J Korean Assoc Maxillofac Plast Reconstr Surg.
2005 Nov;27(6):559-564.
Reduction glossectomy of macroglossia in beckwith-wiedemann syndrome : a case report
- Affiliations
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- 1Department of Oral and Maxillofacial Surgery, College of Dentistry, Chosun University, Korea.
- 2Department of Oral and Maxillofacial Surgery, College of Medicine, Chungnam National University, Korea.
- 3Department of Anesthesiology and Pain Medicine, College of Medicine, Chungnam National University, Korea.
Abstract
- Beckwith-Wiedemann syndrome is an autosomal dominant growth excess disorder, which occurs with a reported incidence of 1 in 13,700 to 1 in 17,000 live births. It constitutes a discrete clinicopathologic entity characterized by macroglossia, abdominal wall defects (omphalocele), visceromegaly, gigantism, hemihypertrophy, hypoglycemia, and the increased risk of solid tumor development from multiple cell lines. A macroglossia is a key component of the syndrome, and can lead to cosmetic, functional and psychologic disorder. This report shows a 5-year-old patient with Beckwith-Wiedemann syndrome, who had macroglossia and received reduction glossectomy.
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