Management of macroglossia in Beckwith-Wiedemann syndrome

The British Journal of Oral & Maxillofacial Surgery
M A KitturD Drake

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.

References

Mar 11, 2003·Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics·Joy WangM Anthony Pogrel
Feb 22, 2007·Plastic and Reconstructive Surgery·Jillian K TomlinsonJohn G Meara
Sep 4, 2007·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·Codruta NeumannHolger Sudhoff
Dec 4, 2008·Plastic and Reconstructive Surgery·Yoav KaufmanJoseph Edmonds
Jun 26, 2009·Head & Neck Oncology·Yoann PonsClaude Conessa

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Citations

Mar 8, 2005·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Carine LarocheBernard Devauchelle
Dec 29, 2015·Paediatric Respiratory Reviews·Andreas Pfleger, Ernst Eber
Sep 8, 2017·Journal of Dental Anesthesia and Pain Medicine·Masanori TsukamotoTakeshi Yokoyama
Mar 30, 2020·Plastic and Reconstructive Surgery·Jennifer L CohenJesse A Taylor
Apr 4, 2017·Paediatric Respiratory Reviews·Christopher M CieloJennifer M Kalish

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Beckwith-Wiedemann syndrome is an imprinting disorder characterized by overgrowth, congenital malformations and predisposition to tumors. Discover the latest research on Beckwith-Wiedemann Syndrome here.