Respiratory culture growth and 3-years lung health outcomes in children with bronchopulmonary dysplasia and tracheostomies.

BACKGROUND: While bacteria identification on respiratory cultures is associated with poor short-term outcomes in children with bronchopulmonary dysplasia (BPD) and tracheostomies, the influence on longer-term respiratory support needs remains unknown. OBJECTIVE: To determine if respiratory culture growth of pathogenic organisms is associated with ongoing need for respiratory support, decannulation, and death at 3 years posttracheostomy placement in children with BPD and tracheostomies. METHODS: This single center, retrospective cohort study included infants and children with BPD and tracheostomies placed 2010-2018 and ≥1 respiratory culture obtained in 36 months posttracheostomy. Primary predictor was any pathogen identified on respiratory culture. Additional predictors were any Pseudomonas aeruginosa and chronic P. aeruginosa identification. Outcomes included continued use of respiratory support (e.g., oxygen, positive pressure), decannulation, and death at 3 years posttracheostomy. We used Poisson regression models to examine the relationship between respiratory organisms and outcomes, controlling for patient-level covariates and within-patient clustering. RESULTS: Among 170 children, 59.4% had a pathogen identified, 28.8% ever had P. aeruginosa, and 3.5% had chronic P. aeruginosa. At 3 years, 33.1% of alive children required ongoing respiratory support and 24.8% achieved decannulation; 18.9% were deceased. In adjusted analysis, any pathogen and P. aeruginosa were not associated with ongoing respiratory support or mortality. However, P. aeruginosa was associated with decreased decannulation probability (adjusted risk ratio 0.48, 95% CI 0.23-0.98). Chronic P. aeruginosa was associated with lower survival probability. CONCLUSION: Our findings suggest that respiratory pathogens including P. aeruginosa may not promote long-term respiratory dysfunction, but identification of P. aeruginosa may delay decannulation.

Characteristics and Outcomes of Surgical Treatment for Bronchial Anomalies.

OBJECTIVES: Bronchial anomalies are rare but challenging conditions to treat in children, encompassing a variety of structural abnormalities that could compromise airway patency. This includes complete rings, absent cartilage, traumatic avulsions, bronchoesophageal fistulas, and cartilaginous sleeves. The objective of this study is to describe the characteristics and outcomes of a series of pediatric cases of bronchial anomalies that were treated by slide tracheobronchoplasty. METHODS: This is a single-institution retrospective case series of pediatric patients with bronchial anomalies who underwent surgical treatment between February 2004 and April 2020. Data extracted from electronic medical records included patient demographics, comorbidities, and surgical outcomes. RESULTS: There were a total of 29 patients included in the study, of which 14 had complete bronchial rings, 8 had absent bronchial rings, 4 had traumatic bronchial avulsions, 2 had bronchoesophageal fistulas, and one had a cartilaginous sleeve. Median follow-up time was 13 months (with a range of 0.5-213 months). The overall mortality rate was 17.2% (5 patients), all of whom had complete bronchial rings. Patients with complete bronchial rings also had a higher rate of not only cardiac (85.7%) and pulmonary comorbidities (85.7%) but also secondary airway lesions (78.6%). CONCLUSION: This is the largest series to date describing surgical treatment for bronchial anomalies. Complete bronchial rings were the most common anomaly treated, followed by absent rings and trauma. Surgical treatment can be successful but mortality rates are higher in patients with complete bronchial rings, possibly due to higher rates of pulmonary and cardiac comorbidities. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:3334-3340, 2023.

The follow up of complex infants in an aerodigestive clinic.

The current available literature evaluating pediatric multidisciplinary aerodigestive programs for the management of aerodigestive disorders in infants was reviewed. Multidisciplinary aerodigestive programs have emerged to provide coordinated and comprehensive care for the growing population of children with aerodigestive conditions, including complex airway, pulmonary, gastrointestinal, and feeding disorders, which are prevalent among infants discharged from the neonatal intensive care unit (NICU). The team approach central to aerodigestive clinics offers a comprehensive diagnostic workup and unified management plan through consolidated interdisciplinary clinics, combined endoscopic procedures, and regular team discussions, leading to improved resource utilization and health care outcomes. We review common conditions presenting in the NICU that benefit from the aerodigestive model of care, including esophageal atresia, prematurity, bronchopulmonary dysplasia with or without tracheostomy or ventilator dependence, and dysphagia.

