Validation of a PHIS Esophageal Atresia and Tracheoesophageal Fistula Cohort in ICD-10.

OBJECTIVE: Validation of a contemporary International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) congenital esophageal atresia/tracheoesophageal atresia (EA/TEF) cohort in the Pediatric Health Information System (PHIS) database. STUDY DESIGN: Database study, validation. SETTING: Tertiary care center. METHODS: Search methods used to validate an ICD-9-CM EA/TEF cohort in PHIS were modified for ICD-10-CM. A retrospectively and prospectively maintained clinical database at a single high-volume EA/TEF center was used for comparison. Patients treated between October 1, 2015 and July 31, 2022 were included. Searches progressively narrowed the cohort by ICD-10-CM diagnosis codes, expansion to include incorrectly coded as 'iatrogenic, age less than 30 days, and use of at least 1 ICD-10-CM procedure code. Results of PHIS data and institution data were compared for accuracy. RESULTS: The most refined search of PHIS and the EA/TEF clinical database yielded 93 and 84 patients, respectively. The sensitivity was 99% and positive predictive value was 94%. A PHIS search using these methods and encompassing 49 children's hospitals yielded an EA/TEF cohort of 2479 patients. CONCLUSION: We present a validated search method in the PHIS database to identify a high-fidelity cohort of EA/TEF patients for multi-institutional study. We have demonstrated that a carefully maintained clinical database may be used to validate cohorts in PHIS. This cohort allows for improved practice variability and outcomes study of EA/TEF patients. Similar methods may be employed to generate other rare disease cohorts in PHIS. LEVEL OF EVIDENCE: Level 4.

Lipid-Laden Macrophage Index as a Diagnostic Tool for Pediatric Aspiration: A Systematic Review.

Objective: Lipid-laden macrophage index (LLMI) has been proposed as a marker for aspiration on bronchoalveolar lavage. It has also been studied as a marker for gastroesophageal reflux and other pulmonary diseases. This review aims to determine the clinical correlation between LLMI and pediatric aspiration. Data Sources: PubMed (MeSH search), Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) portals through December 17th, 2020. Review Methods: Preferred Reporting Items for Systematic Review and Meta-Analysis criteria were followed, and a quality assessment of included studies was performed using the Methodological Index for Non-Randomized Studies. Search criteria included all occurrences in the title or abstract of the terms "pulmonary aspiration" and "alveolar macrophages." Results: Five studies describing 720 patients met inclusion, 3 retrospective case-control studies, and 2 prospective observational studies. Four studies suggested a link between elevated LLMI and aspiration, and 1 found no association. Control groups varied and included healthy nonaspirators to nonaspirators with other pulmonary diseases. Diagnosis of aspiration was not standardized across the studies. Three papers proposed cutoff values for LLMI, all different. Conclusion: The existing literature indicates that LLMI is not a sensitive or specific marker for aspiration. Further study is needed to define the utility of LLMI in pediatric aspiration.

Screening for Vocal Fold Movement Impairment in Children Undergoing Esophageal and Airway Surgery.

INTRODUCTION: Children undergoing cervical and/or thoracic operations are at risk for recurrent laryngeal nerve injury, resulting in vocal fold movement impairment (VFMI). Screening for VFMI is often reserved for symptomatic patients. OBJECTIVE: Identify the prevalence of VFMI in screened preoperative patients prior to an at-risk operation to evaluate the value of screening all patients at-risk for VFMI, regardless of symptoms. METHODS: A single center, retrospective review of all patients undergoing a preoperative flexible nasolaryngoscopy between 2017 and 2021, examining the presence of VFMI and associated symptoms. RESULTS: We evaluated 297 patients with a median (IQR) age of 18 (7.8, 56.3) months and a weight of 11.3 (7.8, 17.7) kilograms. Most had a history of esophageal atresia (EA, 60%), and a prior at-risk cervical or thoracic operation (73%). Overall, 72 (24%) patients presented with VFMI (51% left, 26% right, and 22% bilateral). Of patients with VFMI, 47% did not exhibit the classic symptoms (stridor, dysphonia, and aspiration) of VFMI. Dysphonia was the most prevalent classic VFMI symptom, yet only present in 18 (25%) patients. Patients presenting with a history of at-risk surgery (OR 2.3, 95%CI 1.1, 4.8, p = 0.03), presence of a tracheostomy (OR 3.1, 95%CI 1.0, 10.0, p = 0.04), or presence of a surgical feeding tube (OR 3.1, 95%CI 1.6, 6.2, p = 0.001) were more likely to present with VFMI. CONCLUSION: Routine screening for VFMI should be considered in all at-risk patients, regardless of symptoms or prior operations, particularly in those with a history of an at-risk surgery, presence of tracheostomy, or a surgical feeding tube. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3564-3570, 2023.

