DySMA - an Instrument to Monitor Swallowing Function in Children with Spinal Muscular Atrophy ages 0 to 24 Months: Development, Consensus, and Pilot Testing.

Background: The manifestation of bulbar symptoms, especially swallowing, is important for evaluating disease-modifying therapies for spinal muscular atrophy (SMA). Due to the lack of instruments, the topic is still underrepresented in research. Objective: This study aimed to develop a tool to monitor swallowing development in children aged 0 to 24 months with SMA. Methods: The method was guided by the COSMIN guidelines and followed a multi-stage Delphi process. The first step was a rapid review of swallowing outcomes in children with SMA younger than 24 months. In the second step, online group interviews with experts (n = 7) on dysphagia in infants were conducted, followed by an anonymous online survey among experts in infants with SMA (n = 19). A predefined consensus threshold for nominal scaled voting was set at≥75 % and for 5-point Likert scale voting at 1.25 of the interquartile range. The third step was the pilot test of the instrument, performed with three groups (healthy controls n = 8; pre-symptomatic n = 6, symptomatic n = 6). Results: Based on the multi-level interprofessional consensus, the DySMA comprises two parts (history and examination), ten categories, with 36 items. Implementation and scoring are clearly articulated and easy to implement. The pilot test showed that swallowing development could be recorded in all groups. Conclusion: The DySMA is well suited for monitoring swallowing development in pre-symptomatic and symptomatic treated infants with SMA. It can be performed in a time-efficient and interprofessional manner. The resulting score is comparable to results from other instruments measuring other domains, e.g., motor function.

Narrow Band Imaging in Flexible Endoscopic Evaluation of Swallowing-How Does It Work?

PURPOSE: The purposes of this study were to compare the visibility of various color groups in different dilutions using narrow band imaging (NBI) and white light (WL) and to determine an optimal color combination using NBI and WL for multicolor flexible endoscopic evaluation of swallowing (FEES), for example, to test different consistencies. METHOD: Preliminary examinations were performed in the oral cavity of two healthy volunteers. Various dyes were tested using NBI and WL to evaluate their visibilities. In the case of a clearly discernible color change, the visibility differences in the dilution series under WL and NBI were recorded and compared. Subsequently, an abbreviated dilution series with NBI and WL was performed in a volunteer as part of a swallow endoscopy to determine whether the results from the oral cavity could be transferred to the hypopharynx. RESULTS: The enhancement of visibility using NBI compared with WL can be proven. When NBI was used, yellow and red food dyes and their mixtures showed distinct color changes. The reacting dyes were still visible under NBI, even at a 10-times-higher dilution, requiring a lower dye concentration for FEES. For increased visibility, the dyes used for FEES with NBI must contain colors from a small range in the yellow and red spectra, which are close to the filter maxima of NBI. Both colors are well visible under WL when combining red and green (secondary color of yellow). CONCLUSIONS: Certain food colorings are visible 10 times more diluted under NBI than under WL. In a multicolored approach, optimal visibility under NBI and WL can be achieved by combining green and red. This new high-sensitivity FEES should be highlighted to allow an instant distinction from WL-FEES; we suggest "FEES+." SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.23280290.

Flexible endoscopic evaluation of swallowing in children with type 1 spinal muscular atrophy.