The Impact of Socioeconomic Status on Time to Decannulation Among Children With Tracheostomies.

OBJECTIVE: To determine if time to tracheostomy decannulation differs among children by socioeconomic status. STUDY DESIGN: Case series with chart review. SETTING: Tertiary pediatric medical center. METHODS: Patients (≤21 years old) who underwent tracheostomy from January 1, 2011, to December 31, 2016. Patients were divided into 2 groups based on their socioeconomic status (SES), low SES and high SES. Principal components analysis was used to create an index for SES using census data obtained by the US Census Bureau's American Community Survey 5 year data profile from 2013 to 2017. Statistical analysis was performed using a χ2 for categorical variables and Wilcoxon rank-sum test for continuous variables. A general linear model was constructed to control for clinical factors to understand the independent effect of SES on time to decannulation. RESULTS: In total, 215 patients were included; of these patients, 111 patients (52%) were included in the high-SES group and 104 patients (48%) were included in the low-SES group. There was a significant difference in the time to decannulation for children based on SES status, with those children in the low-SES group taking on average 10 months longer to decannulate (38.7 vs 28.0 months, P = .0007). Median follow-up was 44.1 months (interquartile range, 29.6-61.3 months). CONCLUSION: Health care disparities appear to exist among children undergoing decannulation of their tracheostomy tube. Patients with lower SES had a significantly longer time to decannulation than those with higher SES.

Differences in Flexible and Rigid Bronchoscopy for Assessment of Tracheomalacia.

OBJECTIVES/HYPOTHESIS: Both flexible and rigid bronchoscopy can be used to assess tracheomalacia; however, there is limited evidence comparing the two techniques. The objective of this study was to compare flexible and rigid bronchoscopy for evaluating the location and severity of tracheomalacia in children. STUDY DESIGN: Retrospective case series. METHODS: This was a retrospective study of children with both flexible and rigid bronchoscopy under the same sedation. All bronchoscopies were reviewed by three bronchoscopists for the location and severity of tracheomalacia. The location of collapse was defined as upper, middle, or lower trachea, and the severity of collapse was defined as none (0%-25% collapse), mild/moderate (26%-75% collapse), and severe (>75% collapse). RESULTS: Twenty-one patients were recruited for this study with a variety of neonatal respiratory diseases. There was 94% agreement (κ = 0.64) for assessment of tracheomalacia in the upper trachea. However, agreement was only 75% (κ = 0.50) in the middle trachea and 76% (κ = 0.52) in the lower trachea. In the subset of patients without tracheostomy, agreement improved to 100%, 88%, and 82% for the upper, middle, and lower trachea, respectively. There was poor correlation for tracheomalacia severity in the middle trachea (ρ = 0.30, P = .2) and moderate in the lower trachea (ρ = 0.63, P = .002). CONCLUSIONS: Although there is moderate agreement between flexible and rigid bronchoscopy for evaluating the presence of tracheomalacia, there can be differences in the two techniques, particularly when assessing severity of airway collapse. Future studies will be needed to understand factors that result in the discordance of flexible and rigid bronchoscopy for assessing airway dynamics. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:201-204, 2021.

Pediatric tracheostomy care updates.

PURPOSE OF REVIEW: The aim of this review is to provide an update on the management of pediatric tracheostomies. RECENT FINDINGS: Recent literature has focused on optimization of care for children with tracheostomies including prevention and management of skin breakdown, timing of tracheostomy tube changes, the role of multidisciplinary and team-based approaches to education and management of tracheostomy patients, ideal timing of surveillance endoscopy, and the emerging role of telemedicine in the care of tracheostomy patients. SUMMARY: A focus on quality improvement and a systematic, team-based approach to care has the potential to improve the quality of care for pediatric tracheostomy patients.

