change in the OSA-18 score of 1.15, indicating a moderate, yet significant, clinical change. CONCLUSIONS: HGN stimulation was safe and effective in the study population. Two minor surgical complications were corrected surgically. Overall, these data suggest that pediatric HGN stimulation appears to be a safe and effective therapy for children with DS and refractory severe OSA. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E263-E267, 2020.

Association of Feeding Evaluation With Frenotomy Rates in Infants With Breastfeeding Difficulties.

IMPORTANCE: Inpatient surgical release of lingual frenulums rose 10-fold between 1997 and 2012 despite insufficient evidence that frenotomy for ankyloglossia is associated with improvements in breastfeeding. Clear indications for surgical release remain murky, and best practice guidelines have yet to be developed. OBJECTIVE: To determine whether infants referred for frenotomy to treat breastfeeding difficulties should undergo procedures after comprehensive feeding examination, during which the primary cause of feeding issues was identified, and targeted intervention was provided. DESIGN, SETTING, AND PARTICIPANTS: This observational quality improvement study followed mother-infant dyads between March and December of 2018 who were referred to our tertiary care center for difficulty with breastfeeding. All infants underwent a comprehensive feeding evaluation by speech and language pathologists who examined the infants' ability to breastfeed prior to a surgical consultation for initial frenotomy. Data analysis was performed between January 2019 and May 2019. INTERVENTIONS: A multidisciplinary feeding evaluation that examined infants' oral structure and function and their ability to breastfeed and that offered techniques for mothers to address any feeding difficulties prior to surgical intervention was developed. Infants either found success in feeding and weight gain through this program or underwent procedures. MAIN OUTCOMES AND MEASURES: The primary outcome was the percentage of frenotomy procedures following implementation of a multidisciplinary feeding team evaluation. The secondary outcome was the percentage of infants referred for lingual frenotomy who later had either combined lingual and labial frenotomy or labial frenotomy alone. RESULTS: Included in the study were 115 patients (median age, 34 days [interquartile range, 19-56 days], 68 (59%) were male) referred for surgical division of the lingual frenum. Following the development of a program with feeding examination with a pediatric speech and language pathologist, 72 (62.6%) patients subsequently did not undergo surgical procedures. Although all of the referrals were for lingual frenotomy, 10 (8.7%) underwent labial frenotomy alone and 32 (27.8%) underwent both labial and lingual frenotomy. CONCLUSIONS AND RELEVANCE: The majority of patients referred for ankyloglossia may benefit from alternative intervention strategies following comprehensive feeding evaluation. Close collaboration and formation of multidisciplinary teams are imperative for treating these children.

Comparison of Ibuprofen vs Acetaminophen and Severe Bleeding Risk After Pediatric Tonsillectomy: A Noninferiority Randomized Clinical Trial.

