Recurrent missense variant identified in two unrelated families with MPZL2-related hearing loss, expanding the variant spectrum associated with DFNB111.

MPZL2-related hearing loss is a rare form of autosomal recessive hearing loss characterized by progressive, mild sloping to severe sensorineural hearing loss. Thirty-five previously reported patients had biallelic truncating variants in MPZL2, with the exception of one patient with a missense variant of uncertain significance and a truncating variant. Here, we describe the clinical characteristics and genotypes of five patients from four families with confirmed MPZL2-related hearing loss. A rare missense likely pathogenic variant [NM_005797.4(MPZL2):c.280C>T,p.(Arg94Trp)] located in exon 3 was confirmed to be in trans with a recurrent pathogenic truncating variant that segregated with hearing loss in three of the patients from two unrelated families. This is the first recurrent likely pathogenic missense variant identified in MPZL2. Apparently milder or later-onset hearing loss associated with rare missense variants in MPZL2 indicates that some missense variants in this gene may cause a milder phenotype than that resulting from homozygous or compound heterozygous truncating variants. This study, along with the identification of truncating loss of function and missense MPZL2 variants in several diverse populations, suggests that MPZL2-related hearing loss may be more common than previously appreciated and demonstrates the need for MPZL2 inclusion in hearing loss testing panels.

International pediatric otolaryngology group (IPOG) consensus on approach to aspiration.

OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.

Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia.

OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.

Middle Ear Condition at the Time of Pediatric Myringotomy Tube Placement: Pain Associations Following Intraoperative Fentanyl/Ketorolac and Seasonal Variation.

BACKGROUND: Ketorolac-refractory pain behavior following bilateral myringotomy and pressure equalization tube placement (BMT) is associated with the absence of middle ear fluid. Intraoperative fentanyl/ketorolac affords more reliable pain control than ketorolac alone. We hypothesized that middle ear condition would correlate with postoperative pain despite such combination therapy. We further sought to demonstrate seasonal variation in ear condition and its influence on pain. METHODS: We conducted a single-institution retrospective cohort study of healthy children (9 months-7 years), who underwent BMT by a single surgeon from 2015 to 2020. Anesthetic care included sevoflurane/nitrous oxide/oxygen/air by mask and intramuscular fentanyl/ketorolac. Left/right middle ear fluid status was recorded at the time of BMT, and ear condition (primary exposure) was dichotomized as bilateral infected (mucoid or purulent) or normal/unilateral infected. The primary outcome was maximum postanesthesia care unit Face, Legs, Activity, Cry, and Consolability (FLACC) score: 4-10 (moderate-to-severe pain) versus 0-3 (no-to-low pain). Rescue oxycodone, acetaminophen administration, and emergence agitation were secondary outcomes. Statistical analysis incorporated generalized linear mixed-effect models (GLMMs) with random intercepts to account for clustering by anesthesia provider. A year-over-year monthly time-series analysis was conducted using an autoregressive integrated moving average (ARIMA) regression model. RESULTS: Excluding recurrent cases, 1149 unique evaluable subjects remained. Bilateral infection prevalence was 39.8% (457/1149; 95% confidence interval [CI], 37.0-42.6). Probability of moderate-to-severe pain behavior was 23.5% (270/1149; 95% CI, 21.1-26.0) overall. Compared to patients with bilateral infected middle ears, those with normal/unilateral infected ears were more likely to have a FLACC score ≥4 (26.7% [185/692] versus 18.6% [85/457]; odds ratio [95% CI], 1.7 [1.2-2.3]; P = .002). Variability in pain outcome explained by the multivariable GLMM was 4.7%. Fentanyl dose response was evidenced by oxycodone administration differences ( P ≤ 0.002). Moderate-to-severe pain and emergence agitation were more likely with reduced fentanyl dosing. Bilateral infection prevalence exhibited seasonality, peaking in March and nadiring in July. However, pain outcomes did not vary by season. CONCLUSIONS: Normal/unilateral infected ears at time of pediatric BMT are associated with higher incidence of moderate-to-severe postoperative pain following intraoperative fentanyl/ketorolac administration, but the predictive value of ear condition on pain is limited. Infections were less common in the summer.

Tracheocutaneous Fistula After Pediatric Open Airway Reconstruction.

