Literature DB >> 22341741

Management of macroglossia in Beckwith-Wiedemann syndrome.

M A Kittur1, J Padgett, D 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.
Copyright © 2012 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

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Year:  2012        PMID: 22341741     DOI: 10.1016/j.bjoms.2012.01.015

Source DB:  PubMed          Journal:  Br J Oral Maxillofac Surg        ISSN: 0266-4356            Impact factor:   1.651


  3 in total

Review 1.  Obstructive sleep apnoea and the role of tongue reduction surgery in children with Beckwith-Wiedemann syndrome.

Authors:  Christopher M Cielo; Kelly A Duffy; Aesha Vyas; Jesse A Taylor; Jennifer M Kalish
Journal:  Paediatr Respir Rev       Date:  2017-02-24       Impact factor: 2.726

2.  The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome.

Authors:  Jennifer L Cohen; Christopher M Cielo; Jonida Kupa; Kelly A Duffy; Evan R Hathaway; Jennifer M Kalish; Jesse A Taylor
Journal:  Plast Reconstr Surg       Date:  2020-04       Impact factor: 4.730

3.  Perioperative airway management of a patient with Beckwith-Wiedemann syndrome.

Authors:  Masanori Tsukamoto; Takashi Hitosugi; Takeshi Yokoyama
Journal:  J Dent Anesth Pain Med       Date:  2016-12-31
  3 in total

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