Recalcitrant Stenosis Following Pediatric LTR: Contributing Factors and Clinical Impact.

OBJECTIVES: Laryngotracheal reconstruction (LTR) has revolutionized the management of pediatric subglottic stenosis (SGS). However, postoperative stenosis remains a difficult hurdle to overcome. Our goal is to determine the clinical impact of recalcitrant stenosis after LTR and the factors contributing to postoperative stenosis. STUDY DESIGN: Retrospective review of 181 open LTR patients between 2008 and 2021. SETTING: Stand-alone tertiary children's hospital. METHODS: Recalcitrant stenosis was defined as new or worsening stenosis despite open LTR. Fisher's exact and Mann-Whitney tests were used to detect differences in categorical and continuous clinical data between patients with and without treatment-resistant stenosis. Time-to-decannulation analysis of both groups was performed using Kaplan-Meier analysis and evaluated with log-rank and Cox proportional hazards regression. Multivariate logistical regression was used to assess the validity of associations found in univariate analysis. RESULTS: As expected, the 27 patients with postoperative stenosis were less likely to be decannulated (P < .001, Fisher's Exact), more likely to require a postoperative tracheostomy (P < .001, Fisher's Exact) or revision LTR (P < .001, Fisher's Exact) and had prolonged time to decannulation (P < .001, Log-rank). Children with Grade IV SGS (P = .004, Fisher's Exact), and those with longer suprastomal stent duration (P = .03, Fisher's Exact) were more likely to suffer from recalcitrant stenosis. Stent duration longer than 4 weeks (P = .01) contributed to refractory stenosis when controlling for all aforementioned variables using multivariable logistic regression. Interposition grafts had a protective effect (P = .005). CONCLUSION: Maintaining suprastomal stents over 4 weeks after LTR increases the risk for postoperative stenosis and its sequelae.

Documentation in Pediatric Microlaryngoscopy/Bronchoscopy: International Modified Delphi Consensus.

BACKGROUND: Complete and accurate documentation of surgical procedures is essential for optimizing patient care, yet significant variation in operative notes persists within and across institutions. We sought to reach consensus on the most important components of an operative note for pediatric microlaryngoscopy and bronchoscopy. METHODS: A modified Delphi consensus process was used. A checklist for operative documentation, created by fellowship-trained pediatric otolaryngologists-head and neck surgeons, was sent to surgeons identified as experts in pediatric laryngoscopy and bronchoscopy. In the first round, items were rated as "keep" or "remove". In the second round, each item was rated on a 7-point Likert scale for importance. The mean score of each item was calculated to determine if consensus was reached. RESULTS: Overall, 43/74 (58.1%) surgeons responded to our survey. After two rounds of editing, 28 components reached consensus, 24 were near consensus, and 26 did not reach consensus. Items that reached final consensus had mean (SD) ratings of 6.12 (0.94) (range, 5.31-6.72). CONCLUSION: Pediatric otolaryngologists identified as bronchoscopy experts were able to create a checklist of essential components of an operative note for pediatric laryngoscopy and bronchoscopy using a Delphi method. Items reaching consensus included procedure name, description of breathing, grade of airway view, description of normal anatomic structures, grade of subglottic stenosis if present, presence and description of tracheobronchomalacia, presence of fistulae, cleft and rings, and several special cases including foreign body and tracheostomy management, as well as end of procedure disposition and complications. LEVEL OF EVIDENCE: 5 Laryngoscope, 133:1234-1238, 2023.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of non-tuberculous mycobacterial cervicofacial lymphadenitis.

INTRODUCTION: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. OBJECTIVES: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. METHODS: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. CONCLUSION: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation.

Outcomes of balloon dilation in pediatric subglottic stenosis.

