Orthodontic evaluation of eight cases in Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome is a congenital anomaly. It was reported by Beckwith [Beckwith JB. Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic visceromegaly. In: Bersma D, editor. Part II. Malformation syndromes, birth defects: original article series, vol. 5, no. 2. White Plains: The National Foundation-March of Dimes; 1969. p. 188-96] in 1969 and Wiedemann [Wiedemann HR. Familial malformation complex with umbilical Aherina and macroglossia-a "NEWSYNDROME"? Genet Hum 1964; 13:223-32] in 1964, and is characterized by the triad of exomphalos, macroglossia, and gigantism and hypoglycemia in newborns. This syndrome is, therefore, also known as EMG syndrome [Niikawa N, Ishikiriyama S, Takahashi S. The Wiedemann-Beckwith syndrome: pedigree studies on five families with evidence for autosomal dominant inheritance with variable expressivity. Am J Med Genet 1986; 24:41-55]. Orthodontically, open bite and reversed occlusion are often observed effects of gigantism and macroglossia, although a number of studies have found no common maxillofacial morphological characteristics between these two disorders [Kamogashira K, Itoh T, Nakagawa M. Orthodontic findings in a case of Beckwith-Wiedemann syndrome. J Jpn Orthod Soc 1984;43:564-72; Friede H, Figueroa A. The Beckwith-Wiedemann syndrome: a longitudinal study of the macroglossia and dentofacial complex. J Craniofac Genet Dev Biol Suppl 1985;1:179-87]. We analyzed intraoral findings and lateral cephalograms (cephalometric analysis) obtained at initial consultation in eight patients with Beckwith-Wiedemann syndrome who visited our department. We found the common maxillocraniofacial problem among them to occur solely in the vertical plane (increased gonial angle and open bite). There were no common anteroposterior problems. These differences in the anteroposterior growth of the mandibular body may be closely associated with whether macroglossia, as an external factor, was improved by surgery before eruption of the permanent teeth. In terms of dental problems, the only common finding observed was open bite. The position and function of the tongue and perioral muscles during speech and swallowing may differ among patients, thus resulting in maxillocraniofacial morphology characteristic to each patient. (C) 2006 Elsevier Ltd and the Japanese Orthodontic Society. All rights reserved.