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Management of macroglossia in Beckwith-Wiedemann syndrome.
Br J Oral Maxillofac Surg. 2013 Jan; 51(1):e6-8.BJ

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.

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Authors+Show Affiliations

Kittur MA
Dept. of Oral and Maxillofacial Surgery, Morriston Hospital, Swansea, UK. mkittur@hotmail.com
Padgett J
No affiliation info available
Drake D
No affiliation info available

MeSH

Beckwith-Wiedemann SyndromeFemaleGlossectomyHumansInfantMacroglossiaTongue

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

22341741

Citation

Kittur, M A., et al. "Management of Macroglossia in Beckwith-Wiedemann Syndrome." The British Journal of Oral & Maxillofacial Surgery, vol. 51, no. 1, 2013, pp. e6-8.
Kittur MA, Padgett J, Drake D. Management of macroglossia in Beckwith-Wiedemann syndrome. Br J Oral Maxillofac Surg. 2013;51(1):e6-8.
Kittur, M. A., Padgett, J., & Drake, D. (2013). Management of macroglossia in Beckwith-Wiedemann syndrome. The British Journal of Oral & Maxillofacial Surgery, 51(1), e6-8. https://doi.org/10.1016/j.bjoms.2012.01.015
Kittur MA, Padgett J, Drake D. Management of Macroglossia in Beckwith-Wiedemann Syndrome. Br J Oral Maxillofac Surg. 2013;51(1):e6-8. PubMed PMID: 22341741.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Management of macroglossia in Beckwith-Wiedemann syndrome. AU - Kittur,M A, AU - Padgett,J, AU - Drake,D, Y1 - 2012/02/15/ PY - 2011/12/20/received PY - 2012/01/10/accepted PY - 2012/2/21/entrez PY - 2012/2/22/pubmed PY - 2013/7/6/medline SP - e6 EP - 8 JF - The British journal of oral & maxillofacial surgery JO - Br J Oral Maxillofac Surg VL - 51 IS - 1 N2 - 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. SN - 1532-1940 UR - https://www.unboundmedicine.com/medline/citation/22341741/Management_of_macroglossia_in_Beckwith_Wiedemann_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0266-4356(12)00038-1 DB - PRIME DP - Unbound Medicine ER -