Pediatric Robotic Laryngeal Cleft Repair.

OBJECTIVE: Compare surgical and swallow outcomes in robotic versus traditional laryngeal cleft (LC) repairs. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. METHODS: Pediatric patients who underwent robotic or traditional (open or endoscopic) LC repair between 2010 and 2021 were identified. Patient characteristics, operative times, adverse events, hospital length of stay (LOS), and modified barium swallow study (MBSS) results were compared. RESULTS: Eighteen robotic and thirty traditional LC repairs were identified. Mean surgical (149 vs 111 min, P < .05) and OR times (207 vs 139 min, P < .002) were increased for robotic type I LC repairs, but were similar for type II and III LC. Mean hospital LOS was increased for robotic type I LC repairs (2.6 vs 1.2 days, P < .006), but was decreased for type II (4 vs 12.2 days) and type III (4.3 vs 94.5 days) LC. Postoperative MBSS results were improved for robotic type I LC repairs at 12 months (82% vs 43%, P = .05), and trended toward improvement at 6 months for type II (75% vs 22%), and type III (67% vs 50%) LC repairs, although significance was limited for type II and III LC due to the number of subjects. A robotic approach was used successfully to revise all recurrent LC that failed traditional repairs. CONCLUSION: Robotic type 1 LC repairs demonstrated increased operative times and hospital LOS but improved postoperative swallow outcomes compared to traditional approaches may be particularly useful in cases of recurrent clefts.

Large Nasopharyngeal Hairy Polyp Causing Immediate Respiratory Distress at Birth.

This article presents a rare case of a large hairy polyp, a developmental malformation causing a benign tumor, within the nasopharynx. The patient, born with the polyp obstructing the airway, required immediate intubation and a combined transnasal-transoral surgical approach for excision. The case underscores the challenges in diagnosing and managing such polyps, emphasizing the importance of imaging for surgical planning, and the consideration of multiple approaches to ensure complete resection and prevent recurrence. Laryngoscope, 2024.

Surgical procedure of intratympanic injection and inner ear pharmacokinetics simulation in domestic pigs.

Introduction: One major obstacle in validating drugs for the treatment or prevention of hearing loss is the limited data available on the distribution and concentration of drugs in the human inner ear. Although small animal models offer some insights into inner ear pharmacokinetics, their smaller organ size and different barrier (round window membrane) permeabilities compared to humans can complicate study interpretation. Therefore, developing a reliable large animal model for inner ear drug delivery is crucial. The inner and middle ear anatomy of domestic pigs closely resembles that of humans, making them promising candidates for studying inner ear pharmacokinetics. However, unlike humans, the anatomical orientation and tortuosity of the porcine external ear canal frustrates local drug delivery to the inner ear. Methods: In this study, we developed a surgical technique to access the tympanic membrane of pigs. To assess hearing pre- and post-surgery, auditory brainstem responses to click and pure tones were measured. Additionally, we performed 3D segmentation of the porcine inner ear images and used this data to simulate the diffusion of dexamethasone within the inner ear through fluid simulation software (FluidSim). Results: We have successfully delivered dexamethasone and dexamethasone sodium phosphate to the porcine inner ear via the intratympanic injection. The recorded auditory brainstem measurements revealed no adverse effects on hearing thresholds attributable to the surgery. We have also simulated the diffusion rates for dexamethasone and dexamethasone sodium phosphate into the porcine inner ear and confirmed the accuracy of the simulations using in-vivo data. Discussion: We have developed and characterized a method for conducting pharmacokinetic studies of the inner ear using pigs. This animal model closely mirrors the size of the human cochlea and the thickness of its barriers. The diffusion time and drug concentrations we reported align closely with the limited data available from human studies. Therefore, we have demonstrated the potential of using pigs as a large animal model for studying inner ear pharmacokinetics.

Accuracy and Reliability of 4D-CT and Flexible Laryngoscopy in Upper Airway Evaluation in Robin Sequence.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = none to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, P = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, P < .0001). CONCLUSION: Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.

4D Computed Tomography for Dynamic Upper Airway Evaluation in Robin Sequence.

