Characterizing swallowing and feeding in children with leukodystrophies: Retrospective analysis of 22 cases.

INTRODUCTION

Dysphagia is a frequent and potentially life-threating symptom in children affected by leukodystrophies. However, reports on dysphagia in leukodystrophies are scarce. The study aimed to characterize swallowing and feeding in this population.

METHODS

We retrospectively reviewed the medical charts of 22 children with leukodystrophies referred for swallowing evaluation. The severity of the disease was determined by a child neurologist according to several functional scales. Data on feeding modality and diet type according to the Pediatric Functional Oral Intake Scale (P-FOIS) and the International Dysphagia Diet Standardization Initiative Functional Diet Scale (IDDSI-FDS) were collected and correlated with the severity of the disease. Presence of clinical signs of dysphagia or feeding disorders and related mechanisms were recorded from clinical and/or instrumental swallow examinations.

RESULTS

All children except one had full oral nutrition, although 50% required texture modifications and 18.1% required compensatory strategies during meals. P-FOIS and IDDSI-FDS significantly correlated with all neurological functional scales (r = |0.430-0.886|). Sixteen children (72.7%) exhibited dysphagia, and 7 children (31.8%) had signs of behavioural feeding disorder. Mechanisms of dysphagia were poor oral control (36%), impaired oral preparation (27%), impaired oral transport (23%), reduced airway protection (9%), and prolonged pharyngeal phase (5%). Five patients (22.7%) had a history of aspiration pneumonia.

CONCLUSION

Dysphagia in our cohort seemed mainly characterized by alterations in the oral phase, whereas pharyngeal phase was often preserved, but should be carefully monitored due to the high prevalence of respiratory complications. Diet restrictions were correlated with the neurological severity, although full-oral diet was often possible.