Flexible bronchoscopy in pediatric lung transplantation.

Pediatric lung transplantation represents a treatment option for children with advanced lung disease or pulmonary vascular disorders who are deemed an appropriate candidate. Pediatric flexible bronchoscopy is an important and evolving field that is highly relevant in the pediatric lung transplant population. It is thus important to advance our knowledge to better understand how care for children after lung transplant can be maximally optimized using pediatric bronchoscopy. Our goals are to continually improve procedural skills when performing bronchoscopy and to decrease the complication rate while acquiring adequate samples for diagnostic evaluation. Attainment of these goals is critical since allograft assessment by bronchoscopic biopsy is required for histological diagnosis of acute cellular rejection and is an important contributor to establishing chronic lung allograft dysfunction, a common complication after lung transplant. Flexible bronchoscopy with bronchoalveolar lavage and transbronchial lung biopsy plays a key role in lung transplant graft assessment. In this article, we discuss the application of bronchoscopy in pediatric lung transplant evaluation including historical approaches, our experience, and future directions not only in bronchoscopy but also in the evolving pediatric lung transplantation field. Pediatric flexible bronchoscopy has become a vital modality for diagnosing lung transplant complications in children as well as assessing therapeutic responses. Herein, we review the value of flexible bronchoscopy in the management of children after lung transplant and discuss the application of novel techniques to improve care for this complex pediatric patient population and we provide a brief update about new diagnostic techniques applied in the growing lung transplantation field.

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.

Long-Term Outcomes of Airway Management in 6 Children With Campomelic Dysplasia.

OBJECTIVE: This case series describes the outcomes of airway management, including airway reconstruction, in 6 patients with campomelic dysplasia and tracheostomy/ventilator dependence secondary to multilevel airway obstruction. METHODS: Case series and clinical guidelines are provided for the airway management of patients with campomelic dysplasia. RESULTS: Average age of individuals is 19.4 years. Mean follow-up was 12.2 years. Four individuals underwent open airway reconstruction and achieved decannulation. One patient underwent airway reconstruction with improvement of a complete subglottic stenosis but remains ventilator dependent due to severe scoliosis. The remaining 2 patients did not require additional airway reconstruction, have been liberated from ventilator support, and are under evaluation for tracheostomy tube decannulation. CONCLUSION: Although campomelic dysplasia was historically considered a lethal form of congenital skeletal dysplasia, with many patients succumbing to respiratory failure due to tracheobronchomalacia in the neonatal period, airway reconstruction and long-term survivorship is feasible in children with campomelic dysplasia and significant airway disease.

Caregiver comprehension and satisfaction associated with informed consent for pediatric flexible bronchoscopy: A single-center experience.

Pediatric flexible bronchoscopy (FB) is a relatively common procedure performed in children with respiratory concerns. Despite this, there is no data examining the informed consent process for FB. A prospective study evaluating informed consent comprehension and satisfaction was conducted in 71 primary caregivers of children undergoing outpatient FB. Most caregivers (97%) were overall satisfied with the consent process and reported understanding the potential benefits (97%) and minor risks (94%) associated with the procedure. A smaller percentage (83%) reported understanding major risks associated with FB. Patient safety was reported as the most important aspect of the consent process. Caregivers of children undergoing FB for the first time were more likely to want additional FB-specific information when compared to caregivers of children who had previously undergone FB (53% vs 16%, odds ratio: 5.97). In general, caregivers of children undergoing outpatient FB are satisfied with the informed consent process. Areas for improvement include ensuring adequate understanding of major risks associated with the procedure and considering a tailored approach based on the caregiver's level of experience.

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.

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.

The Tempest: Difficult to Control Asthma in Adolescence.

Severe asthma is associated with significant morbidity and is a highly heterogeneous disorder. Severe asthma in adolescence has some unique elements compared with the features of severe asthma a medical provider would see in younger children or adults. A specific focus on psychological issues and adherence highlights some of the challenges in the management of asthma in adolescents. Treatment of adolescents with severe asthma now includes 3 approved biologic phenotype-directed therapies. Therapies available to adults may be beneficial to adolescents with severe asthma. Research into predictors of specific treatment response by phenotypes is ongoing. Optimal treatment strategies are not yet defined and warrant further investigation.

Response to fluid boluses in the fluid and catheter treatment trial.

BACKGROUND: Recent emphasis has been placed on methods to predict fluid responsiveness, but the usefulness of using fluid boluses to increase cardiac index in critically ill patients with ineffective circulation or oliguria remains unclear. METHODS: This retrospective analysis investigated hemodynamic responses of critically ill patients in the ARDS Network Fluid and Catheter Treatment Trial (FACTT) who were given protocol-based fluid boluses. Fluid responsiveness was defined as ≥ 15% increase in cardiac index after a 15 mL/kg fluid bolus. RESULTS: A convenience sample of 127 critically ill patients enrolled in FACTT was analyzed for physiologic responses to 569 protocolized crystalloid or albumin boluses given for shock, low urine output (UOP), or low pulmonary artery occlusion pressure (PAOP). There were significant increases in mean central venous pressure (9.9 ± 4.5 to 11.1 ± 4.8 mm Hg, P < .0001) and mean PAOP (11.6 ± 3.6 to 13.3 ± 4.3 mm Hg, P < .0001) following fluid boluses. However, there were no significant changes in UOP, and there were clinically small changes in heart rate, mean arterial pressure, and cardiac index. Only 23% of fluid boluses led to a ≥ 15% change in cardiac index. There was no significant difference in the frequency of fluid responsiveness between boluses given for shock or oliguria vs boluses given only for low PAOP (24.0% vs 21.8%, P = .59). There were no significant differences in 90-day survival, need for hemodialysis, or return to unassisted breathing between patients defined as fluid responders and fluid nonresponders. CONCLUSIONS: In this cohort of critically ill patients with ARDS who were previously resuscitated, the rate of fluid responsiveness was low, and fluid boluses only led to small hemodynamic changes.