The Beckwith-Wiedemann syndrome: a longitudinal study of the macroglossia and dentofacial complex.

Abstract

Case reports provide insights into fundamental mechanisms and also assist clinicians in treatment of similarly affected patients [Pruzansky, 1976]. The present investigation examines the natural history of the macroglossia associated with a case of Beckwith-Wiedemann syndrome (BWS) and its influence on dentofacial development. Facial skeletal growth and tongue size were assessed by analyzing cephalometric radiographs from age 2 months to 7.5 years. The data were compared with cephalometric norms and new normative data derived from 13 patients with cleft lip. The major influence of the macroglossia was protrusion of dentoalveolar structures, particularly in the lower jaw. This resulted in an anterior cross-bite in the primary dentition. In addition, an abnormally obtuse gonial angle was observed increasing the effective length of the mandible. Tongue size in BWS was generally greater than the norm, but the increase with age paralleled the mean growth curve of the tongue in the control. Over time the base of the tongue became longer and the hyoid bone moved posteriorly and inferiorly, allowing for accommodation of the tongue within the oral cavity. The changes in tongue shape and dentofacial morphology support the position that early partial glossectomy should be delayed or abandoned. In cases where tongue reduction is considered necessary, the new cephalometric normative data on tongue size provided herein can be used to establish objective criteria for such surgery.