Family-Centered Information Dissemination: A Multidisciplinary Virtual COVID-19 "Town Hall".

Significant misinformation about COVID-19 has been spread on the internet. Parents of children with complex aerodigestive problems have a hard time understanding the information they encounter on the internet and the news media and interpreting how it relates to their child's complex needs. Our multidisciplinary team, at the suggestion of a parent, hosted 3 virtual "town halls" in which families could ask questions directly of pediatric otolaryngology, pediatric pulmonology and case management in order to efficiently obtain factual evidence-based up-to-date advice. The information discussed at the town halls was then annotated and disseminated via active, parent-run aerodigestive social media forums. The information disseminated via the town halls reached 4787 Facebook participants.

Working towards consensus in the management of pediatric chronic rhinosinusitis in cystic fibrosis.

OBJECTIVE: The prevalence of chronic rhinosinusitis (CRS), defined by mucosal thickening on imaging, approaches 100% in the cystic fibrosis (CF) population. CRS is associated with significant morbidity in CF, including its ability to trigger pulmonary exacerbations. CRS in CF is typically managed by pediatricians, otolaryngologists and pulmonologists. This survey evaluates the variance in practice patterns of CRS in CF amongst specialists. METHODS: This is a cross-sectional, electronic survey in which maximum variation purposive sampling was used by a multi-disciplinary group of pediatric, otolaryngology and pulmonology providers in order to select a survey population with expertise in CRS in CF patients. The survey was distributed to 381 practitioners from September to October 2019. RESULTS: 175 participants responded (45% response rate). Ten (of 54) statements achieved 75% consensus agreement. Consensus statements included: The decision to pursue surgical intervention for CRS in CF is a multi-disciplinary approach (94%; n = 146); maximal medical management should include nasal saline irrigation (93%; n = 142), topical steroids (75%; n = 117), maximal medical management should not include intravenous steroids (79%; n = 122); image guidance in surgery is necessary for all surgery involving the frontal sinuses (77%; n = 43), and all revision surgery(80%, n = 45); the appropriate setting for sinus surgery in a CF patient varies depending on patient presentation (89%; n = 133); post-operative regimen should include nasal saline (93%; n = 137); but does depend on the severity of disease discovered intra-operatively (84%; n = 124); post-operative antibiotics should be guided by intra-operative culture data (82%; n = 121). CONCLUSIONS: There is a great deal of variation amongst specialists in the treatment of CRS in CF, however 10 statements met consensus criteria and should be considered when forming clinical care guidelines in this population.

Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis.

BACKGROUND: Despite the significant impact of chronic symptoms on quality of life with cystic fibrosis (CF), the role of palliative care in management of this disease is not well defined. The coping, goal assessment, and relief from evolving CF symptoms (CF-CARES) model is a primary palliative care intervention designed to provide chronic symptom management at all stages of the disease. The goal of this pilot study was to estimate the effectiveness of the CF-CARES intervention on improving chronic symptoms and quality of life for people living with CF. METHODS: A structured assessment was used to guide referral to supportive services intended to address burdensome symptoms. Follow-up assessments were performed approximately 3 and 6 months later. Longitudinal regression analyses of changes in symptoms and quality of life were performed for all participants regardless of utilization of supportive services. Subgroup analyses were performed for subjects participating in mental health and alternative health services. RESULTS: Forty-one subjects completed assessment and referral processes. The mean number of CF-associated symptoms decreased over time, as did respiratory symptom-related distress and depressive symptoms. Subjects utilizing alternative health services reported less psychological distress at follow-up. Among subjects with severe disease, mental health, and quality of life improved, especially for those using mental health services. CONCLUSIONS: The CF-CARES model resulted in significant mental health and quality-of-life benefits, suggesting the value of integrating symptom management interventions into routine CF care. Moreover, mental health services can play a key role in CF-specific primary palliative care, especially for those with advanced disease.

The diagnostic role of triple endoscopy in pediatric patients with chronic cough.

