Tongue Reduction Surgery and Feeding Difficulties in Infants With Beckwith Wiedemann Syndrome: A Case Series.

OBJECTIVE: To profile the pre- and post-operative feeding difficulties in infants with macroglossia in Beckwith Wiedemann Syndrome (m-BWS) who have had tongue reduction surgery (TRS) and to pilot a bespoke feeding rating scale.

DESIGN: Retrospective consecutive case series designed with two pre-operative and one 3-month post-operative feeding assessments. A 4-point Likert-type scale was developed and applied retrospectively to describe the feeding behaviors for liquids, purées, and solids. Descriptive and non-parametric statistics were used.

SETTING: National service for children with m-BWS at a pediatric hospital.

PATIENTS: Twenty-five infants, age range 4 to 12 months at initial assessment, underwent TRS (median age = 16 months).

INTERVENTION: Tongue reduction surgery.

OUTCOME MEASURE: Oral and selected pharyngeal stage feeding behaviors on liquids, purées, and solids.

RESULTS: Pre-operative profile: Most feeding difficulties arose at the oral stage due to the macroglossia impacting important lingual movements. Difficulties were found with lip seal formation, biting, bolus manipulation and tongue lateralization. Aspiration risk was found in >75%. Texture modification was indicated for purées and solids. Post-operative profile: There were statistically significant differences for each consistency pre- and post-operatively. Eighty-four percent of infants had age-appropriate drinking and eating skills. Mild residual difficulties with biting, tongue lateralization, and bolus manipulation remained for solids in four infants.

CONCLUSIONS: Feeding difficulties are common pre-operatively in m-BWS, putting infants at risk of aspiration if left unmanaged. TRS was effective in reducing or eliminating them. This is the first systematic report of infant feeding in m-BWS pre- and post-TRS.