The Natural Evolution of Macroglossia Among Beckwith-Wiedemann Patients: A 30-Year Institutional Review

Macroglossia is a hallmark of Beckwith-Wiedemann syndrome (BWS). Tongue reduction surgery is classically performed to address such issues, but its necessity in patients without airway or feeding compromise remains debated. This study evaluates long-term outcomes of conservative management.

A retrospective review of patients with BWS seen from 1993 to 2023 was performed. Data collected included demographics, comorbidities, occlusion, cephalometrics, and speech outcomes. Dental and dento-skeletal measures were compared with age- and sex-matched standards.

Of 59 patients, 28 met the inclusion criteria (mean age: 14.3±4.0 y). Comorbidities included attention deficit hyperactivity disorder (29%) and autism spectrum disorder (21%). None required eventual tongue reduction, whereas 2 (7.1%) underwent orthognathic surgery for maxillo-mandibular discrepancies. Class III dental (molar) malocclusion persisted in 57%, though mean overbite, overjet, and maximal opening were within normative ranges. Cephalometrics showed mandibular length within expected norms, whereas mandibular width and Wits values were more often outside normal limits. Speech evaluations revealed long-term articulation difficulties in 80%, mainly interdental sigmatism, with less frequent resonance, voice, fluidity, or intelligibility issues. No feeding issues were present in the long term.

In patients with BWS without airway or feeding difficulties, conservative management of macroglossia results in acceptable long-term outcomes. Malocclusion and articulation issues are present but manageable without invasive interventions. Nearly all patients avoid tongue reduction or orthognathic surgery. There seems to be a tendency for tongue accommodation by the mandible in its antegonial width.