Emily M. DeBoer

Automated ct scan scores of bronchiectasis and air trapping in cystic fibrosis

BACKGROUND Computer analysis of high-resolution CT (HRCT) scans may improve the assessment of structural lung injury in children with cystic fibrosis (CF). The goal of this cross-sectional pilot study was to validate automated, observer-independent image analysis software to establish objective, simple criteria for bronchiectasis and air trapping. METHODS HRCT scans of the chest were performed in 35 children with CF and compared with scans from 12 disease control subjects. Automated image analysis software was developed to count visible airways on inspiratory images and to measure a low attenuation density (LAD) index on expiratory images. Among the children with CF, relationships among automated measures, Brody HRCT scanning scores, lung function, and sputum markers of inflammation were assessed. RESULTS The number of total, central, and peripheral airways on inspiratory images and LAD (%) on expiratory images were significantly higher in children with CF compared with control subjects. Among subjects with CF, peripheral airway counts correlated strongly with Brody bronchiectasis scores by two raters (r=0.86, P<.0001; r=0.91, P<.0001), correlated negatively with lung function, and were positively associated with sputum free neutrophil elastase activity. LAD (%) correlated with Brody air trapping scores (r=0.83, P<.0001; r=0.69, P<.0001) but did not correlate with lung function or sputum inflammatory markers. CONCLUSIONS Quantitative airway counts and LAD (%) on HRCT scans appear to be useful surrogates for bronchiectasis and air trapping in children with CF. Our automated methodology provides objective quantitative measures of bronchiectasis and air trapping that may serve as end points in CF clinical trials.

Multidisciplinary care of children with repaired esophageal atresia and tracheoesophageal fistula.

Children with congenital esophageal atresia with tracheoesophageal fistula (TEF) require complex medical and surgical care, but few guidelines exist to guide the long term care of this population. The purpose of this study is to describe the findings and initial management of a comprehensive aerodigestive team in order to understand the ongoing needs of children with repaired TEF.

Surgical management of type I and II laryngeal cleft in the pediatric population

INTRODUCTION Laryngeal clefts (LC) are congenital malformations of the upper aerodigestive tract that can cause dysphagia, aspiration, and subsequent pulmonary complications. This investigation assessed the outcomes of multidisciplinary evaluation and surgical management of type I and II LC (LC1-2) at a tertiary childrens hospital. MATERIALS AND METHODS A retrospective chart review was used to identify patients who underwent repair of LC1-2 by a single surgeon. RESULTS Twenty-five patients who underwent surgical management for LC1-2 were identified. Twenty patients with LC-1 and 5 with LC-2 were included. Most common presentations were aspiration on modified barium swallow (MBS) (76%) and recurrent pulmonary infections (60%). Gastroesophageal reflux disease (GERD) was a concurrent co-morbidity in 68%. Of the 19 patients who presented with preoperative aspiration on MBS, 52.6% had evidence of improvement on their first MBS. This improved to 78.9% at the time of their most recent MBS (10 with resolution of aspiration, 5 with improved tolerance of consistencies). Two patients failed to improve from surgical repair and two had worsened aspiration on most recent MBS. CONCLUSION Endoscopic repair of laryngeal clefts is a well-tolerated procedure. Concurrent GERD and positive bronchoalveolar lavage (BAL) cultures are common and require management prior to definitive repair. Given the multidimensional nature of swallowing disorders and aspiration, a comprehensive team approach in the evaluation, management, and surveillance of these patients is a valuable complement to surgical repair since not all aspiration may resolve.

Structure and functions of pediatric aerodigestive programs: A consensus statement

We present a multi-disciplinary consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

Measuring Pediatric Bronchoscopy Outcomes Using an Electronic Medical Record

RATIONALE Bronchoscopy procedures should be tracked for safety, quality improvement, and federal regulations. OBJECTIVE The aim of this study was to develop and test a method for evaluating flexible bronchoscopy use and outcomes using the electronic medical record (EMR) system in current clinical use at a large childrens hospital. METHODS We created a custom bronchoscopy procedure note for our EMR system (Epic Systems Corporation) to track demographics, bronchoscopist, coordinated procedures, and outcome. Unplanned outcomes in children were defined as a disposition (admission to the hospital or elevation of care) after flexible bronchoscopy that differed from the preoperative plan. Readmissions to the hospital and emergency visits within our hospital system were also tracked electronically. Unplanned outcomes and readmissions were evaluated by a bronchoscopy quality team. MEASUREMENTS AND MAIN RESULTS Over 2.5 years, we tracked 1,297 bronchoscopic procedures performed on 1,161 patients (60% male, 78% American Society of Anesthesiologists class 2 or 3, mean age 5.5 yr [range, 0.02-40 yr]). Overall, 27 unplanned outcomes occurred (2.1%). The risk of unplanned outcomes did not appear to be different between procedures performed by a trainee with faculty oversight and those performed by a faculty member alone. Patients with multiple same-day procedures were more likely to have unplanned outcomes (21 of 27 [78%], P = 0.004) than were patients who had flexible bronchoscopy alone. The relative risk (RR) of having an unplanned outcome was not different from flexible bronchoscopy alone in the subset of patients with multiple procedures coordinated through our multidisciplinary aerodigestive clinic (RR 0.7; 95% CI, 0.1-3.4). The risk of unplanned events was significantly elevated in children with coordinated procedures scheduled outside the aerodigestive group (RR, 5.8; 95% CI, 2.4-14.5). Ten patients (<1%) were readmitted or seen urgently within 1 week; three of these unplanned outcomes were attributed to complications of the bronchoscopy. CONCLUSIONS An EMR system may be used to track procedural outcomes. Unplanned outcomes after flexible bronchoscopy were infrequent at our institution. Children who underwent multiple procedures had unplanned outcomes more often; however, the subset of children who underwent coordinated procedures through our multidisciplinary aerodigestive clinic did not demonstrate this increased risk.

Evaluating the yield of gastrointestinal testing in pediatric patients in aerodigestive clinic

To improve understanding of the interrelatedness of airway and esophageal diagnoses by evaluating the yield of procedural and radiographic testing of the gastrointestinal tract in children with airway conditions by their referring diagnoses in a pediatric aerodigestive clinic.

Pediatric tracheomalacia and the perioperative anesthetic management of thoracoscopic posterior tracheopexy.

Tracheomalacia is a broad term used to describe an abnormally compliant trachea that can lead to exaggerated collapse and obstruction with expiration. We describe the perioperative management of a complex pediatric patient undergoing a posterior tracheopexy which is a relatively new surgical treatment, with a novel surgical approach—thoracoscopy. This procedure has competing surgical and anesthetic needs and presents unique challenges to the physicians involved in caring for these patients. We also review the current literature on pediatric tracheomalacia and examine the newest treatment options to highlight the potential anesthetic challenges and pitfalls associated with management.

Erratum to: Analysis of pediatric airway morphology using statistical shape modeling.

regression modeling using all subjects showed a good fit (ROC AUC = 0.85) and revealed significant differences in SSM parameters between control and CF groups. The largest mode of shape variation was highly correlated with subject size (R = 0.95, p < 0.001). SSM methodology can be applied to identify shape differences in the airway between two populations. This method suggests that subtle shape differences exist between the CF airway and disease control.