A multidisciplinary approach to the treatment of oral manifestations associated with Beckwith-Wiedemann syndrome: a long-term case report

Abstract

Background: Beckwith-Wiedemann syndrome (BWS) is a congenital disorder that involves a somatic overgrowth during the patient's first years of life. Exomphalos, macroglossia and gigantism are the main clinical symptoms.

Case description: The authors describe a 15-year follow-up in a patient with BWS. They focus on a multidisciplinary approach to treating the patient's oral manifestations from age 9 months. The approach included an initial physiotherapy treatment, a partial glossectomy, a first phase of orthopedic treatment with a tongue crib and chin cap, and a second phase of orthodontic treatment with an edgewise appliance.

Clinical implications: To obtain long-term positive and stable results, an appropriate treatment plan for patients with BWS and dentoskeletal alterations, including macroglossia, requires surgical tongue reduction when the patient is young, combined with physiotherapeutic phases and orthopedic and orthodontic treatment.