Rate and management of tympanic membrane perforations in children with Down syndrome and middle ear disorder.

OBJECTIVE: To characterize the management and outcomes of observation versus surgical intervention of tympanic membrane (TM) perforations in children with Down syndrome (DS). In addition, to estimate the prevalence of TM perforations in children with DS. METHODS: Retrospective case review analysis of TM perforation rate in children with DS with history of tympanostomy tube (TT) insertion at a tertiary pediatric referral center. Patients were divided into observation or surgical intervention groups and then further evaluated for the type of intervention, the number of required procedures, and success rate of hearing improvement. Risk factors contributing to perforations were analyzed, including TT type, number of TT surgeries, and perforation size. RESULTS: The TM perforation rate in children with DS with TT history was 7.0 %. Tympanoplasty was performed in 41.5 % of perforated ears with a success rate of 53.1 %. There was no statistical difference between the surgical intervention and observation groups regarding perforation characteristics or TT number and type, but the surgical intervention cohort was older. Hearing improvement based on postoperative pure tone average (PTA) threshold was noted in the successful surgical intervention group. CONCLUSION: The rate of TM perforations in children with DS after TTs is comparable to the general population. Improved PTA thresholds were noted in the surgical success group influencing speech development. The overall lower success rate of tympanoplasty in patients with DS emphasizes the need to factor in the timing of surgical intervention based on the predicted age of Eustachian tube maturation.

Measuring the Impact of a Delay in Care on Pediatric Otolaryngologic Surgery Completion.

Objective: To determine if postponement of elective pediatric otorhinolaryngology surgeries results in a change in overall healthcare utilization and if there is any commensurate impact on disease progression. Methods: We identified patients ≤18 years of age whose surgeries were postponed at the onset of the COVID-19 pandemic-related shutdown. We then tracked patients' rate of and patterns of rescheduling surgery. Surveys were also sent to caregivers to better characterize his/her decision regarding moving forward with his/her child's surgery during COVID-19. Results: A total of 1915 pediatric patients had elective surgeries canceled, of which 992 (51.8%) were rescheduled within 4 months. No difference in rates of rescheduling was identified based on race or ethnicity. Patients who were scheduled for tonsillectomies and/or adenoidectomies were 1.22 times more likely to reschedule compared to those patients with other planned procedures (CI: 1.02-1.46). A total of 95 caregivers at two hospitals completed surveys: 44 (47.4%) rescheduled their child's surgery. Most caregivers who rescheduled were concerned their child's disease could impact their future (n = 14, 32%). Conclusions: Just over half of patients who had pediatric otolaryngologic surgery canceled during a period of social distancing went on to have surgery within a 4-month timeframe. This reflects the dependence of pediatric otolaryngologic surgery on environmental exposures and may represent a potential target for prevention and management of some pediatric otolaryngology diseases.

Prevalence and surgical management of cholesteatoma in Down Syndrome children.

OBJECTIVE: The objective was to report the outcomes of surgical management of cholesteatoma in Down Syndrome (DS) children and to determine the prevalence of cholesteatoma in DS children at three academic tertiary care centers. METHODS: A retrospective chart review was performed of all DS children diagnosed with cholesteatoma from three tertiary care academic centers. Demographic data, comorbidities, imaging findings, surgical management, and pre- and postoperative pure tone averages were collected. RESULTS: The prevalence of cholesteatoma in DS patients at these three institutions was calculated to be 8/802 (0.998%), 4/448 (0.893%), and 4/1016 (0.394%), with an average prevalence of 0.762%. Of the 16 patients with cholesteatoma identified, the average age of diagnosis was 10.8 years, and 5/16 (31%) had at least three sets of pressure equalizing tubes (PETs) placed. Of the 13 patients with preoperative CTs, 4/13 (30.8%) had blunting of the scutum, 4/13 (30.8%) had tegmen involvement, 1/13 (7.7%) had erosion of the semicircular canal, 2/13 had facial nerve dehiscence, and 7/13 (53.8%) had ossicular chain erosion. For surgical management, disease control was attained in 4/16 children with tympanoplasty only and canal wall up (CWU) tympanomastoidectomy in 11/16 patients. Available pre- and postoperative audiograms were evaluated; however, there was insufficient audiological data to contribute to outcomes. CONCLUSION: Cholesteatoma occurs in nearly 1% of DS patients, and the disease tends to be advanced with tegmen involvement in nearly a third of patients, and ossicular erosion seen in more than half the patients. A CWU procedure can be successful in managing DS patients with cholesteatoma when consistent long term follow-up is planned. Close monitoring and a high index of suspicion for cholesteatoma are essential to prevent delays in diagnosis and treatment of DS patients with a history of multiple sets of PET placement.

