Outcomes of Tongue Reduction Surgery in Beckwith-Wiedemann Syndrome: A Systematic Review.

Abstract

Introduction: Macroglossia is a frequent clinical feature of Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth disorder. Macroglossia can lead to abnormal breathing, feeding, speech, and dentoskeletal development. Partial glossectomy is a common intervention aimed at reducing these abnormalities. The optimal timing of partial glossectomy remains controversial due to the potential need for secondary surgery and the ongoing growth of the tongue in early childhood.

Materials and methods: After PRISMA-ScR and PRISMA-S reporting standards, this systematic review included English language studies of patients with BWS who underwent partial glossectomy. Data were extracted, including patient age, clinical outcomes, and follow-up. Study evidence levels were categorized based on a recognized hierarchy, and bias was assessed using the MINORS criteria.

Results: Early tongue reduction surgery (<24 mo) was associated with a lower incidence of class 3 occlusion and anterior open bite compared with later surgery. Improvements in speech intelligibility, tongue mobility, and breathing outcomes, including a reduction in obstructive sleep apnea, were observed, especially in early surgical groups. Feeding and drooling outcomes improved across both early and late surgical interventions, although no direct comparisons were made between the 2. Overall, tongue reduction surgery demonstrated benefits in functional outcomes, whereas dentoskeletal improvements remained variable.

Conclusion: Although there is a lack of consensus to the optimal age for the procedure, overall tongue reduction surgery in BWS seems to have functional benefits, including in speech, feeding, and breathing. Dentoskeletal outcomes are more variable. Variability in macroglossia severity, surgical technique, and surgeon experience may account for differences in reported outcomes across studies.