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REDUCTION GLOSSECTOMY OF MACROGLOSSIA IN BECKWITH-WIEDEMANN SYNDROME : A CASE REPORT  

Kim, Hak-Kyun (Department of Oral and Maxillofacial Surgery, College of Dentistry, Chosun University)
Kim, Eun-Seok (Department of Oral and Maxillofacial Surgery, College of Medicine, Chungnam National University)
Ko, Young-Kwon (Department of Anesthesiology and Pain Medicine, College of Medicine, Chungnam National University)
Kim, Su-Gwan (Department of Oral and Maxillofacial Surgery, College of Dentistry, Chosun University)
Publication Information
Maxillofacial Plastic and Reconstructive Surgery / v.27, no.6, 2005 , pp. 559-564 More about this Journal
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.
Keywords
Beckwith-Wiedemann syndrome; Macroglossia; Reduction glossectomy;
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