Screening and evaluation tools of dysphagia in children with neuromuscular diseases: a systematic review

Abstract

Aim: Dysphagia is frequent in paediatric patients with neuromuscular diseases (pNMD). Its detection is important for initiating early diagnosis and treatment as well as for minimizing related complications. The aim of this study was to review the literature on dysphagia screening and evaluation tools in pNMD.

Method: A systematic review was performed on the basis of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Three databases (PubMed, CINAHL, and ScienceDirect) were searched. Measurement properties of tools and the quality index developed by Downs and Black were considered.

Results: Our search yielded four studies and four different tools for paediatric patients with Duchenne muscular dystrophy (DMD). The Sydney Swallow Questionnaire, surface electromyography, Neuromuscular Disease Swallowing Status Scale, and videofluoroscopic swallow study showed interesting properties for DMD. No data were available for other NMD and children under 9 years. The mean total score for the quality index was 17.5.

Interpretation: We did not identify any superior validated tools, either for screening or for evaluation of dysphagia, and no widely accepted protocol. Further studies are needed to identify the simplest assessment with the best psychometric properties for pNMD. We recommend establishing a specific tool for pNMD.