OBJECTIVES: Tracheocutaneous fistula (TCF) is a common occurrence after pediatric tracheostomy decannulation. However, the persistence of TCF after staged reconstruction of the pediatric airway is not well-described. The primary objective was to determine the rate of persistent TCF after successful decannulation in children with staged open airway reconstruction. METHODS: A case series with chart review of children who underwent decannulation after double-stage laryngotracheal reconstruction between 2017 and 2019. RESULTS: A total of 26 children were included. The most common open airway procedure was anterior and posterior costal cartilage grafting (84.6%, 22/26). Median age at decannulation was 3.4 years (IQR: 2.8-4.3) and occurred 7.0 months (IQR: 4.3-10.4) after airway reconstruction. TCF persisted in 84.6% (22/26) of children while 15.4% (4/26) of stomas closed spontaneously. All closures were identified by the one-month follow-up visit. There was no difference in age at tracheostomy (P = .86), age at decannulation (P = .97), duration of tracheostomy (P = .43), or gestational age (P = .23) between stomas that persisted or closed. Median diameter of stent used at reconstruction was larger in TCFs that persisted (7.0 mm vs 6.5 mm, P = .03). Tracheostomy tube diameter (P = .02) and stent size (P < .01) correlated with persistence of TCF on multivariable logistic regression analysis. There were 16 surgical closure procedures, which occurred at a median of 14.4 months (IQR: 11.4-15.4) after decannulation. Techniques included 56.3% (9/16) by primary closure, 18.8% (3/16) by secondary intention and 25% (4/16) by cartilage tracheoplasty. The overall success of closure was 93.8% (15/16) at latest follow-up. CONCLUSIONS: Persistent TCF occurs in 85% of children who are successfully decannulated after staged open airway reconstruction. Spontaneous closure could be identified by 1 month after decannulation and was more likely when smaller stents and tracheostomy tubes were utilized. Surgeons should counsel families on the frequency of TCF and the potential for additional procedures needed for closure.

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.

Timing the First Pediatric Tracheostomy Tube Change: A Randomized Controlled Trial.

OBJECTIVE: The first pediatric tracheostomy tube change often occurs within 7 days after placement; however, the optimal timing is not known. The primary objective was to determine the rate of adverse events of an early tube change. Secondary objectives compared rates of significant peristomal wounds, sedation requirements, and expedited intensive care discharges. STUDY DESIGN: Prospective randomized controlled trial. SETTING: Tertiary children's hospital between October 2018 and April 2020. METHODS: A randomized controlled trial enrolled children under 24 months to early (day 4) or late (day 7) first tracheostomy tube changes. RESULTS: Sixteen children were enrolled with 10 randomized to an early change. Median age was 5.9 months (interquartile range, 5.4-8.3), and 86.7% required tracheostomy for respiratory failure. All tracheostomy tube changes were performed without adverse events. There were no accidental decannulations. Significant wounds developed in 10% of children with early tracheostomy tube changes and 83.3% of children with late tracheostomy tube changes (odds ratio [OR], 45.0; 95% CI, 2.3-885.6; P = .01). This significant reduction in wound complications justified concluding trial enrollment. Hours of dexmedetomidine sedation (P = .11) and boluses of midazolam during the first 7 days (P = .08) were no different between groups. After the first change, 90% of the early group were discharged from intensive care within 5 weeks compared to 33.3% of patients in the late group (OR, 18.0; 95% CI, 1.2-260.9; P = .03). CONCLUSION: The first tracheostomy tube change in children can occur without adverse events on day 4, resulting in fewer significant peristomal wounds and earlier intensive care discharge.

Diagnostic considerations prior to pediatric tracheocutaneous fistula closure.

INTRODUCTION: An airway assessment often occurs prior to tracheocutaneous fistula (TCF) closure in children. Bronchoscopy (MLB) with or without fistula-occluded polysomnography (PSG) helps determine candidacy and localize potential obstruction. To date, little has been published on MLB or PSG findings in children before surgically closing a TCF. METHODS: A case series with chart review of children between 2017 and 2020 who underwent repair of a TCF after tracheostomy decannulation. RESULTS: Thirty-six children were included for review. Mean age was 5.9 years (95% CI: 4.5-7.3), 58.3% were male, and 50% had chronic lung disease. Surgery occurred 13.3 months (95% CI: 11.9-14.8) after decannulation, with 80.6% by primary closure and 19.4% by secondary intention. There was one unsuccessful closure and two patients (5.6%) presented with a postoperative complication. An MLB was performed in 97.2% of children, where 22.9% identified supraglottic pathology, 11.4% had grade 2 subglottic stenosis, and 11.4% had difficult exposure of the larynx. Further, one child had a non-obstructing subglottic cyst, one had a supraglottoplasty for redundant arytenoid mucosa, and two children had suprastomal granulomas requiring removal. A PSG was obtained in 36.1%, with a mean Apnea-Hypopnea Index of 2.4 events/hour (95% CI: 0.9-3.9), nadir Oxygen saturation of 90.5% (95% CI: 87.9-93.0), and peak end-tidal CO2 of 46.1 mmHg (95% CI: 43.7-48.5). CONCLUSION: The selection of candidates for pediatric TCF closure requires careful evaluation of the airway. Surgeons should be familiar with the potential findings on MLB and PSG prior to closure.

