Psychosocial, feeding, and drooling outcomes in children with Beckwith Wiedemann syndrome following tongue reduction surgery.

OBJECTIVE

Macroglossia is a common feature of Beckwith Wiedemann Syndrome (BWS). Tongue reduction surgery (TRS) is advocated to overcome, or reduce, the secondary effects of macroglossia. Macroglossia may affect a child's cosmetic appearance, feeding, and drooling function. However, no study has systematically reported on feeding, drooling, or psychosocial outcomes preoperatively and postoperatively in this group. This study aimed to describe the presurgical and postsurgical psychosocial, feeding, and drooling outcomes of children with macroglossia associated with BWS and to determine the effect of TRS on these areas.

DESIGN

Clinical cohort study.

PARTICIPANTS

Ten consecutively admitted children with BWS (age at surgery from 9 months to 4 years, 9 months [4;9]; mean, 2;7) were assessed preoperatively, 3 months postoperatively, and at long-term follow-up.

MAIN OUTCOME MEASURES: A parental report questionnaire, the Brodsky drooling scale, and a feeding rating scale.

RESULTS

Parents reported that macroglossia had a negative impact cosmetically that was ameliorated following surgery. Macroglossia caused a range of feeding difficulties presurgically by preventing lip seal and bolus manipulation during the oral preparatory phase. Excessive drooling was present in all cases presurgically. This resolved partially across the group postsurgically, with almost complete recovery at longer-term follow-up assessment.

CONCLUSIONS

Presurgically, children show a common profile of feeding and drooling impairment with negative effects on cosmetic appearance. Our preliminary results demonstrate that TRS has a positive impact on these features with good outcomes for children with BWS.