OBJECTIVES: We report outcomes of balloon dilation in the endoscopic management of pediatric subglottic stenosis (SGS) and discuss the role of balloon dilation in both primary and adjuvant therapy. METHODS: We performed a retrospective review of treatment with noncompliant, high-pressure balloons for SGS in the past 2 years at a tertiary pediatric hospital. Fifty-one dilations were performed in 28 children with SGS. The children's mean age was 42 months. The mean SGS grade was 2.46. RESULTS: Fifteen children had primary balloon dilation, and 13 had adjuvant balloon dilation. Overall, 16 children (57.1%) had successful balloon dilation. Of those who underwent primary dilation, 9 (60.0%) were able to avoid open reconstruction or tracheotomy and 6 had their symptoms temporarily improved (average, 36 days) until definitive open reconstruction. Of the patients who underwent adjuvant dilation, 7 (53.8%) were successfully decannulated. Nine of the 12 failed balloon dilations were in children who had concomitant airway disorders; in contrast, only 6 of 16 children in whom treatment was successful had concomitant airway disorders (p = 0.048). There was no statistical association between successful versus failed treatment and age (51.6 versus 27.9 months; p = 0.23), degree of stenosis (grade 2.3 versus grade 2.6; p = 0.41), presence of lung disease (33.3% versus 70%; p = 0.07), or soft versus firm stenosis (60.0% versus 53.1%; p = 0.71). CONCLUSIONS: Balloon dilation plays an important role in the primary and adjuvant management of pediatric SGS. The presence of concomitant airway lesions is significantly associated with failure of balloon dilation treatment. Meticulous surveillance of the dilated airway is necessary, given this failure rate.

"Natural" decannulation in patients with Robin sequence and severe airway obstruction.

OBJECTIVES: Airway management in infants with Robin sequence (RS) remains controversial, ranging from conservative to operative methods. In some centers, tracheostomy remains the mainstay for those infants with severe respiratory obstruction. The goals of this retrospective case cohort study were to determine the length of time to decannulation without further surgical intervention (ie, "natural" decannulation) in patients with severe RS who underwent tracheostomy and to investigate potential factors associated with successful decannulation. METHODS: We reviewed all infants with RS treated at a large tertiary center from 1994 to 2010. Patients who had undergone tracheostomy were identified. Baseline demographics, complications, deaths, and time to "natural" decannulation were recorded. Statistical analysis was performed with univariate analysis and Kaplan-Meier log-rank tests. RESULTS: Of 61 infants with RS with obstructive events, 25 infants (14 with isolated RS and 11 with syndromic RS) required tracheostomy. At a mean follow-up of 4 years, the rate of tracheostomy-specific complications was 52%; the tracheostomy-specific mortality rate was 12%. Overall, 13 of 25 infants (52%) were "naturally" decannulated, with a median time to decannulation of 97 months. Patients with syndromic RS had a significantly longer median time to decannulation than did those with isolated RS (more than 73 months versus 19 months, respectively; p = 0.019). In addition, patients with long-term tracheostomy dependence had significantly higher maximum carbon dioxide levels before tracheostomy than did patients who were successfully decannulated (82.4 versus 63.2 mEq/L, respectively; p = 0.02). CONCLUSIONS: Tracheostomy in infants with RS is associated with inordinately high rates of mortality, morbidity, and long-term tracheostomy dependence, particularly in patients with syndromic RS and in those with high maximum carbon dioxide levels before tracheostomy.

Stridor in the Infant Patient.

Stridor is a high-pitched extrathoracic noise associated with turbulent airflow, commonly associated with respiratory distress in infants. Workup for stridor requires evaluation of the upper-respiratory airway, with severe distress requiring evaluation under anesthesia. The differential diagnosis of stridor depends on location of the obstruction, age of the patient, and acuity of the symptoms. The most common reason is laryngomalacia; most patients can be managed conservatively with resolution of symptoms by 2 years of age. In children who do not improve or have severe disease, supraglottoplasty is the treatment of choice, and the majority will have resolution of stridor postoperatively.

Presenting Symptom as a Predictor of Clinical course in Juvenile Onset Recurrent Respiratory Papillomatosis.