Pediatric Otolaryngology in the COVID-19 Era.

Although the majority of attention to the health care impact of COVID-19 has focused on adult first responders and critical care providers, the pandemic has had a profound effect on the entire health care industry, including the pediatric otolaryngology community. This article highlights the unique ramifications of COVID-19 on pediatric otolaryngology, with a focus on the immediate and potential long-term shifts in practice. Specifically, the article is divided into 3 sections (care for the patient, care for the practitioner, and care for the practice) and details the unique effects of the pandemic on the pediatric otolaryngology specialty.

Predictors of the need for tracheostomy in the neonatal intensive care unit.

OBJECTIVES: Many infants in the neonatal intensive care unit (NICU) require prolonged periods of respiratory support. Microlaryngoscopy and bronchoscopy (MLB) is performed to evaluate for airway pathology and facilitate decision-making regarding further airway interventions or tracheostomy. The objectives of this study are to describe the operative findings of MLB performed on infants in the NICU and determine which pre-operative characteristics or operative findings are predictive of the need for tracheostomy. METHODS: The medical records of preterm inpatients in the NICU at a single tertiary care hospital who underwent MLB between January 1, 2013 and January 7, 2016 were reviewed. Baseline and demographic characteristics and intra-operative findings were compared between patients who underwent tracheostomy and those who were successfully weaned from respiratory support. RESULTS: Seventy-three preterm patients underwent MLB for respiratory failure, of whom 41 (56.2%) underwent tracheostomy. Patients who underwent tracheostomy had lower mean gestational age (27.4 vs. 30.5 weeks), higher prevalence of bronchopulmonary dysplasia (73.2% vs. 37.5%), lower mean birth weight (1.1 kg vs. 1.6 kg), and a greater number of extubation events (5.2 vs. 3.0) than those who weaned from respiratory support. Abnormal MLB findings were common in both groups, though no single MLB finding differed significantly between groups. CONCLUSIONS: Preterm infants in the NICU with gestational age ≤30 weeks, birth weight <1.5 kg, severe pulmonary disease, and who have failed more than 3 extubation attempts are more likely to require tracheostomy.

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

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.

Slide tracheoplasty: Predictors of outcomes and literature review.

OBJECTIVE: Determine preoperative comorbidities and intraoperative parameters associated with adverse postoperative outcomes. METHODS: Retrospective case series at a single tertiary care children's hospital from 2010 through 2017. RESULTS: Twenty-six patients with median age of 6 months and median weight of 7.1 kg underwent slide tracheoplasty. Median time to extubation, length of intensive care unit admission, and length of hospitalization were 7, 27, and 30 days, respectively. Twenty-two (85%) required no additional intervention. Overall success was 87%. One (4%) patient required open revision, and 3 (11%) required tracheostomy. Concomitant cardiac surgery was associated with postoperative tracheostomy (p = 0.04). Age and weight at surgery were inversely correlated with length of intubation (p = 0.03) and length of hospital stay (p = 0.001, p = 0.002) respectively. Hospital stay was 2.2 times longer if preoperative mechanical ventilation was required (p = 0.01) and 39% longer for every 1 mm decrease in airway diameter at the narrowest portion of the stenosis (p = 0.005). There were no deaths related to persistent tracheal stenosis with a median follow-up of 24 months. CONCLUSION: Slide tracheoplasty is safe and effective. Concomitant cardiac surgery was associated with postoperative tracheostomy. Lower age and weight at surgery were correlated with longer length of intubation and hospital stay. Preoperative mechanical ventilation and smaller airway diameter were associated with longer hospital stay. This information may be helpful in counseling families and planning future prospective studies.

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.

Diagnosis and Management of Laryngotracheoesophageal Cleft.

Laryngotracheoesophageal cleft (LTEC) is a rare anatomic cause of aspiration in the newborn and infant. The clinical presentation of infants with LTEC is variable and will usually vary with the degree of anatomic communication between the airway and esophagus. The diagnosis of LTEC is often challenging, particularly in cases where the presentation and pathology are more subtle. Management of LTEC depends upon the clinical presentation, degree of aspiration, and anatomic depth of the cleft. The goal of this review is to present the clinician with a practical approach to diagnosis and management of this rare but challenging congenital airway anomaly.