PURPOSE: This study aimed to report on implementing flexible endoscopic evaluation of swallowing (FEES) in infants and toddlers with type 1 spinal muscular atrophy (SMA). In addition, a comparison of FEES results and clinical scores was carried out. METHODS: A prospective pilot study was conducted including ten symptomatic children with SMA type 1 (two SMN2 copies). They started treatment with one of the three currently approved therapies for SMA at a median age of 3.8 months (range 0.7-8.9). FEES was performed according to a standard protocol using Penetration-Aspiration Scale (PAS) and Murray Secretion Scale as a primary outcome. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) for motor function, Neuromuscular Disease Swallowing Status Scale (NdSSS), Oral and Swallowing Abilities Tool (OrSAT), and single clinical swallowing-related parameters were also assessed. RESULTS: Distinct swallowing disorders were already evident in eight children at inclusion. The most common findings from FEES were pharyngeal secretion pooling, penetration, and aspiration of saliva and food as well as delayed initiation of swallowing. Despite an average increase in motor function, no comparable improvement was found in swallowing function. None of the surveyed clinical scores showed a significant dependence on PAS in a mixed linear model. CONCLUSIONS: Valuable information regarding the status of dysphagia can be gathered endoscopically, particularly concerning secretion management and when oral intake is limited. Currently available clinical tools for children with type 1 may represent a change in nutritional status but are not yet mature enough to conclude swallowing ability. Further development is still required.

Implementation of Pediatric Flexible-Endoscopic Evaluation of Swallowing: A Systematic Review and Recommendations for Future Research.

BACKGROUND: Although pediatric flexible-endoscopic evaluation of swallowing (FEES) has developed into a standard in dysphagia diagnostics, there are no valid protocols and procedures for children available to date. OBJECTIVE: This systematic PROSPERO-registered review aimed to identify implementation protocols for pediatric FEES described in research studies, and to analyze them in detail concerning procedural steps, equipment, and reported outcome. METHODS: Included were all studies reporting a pediatric FEES protocol for children aged 0-18 years, if they described at least two criteria defined in advance. The databases MEDLINE and CINHAL were searched systematically from January 2000 to February 2021. Risk of bias for included studies was assessed using the National Institutes of Health (NIH) quality assessment tool for observational cohort and cross-sectional studies. A narrative synthesis of the FEES protocols was conducted and the results compared in tabular form. RESULTS: In total 22 studies were included, reporting on FEES in 1547 infants, children, and adolescents with a wide range of diagnoses. It was possible to identify protocols related to all age groups in general as well as to particular groups such as breastfed or bottle-fed infants. None of the included studies demonstrated a good methodological quality; all studies had missing data. Uniform implementation for sub-groups could not be determined. The reported outcome of FEES examinations could not be compared. DISCUSSION: None of the included studies showed good methodological quality and a significant amount of data were missing; the review still offers a systematic basis for future research to close the serious gap in the area of pediatric FEES. A proposal is made for a minimum requirement for pediatric FEES protocols in scientific studies.

Pediatric Flexible Endoscopic Evaluation of Swallowing: Critical Analysis of Implementation and Future Perspectives.

This study aimed to critically review pediatric swallowing assessment data to determine the future need for standardized procedures. A retrospective analysis of 152 swallowing examinations in 128 children aged 21 days to 18 years was performed. The children were presented at a university dysphagia center between January 2015 and June 2020 for flexible-endoscopic evaluation of swallowing (FEES). Descriptive analysis was conducted for the sample, swallowing pathologies, diagnosis, and missing values. Using binary logistic regression, the relationship between dysphagia and underlying diseases was investigated. The largest group with a common diagnosis in the cohort were children with genetic syndromes (n = 43). Sixty-nine children were diagnosed with dysphagia and 59 without dysphagia. The non-dysphagic group included 15 patients with a behavioral feeding disorder. The presence of an underlying disease significantly increased the chance of a swallowing problem (OR 13.08, 95% CI 3.66 to 46.65, p = .00). In particular, the categories genetic syndrome (OR 2.60, 95% CI 1.15 to 5.88) and neurologic disorder (OR 4.23, 95% CI 1.31 to 13.69) were associated with higher odds for dysphagia. All pediatric FEES were performed without complications, with a completion rate of 96.7%, and with a broad variability of implementation. Several charts lacked information concerning swallowing pathologies, though. Generally, a more standardized protocol and documentation for pediatric FEES is needed to enable better comparability of studies on epidemiology, assessment, and treatment outcomes in future.