OBJECTIVE/HYPOTHESIS: Determine if the presentation of voice versus airway symptoms at the time of diagnosis of juvenile recurrent respiratory papillomatosis (JORRP) correlates with treatment and outcomes. STUDY DESIGN: Original report, Retrospective review. METHODS: Twenty year retrospective review of all patients with JORRP presenting at any time in treatment to a single tertiary children's hospital between 1997 and 2017. RESULTS: Fifty-four patients presented with a diagnosis of JORRP during the 20 year study period. Of these, 32 were female and 22 were male. African American children made up the majority of the patients (65%), with 19% identifying as Caucasian, 9% Hispanic, and 7% Asian. Fifteen of the children presented initially to the ENT clinic (87% with hoarseness), 12 to the emergency department (92% with airway concerns), 11 to the voice clinic, and the remaining 12 at outside hospitals or clinics and care were transferred. Voice symptoms, namely hoarseness, was the presenting symptom in 31 (57%), airway symptoms, namely respiratory distress or stridor was predominate in 17 (32%), and the presenting symptom was unknown in six cases (11%). Children presenting with airway symptoms were younger at presentation (median 2.1 years, range 0.38-8.77 years) than those presenting with voice symptoms (median 6.7 years, range 0.98-15.13 years), and after adjusting for age underwent a greater number of surgeries in the first year to control disease. CONCLUSIONS: Patients who present with airway symptoms present younger and have an increased number of surgeries in the first year compared to those presenting with voice complaints. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1670-1675, 2021.

Barriers to Decannulation After Double-Stage Laryngotracheal Reconstruction.

OBJECTIVES/HYPOTHESIS: To identify any potential barriers for decannulation in children undergoing double-staged laryngotracheal reconstruction (dsLTR) beyond the severity of disease itself. STUDY DESIGN: Case series with chart review. METHODS: We performed a retrospective chart review from 2008 to 2018 of 41 children who had undergone dsLTR as primary treatment for laryngotracheal stenosis at a stand-alone tertiary children's hospital. We examined the effect of demographic, medical, and surgical factors on successful decannulation and time to decannulation after dsLTR. RESULTS: Of the 41 children meeting inclusion criteria who underwent dsLTR, 34 (82%) were decannulated. Age, gender, race, insurance status, medical comorbidity, and multilevel stenosis did not predict overall decannulation. Insurance status did not impact time to decannulation (P = .13, Log-rank). Factors that increased length of time to decannulation were the use of anterior and posterior cartilage grafts (P = .001, Log-rank), history of pulmonary disease (P = .05, Log rank), history of cardiac disease (P = .017, Log-rank), and race/ethnicity (P = .001 Log-rank). CONCLUSION: In a cohort with a similar decannulation rates to previous dsLTR cohorts, we identified no demographic or medical factors that influenced overall decannulation. We did observe that pulmonary comorbidity, cardiac comorbidity, and race/ethnicity lengthens time to decannulation. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2141-2147, 2021.

International Pediatric Otolaryngology Group (IPOG) survey: Efforts to avoid complications in home tracheostomy care.

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

International Pediatric Otolaryngology Group (IPOG) management recommendations: Pediatric tracheostomy decannulation.

OBJECTIVES: To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients. METHODS: An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group. RESULTS: Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation. CONCLUSION: Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies.

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.

Systemic bevacizumab for end-stage juvenile recurrent respiratory papillomas: A case report.

We report on two cases of severe recurrent respiratory papillomatosis (RRP) involvement where the anti-vascular endothelial growth factor (VEGF) antibody bevacizumab IV has been administered for treatment. Notably, there have not been any episodes of urgent transfer to the hospital for obstructive events since the bevacizumab was started. It is important to note however, that the disease has not been eliminated. While some pulmonary nodules continue to grow, they have become cavitated. Overall, the number and size of pulmonary lesions has decreased. Therefore, VEGF-targeted therapies, Bevacizumab, could prove to be a promising novel approach for long-term treatment of severe RRP.

An evaluation of arytenoid prolapse following laryngotracheal reconstruction.