Continuous Positive Airway Pressure Titration During Pediatric Drug Induced Sleep Endoscopy.

OBJECTIVE: To observe the degree of airway collapse at varying levels of continuous positive airway pressure (CPAP) during drug pediatric induced sleep endoscopy. METHODS: Using our institutional anesthesia protocol for pediatric DISE procedures, patients were anesthetized followed by evaluation of the nasal airway, nasopharynx, velum, hypopharynx, arytenoids, tongue base, and epiglottis. CPAP titration was performed under vision to evaluate the degree of airway collapse at the level of the velum. Comparison was made with pre-operative polysomnography findings. RESULTS: Twelve pediatric patients underwent DISE with intraoperative CPAP titration. In 7/12 patients, DISE observed CPAP titration was beneficial in elucidating areas of obstruction that were observed at pressures beyond those recommended during preoperative sleep study titrations. In 3 patients, DISE observations provided a basis for evaluation in children not compliant with sleep study CPAP titration testing. With regard to regions effected, airway collapse was observed at the velum and oropharynx to a greater degree when compared with the tongue base and epiglottis. CONCLUSION: DISE evaluation of the pediatric patient with obstructive sleep apnea may present a source for further patient evaluation with respect to CPAP optimization and severity of OSA assessment, particularly in syndromic patients.

Extracapsular versus intracapsular tonsillectomy: Outcomes in children with a focus on developmental delay.

INTRODUCTION: Outcomes following intracapsular tonsillectomy (IT) have not been well established in children with developmental delays. The objective of this study was to compare outcomes and complications between intracapsular and extracapsular tonsillectomy (TT) in pediatric patients with developmental delay (DD) in comparison to non-developmentally delayed children. METHODS: This is a retrospective study of pediatric patients with DD undergoing tonsillectomy between 2016 and 2019 at a tertiary care hospital. This group included patients with Down Syndrome, Autism Spectrum Disorder, other genetic syndromes, and patients with a diagnosis of global developmental delay. Outcomes and complications were analyzed for IT and TT. RESULTS: 2267 charts were reviewed, and 320 patients were identified with DD. Of those, 72 patients underwent IT and 248 underwent TT. In the DD cohort, the IT group had a shorter length of stay (0.97 vs 1.7 days, p < .0001) and was less likely to receive post-operative narcotic medication (2.8% vs 35%, p < .0001) and corticosteroids (9.7% vs 64%, p < .0001) during their hospital stay. Reductions in emergency room (ER) visits (5.6% vs 10%, p = .21) and post-op bleeding (PTH) (1.4% vs 4.8%, p = .31) for IT vs TT were not statistically significant in the DD group. In the NDD group, fewer patients undergoing IT returned to the ER (11% vs 2.3%, p < .0001) or had PTH (4.8% vs 0.25%, p, 0.0001) as compared to those children undergoing TT. There was no difference between parental report of symptom improvement between the groups (39% vs 33%, p = .39). Analysis of 180 patients with preoperative and postoperative sleep study data revealed post-op Apnea Hypopnea Index (AHI) improved with both techniques (74% TT vs 79% IT, p = .7). There were no differences noted for persistent obstructive sleep apnea (OSA) among the two techniques for both study groups (p = .63). CONCLUSION: Children with DD undergoing IT have reduced length of stay and reduced inpatient administration of post-operative opioids and steroids. IT has comparable efficacy to TT in treating symptoms of pediatric sleep apnea with a better safety profile. Overall, children undergoing IT return to the operating room less frequently than those undergoing TT. Longer follow-up studies will be needed to evaluate rate of tonsil regrowth, risk of revision surgery and persistence of OSA in these patients.

Volumetric brain magnetic resonance imaging analysis in children with obstructive sleep apnea.

