Final Tongue Size in the Treatment of Macroglossia Associated With Beckwith-Wiedemann Syndrome

Vollständiger Abstract

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Beckwith-Wiedemann syndrome (BWS) is a rare congenital disorder whose main clinical feature is macroglossia. This frequent manifestation may impair respiratory, feeding, speech, and orofacial developmental functions. When significant functional and/or aesthetic repercussions are evident, surgical excision is the primary management approach, with several techniques described. In this study, we analyzed medical records from 29 patients diagnosed with BWS and macroglossia, and evaluated 26 cases of partial glossectomy performed between 2015 and 2025. Indications, surgical technique, postoperative course, and short-term and medium-term outcomes are discussed, emphasizing the importance of early and standardized intervention. A new clinical grading for macroglossia was developed in BWS. All procedures were performed by a single surgeon in a craniofacial surgery reference center in Southern Brazil, with patients aged between 5 months and 8.9 years. Epidemiologically, 65.4% of the patients were female and the average age of surgery was 18.5 months, the median was 10 months. Macroglossia was classified as mild in 11.5%, moderate in 46.2% and severe in 42.3% of cases. The average tongue reduction was 31.6% in length and 21% in width. Retrospective analysis of medical records demonstrated appropriate functional improvement, a low complication rate, and satisfactory aesthetic and respiratory outcomes in most cases. The findings reinforce the safety and effectiveness of partial glossectomy in managing macroglossia associated with BWS.