Assessment of swallowing function in pediatric patients with Wilson's disease: Results of a videofluoroscopic swallowing study

Abstract

Background and study aims: Wilson's disease (WD) is a complex disorder related to copper metabolism and neurological involvement may lead to swallowing disorders. The purpose of this study was to evaluate swallowing function in pediatric patients with WD by using videofluoroscopic swallowing study (VFSS).

Patients and methods: A total of 21 patients were included in the study, prospectively. The VFSS was conducted to evaluate swallowing function of the patients. The penetration-aspiration scale (PAS) was used to assess penetration-aspiration severity.

Results: According to the VFSS, abnormal results were detected in nine patients (42.9%) with WD. Of these nine patients, oral phase dysfunction was present in one patient, laryngeal penetration was present in one patient and moreover, abnormal esophageal body function was detected in all nine patients. Of these nine patients, five had neurological presentation at the time of diagnosis, and remaining four patients had hepatic presentation. Mean PAS score of the patients was 1.

Conclusion: The current study results suggest that subclinical swallowing dysfunction may be observed in patients with either neurological or hepatic WD. Further studies are necessary to reveal the real incidence of esophageal phase problems of swallowing function in pediatric patients with WD.

Keywords: Childhood; Swallowing; Videofluoroscopic swallowing study; Wilson’s disease.