Characterization of tracheobronchomalacia in infants with hypophosphatasia.

BACKGROUND: Perinatal and infantile hypophosphatasia (HPP) are associated with respiratory failure and respiratory complications. Effective management of such complications is of key clinical importance. In some infants with HPP, severe tracheobronchomalacia (TBM) contributes to respiratory difficulties. The objective of this study is to characterize the clinical features, investigations and management in these patients. METHODS: We report a case series of five infants with perinatal HPP, with confirmed TBM, who were treated with asfotase alfa and observed for 3-7 years. Additionally, we reviewed respiratory function data in a subgroup of patients with perinatal and infantile HPP included in the clinical trials of asfotase alfa, who required high-pressure respiratory support (positive end-expiratory pressure [PEEP] ≥6 cm H2O and/or peak inspiratory pressure ≥18 cm H2O) during the studies. RESULTS: The case series showed that TBM contributed significantly to respiratory morbidity, and prolonged respiratory support with high PEEP was required. However, TBM improved over time, allowing weaning of all patients from ventilator use. The review of clinical trial data included 20 patients and found a high degree of heterogeneity in PEEP requirements across the cohort; median PEEP was 8 cm H2O at any time and some patients presented with high PEEP (≥8 cm H2O) over periods of more than 6 months. CONCLUSION: In infants with HPP presenting with persistent respiratory complications, it is important to screen for TBM and initiate appropriate respiratory support and treatment with asfotase alfa at an early stage. TRIAL REGISTRATION: ClinicalTrials.gov numbers: NCT00744042 , registered 27 August 2008; NCT01205152 , registered 17 September 2010; NCT01176266 , registered 29 July 2010.

Moral distress in pediatric otolaryngology: A pilot study.

OBJECTIVES: SUBJECTS/METHODS: Moral distress is defined as "when one knows the right thing to do, but institutional constraints make it nearly impossible to pursue the right course of action". The Moral Distress Survey-Revised (MDS-R) is a validated 21-question survey measuring moral distress in pediatrics. The MDS-R was anonymously distributed to pediatric otolaryngology faculty and fellows at a tertiary institution. Descriptive statistics, bivariate and multivariate analysis were performed. RESULTS: Response rate was 89% (16/18). Overall MDS-R score was 40 (range 14-94), which is lower than that found in the literature for pediatric surgeons (reported mean 72), pediatric intensivists (reported means 57-86), and similar to pediatric oncologists (reported means 42-52). Fellows had a significantly higher level of moral distress than faculty (mean 69 vs. 26, p < 0.05). Factors leading to higher degrees of distress involved communication breakdowns and pressure from administration/insurance companies to reduce costs. CONCLUSION: Pediatric Otolaryngologists at our institution have lower degrees of moral distress compared to other pediatric subspecialists. Fellows had higher levels of distress compared to faculty. Further research is necessary to determine degrees of distress across institutions and to determine its impact on the wellness of pediatric otolaryngologists.

Timing of Initial Posttracheostomy Surveillance Endoscopy in Pediatric Patients.

OBJECTIVE: To determine the optimal timing of the first posttracheostomy microlaryngoscopy and bronchoscopy (MLB). STUDY DESIGN: Case series with chart review. SETTING: Tertiary pediatric medical center. SUBJECTS AND METHODS: Patients (<21 years of age) who underwent tracheostomy placement from January 1, 2011, to December 31, 2016. Patients were divided into early and late posttracheostomy surveillance groups (<6 weeks vs 6 to 14 weeks, respectively) based on the timing of their first posttracheostomy MLB. The primary outcome was to ascertain the clinical yield of the initial posttracheostomy MLB by documenting whether a medical or surgical treatment decision was made based on MLB findings. RESULTS: In total, 202 patients were included; of these patients, 162 met criteria for placement in the early group and 40 met criteria for the late group. There was no significant difference between the early and late groups regarding whether a medical or surgical decision was made at the time of the first MLB (21.5% vs 19%, respectively; P = .49). Multiple logistic regression identified that the presence of tracheostomy-related symptoms prior to MLB (odds ratio, 6.75; 95% confidence interval, 2.78-16.39) was the sole predictor of a medical or surgical decision being made at the first posttracheostomy MLB. CONCLUSION: The presence of tracheostomy-related symptoms was predictive of a medical or surgical decision being made using information obtained at the time of the first posttracheostomy MLB. We thus recommend that surveillance endoscopy be initiated when tracheotomized children start to develop tracheostomy-related symptoms.