A Critical Period in Postnatal Neuroplasticity of Olfaction: A Pediatric Tracheostomy Model.

IMPORTANCE: There is controversy over whether a critical period in the development of olfaction exists, as there is in hearing and vision, whereby early stimulation of the olfactory nerve is necessary for normal olfactory performance later in life. Children who undergo tracheotomy early in life are deprived of airflow through the nasal cavity during a critical period of development. Persistent olfactory dysfunction in this patient group after decannulation would provide evidence that postnatal stimulation of the olfactory nerve is critical to normal development. OBJECTIVE: To determine whether children who undergo early tracheotomy have persistent olfactory dysfunction following decannulation and to validate a prior study showing olfactory deficits in cannulated patients. DESIGN, SETTING, AND PARTICIPANTS: This was a cross-sectional study of smell function in pediatric patients with either long-term tracheostomy (cannulated), decannulated patients after long-term tracheostomy, and healthy age- and sex-matched controls, conducted in a tertiary care academic referral center, using data that were collected between 2013 and 2015. All patients were without coexisting nasal abnormalities or developmental delay that would prevent completion of testing. INTERVENTIONS: Administration of a validated pediatric smell test to all 3 patient groups. MAIN OUTCOMES AND MEASURES: Mean percentage correct on a validated pediatric smell test. RESULTS: In 18 patients ages 6 to 18 years, there was a statistically significant difference (P = .007) in mean percentage of correct responses on the smell test between cannulated (67%; 95% CI, 54%-79%, N = 6), decannulated (61%; 95% CI, 42%-80%, N = 6), and age-matched controls (94%; 95% CI, 90%-99%, N = 6). Analysis between groups showed statistically significant differences between both control and cannulated patients (P = .002) and between control and decannulated patients (P = .006). There was no significant difference between scores in the cannulated and decannulated groups (P = .64). CONCLUSIONS AND RELEVANCE: This pilot study suggests that olfactory deficits from early chronic tracheostomy persist following decannulation and provides early data suggestive of a critical period in the postnatal development and neuroplasticity of olfaction.

Is inpatient admission necessary following removal of airway foreign bodies?

OBJECTIVE: To determine the need for postoperative admission following airway foreign body retrieval by examining the preoperative presentation, operative details, and postoperative recovery. INTRODUCTION: Inpatient admission following foreign body removal is common, however little evidence supports this practice. In the era of cost containment and prudent utilization of hospital resources, careful examination of the postoperative course following airway foreign body removal is required. METHODS: A retrospective review of cases over a four year period from a pediatric tertiary care pediatric hospital was performed. All children presenting with concerns for airway foreign bodies were included. Children without identification of an airway foreign body during bronchoscopy were excluded. Microlaryngoscopy and bronchoscopy with airway foreign body extraction was performed. Details concerning demographics, operative findings, and pre and postoperative course including pulse oximetry were collected. All respiratory events, intubations, and persistent oxygen requirements were investigated. RESULTS: Thirty five children underwent successful airway foreign body removal. The mean age was 3.2±2.6 years with a preponderance of males (68.6%). The retrieved items included: nuts (31.4%), popcorn (14.3%), seeds (8.6%), and inorganic materials (34.3%). Mean operative time was 29.7±25.6min. The mean length of stay following surgery was 1.3±1.9 days. Most patients (31/35) (88.6%) were extubated prior to transfer to recovery. 30/35 (85.7%) patients required no supplemental oxygen without desaturations following post anesthesia care unit (PACU) recovery. One patient developed laryngospasm requiring reintubation within 15min of surgery. Two patients were intubated prior to transfer for respiratory distress and remained intubated following surgery. Two patients breathing spontaneously prior to surgery were left intubated following surgery secondary to prolonged pneumonia treatment or multifocal foreign bodies with airway edema. All patients subsequently extubated without complication. In total, 30/31 (96.7%) of patients extubated in the operating room returned to room air oxygenation within 2h of surgery. CONCLUSIONS: PACU observation and discharge is feasible in select children following airway foreign body extraction. Patients carefully monitored in the recovery unit without oxygen requirement are candidates for discharge. Inpatient monitoring is advised in 'children with preoperative respiratory distress or a complicated operative course.

Inpatient observation for elective decannulation of pediatric patients with tracheostomy.

