title: Systemic and maxillofacial characteristics of patients with Beckwith-Wiedemann syndrome not treated with glossectomy.
abstract: BACKGROUND
  Beckwith-Wiedemann syndrome (BWS) is a genetic disorder characterized by exomphalos, macroglossia and gigantism. Previous studies reported a wide variation in the skeletal and occlusal characteristics in individuals affected by BWS. However, these studies were performed by analyzing both individuals who had and those had not received a glossectomy, which has a high impact on the jaw growth and occlusion. To highlight the intrinsic characteristics of BWS, 7 Japanese affected individuals without glossectomy were analyzed in this study.
  
  
  METHODS
  Seven individuals who had been diagnosed with BWS by medical specialists and had not undergone glossectomy were analyzed. Cephalograms and dental casts were taken and systemic complications were recorded at the first visit.
  
  
  RESULTS
  Individuals uniformly showed a higher birth height and weight, macroglossia, large anterior cranial base, and mandibular body. They exhibited a wide dental arch and an anterior open bite due to the undererupted and proclined anterior teeth. A wide variation was seen in the gonial angle, but the facial height was large overall.
  
  
  CONCLUSIONS
  As intrinsic characteristics of BWS, individuals exhibited macroglossia resulting in an anterior open bite and a wide dental arch. A long facial height and an enlarged anterior cranial base and mandibular body were also noted.
authors: Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan.
authors: Akiko
authors: A
authors: Kawafuji
authors: Naoto
authors: N
authors: Suda
authors: Naoko
authors: N
authors: Ichikawa
authors: Sumako
authors: S
authors: Kakara
authors: Tsuyoshi
authors: T
authors: Suzuki
authors: Yoshiyuki
authors: Y
authors: Baba
authors: Takuya
authors: T
authors: Ogawa
authors: Michiko
authors: M
authors: Tsuji
authors: Keiji
authors: K
authors: Moriyama
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000473:pathology*; D001724:Birth Weight; D001827:Body Height; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D003481:Cuspid / Q000473:pathology; D003724:Dental Arch / Q000473:pathology; D005145:Face; D005147:Facial Bones / Q000254:growth & development / Q000473:pathology*; D005260:Female; D005927:Glossectomy; D006801:Humans; D007180:Incisor / Q000473:pathology; D008260:Macroglossia / Q000473:pathology; D008297:Male; D008310:Malocclusion / Q000473:pathology*; D008334:Mandible / Q000473:pathology; D003765:Models, Dental; D024343:Open Bite / Q000473:pathology; D011378:Prognathism / Q000473:pathology; D019291:Skull Base / Q000473:pathology