Thorough assessment of dynamic upper airway obstruction (UAO) in Robin sequence (RS) is critical, but traditional evaluation modalities have significant limitations. Four-dimensional computed tomography (4D-CT) is promising in that it enables objective and quantitative evaluation throughout all phases of respiration. However, there exist few protocols or analysis tools to assist in obtaining and interpreting the vast amounts of obtained data. A protocol and set of data analysis tools were developed to enable quantification and visualization of dynamic 4D-CT data. This methodology was applied to a sample case at 2 time points. In the patient with RS, overall increases in normalized airway caliber were observed from 5 weeks to 1 year. There was, however, continued dynamic obstruction at all airway levels, though objective measures of UAO did improve at the nasopharynx and oropharynx. Use of 4D-CT and novel analyses provide additional quantitative information to evaluate UAO in patients with RS.

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.

IVORY Guidelines (Instructional Videos in Otorhinolaryngology by YO-IFOS): A Consensus on Surgical Videos in Ear, Nose, and Throat.

OBJECTIVES/HYPOTHESIS: Otolaryngology instructional videos available online are often of poor quality. The objective of this article was to establish international consensus recommendations for the production of educational surgical videos in otolaryngology. STUDY DESIGN: DELPHI survey. METHODS: Twenty-seven international respondents participated in this study from 12 countries. Consensus was reached after three rounds of questionnaires following the Delphi methodology. The proposals having reached the 80% agreement threshold in the third round were retained. RESULTS: The main recommendations are as follows: 1) Ethics: patients must be anonymized and unrecognizable (apart from plastic surgery if necessary). A signed authorization must be obtained if the person is recognizable. 2) Technical aspects: videos should be edited and in high-definition (HD) quality if possible. Narration or subtitles and didactic illustrations are recommended. 3) Case presentation: name of pathology and procedure must be specified; the case should be presented with relevant workup. 4) Surgery: surgical procedures should be divided into several distinct stages and include tips and pitfalls. Pathology should be shown if relevant. Key points should be detailed at the end of the procedure. 5) Organ-specific: type of approach and bilateral audiometry should be specified in otology. Coronal plane computed tomography scans should be shown in endonasal surgery. It is recommended to show pre- and postoperative videos in voice surgery and preoperative drawings and photos of scars in plastic surgery, as well as the ventilation method in airway surgery. CONCLUSIONS: International recommendations have been determined to assist in the creation and standardization of educational surgical videos in otolaryngology and head and neck surgery. LEVEL OF EVIDENCE: 5 Laryngoscope, 131:E732-E737, 2021.

Idiopathic, Refractory Sweet's Syndrome Associated with Common Variable Immunodeficiency: a Case Report and Literature Review.

PURPOSE OF REVIEW: Sweet's syndrome (SS) is classically considered a hypersensitivity reaction often associated with autoimmune disorders and malignancy. SS has also been increasingly reported to occur with immunodeficiencies. We present a case of treatment-refractory, systemic SS as the initial manifestation in a young child with common variable immunodeficiency (CVID). We also review current literature about SS and concurrent immunodeficiencies and autoimmunity in CVID patients. RECENT FINDINGS: Few case reports exist regarding the co-occurrence of Sweet's syndrome and primary immunodeficiencies. SS is characterized by a pro-inflammatory state with a neutrophil predominance resulting in a spectrum of clinical manifestations. CVID is a multifactorial antibody deficiency that can be associated with autoimmunity, which some studies have proposed to be secondary to altered CD21 expression. SS occurring in patients with CVID has been infrequently reported, and one case study demonstrated improvement of Sweet's associated skin lesions with immunoglobulin replacement. In our case, the patient had multi-system SS refractory to multiple immunomodulatory therapies. To our knowledge, this is the first report of the effective and safe use of intravenous tocilizumab and oral lenalidomide to treat SS in a child with CVID. Immunoglobulin replacement reduced the frequency of infections and may have contributed to the opportunity to wean the immunosuppressive therapies for Sweet's syndrome. Sweet's syndrome as an initial manifestation of co-occurring immunodeficiencies is rare, and providers need a high index of suspicion. In addition, treatment of SS associated with an immunodeficiency can be a challenge. Treatment with immunoglobulin replacement reduces the frequency of infections, and in some patients with concurrent SS may improve skin lesions and reduce the need for immunomodulator therapy. Further study is necessary to better understand the pathogenesis of CVID in patients with SS and to identify possible biomarkers that predict who with SS are at risk for developing hypogammaglobulinemia.