Importance: Ibuprofen is an effective analgesic after tonsillectomy alone or tonsillectomy with adenoidectomy, but concerns remain about whether it increases postoperative hemorrhage. Objective: To investigate the effect of ibuprofen compared with acetaminophen on posttonsillectomy bleeding (PTB) requiring surgical intervention in children. Design, Setting, and Participants: A multicenter, randomized, double-blind noninferiority trial was conducted at 4 tertiary medical centers (Massachusetts Eye and Ear Infirmary, Boston; Naval Medical Center, San Diego, California; Naval Medical Center, Portsmouth, Virginia; Madigan Army Medical Center, Tacoma, Washington). A total of 1832 children were assessed for eligibility (presence of sleep-disordered breathing or obstructive sleep apnea, adenotonsillar hypertrophy, or infectious tonsillitis undergoing extracapsular tonsillectomy by electrocautery). Of these, 1091 were excluded because they did not meet eligibility criteria (n = 681) or refused to participate (n = 410); thus, 741 children aged 2 to 18 years undergoing tonsillectomy alone or tonsillectomy with adenoidectomy were enrolled between May 3, 2012, and January 20, 2017. Interventions: Participants were randomized to receive ibuprofen, 10 mg/kg (n = 372), or acetaminophen, 15 mg/kg (n = 369), every 6 hours for the first 9 postoperative days. Main Outcomes and Measures: Rate and severity of posttonsillectomy bleeding were recorded using a postoperative bleeding severity scale: type 1 (bleeds that were observed at home or evaluated in the emergency department without further intervention), type 2 (bleeds that required readmission for observation), and type 3 (bleeds that required a return to the operating room for control of hemorrhage). Type 3 bleeding was the main outcome measure. The noninferiority margin was set at 3%, and modified intention-to-treat analysis was used. Results: Of the 741 children enrolled, 688 children (92.8%) (median [interquartile range] age, 5 [4] years; 366 boys [53.2%]) received the study medication and were included in a modified intention-to-treat analysis. The rate of bleeding requiring operative intervention was 1.2% in the acetaminophen group and 2.9% in the ibuprofen group (difference, 1.7%; 97.5% CI upper limit, 3.8%; P = .12 for noninferiority). There were no significant adverse events or deaths. Conclusions and Relevance: This study could not exclude a higher rate of severe bleeding in children receiving ibuprofen after tonsillectomy alone or tonsillectomy with adenoidectomy. This finding should be considered when selecting a postoperative analgesic regimen. Further studies are needed to understand if bleeding risk is affected when ibuprofen is used for a shorter duration or in combination with acetaminophen for postoperative analgesia. Trial Registration: ClinicalTrials.gov identifier: NCT01605903.

The diagnostic role of triple endoscopy in pediatric patients with chronic cough.

OBJECTIVE/HYPOTHESIS: Multi-disciplinary aero-digestive centers provide high quality health care through improved outcomes and treatment costs over separate sub specialty clinics. These outcomes are often the result of a common investigative tool known as triple endoscopy: a rigid bronchoscopy performed by an otolaryngologist, flexible bronchoscopy and lavage obtained by a pulmonologist, and an endoscopy with guided biopsies performed by a gastroenterologist. Combining such procedures into one 'triple endoscopy' allows for diagnoses which otherwise might have been missed with just one procedure. The goal of our study was to describe the efficacy of the triple endoscopy procedure in diagnosing recalcitrant aero-digestive conditions, specifically chronic cough. STUDY DESIGN: Retrospective chart review METHODS: Multiple charts from children who underwent the triple endoscopy for chronic cough were retrospectively reviewed from 2005 and 2017. Complete data from the triple procedure was gathered on 243 patients, including findings by sub specialty (otolaryngology, pulmonology, and gastroenterology). RESULTS: Of the 243 patients with complete data who underwent triple endoscopy, 203 (83.5%) children had at least one positive finding. Of these children, 101 (41.5%) had one specialty specific diagnosis, and 102 (42%) had multiple cross specialty diagnoses. When describing the diagnoses, 63 children had gastro esophageal reflux (GER), 14 had eosinophilic esophagitis (EoE), 118 had tracheomalacia, 54 had laryngeal clefts, and 102 children had positive bronchoalveolar lavages. Outcome data was available on 226 patients (93%), of these patients, 188 patients had a diagnosis from the triple scope. Of those patients with a diagnosis and outcome data, 144 (76.6%) children had an improved outcome as a result of a treatment plan targeting that diagnosis, while 16 of the 37 patients without a diagnosis improved. This difference was significant by chi square analysis (p<0.0001). CONCLUSION: The triple scope procedure is a useful investigative tool for patients with recalcitrant aero-digestive complaints like chronic cough. In particular, triple scope can yield more than one specialty specific diagnosis, normally missed by one procedure. The triple scope also leads to improved parental satisfaction by improved cost and healthcare outcomes.

Analysis of Vocal Fold Motion Impairment in Neonates Undergoing Congenital Heart Surgery.