OBJECTIVE/HYPOTHESIS: Multi-disciplinary aero-digestive centers provide high quality health care through improved outcomes and treatment costs over separate sub specialty clinics. These outcomes are often the result of a common investigative tool known as triple endoscopy: a rigid bronchoscopy performed by an otolaryngologist, flexible bronchoscopy and lavage obtained by a pulmonologist, and an endoscopy with guided biopsies performed by a gastroenterologist. Combining such procedures into one 'triple endoscopy' allows for diagnoses which otherwise might have been missed with just one procedure. The goal of our study was to describe the efficacy of the triple endoscopy procedure in diagnosing recalcitrant aero-digestive conditions, specifically chronic cough. STUDY DESIGN: Retrospective chart review METHODS: Multiple charts from children who underwent the triple endoscopy for chronic cough were retrospectively reviewed from 2005 and 2017. Complete data from the triple procedure was gathered on 243 patients, including findings by sub specialty (otolaryngology, pulmonology, and gastroenterology). RESULTS: Of the 243 patients with complete data who underwent triple endoscopy, 203 (83.5%) children had at least one positive finding. Of these children, 101 (41.5%) had one specialty specific diagnosis, and 102 (42%) had multiple cross specialty diagnoses. When describing the diagnoses, 63 children had gastro esophageal reflux (GER), 14 had eosinophilic esophagitis (EoE), 118 had tracheomalacia, 54 had laryngeal clefts, and 102 children had positive bronchoalveolar lavages. Outcome data was available on 226 patients (93%), of these patients, 188 patients had a diagnosis from the triple scope. Of those patients with a diagnosis and outcome data, 144 (76.6%) children had an improved outcome as a result of a treatment plan targeting that diagnosis, while 16 of the 37 patients without a diagnosis improved. This difference was significant by chi square analysis (p<0.0001). CONCLUSION: The triple scope procedure is a useful investigative tool for patients with recalcitrant aero-digestive complaints like chronic cough. In particular, triple scope can yield more than one specialty specific diagnosis, normally missed by one procedure. The triple scope also leads to improved parental satisfaction by improved cost and healthcare outcomes.

Assessment of the feeding Swallowing Impact Survey as a quality of life measure in children with laryngeal cleft before and after repair.

OBJECTIVE: To cross-validate the Feeding Swallowing Impact Survey (FSIS), a quality of life instrument, specifically to a subpopulation of children who aspirate due to laryngeal cleft. INTRODUCTION: The FSIS is a recently validated instrument used to describe caregiver quality of life (QOL) in children with aspiration due to various causes. To cross-validate the FSIS specifically to the subpopulation of children who aspirate due to laryngeal cleft, we tested the hypotheses that caregivers would report significant different scores form baseline if their children improved at the one year mark postintervention due to either successful conservative or surgical measures (discriminant validity) and would not report significant differences in their FSIS reporting if there was no change in their child's aspiration at the one year mark post intervention (convergent validity). METHODS: The FSIS was administered to the caregivers of 35 children (19 male, 16 female; age range: 5-79 months) who aspirate secondary to known laryngeal cleft (diagnosed by suspension laryngoscopy and inspection). All children had a baseline VFSS demonstrating aspiration and documenting what feeding plan to follow and caregivers completed the FSIS at this point as well. All children regardless of whether they were treated by conservative or surgical intervention underwent a follow-up VFSS at the one year post-intervention mark and the caregivers completed a FSIS at this time point as well. RESULTS: Among two distinct sub-populations of children who underwent either successful conservative or surgical treatment for their laryngeal cleft and demonstrated improvement at the one year mark (as defined by a VFSS documented decreased need for thickener by at least one consistency or more) and where we hypothesized that FSIS scores would not be significantly different, the caregivers reported no significant differences in FSIS scores cleft repair (mean FSIS scores 2.45 (SD 0.88)/2.1 (SD 0.94); p = 0.28). Moreover, as another test to convergent validity, for children who underwent either unsuccessful conservative treatment (and subsequently went on to need surgery) or who were not successfully surgically treated for their laryngeal cleft and demonstrated no significant improvement at the one year mark (as defined by a VFSS documented decreased need for thickener by at least one consistency or more), the caregivers reported no significant differences in FSIS scores cleft repair (mean FSIS scores 2.8(SD 0.79)/2.5(SD 0.88); p = 0.69). For divergent validity, two distinct sub-populations of children who underwent either successful or not successful surgical treatment for their laryngeal cleft (demonstrated by either improvement or lack of improvement at the one year mark VFSS as defined by a decreased need for thickener by at least one consistency or more) revealed significant differences in caregiver FSIS scores cleft repair (mean FSIS scores 1.38 (SD 0.32); 32.8 (SD 0.79); p=<0.0002). DISCUSSION: This results of this study provide convergent and divergent validity supporting the cross-validation of the FSIS instrument to be utilized as a validated QOL instrument to evaluate children with aspiration specifically due laryngeal cleft as another tool with which to evaluate the outcomes of medical or surgical interventions for this disorder.