Considering a Weight Criterion for Neonatal Tracheostomy: An Analysis of the ACS NSQIP-P.

OBJECTIVES: Neonates weighing < 2.5 kg have known higher rates of surgical mortality and morbidity, but this remains unexamined specifically for tracheostomy. We present outcomes of neonates undergoing tracheostomy stratified by weight. METHODS: Retrospective review of the American College of Surgeons National Surgical Quality Improvement Program-Pediatric from 2012 to 2014. Patients undergoing tracheostomy were included. Thirty-day mortality and major/minor complication rates were stratified by weight (group 1: < 2.5 kg; group 2: ≥ 2.5 kg and < 4 kg; group 3: ≥ 4 kg). Patient comorbidities were assessed for independent risk factors of morbidity and mortality. RESULTS: Of 183,233 patients, 543 underwent tracheostomy. Forty-four patients were group 1 (mean: 2.2 kg ± 0.25); 170 patients were group 2 (mean: 3.31 kg ± 0.42); and 329 patients were group 3 (mean: 6.4 kg ± 2.7). Between groups 1 and 2, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.99), or minor complication rates (P = 0.64). In comparing all three groups, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.80), or minor complication rates (P = 0.77). The overall 30-day mortality for all patients was 4.24%. In a multivariate logistical regression model, weight group did not change the odds of all negative outcomes (group 1: odds ratio [OR] of 0.71; 95% confidence interval [CI], 0.33-1.53 and group 2: OR of 0.78; 95% CI, 0.50-1.22). Bronchopulmonary dysplasia was the only independent significant predictor of major complications (OR, 1.69; 95% CI, 1.02-2.79) (P = 0.04). CONCLUSION: Our data indicate that 30-day mortality and morbidity outcomes for neonatal tracheostomy are not affected by weight. Overall 30-day mortality should be discussed with caregivers preoperatively. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:500-505, 2019.

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.

Transoral robotic surgery for upper airway pathology in the pediatric population.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to present one of the largest case series of pediatric transoral robotic surgery (TORS) in the upper airway demonstrating a wide range of ages and indications. STUDY DESIGN: A retrospective case series at an academic tertiary referral center from August 2010 to September 2014. METHODS: The da Vinci surgical robot (Intuitive Surgical, Inc., Sunnyvale, CA) was used on 16 pediatric patients for 18 procedures. A variety of upper airway pathologies and reconstructions in children with a wide range of ages and weights were treated. No lingual tonsillectomies or base-of-tongue reductions were included. RESULTS: Sixteen children (6 males) underwent 18 TORS procedures, including resection of hamartoma (n = 1), repair of laryngeal cleft (n = 7), removal of saccular cyst (n = 2), release of pharyngeal or esophageal strictures (n = 2), and excision of lymphatic malformations (n = 4). Patient ages ranged from 14 days to 15 years. There were no intraoperative complications. All patients had successful robotic access, and no patients had conversions to open or traditional endoscopic surgery. Hospital courses varied with duration ranging from 1 to 20 days. The median follow up was 22 months. CONCLUSION: Applying TORS to the pediatric population can be feasible and safe for appropriate airway pathologies. Because many patients are small in size, there is inherent risk in using robotic instruments and scopes transorally. Pearls in this series include a standardized two-robot experienced attending team and longitudinal airway follow-up. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:247-251, 2017.

The effect of interdevice interval on speech perception performance among bilateral, pediatric cochlear implant recipients.