OBJECTIVES: Pediatric Obstructive Sleep Apnea (OSA) is associated with neurocognitive deficits. Cerebral structural alterations in the frontal cortex, cerebellum, and hippocampus have been reported in adult OSA patients. These brain areas are important for executive functioning, motor regulation of breathing, and memory function, respectively. Corresponding evidence comparing cerebral structures in pediatric OSA patients is limited. The objective of this study is to investigate MRI differences in cortical thickness and cortical volume in children with and without OSA. STUDY DESIGN: Prospective, single institutional case-control study. METHODS: Forty-five children were recruited at a pediatric tertiary care center (27 with OSA; mean age 9.9 ± 1.9 years, and 18 controls; mean age 10.5 ± 1.0 years). The OSA group underwent magnetic resonance imaging (MRI), polysomnography (PSG) and completed the Pediatric Daytime Sleepiness Scale (PDSS) and the Child's Sleep Habits Questionnaire (CSHQ). High-resolution T1-weighted MRI was utilized to examine cortical thickness and gray and white matter volume in children with OSA compared to a healthy group of demographically-comparable children without OSA selected from a pre-existing MRI dataset. RESULTS: Children with OSA showed multiple regions of cortical thinning primarily in the left hemisphere. Reduced gray matter (GM) volume was noted in the OSA group in multiple frontal regions of the left hemisphere (superior frontal, rostral medial frontal, and caudal medial frontal regions). Reduced white matter (WM) volume in both the left and right hemisphere was observed in regions of the frontal, parietal, and occipital lobes in children with OSA. CONCLUSION: This study noted differences in cortical thickness and GM and WM regional brain volumes in children with OSA. These findings are consistent with other pediatric studies, which also report differences between healthy children and those with OSA. We found that the severity of OSA does not correlate with the extent of MRI alterations.

International Pediatric Otolaryngology group (IPOG) consensus on the diagnosis and management of pediatric obstructive sleep apnea (OSA).

OBJECTIVE: To develop an expert-based consensus of recommendations for the diagnosis and management of pediatric obstructive sleep apnea. METHODS: A two-iterative Delphi method questionnaire was used to formulate expert recommendations by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: Twenty-six members completed the survey. Consensus recommendations (>90% agreement) are formulated for 15 different items related to the clinical evaluation, diagnosis, treatment, postoperative management and follow-up of children with OSA. CONCLUSION: The recommendations formulated in this IPOG consensus statement may be used along with existing clinical practice guidelines to improve the quality of care and to reduce variation in care for children with OSA.

Polysomnography in children with obstructive sleep apnoea and neurocognitive disorders.

OBJECTIVE: The neurocognitive associations in paediatric obstructive sleep apnoea (OSA) are well known; however, whether polysomnographic features can predict these associations is unknown. Therefore, the primary objective of this study was to compare common polysomnographic parameters in children with OSA in the presence and absence of neurocognitive dysfunction. METHODS: Polysomnography data for children ages 3-6 years with mild-moderate OSA who as defined by AHI between 5 and 10 were analysed from a single sleep centre at a tertiary paediatric hospital from January 2016 to December 2018. The following parameters were identified: arousals per hour, percentage of time asleep, apnoea-hypopnoea index (AHI), oxygen desaturation nadir during sleeps, baseline oxygen saturation during sleep, time spent with SpO2 less than 90%, maximum transcutaneous CO2, per cent of the total sleep time spent with TcCO2 greater than 50 mmHg, age, body mass index (BMI), gender and type of disability in the neurocognitive dysfunction group. Neurocognitive diagnoses were recorded. Those with syndromic comorbidities were excluded. The study cohort was then compared to a cohort of 200 subjects with OSA and no neurocognitive disorders matched for age, gender and BMI. A paired column analysis by chi-squared analysis was then undertaken between the two groups. RESULTS: A total of 200 children were identified (126 males and 74 females) in the neurocognitive dysfunction group (OSA with neurocognitive dysfunction) and compared with 200 children in the control group (OSA without neurocognitive dysfunction) (113 males and 87 females). There were no statistical differences between groups. CONCLUSION: Commonly used polysomnographic indices are not predictive of neurocognitive dysfunction in paediatric OSA.

Pediatric otolaryngology workflow changes in a community hospital setting to decrease exposure to novel coronavirus.