OBJECTIVES/HYPOTHESIS: Laryngotracheal reconstruction (LTR) is a collection of procedures used to treat pediatric laryngotracheal stenosis. Arytenoid prolapse is a potential postoperative complication that may lead to upper airway obstruction. This study investigates the incidence, risk factors, and need for surgical intervention for post-LTR arytenoid prolapse. STUDY DESIGN: Retrospective chart review. METHODS: The charts of 107 patients who underwent a total of 119 LTR procedures between 2005 and 2018 at a tertiary free-standing children's hospital were reviewed. RESULTS: The incidence of post-LTR arytenoid prolapse was 7.6%. Age, glottic involvement, tracheostomy status, vocal cord paralysis, and balloon dilation had no effect on arytenoid prolapse development. Sixteen percent of procedures performed in children <8 kg resulted in arytenoid prolapse, versus 4.5% of procedures performed in children >8 kg (P = .036). One hundred percent 100% of patients with post-LTR arytenoid prolapse had placement of a posterior graft. Multivariate analysis confirmed both weight <8 kg and posterior graft use to be significantly associated with arytenoid prolapse (P = .027 and .039, respectively). Three of the nine patients with arytenoid prolapse were symptomatic and weighed <8 kg at time of surgery. One required voice therapy; two required surgical intervention. CONCLUSIONS: The incidence of arytenoid prolapse is 7.6% following LTR. Patients undergoing posterior graft LTR or weigh <8 kg at time of surgery are more likely to develop arytenoid prolapse. Children <8 kg at time of surgery who develop arytenoid prolapse are more likely to be symptomatic and require further intervention. These findings are valuable for preoperative risk assessment of pediatric laryngotracheal stenosis management. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:247-251, 2020.

Sedation withdrawal following single stage laryngotracheal reconstruction: Does dexmedetomidine help?

OBJECTIVES: Single-stage laryngotracheal reconstruction (SS-LTR) requires a period of post-operative intubation, during which time adequate sedation is needed to ensure graft healing. Commonly used agents include benzodiazepines, opioids, and more recently, dexmedetomidine, a centrally-acting α2 adrenoreceptor. This study aims to compare withdrawal outcomes between various sedation regimens following SS-LTR. METHODS: Retrospective chart review of 56 patients who underwent SS-LTR between 2008 and 2018 at a tertiary free-standing children's hospital was performed. Of 47 patients with complete records, 18 patients received dexmedetomidine for >75% of their intubation period with midazolam (DexWM), 9 received dexmedetomidine for >75% without midazolam (DexWOM), and 20 received dexmedetomidine for <75% with midazolam (noDex). RESULTS: There was no significant difference in length of PICU or hospital stay between the groups. The noDex group trended toward a higher re-intubation rate of 25%, as compared with 11% of DexWOM and 5.6% of DexWM (p = 0.21). There was no significant difference in days of oral sedation taper required or Withdrawal Assessment Tool (WAT-1) score for post-extubation days 1 and 3. By post-extubation day 5, 100% of the DexWM group had WAT-1 scores <3 as compared with 71.4% of the noDex group (p = 0.037). Notably, lower average daily doses of dexmedetomidine and midazolam were used in the DexWM group, as compared with the DexWOM and noDex groups, respectively. CONCLUSION: Dexmedetomidine as a primary sedation agent with midazolam allows for adequate sedation following SS-LTR. The combination of the two drugs in the DexWM group not only reduced the dosage of each drug needed, but also significantly improved WAT-1 scores by post-extubation day 5, as compared with the alternative sedation regimens.

International Pediatric ORL Group (IPOG) Robin Sequence consensus recommendations.

OBJECTIVE: To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management. CONCLUSION: Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

International Pediatric Otolaryngology Group (IPOG): Consensus recommendations on the prenatal and perinatal management of anticipated airway obstruction.

OBJECTIVE: To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery. METHODS: Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. "Consensus" was defined by >80% respondent affirmative responses, "agreement" by 51-80% affirmative responses, and "no agreement" by 50% or less affirmative responses. RESULTS: Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure. CONCLUSIONS: Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.

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.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

International Pediatric Otolaryngology Group (IPOG): Juvenile-onset recurrent respiratory papillomatosis consensus recommendations.

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

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.