Journal of the Korean Association of Oral and Maxillofacial Surgeons

The Korean Association of Oral and Maxillofacial Surgeons (대한구강악안면외과학회)
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A CASE REPORT OF MULTIPLE ODONTOGENIC KERATOCYSTS ASSOCIATED WITH BASAL CELL NEVUS SYNDROME

기저세포모반증후군과 관련된 악골의 다발성 치성각화낭종의 치험례

  • Byun, June-Ho (Dept. of Oral & Maxillofacial Surgery, College of Dentistry, Pusan National University) ;
  • Park, Seong-Hee (Dept. of Oral & Maxillofacial Surgery, College of Dentistry, Pusan National University) ;
  • Kim, Uc-Kyu (Dept. of Oral & Maxillofacial Surgery, College of Dentistry, Pusan National University) ;
  • Park, Hae-Ryoun (Dept. of Oral Pathology, College of Dentistry, Pusan National University) ;
  • Chung, In-Kyo (Dept. of Oral & Maxillofacial Surgery, College of Dentistry, Pusan National University)
  • 변준호 (부산대학교 치과대학 구강악안면외과학교실) ;
  • 박성희 (부산대학교 치과대학 구강악안면외과학교실) ;
  • 김욱규 (부산대학교 치과대학 구강악안면외과학교실) ;
  • 박혜련 (부산대학교 치과대학 구강병리학교실) ;
  • 정인교 (부산대학교 치과대학 구강악안면외과학교실)
  • Published : 2000.06.30
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Abstract

The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.

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