OBJECTIVES: To develop a workflow algorithm for physicians and staff to decrease cross contamination and minimize exposure to Coronavirus Disease -19 (COVID-19) in pediatric otolaryngology at a community pediatric hospital. METHODS: PubMed and Google Scholar searches were carried out using the keywords. Pertinent clinical information related to pediatric otolaryngology was reviewed from journals, Texas Medical Board guidelines as well as institutional guidelines. Updated information from the Centers for Disease Control through social media was identified via Google search. STUDY DESIGN: Safety measures and clinical work-flow protocols were developed to protect patients, families, and staff. Protocols included a rotation schedule for providers and ancillary staff, guidelines for in-clinic visits and alteration to surgical block and surgical case cadence to minimize exposure of providers and staff to COVID-19. Algorithms and guidelines were reviewed and revisions made at each phase of the pandemic related to in-clinic visits, telemedicine visits, and surgical cases. CONCLUSION: In the era of the COVID-19 pandemic, otolaryngologists and pediatric otolaryngologists are amongst sub-specialties with an increased risk of exposure. It is imperative that a modification in clinical workflow is created to minimize the risk of exposure to providers and staff while continuing to provide care to patients. PRACTICE IMPLICATIONS: The COVID-19 pandemic continues to evolve and change rapidly. The described guidelines and workflow algorithm serve as tools to help pediatric otolaryngologists protect themselves and their staff and patients during this global crisis.

Pediatric obstructive sleep apnea: Preoperative and neurocognitive considerations for perioperative management.

Obstructive sleep apnea (OSA) affects up to 7.5% of the pediatric population and is associated with a variety of behavioral and neurocognitive sequelae. Prompt diagnosis and treatment is critical to halting and potentially reversing these changes. Depending on the severity of the OSA and comorbid conditions, different treatment paradigms can be pursued, each of which has its own unique risk:benefit ratio. Adenotonsillectomy is first-line recommended surgical treatment for pediatric OSA. However, it carries its own perioperative risks and the decision regarding surgical timing is therefore made in the context of procedural risk versus patient benefit. This article presents the seminal perioperative and neurocognitive risks from pediatric OSA to aid with perioperative management.

Airway protection & patterns of dysphagia in infants with down syndrome: Videofluoroscopic swallow study findings & correlations.

INTRODUCTION: Down syndrome is a genetic condition that affects 1:737 births. Along with cardiac, otolaryngologic, and developmental anomalies, infants with Down syndrome can have swallowing difficulties resulting in respiratory infections. This study aims to characterize the airway protection and dysphagia seen in infants with Down syndrome. METHODS: This is a retrospective chart review of infants with Down syndrome who underwent videofluoroscopic swallow studies (VFSS) from 2008 to 2018 at a tertiary children's hospital. Demographic data and VFSS findings were collected. RESULTS: 89.8% (114/127) of infants presented with at least one element of oral dysphagia, while 72.4% (92/127) had at least one element of pharyngeal dysphagia. Sucking skills were classified as abnormal in 63.7% of the patients and bolus formation-control was determined to be deficient (abnormal) in 62.2% of the patients. Oral residuals were present in 37.8% of the patients. With regard to pharyngeal phase, the swallow initiation was considered abnormal in 53.5% of the patients. Pharyngeal residue was present in 17.3% and pharyngo-nasal reflux was present in 27.5% of the patients. CONCLUSIONS: Swallowing assessments in infants with Down syndrome suspected of dysphagia should be considered, especially in those with any alterations in pulmonary health.

Pediatric Drug Induced Sleep Endoscopy: A Simple Sedation Recipe.

OBJECTIVE: To describe a minimalist approach to sedating children for DISE procedures. METHODS: We searched existing literature and derived and tested our algorithm on patients using evidence-based studies. RESULTS: We were able to successfully sedate, without airway intervention, 15 highly complex children with a variety of comorbidities for DISE procedures. CONCLUSION: We describe a minimalistic sedation approach for DISE procedures in highly complex children. Further studies are required to compare this regimen to natural sleep states.

Screening for undiagnosed bleeding disorders in post-tonsillectomy bleed patients: Retrospective review and systematic review of the literature.