IMPORTANCE: The incidence and timing patterns of decannulation failure in children are unknown. There is substantial variability in the duration of inpatient hospitalization for patients undergoing decannulation, which represents an opportunity for improved resource use. OBJECTIVE: To determine the incidence and timing patterns of elective decannulation failure in the pediatric population and to determine an appropriate interval of inpatient observation following decannulation that optimizes both patient safety and resource use. DESIGN, SETTING, AND PARTICIPANTS: Retrospective review of medical records of consecutive patients 18 years or younger hospitalized for elective, inpatient decannulation between January 1, 2012, and October 31, 2013, at a quaternary care pediatric hospital. MAIN OUTCOMES AND MEASURES: Duration of decannulation hospitalization, failure of elective decannulation (decision not to decannulate or reinsertion of tracheostomy tube after decannulation), time interval from decannulation to failure. RESULTS: Forty-six patients completed 50 elective decannulation hospitalizations during the study period. The median duration of hospitalization for decannulation was 3.0 days. The hospitalization-specific failure rate was 16% (8 of 50), and the overall failure rate was 9% (4 of 46). Four patients were not able to tolerate capping of the tracheostomy tube and were discharged with their original tracheostomy tubes in place. Three of these patients were decannulated at a later hospitalization. In 4 patients, decannulation failed and they had to have their tracheostomy tubes replaced prior to discharge. Patients who did not tolerate decannulation were younger (mean [SD] age, 45.7 [17.0] months) than patients whose decannulation was successful (68.2 [48.0] months). All patients with unsuccessful decannulation attempts were symptomatic during capping. The longest interval from decannulation to tracheostomy reinsertion was 11 hours. CONCLUSIONS AND RELEVANCE: Elective decannulation failure occurred in 9% of this population and may be more common in younger patients and those with a diagnosis of vocal fold paralysis. Patients who are symptomatic during predecannulation capping are at high risk for decannulation failure. Inpatient observation for a 24-hour asymptomatic interval after decannulation may be sufficient because late failures were not observed in this sample.

Risk of otitis externa following manual cerumen removal.

OBJECTIVES: Based on an interpretation of a recent Joint Commission protocol to sterilize instruments coming into contact with mucous membranes, there has been institutional interest in sterile packaging of cerumen curettes used for manual cerumen removal. To date, there are no studies that have assessed the risk of otitis externa (OE) following cerumen removal and the utility of sterile packaging is questionable. The objective of this study is to assess the incidence of OE following cerumen disimpaction prior to the implementation of sterile packaging at our institution. METHODS: This was a retrospective chart review. Over a I-year period, 1457 episodes of manual cerumen removal took place in the otolaryngology clinic. Charts were assessed for signs or symptoms of OE within 2 weeks of the procedure through follow-up phone calls and clinic visits in the otolaryngology division. RESULTS: There were no patients who followed up with symptoms or signs suggestive of OE in the 2-week postprocedure period. CONCLUSION: There is no evidence that OE is a complication of manual cerumen removal when performed by otolaryngologists using clean technique. Unnecessary sterilization of tools leads to increased cost and time for this common outpatient procedure performed by the otolaryngologist.

Laryngeal obstruction by cervical and endolaryngeal lymphatic malformations in children: proposed staging system and review of treatment.

OBJECTIVES: We review and stage a series of congenital lymphatic malformations (LMs) that caused laryngeal obstruction according to a proposed staging system, review the treatment modalities utilized, and correlate the stage of disease with clinical outcomes and tracheotomy statuses. METHODS: We present a detailed description of the clinical presentation, management approach, and clinical outcome and tracheotomy status of a series of patients with laryngeal obstruction by LMs. A 4-part staging system (stages I to IV) for congenital LMs obstructing the larynx, based upon our clinical experience, is proposed. The patients were retrospectively reviewed and staged according to the proposed system, and clinical management and outcomes were correlated with the stage of disease. RESULTS: We identified 16 patients with laryngeal obstruction by LMs. Eighty-one percent (13 of 16) received sclerotherapy, and 50% (8 of 16) underwent operative excision or debridement. Forty percent (2 of 5) of stage I lesions, 75% (3 of 4) of stage II lesions, 100% (4 of 4) of stage III lesions, and 100% (3 of 3) of stage IV lesions were in patients who required tracheotomy. All patients who had stage I and II lesions and required tracheotomy have been decannulated, whereas only 2 of the 4 patients with stage III lesions and no patients with stage IV lesions have been successfully decannulated. CONCLUSIONS: Lymphatic malformations obstructing the larynx require a careful and often staged management approach. A proposed staging system helps to predict the need for tracheotomy and the likelihood of long-term tracheotomy dependence.