Maxillofacial Plastic and Reconstructive Surgery

Korean Association of Maxillofacial Plastic and Reconstructive Surgeons (대한악안면성형재건외과학회)
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Treatment of Keratocystic Odontogenic Tumors in Nevoid Basal Cell Carcinoma Syndrome

  • Ryu, Hyun-Ho (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Shin, Dong-Yoon (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Ryu, Seok-Hwan (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Kim, Taek-Woo (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Lee, Jong-Bum (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Kim, Bok-Joo (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center) ;
  • Kim, Chul-Hoon (Division of Oral and Maxillofacial Surgery, Department of Dentistry, Dong-A University Medical Center)
  • Received : 2013.08.02
  • Accepted : 2014.01.10
  • Published : 2014.01.30
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Abstract

Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.

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References

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