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
  • 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.

Keyword

Beckwith-Wiedemann syndrome; Macroglossia; Reduction glossectomy

MeSH Terms

Abdominal Wall
Beckwith-Wiedemann Syndrome*
Cell Line
Child, Preschool
Gigantism
Glossectomy*
Humans
Hypoglycemia
Incidence
Live Birth
Macroglossia*
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