Comparative Effectiveness of Recurrent Laryngeal Nerve Monitoring Techniques in Pediatric Surgery.

OBJECTIVES/HYPOTHESIS: The recurrent laryngeal nerve (RLN) is at risk during pediatric cervical, thoracic, and cardiac surgery. We aim to determine the feasibility and effectiveness of RLN monitoring techniques in all pediatric patients. STUDY DESIGN: Retrospective case series. METHODS: Retrospective review of patients/procedures with RLN(s) at risk and RLN monitoring at Boston Children's Hospital July 2019-October 2020. PRIMARY OUTCOMES: pre/postoperative vocal fold mobility by awake flexible fiberoptic laryngoscopy (FFL). RESULTS: One hundred one patients (median [interquartile range, IQR] age 14.6 months [4.6-49.7 months], weight 10 kg [5.2-16.2 kg]) underwent 122 procedures with RLN(s) at risk. RLN monitoring attempted 111 cases, successful 96 (84%). Surgical indications: esophageal atresia/tracheoesophageal fistula, and tracheobronchomalacia. Sixty-two (56%) procedures in reoperative field. Median follow-up 112 days (IQR 41-230). Pre/postoperative FFL performed 84 procedures (69%), 19 new postoperative RLN injuries (16%), median age 12 months, reoperative fields 11 (18%). Prass probes: 34 cases (28 successful, 82%), 6 injuries (18%), age 12.2 (5.8-23.6) months. Dragonfly electrodes: 45 cases (37 successful, 82%), 8 injuries (18%), age 7.5 (3.8-19) months. Nerve integrity monitoring (NIM) integrated electrode endotracheal tube: 33 cases (33 successful, 100%), 5 injuries (15%), age 90 (58.8-136.7) months. Automatic periodic stimulation (APS): 16 cases, 13 successful (81%), four injuries (25%), age 7.2 (5.3-20.6) months. NIM RLN monitoring is significantly more successful than Prass, Dragonfly (95%CI -0.3 to 0.02, P = .02; and 95%CI 0.05-0.31, P = .008). Rates of injury are not different between types of RLN monitoring (P = .94), with APS use (P = .47), or with monitoring success (95%CI -0.36 to 0.09, P = .28). CONCLUSIONS: RLN monitoring is feasible in pediatric patients of all ages. Although NIM type RLN monitoring success is superior, all forms offer similar rates of nerve protection. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:889-894, 2022.

Effect of Posterior Tracheopexy on Risk of Recurrence in Children after Recurrent Tracheo-Esophageal Fistula Repair.

BACKGROUND: A recurrent tracheo-esophageal fistula can complicate esophageal atresia and tracheo-esophageal fistula (TEF) repair in children. Therapeutic approaches and the rate of recurrence vary widely. Most reports are limited by small cohorts and short-term follow-up, and rates of re-recurrence are substantial, making it difficult to select the treatment of choice. We aimed to review our experience with the treatment of recurrent TEF using posterior tracheopexy, focusing on operative risks and long-term outcomes. STUDY DESIGN: We conducted a retrospective review of patients with esophageal atresia TEF with recurrent TEF treated at 2 institutions from 2011 to 2020. We approach recurrent TEFs surgically. Once the TEF is divided and repaired, the membranous trachea is sutured to the anterior longitudinal ligament of the spine (posterior tracheopexy) and the esophagus is rotated into the right chest (rotational esophagoplasty), separating the suture lines widely. To detect re-recurrence, patients undergo endoscopic surveillance during follow-up. RESULTS: Sixty-two patients with a recurrent TEF were surgically treated (posterior tracheopexy/rotational esophagoplasty) at a median age of 14 months. All had significant respiratory symptoms. On referral, 24 had earlier failed endoscopic and/or surgical attempts at repair. Twenty-nine required a concomitant esophageal anastomotic stricturoplasty or stricture resection. Postoperative morbidity included 3 esophageal leaks, and 1 transient vocal cord dysfunction. We have identified no recurrences, with a median follow-up of 2.5 years, and all symptoms have resolved. CONCLUSIONS: The surgical treatment of recurrent TEFs that incorporates a posterior tracheopexy and rotational esophagoplasty is highly effective for preventing re-recurrence with low perioperative morbidity.

Intraoperative Recurrent Laryngeal Nerve Monitoring During Pediatric Cardiac and Thoracic Surgery: A Mini Review.