OBJECTIVES/HYPOTHESIS: To determine if prolongation of the interdevice interval in children receiving bilateral cochlear implants adversely affects speech perception outcomes. STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review of our pediatric cochlear implant database was performed. Children who had undergone revision surgery or had less than 12 months listening experience with either the first or second implant were excluded. The interdevice interval, best Phonetically Balanced Kindergarten word lists (PBK) score from each ear, and demographic data about each patient were collected. A ratio of PBK was generated (PBK second side/PBK first side) to minimize potential confounding from other individual patient factors that affect speech outcomes. RESULTS: Two hundred forty children met the study criteria. Mean age at first cochlear implantation (CI) was 3.2 years (0.6-17.9), and the second was 6.6 years (0.8-22.4). Mean best PBK score from the first CI side was 83.8% (0-100), and the second was 67.5% (0-100) (P < .001). When the PBK ratio was plotted against interdevice interval, R(2) was 0.47 (P < .001). When analyzed for hearing stability, those with a progressive loss history demonstrated less influence of prolonged interdevice interval on performance. Multivariate analysis did not identify other factors influencing the ratio. A line of best fit for those with stable hearing loss suggested best outcomes were with an interdevice interval less than 3 to 4 years. Beyond 7 to 8 years, very few achieved useful speech recognition from the second CI. CONCLUSIONS: Where possible, the second implant should be received within 3 to 4 years of the first to maximize outcome in those with stable, severe to profound sensorineural hearing loss. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:2389-2394, 2016.

A Multidisciplinary Children's Airway Center: Impact on the Care of Patients With Tracheostomy.

BACKGROUND: Children with complex airway problems see multiple specialists. To improve outcomes and coordinate care, we developed a multidisciplinary Children's Airway Center. For children with tracheostomies, aspects of care targeted for improvement included optimizing initial hospital discharge, promoting effective communication between providers and caregivers, and avoiding tracheostomy complications. METHODS: The population includes children up to 21 years old with tracheostomies. The airway center team includes providers from pediatric pulmonology, pediatric otolaryngology/head and neck surgery, and pediatric gastroenterology. Improvement initiatives included enhanced educational strategies, weekly care conferences, institutional consensus guidelines and care plans, personalized clinic schedules, and standardized intervals between airway examinations. A patient database allowed for tracking outcomes over time. RESULTS: We initially identified 173 airway center patients including 123 with tracheostomies. The median number of new patients evaluated by the center team each year was 172. Median hospitalization after tracheostomy decreased from 37 days to 26 days for new tracheostomy patients <1 year old discharged from the hospital. A median of 24 care plans was evaluated at weekly conferences. Consensus protocol adherence increased likelihood of successful decannulation from 68% to 86% of attempts. The median interval of 8 months between airway examinations aligned with published recommendations. CONCLUSIONS: For children with tracheostomies, our Children's Airway Center met and sustained goals of optimizing hospitalization, promoting communication, and avoiding tracheostomy complications by initiating targeted improvements in a multidisciplinary team setting. A multidisciplinary approach to management of these patients can yield measurable improvements in important outcomes.

Connexin-related (DFNB1) hearing loss: is routine computed tomography imaging necessary?

OBJECTIVES: Determine if routine computed tomography (CT) imaging is necessary in the workup for children with connexin-related (DFNB1) sensorineural hearing loss (SNHL). STUDY DESIGN: Case-control retrospective chart and imaging review. SETTING: Tertiary care otolaryngology practice. SUBJECTS AND METHODS: High-resolution temporal bone CT scans of children (n = 21) with DFNB1 SNHL were compared to age-matched controls with either conductive hearing loss (CHL, n = 33) or a nonsyndromic, non-DFNB1 SNHL (n = 33). Sixteen measurements of cochleo-vestibular structures were recorded. Statistical analysis was performed using a repeated analysis of variance model that controlled for both age and gender. Area under the curve (AUC) and multidimensional AUC (MAUC) analyses were also performed. RESULTS: Overall, no statistically significant differences were found between the 3 experimental groups. In addition, comparisons between the DFNB1 and CHL groups, DFNB1 and non-DFNB1 SNHL groups, and CHL and non-DFNB1 SNHL groups failed to demonstrate any statistically significant differences. AUC and MAUC analyses also failed to detect any significant differences between the 3 groups. CONCLUSIONS: Patients with DFNB1 SNHL do not have significant anatomic differences on temporal bone CT scans when compared to non-DFNB1 SNHL and CHL control groups. Based on the above analysis, it is reasonable to avoid routine CT imaging of the temporal bones in children with known DFNB1 SNHL unless otherwise clinically indicated.