IMPORTANCE: Vocal fold motion impairment (VFMI) is a known risk factor following congenital heart surgery (CHS). The impact of this diagnosis on utilization and outcomes is unknown. OBJECTIVE: To evaluate the cost, postprocedure length of stay (PPLOS), and outcomes for neonates with VFMI after CHS. DESIGN, SETTING, AND PARTICIPANTS: A cross-sectional analysis of the 2012 Kids' Inpatient Database (KID) of neonates who underwent CHS was carried out. The KID is an administrative data set of patients, aged 20 years or younger, and contains data on more than 10 million hospitalizations from 44 states. The KID is limited to inpatient hospitalization and contains discharge summary level of data. Patients were limited to those who were born during the hospitalization and those who were aged 28 days or younger at the time of admission for CHS. A weighted total of 4139 neonates who underwent CHS were identified, of which 3725 survived. The proportion of neonates diagnosed with VFMI was 264 (6.92%) of 3725. EXPOSURES: Congenital heart surgery. MAIN OUTCOMES AND MEASURES: Cost of inpatient hospital stay, postprocedure length of stay, odds of pneumonia, gastrostomy tube placement, and tracheostomy tube placement. Risk-adjusted generalized linear models examined differences in cost and PPLOS between neonates who underwent CHS and were diagnosed with VFMI and those who were not. Risk-adjusted logistic regression compared the odds of selected outcomes (gastrostomy, tracheostomy, pneumonia). Models were weighted to provide national estimates. RESULTS: Of 3725 neonates (aged 0-28 days), 2203 (59.1%) were male and 1517 (40.7%) were female. Neonates diagnosed with VFMI had significantly higher total cost by $34 000 (95% CI, 2200-65 000) and PPLOS by 9.1 days (95% CI, 4.6-13.7) compared with those who did not. When PPLOS was included as a covariate in the model for cost, presence of VFMI was no longer significant. There were no differences in odds of pneumonia, gastrostomy, or tracheostomy. CONCLUSIONS AND RELEVANCE: Vocal fold motion impairment after CHS was associated with significant increases in cost owing to increased PPLOS. These findings provide a foundation to further investigate standardized screening for VFMI following CHS; early identification and treatment may decrease cost and PPLOS.

Modified surgical approach to hypoglossal nerve stimulator implantation in the pediatric population.

Upper airway stimulation with the hypoglossal nerve stimulator is a promising treatment modality for severe obstructive sleep apnea in carefully selected patients with Down syndrome. The pediatric population presents a greater variety in body habitus, including thorax size. A modified surgical approach, utilizing a medially placed, single chest incision, instead of two separate chest incisions, provides an alternative that is particularly useful for pediatric patients with small stature. As this technology is evaluated for Food and Drug Administration clearance in the pediatric population, it is important to consider modifications in surgical technique, partnering prior surgical experience with the technical support of company representatives. Laryngoscope, 128:1490-1492, 2018.

Hypoglossal Nerve Stimulation in Adolescents With Down Syndrome and Obstructive Sleep Apnea.

IMPORTANCE: Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS) and may persist in half of patients after adenotonsillectomy. Children with DS who have persistent OSA often do not tolerate treatment with positive pressure airway support devices or tracheotomy for their residual moderate to severe OSA. The hypoglossal nerve stimulator is an implantable device that delivers an electrical impulse to anterior branches of the hypoglossal nerve in response to respiratory variation, resulting in tongue base protrusion that alleviates upper airway obstruction in adults. OBJECTIVE: To determine whether hypoglossal nerve stimulation is safe and effective in children with DS. DESIGN, SETTING, AND PARTICIPANTS: Case series of the first 6 adolescents with DS to undergo hypoglossal nerve stimulator implantation. Participants were 6 children and adolescents (12-18 years) with DS and severe OSA (apnea hypopnea index [AHI] > 10 events/h) despite prior adenotonsillectomy. INTERVENTION: Inspire hypoglossal nerve stimulator placement. MAIN OUTCOMES AND MEASURES: Patients were monitored for adverse events. Adherence to therapy was measured by hours of use recorded by the device. Efficacy was evaluated by comparing AHI and OSA-18, a validated quality-of-life instrument, scores at baseline and follow-up. RESULTS: In 6 patients (4 male, 2 female; aged 12-18 years), hypoglossal nerve stimulator therapy was well tolerated (mean use, 5.6-10.0 h/night) and effective, resulting in significant improvement in OSA. At 6- to 12-month follow-up, patients demonstrated a 56% to 85% reduction in AHI, with an overall AHI of less than 5 events/h in 4 children and less than 10 events/h in 2 children. Children also demonstrated a clinically significant improvement (mean [SD] overall change score, 1.5 [0.6]; range, 0.9-2.3) on the OSA-18, a validated quality-of-life instrument. CONCLUSIONS AND RELEVANCE: Hypoglossal nerve stimulation was well tolerated and effective in the study population, representing a potential therapeutic option for patients with DS and refractory OSA after adenotonsillectomy who are unable to tolerate positive pressure airway devices. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT2344108.