OBJECTIVES: There is currently no standard for screening children with post-tonsillectomy bleeds (PTB) for coagulopathy disorders. This study aims to identify children with occult coagulopathy diagnosed at PTB and to identify factors associated with diagnosis. A systematic review of the literature further identified trends in this topic. METHODS: A retrospective chart review of patients returning to the operating room for PTB at a tertiary children's hospital was undertaken from 2012 to 2016. A systematic review using Medline OVID was subsequently performed. RESULTS: Of 12,503 tonsillectomies, 311 children (52% male, mean age 8 years) required surgery for PTB (2.5% rate). Twenty-one patients (7%) had multiple episodes. Only two patients (0.6%) (both with known coagulopathy) underwent pre-tonsillectomy labs and 260 (84%) had labs at PTB. Six patients (2%) were diagnosed with a new coagulopathy, most commonly von Willebrand's Disease (vWD) in five (2%). Three patients (1%) were diagnosed at first PTB and three (1%) at second PTB. Of the three diagnosed at second PTB, two had normal partial thromboplastin time (PTT). In systematic review, 1243 manuscripts were reviewed and 8 papers discussing this topic are presented. CONCLUSION: Occult coagulopathy was rarely diagnosed at PTB, but this may be limited by inconsistent screening. PT and PTT are not sensitive tests for vWD, and normal coagulation labs may lead to delayed diagnosis. The literature reveals occult coagulopathy is rare but often diagnosed after severe or recurrent hemorrhage. In order to provide efficient care and medical management, a standardized algorithm and sensitive labs for screening PTB patients are needed.

Otolaryngologic management of Down syndrome patients: what is new?

PURPOSE OF REVIEW: The management of children with Down syndrome as it pertains to the otolaryngologist continues to evolve. Obstructive sleep apnea (OSA) has dominated the recent literature, but other topics including hearing loss, swallowing, and perioperative considerations are also reported. RECENT FINDINGS: The prevalence of OSA in children with Down syndrome ranges from 57 to 73% in certain cohorts, and, whereas adentonsillectomy can decrease Apnea-Hypopnea Index, up to 80% may have persistent OSA. Surgical techniques involving reduction of the base of tongue are effective for those who fail adenotonsillectomy, and it is expected that drug-induced sleep endoscopy may improve outcomes. New technology is also on the horizon that can assist with diagnosis and treatment including computational modelling and upper airway stimulation. Children with Down syndrome may not respond to medical management of eustachian tube dysfunction as well as normally developing children. In addition, there is a high prevalence of inner ear anomalies, increasing the risk for sensorineural hearing loss. SUMMARY: Questions remain pertinent to the otolaryngologist regarding the ideal management of children with Down syndrome. Additional studies are necessary, to optimize understanding and treatment of this complex population, in particular as opportunities develop with technological advances.

The reliability of flexible nasolaryngoscopy in the identification of vocal fold movement impairment in young infants.

OBJECTIVE: Flexible nasolaryngoscopy (FNL) is considered the gold standard for evaluation of vocal fold mobility but there has been no data on the reliability of interpretation in the infant population. Visualization may be limited by excessive movement, secretions, or floppy supraglottic structures that prevent accurate diagnosis of vocal fold movement impairment (VFMI). We sought to evaluate the inter- and intra-rater reliability of FNL for the evaluation of VFMI in young infants. STUDY TYPE: Case-control. METHODS: Twenty infants were identified: 10 with VFMI and 10 normal as seen on FNL. Three pediatric otolaryngologists reviewed the video without sound and rated the presence and/or degree of vocal fold mobility. Twelve videos were repeated to assess intra-rater reliability. RESULTS: There was substantial agreement between the reviewers regarding the identification normal vs. any type of VFMI (kappa = 0.67) but only moderate agreement regarding the degree of vocal fold movement (kappa = 0.49). Intra-rater reliability ranges from moderate to perfect agreement (kappa = 0.48-1). CONCLUSION: FNL in infants is an extremely challenging procedure. Clinically, physicians frequently use the quality of the cry and the past medical and surgical history to help make a judgment of vocal fold movement when the view is suboptimal. These other factors, however, may bias the interpretation of the FNL. Without sound, there is only moderate inter-rater and variable intra-rater reliability for the identification of degree of movement on FNL. Otolaryngologists must be cognizant of the limitations of FNL when using it as a clinical tool or as a "gold standard" against which other modalities are measured.