Comprehensive quality of life outcomes for pediatric patients undergoing endoscopic sinus surgery.

BACKGROUND: Limited quality of life data exist for pediatric chronic rhinosinusitis (CRS) patients undergoing endoscopic sinus surgery (ESS). Further exploration of the following areas will enhance understanding and support clinical decision-making: baseline and post-ESS general and disease-specific quality of life, parent vs. child report, and correlation of nasal endoscopy to sinus CT scores. METHODOLOGY: A prospective cohort study evaluated CRS patients age 5-18 undergoing ESS. Surveys were completed at two timepoints: (1) pre-ESS and (2) 30-90 days post-ESS, with parents completing general (PedsQLTM) and CRS-specific (SNOT-16 and SN-5) quality of life surveys and children completing PedsQLTM and SNOT-16 surveys. Preoperative Lund-Kennedy nasal endoscopy and Lund-Mackay sinus CT scores were calculated. Where appropriate, outcomes were stratified by cystic fibrosis status. RESULTS: Impaired preoperative general quality of life was evidenced by parent proxy-report of PedsQLTM scores in 10 cystic fibrosis and 11 non-CF patients. ESS was associated with decreased sinus symptoms at 1-3 months postoperatively with SN-5 change scores of -1.85 and -2.2, in CF and non-CF patients, respectively. Parents reported worse CRS symptoms via higher preoperative SNOT-16 scores than their children did. Nasal endoscopy and sinus CT scores correlated with a Spearman correlation coefficient of 0.51. Scores not reaching statistical significance included CF-related CRS SNOT-16 change scores and PedsQLTM general quality of life change scores. CONCLUSION: In pediatric patients with CRS electing ESS, general quality of life is impaired preoperatively and sinus symptoms improve significantly 1-3 months after sinus surgery. Parents report statistically worse CRS symptom scores than their children do. Nasal endoscopy scores in this cohort correlated with sinus CT scores.

Statistical atlas construction via weighted functional boxplots.

Atlas-building from population data is widely used in medical imaging. However, the emphasis of atlas-building approaches is typically to estimate a spatial alignment to compute a mean/median shape or image based on population data. In this work, we focus on the statistical characterization of the population data, once spatial alignment has been achieved. We introduce and propose the use of the weighted functional boxplot. This allows the generalization of concepts such as the median, percentiles, or outliers to spaces where the data objects are functions, shapes, or images, and allows spatio-temporal atlas-building based on kernel regression. In our experiments, we demonstrate the utility of the approach to construct statistical atlases for pediatric upper airways and corpora callosa revealing their growth patterns. We also define a score system based on the pediatric airway atlas to quantitatively measure the severity of subglottic stenosis (SGS) in the airway. This scoring allows the classification of pre- and post-surgery SGS subjects and radiographically normal controls. Experimental results show the utility of atlas information to assess the effect of airway surgery in children.

Comparison of endoscopic versus 3D CT derived airway measurements.

OBJECTIVES/HYPOTHESIS: To understand: 1) how endoscopic airway measurements compare to three-dimensional (3D) CT derived measurements; 2) where each technique is potentially useful; and 3) where each has limitations. STUDY DESIGN: Compare airway diameters and cross-sectional areas from endoscopic images and CT derived 3D reconstructions. METHODS: Videobronchoscopy was performed and recorded on an adult-sized commercially available airway mannequin. At various levels, cross-sectional areas were measured from still video frames using a referent placed via the biopsy port. A 3D reconstruction was generated from a high resolution CT of the mannequin; planar sections were cut at similar cross-sectional levels; and cross-sectional areas were obtained. RESULTS: At three levels of mechanically generated tracheal stricture, the differences between the endoscopic measurement and CT-derived cross-sectional area were 1%, 0%, and 7% (1.8, 0.8, and 14 mm²). At the vocal folds, the difference was 9% (7.8 mm²). The tip of the epiglottis and width of the epiglottis differed by 27% and 10% (18.73 mm², 0.40 mm). The airway measurements at the base of tongue, minimal cross-sectional area of the pharynx, and choana differed by 26%, 36%, and 30% (101.40 mm², 36.67 mm², 122.71 mm²). CONCLUSIONS: Endoscopy is an effective tool for obtaining airway measurements compared with 3D reconstructions derived from CT. Concordance is best in geometrically simple areas where the entire cross-section measured is visible within one field of view (trachea, round; vocal folds, triangular) versus geometrically complex areas that encompass more than one field of view (i.e. pharynx, choana).

