Maxillofacial Plastic and Reconstructive Surgery

Korean Association of Maxillofacial Plastic and Reconstructive Surgeons (대한악안면성형재건외과학회)
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REDUCTION GLOSSECTOMY OF MACROGLOSSIA IN BECKWITH-WIEDEMANN SYNDROME : A CASE REPORT

Beckwith-Wiedemann 증후군 환자에서의 거대설 절제술

  • 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)
  • 김학균 (조선대학교 치과대학 구강악안면외과학교실) ;
  • 김은석 (충남대학교 의과대학 구강악안면외과학교실) ;
  • 고영권 (충남대학교 의과대학 마취통증의학교실) ;
  • 김수관 (조선대학교 치과대학 구강악안면외과학교실)
  • Published : 2005.12.31
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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

References

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