Ultrashort Echo-Time MRI for the Assessment of Tracheomalacia in Neonates.

BACKGROUND: Bronchoscopy is the gold standard for evaluating tracheomalacia; however, reliance on an invasive procedure limits understanding of normal airway dynamics. Self-gated ultrashort echo-time MRI (UTE MRI) can assess tracheal dynamics but has not been rigorously evaluated. METHODS: This study was a validation of UTE MRI diagnosis of tracheomalacia in neonates using bronchoscopy as the gold standard. Bronchoscopies were reviewed for the severity and location of tracheomalacia based on standardized criteria. The percent change in cross-sectional area (CSA) of the trachea between end-inspiration and end-expiration was determined by UTE MRI, and receiver-operating curves were used to determine the optimal cutoff values to predict tracheomalacia and determine positive and negative predictive values. RESULTS: Airway segments with tracheomalacia based on bronchoscopy had a more than threefold change in CSA measured from UTE MRI (54.4 ± 56.1% vs 14.8 ± 19.5%; P < .0001). UTE MRI correlated moderately with bronchoscopy for tracheomalacia severity (ρ = 0.39; P = .0001). Receiver-operating curves, however, showed very good ability of UTE MRI to identify tracheomalacia (area under the curve, 0.78). A "loose" definition (> 20% change in CSA) of tracheomalacia had good sensitivity (80%) but low specificity (64%) for identifying tracheomalacia based on UTE MRI, whereas a "strict" definition (> 40% change in CSA) was poorly sensitive (48%) but highly specific (93%). CONCLUSIONS: Self-gated UTE MRI can noninvasively assess tracheomalacia in neonates without sedation, ionizing radiation, or increased risk. This technique overcomes major limitations of other diagnostic modalities and may be suitable for longitudinal population studies of tracheal dynamics.

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections in Children with Cystic Fibrosis.

Culture and sequencing have produced divergent hypotheses about cystic fibrosis (CF) lung infections. Culturing suggests that CF lungs are uninfected before colonization by a limited group of CF pathogens. Sequencing suggests diverse communities of mostly oral bacteria inhabit lungs early on and diversity decreases as disease progresses. We studied the lung microbiota of CF children using bronchoscopy and sequencing, with measures to reduce contamination. We found no evidence for oral bacterial communities in lung lavages that lacked CF pathogens. Lavage microbial diversity varied widely, but decreases in diversity appeared to be driven by increased CF pathogen abundance, which reduced the signal from contaminants. Streptococcus, Prevotella, and Veillonella DNA was detected in some lavages containing CF pathogens, but DNA from these organisms was vastly exceeded by CF pathogen DNA and was not associated with inflammation. These findings support the hypothesis that established CF pathogens are primarily responsible for CF lung infections.

Thoracoscopic posterior tracheopexy for tracheomalacia: A minimally invasive technique.

BACKGROUND: Tracheomalacia is the most common congenital abnormality of the trachea. Posterior tracheopexy to alleviate posterior intrusion contributing to dynamic tracheal collapse has been reported using thoracotomy or median sternotomy. Here we describe the minimally invasive operative technique of thoracoscopic posterior tracheopexy with bronchoscopic guidance. OPERATIVE TECHNIQUE: After preoperative computed tomography and bronchoscopy, a right thoracoscopic approach is utilized. The esophagus is mobilized and the membranous trachea is sutured to the prevertebral fascia under direct bronchoscopic visualization. Immediate improvement in tracheal collapse is noted. No major complications are reported and length of stay is short. Aortopexy may also be required to address anterior vascular compression. CONCLUSION: Thoracoscopic posterior tracheopexy is safe and feasible. Further studies with more patients and longer follow-up are needed to assess durability.