Assessment of the feeding Swallowing Impact Survey as a quality of life measure in children with laryngeal cleft before and after repair.

OBJECTIVE: To cross-validate the Feeding Swallowing Impact Survey (FSIS), a quality of life instrument, specifically to a subpopulation of children who aspirate due to laryngeal cleft. INTRODUCTION: The FSIS is a recently validated instrument used to describe caregiver quality of life (QOL) in children with aspiration due to various causes. To cross-validate the FSIS specifically to the subpopulation of children who aspirate due to laryngeal cleft, we tested the hypotheses that caregivers would report significant different scores form baseline if their children improved at the one year mark postintervention due to either successful conservative or surgical measures (discriminant validity) and would not report significant differences in their FSIS reporting if there was no change in their child's aspiration at the one year mark post intervention (convergent validity). METHODS: The FSIS was administered to the caregivers of 35 children (19 male, 16 female; age range: 5-79 months) who aspirate secondary to known laryngeal cleft (diagnosed by suspension laryngoscopy and inspection). All children had a baseline VFSS demonstrating aspiration and documenting what feeding plan to follow and caregivers completed the FSIS at this point as well. All children regardless of whether they were treated by conservative or surgical intervention underwent a follow-up VFSS at the one year post-intervention mark and the caregivers completed a FSIS at this time point as well. RESULTS: Among two distinct sub-populations of children who underwent either successful conservative or surgical treatment for their laryngeal cleft and demonstrated improvement at the one year mark (as defined by a VFSS documented decreased need for thickener by at least one consistency or more) and where we hypothesized that FSIS scores would not be significantly different, the caregivers reported no significant differences in FSIS scores cleft repair (mean FSIS scores 2.45 (SD 0.88)/2.1 (SD 0.94); p = 0.28). Moreover, as another test to convergent validity, for children who underwent either unsuccessful conservative treatment (and subsequently went on to need surgery) or who were not successfully surgically treated for their laryngeal cleft and demonstrated no significant improvement at the one year mark (as defined by a VFSS documented decreased need for thickener by at least one consistency or more), the caregivers reported no significant differences in FSIS scores cleft repair (mean FSIS scores 2.8(SD 0.79)/2.5(SD 0.88); p = 0.69). For divergent validity, two distinct sub-populations of children who underwent either successful or not successful surgical treatment for their laryngeal cleft (demonstrated by either improvement or lack of improvement at the one year mark VFSS as defined by a decreased need for thickener by at least one consistency or more) revealed significant differences in caregiver FSIS scores cleft repair (mean FSIS scores 1.38 (SD 0.32); 32.8 (SD 0.79); p=<0.0002). DISCUSSION: This results of this study provide convergent and divergent validity supporting the cross-validation of the FSIS instrument to be utilized as a validated QOL instrument to evaluate children with aspiration specifically due laryngeal cleft as another tool with which to evaluate the outcomes of medical or surgical interventions for this disorder.