Cochlear implantation in unique pediatric populations.

PURPOSE OF REVIEW: Over the last decade, the selection criteria for cochlear implantation have expanded to include children with special auditory, otologic, and medical problems. Included within this expanded group of candidates are those children with auditory neuropathy spectrum disorder, cochleovestibular malformations, cochlear nerve deficiency, associated syndromes, as well as multiple medical and developmental disorders. Definitive indications for cochlear implantation in these unique pediatric populations are in evolution. This review will provide an overview of managing and habilitating hearing loss within these populations with specific focus on cochlear implantation as a treatment option. RECENT FINDINGS: Cochlear implants have been successfully implanted in children within unique populations with variable results. Evaluation for cochlear implant candidacy includes the core components of a full medical, audiologic, and speech and language evaluations. When considering candidacy in these children, additional aspects to consider include disorder-specific surgical considerations and child/caregiver counseling regarding reasonable postimplantation outcome expectations. SUMMARY: Cochlear implants are accepted as the standard of care for improving hearing and speech development in children with severe-to-profound hearing loss. However, children with sensorineural hearing loss who meet established audiologic criteria for cochlear implantation may have unique audiologic, medical, and anatomic characteristics that necessitate special consideration regarding cochlear implantation candidacy and outcome. Individualized preoperative candidacy and counseling, surgical evaluation, and reasonable postoperative outcome expectations should be taken into account in the management of these children.

Influence of an intraoperative perilymph gusher on cochlear implant performance in children with labyrinthine malformations.

OBJECTIVES: To assess the effect of an intraoperative perilymph fluid gusher during cochlear implantation on speech perception abilities in pediatric patients with labyrinthine anomalies. SETTING: Tertiary care academic referral center. METHODS: Seventy subjects with labyrinthine malformations who received a cochlear implant were identified in our pediatric cochlear implant database. In 30 cases, an intraoperative perilymph fluid gusher was encountered during surgery. Fifteen children with GJB2 positive hereditary hearing loss served as controls. Multiple speech perception measures were obtained with the cochlear implant. The best score for each subject over time was determined as a speech reception index in quiet. This index was compared among groups and malformation types. RESULTS: The speech reception index in quiet demonstrated overall good performance scores of cochlear implantation in children with incomplete partitioning/enlarged vestibular aqueduct type malformations. Children with hypoplastic malformations, on the other hand, showed variable outcomes with many children demonstrating only limited long-term speech discrimination abilities. The presence or absence of a perilymph gusher did not significantly influence results after cochlear implantation. CONCLUSION: This report documents the variable outcomes of pediatric cochlear implantation in children with inner ear malformations. More importantly, anatomic parameters, such as the classification of the anomaly and the presence of a viable cochlear nerve, seem to influence performance measures substantially. The presence of a perilymph gusher did not influence outcomes in both hypoplastic and incomplete partitioning/enlarged vestibular aqueduct type malformations. LEVEL OF EVIDENCE: 2b Individual retrospective cohort study.

Pediatric airway abnormalities: evaluation and management.

Sleep disordered breathing syndromes in pediatric patients can lead to adverse effects in the cardiovascular system, neurocognitive function, growth, and behavior. These syndromes occur more frequently in patients with craniofacial disorders. A high index of suspicion as well as early recognition, detection, and treatment of these syndromes are considered integral to care of children with craniofacial disorders.