Bronchiectasis, Chronic Suppurative Lung Disease and Protracted Bacterial Bronchitis.

Bronchiectasis is a structural airway disease characterized by dilated bronchi and bronchioles due to severe or recurrent lower airways inflammation. Bronchiectasis can occur as a result of chronic pulmonary aspiration. Bronchiectasis may also be associated with a wide variety of systemic diseases, which should be considered in the differential diagnosis. Children with bronchiectasis typically have a chronic or recurrent productive cough and carry a significant burden of disease with a considerable impact on quality of life. The diagnosis of bronchiectasis is made by high-resolution chest computerized tomography. Aggressive management of bronchiectasis is necessary to reduce the daily symptom burden and frequency of exacerbations. Chronic suppurative lung disease may be a precursor to bronchiectasis, only lacking the defining radiographic features of bronchiectasis. Children with chronic suppurative lung disease may have the same symptoms as children with bronchiectasis and should be treated similarly. Protracted bacterial bronchitis is defined as a cough lasting at least four weeks that responds to antibiotic therapy. Protracted bacterial bronchitis may occur following a viral respiratory tract infection. Protracted bacterial bronchitis can be treated with a prolonged course of empiric antibiotics. Further evaluation is necessary if a child with suspected protracted bacterial bronchitis does not adequately respond to antibiotics as chronic suppurative lung disease or bronchiectasis must be considered.

Life-threatening airway bleeding after palliation of single ventricle congenital heart disease.

OBJECTIVE: To describe acute and mid-term outcomes following presentation with, and treatment for, life-threatening airway bleeding (hemoptysis) in palliated single ventricle congenital heart disease (SV-CHD). METHODS: Case series of patients with SV-CHD who presented to a large congenital heart centre with hemoptysis between 2004 and 2015. RESULTS: Twenty-one episodes of hemoptysis occurred in 12 patients (58% female, median 10.5 (IQR 7.2, 16.4) years). First hemoptysis episode occurred after Fontan completion (n=8), after superior cavopulmonary anastomosis (SCPA, n=3) and in one shunt-dependent patient. Bronchoscopy was performed in conjunction with catheterisation in 14/21 (67%) initial catheterisations. A specific anatomic source of airway bleeding was identified in 95% of bronchoscopy cases and was uniformly distributed in all lobar segments. Transcatheter intervention with systemic-to-pulmonary collateral artery (SPC) occlusion was performed in 28/30 catheterisations. Apart from increased airway bleeding during interventional bronchoscopy (37%), there were no procedural complications. Median hospital length of stay was 9.0 (3.5, 14.5) days with patients undergoing 1.0 (1.0,2.0) catheterisations per episode of hemoptysis. Two SCPA patients did not survive to discharge. During a median follow-up of 32.5 (12.5, 87.5) months, freedom from mortality was 75%, with all three deaths occurring in the SCPA group by 4 months posthemoptysis. Recurrent hemoptysis occurred in 60% of patients. CONCLUSIONS: Despite the potentially life-threatening nature of hemoptysis in patients with SV-CHD, a policy of bronchoscopic evaluation and transcatheter treatment is safe and may contribute to low mortality at mid-term follow-up in Fontan patients. Hemoptysis in SCPA patients may portend a poor prognosis. Recurrent hemoptysis is common.

Segmental deficiency of cervical tracheal rings masquerading as complete tracheal rings: A case report and literature review.

Congenital deficiency of distal tracheal rings is a rare anomaly and has been previously reported in the literature. Here we report the first case deficient tracheal rings confined to the cervical trachea. Patient was transferred to our institution for management of what was initial thought to be complete tracheal rings. The patient was successfully managed with a tracheal resection and short segment cervical slide tracheoplasty. Presentation, surgical approach, histological findings, and literature review are described.