Objective: Review techniques for intraoperative recurrent laryngeal nerve (RLN) monitoring during pediatric surgery for esophageal atresia, tracheoesophageal fistula, tracheobronchomalacia, and cardiac surgery. Summary Background Data: Literature was reviewed for reports of intraoperative recurrent laryngeal nerve monitoring in cervical, thoracic, and cardiac surgical procedures which place the RLNs at risk for injury. Methods: Review paper. Results: The RLN is at risk during pediatric surgery for esophageal atresia, tracheoesophageal fistula, tracheobronchomalacia, and cardiac surgery. Intraoperative nerve monitoring has decreased rates of RLN injury in thyroid surgery. Intraoperative RLN monitoring techniques appropriate for pediatric surgery are discussed, including endotracheal tubes with integrated surface electrodes, adhesive surface electrodes for smaller endotracheal tubes, endolaryngeal electrodes, and automatic periodic continuous intra-operative stimulation. Conclusions: Multiple techniques exist to monitor the RLN in children undergoing cervical, cardiac, and thoracic surgery. Monitoring the RLN during procedures that place the RLNs at risk may help decrease the rate of RLN injury.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Management of suprastomal collapse in the pediatric population.

INTRODUCTION: Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation. OBJECTIVE: Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy. METHODS: Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method. RESULTS: Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: CONCLUSION: This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.

Evaluating gender parity in operative experience for otolaryngology residencies in the United States.

OBJECTIVES: Gender disparity exists in medicine, such as differences in pay and promotion opportunities. We hypothesize that there is also a gender difference in graduate medical education as manifested by operative case volume. This study compares surgical case volume by gender for graduating US otolaryngology residents. STUDY DESIGN: Cohort study. METHODS: With data use approval from the Accreditation Council for Graduate Medical Education, we evaluated the key indicator case log summaries of graduating otolaryngology residents from 2009-2017. Mean and standard deviation were used for all cases, and t-tests were used to compare cases by resident gender. The Bonferroni method was used to adjust for multiple comparisons across years. RESULTS: Data from 1740 male and 804 female residents were evaluated. Across all years, the average number of key indicator cases reported was 778.8 and 813.6 by female and male residents, respectively, with an average difference of 34.8 cases per graduating year (95% confidence interval [CI] 19.4, 50.2; P < .001). When a resident self-reported the role of resident surgeon/supervisor, the average number of key indicator cases reported was 602.6 and 643.9 by female and male residents, respectively, with an average difference of 41.3 cases per graduating year (95% CI, 28.0, 54.6; P < .001). CONCLUSION: Gender-based discrepancies in surgical case volume exist among graduating otolaryngology residents. This disparity is partially attributed to the self-reported role in the surgery. This study has identified those discrepancies so that training programs can implement strategies to ensure improved gender parity. LEVEL OF EVIDENCE: 2b Laryngoscope, 130:1651-1656, 2020.

Laryngeal Web in the Pediatric Population: Evaluation and Management.

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

Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.

BACKGROUND: Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. METHODS: A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. RESULTS: We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. CONCLUSION: In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.

Otolaryngology boot camps: Current landscape and future directions.

OBJECTIVES: Simulation-based boot camps have gained popularity over the past few years, with some surgical specialties implementing mandatory national boot camps. However, there is no consensus in otolaryngology on boot camp timing, learner level, or curriculum. The purpose of this study is to examine the current landscape and gather opinions regarding future curriculum and standardization of boot camps in otolaryngology. METHODS: A survey was developed to examine current resident participation and boot camp content while also seeking opinions regarding improving boot camp enrollment and standardizing curriculum. A cross-sectional survey of all otolaryngology residency program directors in the United States and Puerto Rico was performed via SurveyMonkey. Responses were collected anonymously, and results were analyzed by descriptive statistical analysis. RESULTS: Of the 45% (48 of 106) who responded, 76.6% reported their residents participate in boot camps. The most common skills taught were basic suturing and airway management skills. The majority (95%) was likely to send residents to a local boot camp, with 56% favoring early postgraduate year (PGY)-1 participation and 42% favoring a 1-day boot camp. Subsidized expenses, improved regional access, and supplementary boot camp information would help the program director in their decision to send residents to boot camp. Only 32% felt boot camps should be standardized, and 27% felt they should be mandatory. CONCLUSION: Many otolaryngology residency programs participate in boot camps. Additional data on the benefits of boot camps, improved access, and reduced financial burden may improve participation. Further discussion of ideal timing, PGY level, and standardized curriculum should occur in conjunction with the otolaryngology academic societies and oversight from accreditation and certifying bodies. LEVEL OF EVIDENCE: NA Laryngoscope, 129:2707-2712, 2019.