Radiation exposure from videofluoroscopic swallow studies in children with a type 1 laryngeal cleft and pharyngeal dysphagia: A retrospective review.

INTRODUCTION: Radiation exposure is recognized as having long term consequences, resulting in increased risks over the lifetime. Children, in particular, have a projected lifetime risk of cancer, which should be reduced if within our capacity. The objective of this study is to quantify the amount of ionizing radiation in care for children being treated for aspiration secondary to a type 1 laryngeal cleft. With this baseline data, strategies can be developed to create best practice pathways to maintain quality of care while minimizing radiation exposure. METHODS: Retrospective review of 78 children seen in a tertiary pediatric aerodigestive center over a 5 year period from 2008 to 2013 for management of a type 1 laryngeal cleft. The number of videofluoroscopic swallow studies (VFSS) per child was quantified, as was the mean effective dose of radiation exposure. The 78 children reviewed were of mean age 19.9 mo (range 4 mo-12 years). All children were evaluated at the aerodigestive center with clinical symptomatology and subsequent diagnosis of a type 1 laryngeal cleft. Aspiration was assessed via VFSS and exposure data collected. Imaging exams where dose parameters were not available were excluded. RESULTS: The mean number of VFSS each child received during the total course of treatment was 3.24 studies (range 1-10). The average effective radiation dose per pediatric VFSS was 0.16 mSv (range: 0.03 mSv-0.59 mSv) per study. Clinical significance was determined by comparison to a pediatric CXR. At our facility a CXR yields an effective radiation dose of 0.017 mSv. Therefore, a patient receives an equivalent total of 30.6 CXR over the course of management. CONCLUSIONS: Radiation exposure has known detrimental effects particularly in pediatric patients. The total ionizing radiation from VFSS exams over the course of management of aspiration has heretofore not been reported in peer reviewed literature. With this study's data in mind, future developments are indicated to create innovative clinical pathways and limit radiation exposure.

Modified Best-Practice Algorithm to Reduce the Number of Postoperative Videofluoroscopic Swallow Studies in Patients With Type 1 Laryngeal Cleft Repair.