PD-L1 expression and CD8+ infiltration shows heterogeneity in juvenile recurrent respiratory papillomatosis.

INTRODUCTION: Tumor immunotherapy have broadened therapeutic options for tumor treatment. The role of immune function in juvenile recurrent respiratory papillomatosis (JRRP) has not been investigated. Applying immunoblockade inhibitors as a novel disease treatment is unclear. Our study, for the first time, evaluates immune infiltration and immuno-suppressive molecule expression in JRRP. Our study provides insights in possibly treating this disease with tumor immunotherapies. We aimed to determine expression of programmed death-ligand 1 (PD-L1), a cancer escape protein, and presence of CD8+ T cell infiltration in tumor microenvironment. MATERIAL AND METHODS: Seven patients with JRRP (mean age: 7.43; age range 3-17) in this study routinely have their tumors surgical debulked at Massachusetts Eye and Ear Infirmary. Following surgery, samples were de-identified and sent to pathology where they were stained and analyzed. RESULTS: Six out of seven patients expressed PD-L1 on tumor cells to various extents. Three patients showed concurrent PD-L1 expression on tumor cells and abundant CD8+ tumor infiltrating lymphocytes as well as PD-L1+ stromal lymphocytes, while PD-L1 expression on tumor cells were not associated with CD8+ tumor infiltrating T cells nor PD-L1+ stromal lymphocytes in the other three patients. HPV 6/11 and p16 was detected in all the patients. There appeared to be no correlation between either PD-L1 expression and CD8+ infiltration and clinical severity as measured by both the number of surgeries per year or Derkay score. CONCLUSIONS: Despite a small cohort, the expression of p16 and HPV 6/11 in all of the patients confirms the tissues were HPV tumor cells. PD-L1 expression was detected in the vast majority of tumor samples, while inflammatory cell compartments showed a higher degree of variation. Expression of PD-L1 on tumor cells but not inflammatory cells raises the possibility of a tumor cell intrinsic manner of PD-L1 expression. In contrast, a group of patients showed PD-L1 positivity in both tumor and inflammatory cells along with abundant CD8+ tumor infiltrating lymphocytes, suggesting adoptive immune resistance in these tumors and potential benefits from tumor immunotherapy.

Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea.

Obstructive sleep apnea (OSA) is more common in children with Down syndrome, affecting up to 60% of patients, and may persist in up to 50% of patients after adenotonsillectomy. These children with persistent moderate to severe OSA require continuous positive airway pressure, which is often poorly tolerated, or even tracheotomy for severe cases. The hypoglossal nerve stimulator is an implantable device that produces an electrical impulse to the anterior branches of the hypoglossal nerve, resulting in tongue protrusion in response to respiratory variation. It is an effective treatment of sleep apnea in select adult patients because it allows for alleviation of tongue base collapse, improving airway obstruction. Herein we describe the first pediatric hypoglossal nerve stimulator implantation, which was performed in an adolescent with Down syndrome and refractory severe OSA (apnea hypopnea index [AHI]: 48.5 events/hour). The patient would not tolerate continuous positive airway pressure and required a long-standing tracheotomy. Hypoglossal nerve stimulator therapy was well tolerated and effective, resulting in significant improvement in the patient's OSA (overall AHI: 3.4 events/hour; AHI: 2.5-9.7 events/hour at optimal voltage settings depending on sleep stage and body position). Five months after implantation, the patient's tracheotomy was successfully removed and he continues to do well with nightly therapy.

A case of pediatric parapharyngeal space ganglioneuroma.

Ganglioneuromas are rare, benign neoplasms derived from sympathetic neural crest progenitor cells. In the pediatric population, ganglioneuromas usually develop in the mediastinum or retroperitoneum. We report the case of a 3-year-old boy who presented with a painless enlarging neck mass, which was found to be a parapharyngeal space ganglioneuroma that extended to the skull base. We summarize the current principles regarding the diagnostic workup and treatment of these neoplasms, and we briefly review the literature.