The Impact of Timing of Tympanostomy Tube Placement on Sequelae in Children With Cleft Palate.

OBJECTIVE: To describe the impact of timing of tympanostomy tube insertion on the number of tubes received and complications in children with routine tube placement. DESIGN: Retrospective case series. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from a consecutive sample of 401 children with cleft palate were reviewed. Sixty-five patients with isolated cleft palate and 82 patients with cleft lip and palate had follow-up until 5 years of age and were included. INTERVENTIONS: Tympanostomy tubes. MAIN OUTCOME MEASURE(S): Number of tubes received and tube-related complications. The hypothesis was formulated prior to data collection. RESULTS: Males comprised 55.8% of included patients, and tubes were placed in 98.6% of patients at a median age of 6.5 months. Effusion was documented at first tube placement for 96.5% of patients. Most (67.4%) patients required replacement of tubes, and 10.6% required long-term tubes. Complications included otorrhea (71.0%), myringosclerosis (35.2%), granulation (22.8%), perforation (17.9%), retained tubes (5.5%), and cholesteatoma (1.4%). Cleft lip and palate (P < .001) and otorrhea (P = .023) were associated with tube placement before palatoplasty. Patients with tube placement before palatoplasty (P = .033), genetic disorders (P = .007), failed newborn hearing screen (P = .012), otorrhea (P < .001), and granulation (P < .001) received more tubes. CONCLUSIONS: Nearly universal effusion in patients with cleft palate supports the need for routine tube placement. The potential for otorrhea and requiring more tubes should be weighed against the risks associated with prolonged effusion when considering tube placement before palatoplasty.

Should Children With Cleft Palate Receive Early Long-Term Tympanostomy Tubes: One Institution's Experience.

OBJECTIVES: To determine whether children with cleft palate might benefit from early long-term tympanostomy tubes with the hypothesis that receiving multiple tubes is associated with shorter duration of first tubes. DESIGN: Retrospective cohort study. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from 401 consecutive children with cleft palate ± cleft lip, born April 2005 to April 2010, were reviewed. After exclusion of children with cleft repair at an outside hospital, no follow-up after 5 years of age, intact secondary palate, no tubes, or tube replacement at palatoplasty, 105 children remained. MAIN OUTCOME MEASURE: Number of tubes. RESULTS: Armstrong grommet tubes were placed at a median age of 6.7 months (range 2.3-19.6 months). Tubes were replaced in 55.3% of patients, with 34.0% receiving ≥3 sets. Duration of first tubes was significantly longer for children with 1 set of tubes compared with those with multiple sets (median 26 vs 19 months, P = .004). Otorrhea, but not perforation, was associated with longer duration of first tubes (median 27 vs 20.5 months, P = .028). Cleft type did not impact the proportion of patients with multiple tubes. Median age at last tube placement for children with multiple tubes was 5.0 years (range 1.9-8.7 years). CONCLUSION: Short duration of first tubes is associated with receiving multiple tubes. Because most patients require repeat tubes and many require tubes until school age, there is a significant need for controlled, prospective trials of early long-term tube placement in this population.

The Impact of Tympanostomy Tubes on Speech and Language Development in Children with Cleft Palate.

Objective Describe the impact of hearing loss, tympanostomy tube placement before palatoplasty, and number of tubes received on speech outcomes in children with cleft palate. Study Design Case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods Records from 737 children born between April 2005 and April 2015 who underwent palatoplasty at a tertiary children's hospital were reviewed. Exclusion criteria were cleft repair at an outside hospital, intact secondary palate, absence of postpalatoplasty speech evaluation, sensorineural or mixed hearing loss, no tubes, first tubes after palatoplasty, or first clinic after 12 months of age. Data from 152 patients with isolated cleft palate and 166 patients with cleft lip and palate were analyzed using Wilcoxon rank-sum, χ2, and Fisher exact test and logistic regression. Results Most patients (242, 76.1%) received tubes before palatoplasty. Hearing loss after tubes, but not before, was associated with speech/language delays at 24 months ( P = .005) and language delays ( P = .048) and speech sound production disorders (SSPDs, P = .040) at 5 years. Receiving tubes before palatoplasty was associated with failed newborn hearing screen ( P = .001) and younger age at first posttubes type B tympanogram with normal canal volume ( P = .015). Hearing loss after tubes ( P = .021), language delays ( P = .025), SSPDs ( P = .003), and velopharyngeal insufficiency ( P = .032) at 5 years and speech surgery ( P = .022) were associated with more tubes. Conclusion Continued middle ear disease, reflected by hearing loss and multiple tubes, may impair speech and language development. Inserting tubes before palatoplasty did not mitigate these impairments better than later tube placement.