IMPORTANCE: There is no consensus as to the timing of videofluoroscopic swallow studies (VFSSs) in determining resolving aspiration after laryngeal cleft repair. There is a growing literature on the effect of radiation exposure in children. OBJECTIVE: To modify a previously published best-practice algorithm based on a literature review and our clinical experience to maintain the quality of care provided after successful type 1 laryngeal cleft repair, while reducing the total number of postoperative VFSSs by 10% or greater. DESIGN, SETTING, AND PARTICIPANTS: The previously published algorithm was modified by a multidisciplinary group at a tertiary care academic medical center (Massachusetts Eye and Ear) and was prospectively applied to 31 children who underwent type 1 laryngeal cleft repair from January 1, 2013, to February 28, 2015. MAIN OUTCOMES AND MEASURES: The number of VFSSs obtained in the first 7 months after surgery was compared with the peer-reviewed literature and with a retrospective cohort of 27 patients who underwent type 1 laryngeal cleft repair from January 1, 2008, to December 31, 2012. RESULTS: The study cohort comprised 31 patients. Their ages ranged from 10 to 48 months, with a mean (SD) age of 23.94 (9.93) months, and 19% (6 of 31) were female. The mean (SD) number of postoperative VFSSs per patient before and after implementation of the algorithm was 1.22 (0.42) and 1.03 (0.55), respectively. The use of the algorithm reduced the number of VFSSs by 0.19 (95% CI, -0.07 to 0.45). This reduction in radiation exposure is equivalent to 1.47 chest radiographs per child per course of care. Surgical success was 87% (27 of 31) compared with our group's previously published success rate of 78% (21 of 27) (absolute difference, 0.09; 95% CI, -0.17 to 0.34). CONCLUSIONS AND RELEVANCE: This modified algorithm to help guide decisions on when and how often to obtain VFSSs after type 1 laryngeal cleft repair can limit patients' radiation exposure, while maintaining high surgical success rates.

Prevalence of eosinophilic esophagitis in children with refractory aerodigestive symptoms.

IMPORTANCE: Eosinophilic esophagitis (EoE) is an increasingly important diagnosis for children; it has a remarkable impact on their quality of life and can present with aerodigestive symptoms commonly evaluated by otolaryngologists. OBJECTIVES: To evaluate the prevalence of EoE in children presenting to a pediatric aerodigestive clinic, to describe their presentation, and to review the role of subsequent food allergy evaluation and treatment. DESIGN: Review of a prospective database. SETTING: Tertiary pediatric multispecialty aerodigestive center. PATIENTS: Children with aerodigestive symptoms refractory to medical treatment who underwent direct laryngoscopy with rigid or flexible bronchoscopy and esophagoscopy with or without pH probe study. MAIN OUTCOMES AND MEASURES: Diagnosis of EoE. RESULTS: Between 2003 and 2012, 376 of 1540 children seen in the center (mean [range] age, 4.54 [0-18.6] years; male to female ratio, 1.72:1) remained symptomatic despite medical therapy and thus underwent triple endoscopic evaluation. Of the 376 children, 14 (3.7%) were eventually diagnosed as having EoE, as defined by 15 or more eosinophils per high-power field on esophageal biopsy and either a negative pH study result or nonresponse to a trial of high-dose proton pump inhibitors. The subpopulation with EoE presented with airway symptoms and diagnoses, most commonly cough (n = 6; 42.9%). Inflammatory subglottic stenosis due to EoE was identified in 1 patient. Of the 14 children with EoE, 6 presented with gastrointestinal symptomatology, most commonly choking or gagging. Subsequent treatment including food allergy challenge and elimination diet resulted in a clinical improvement in half of the cases identified. CONCLUSIONS AND RELEVANCE: This represents the largest multispecialty clinic epidemiologic study evaluating the prevalence of EoE in children presenting not strictly with gastrointestinal symptoms but rather with aerodigestive symptoms that are frequently evaluated by pediatric otolaryngologists. Although the prevalence is low, EoE should be considered for children with appropriate symptoms in whom other medical therapies fail.

Flexible bronchoscopy.

Flexible bronchoscopy is an important procedure for evaluating the pediatric airway, allowing a dynamic view from the trachea through the lower bronchi. The flexible bronchoscope offers greater maneuverability than the rigid bronchoscope and may be done in conjunction with other procedures, such as bronchoalveolar lavage, transbronchial biopsy, endobronchial ultrasound, electrocautery or laser treatments. Common indications for flexible bronchoscopy include recurrent croup or pneumonia, aspiration, foreign body and chronic cough. Although bronchospasm, transient fever, infection and pneumothorax are rare complications of flexible bronchoscopy, these risks are minimized with careful technique and an ongoing dialogue with the anesthesiologist. Flexible bronchoscopy is an important tool in the diagnosis, monitoring and therapy of certain pulmonary diseases and should be considered in the management of carefully selected pediatric patients.