title: Management of macroglossia in Beckwith-Wiedemann syndrome.
abstract: Beckwith-Wiedemann syndrome (BWS) is a rare congenital disease of low prevalence. Its most common feature is macroglossia, being present in most cases. Clinically macroglossia can compromise the airway, cause dysphagia, drooling and poor cosmesis. Functionally it can cause dentoskeletal discrepancy, protrusion of teeth, and speech abnormalities leading significant psychological issues both with the patient and their families. Surgical excision remains the mainstay of management and a variety of techniques have been described. Because of the high vascularity of the tongue, intra-operative blood loss could be significant and have high morbidity in the pediatric patient. We present a technique for tongue reduction in macroglossia associated with BWS using the Ultrasonic Dissector (Harmonic Scalpel). The principles of Ultrasonic dissection are discussed along with the potential advantages of the technique.
authors.affiliation: Dept. of Oral and Maxillofacial Surgery, Morriston Hospital, Swansea, UK. mkittur@hotmail.com
authors.forename: M A
authors.initials: MA
authors.lastname: Kittur
authors.forename: J
authors.initials: J
authors.lastname: Padgett
authors.forename: D
authors.initials: D
authors.lastname: Drake
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D005260:Female; D005927:Glossectomy / Q000295:instrumentation / Q000379:methods*; D006801:Humans; D007223:Infant; D008260:Macroglossia / Q000151:congenital* / Q000601:surgery; D014059:Tongue / Q000002:abnormalities / Q000601:surgery*