NEVOID BASAL CELL CARCINOMA SYNDROME : A REPORT OF CASE

Heo, Su-Kyung; Choi, Nam-Ki; Kim, Seon-Mi; Yang, Kyu-Ho

Journal of the Korean Academy of Pediatric Dentistry 2008;35(4):725-730.
Published online November 30, 2008.

NEVOID BASAL CELL CARCINOMA SYNDROME : A REPORT OF CASE

Su-Kyung Heo, Nam-Ki Choi, Seon-Mi Kim, Kyu-Ho Yang

Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 2

기저세포모반 증후군 환아의 증례보고

허수경, 최남기, 김선미, 양규호

전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21

Correspondence: Nam-Ki Choi, Tel: 062-530-5668, Email: hellopedo@hanmail.net

Received: 20 March 2008 • Accepted: 24 July 2008

Abstract

Nevoid basal cell carcinoma syndrome is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. The syndrome is an autosomal dominant inherited, with a high penetration and visible expression. The syndrome is characterized by a series of associated anomalies such as cutaneous, dentofacial, skeletal, ophthalmologic, neurological, and genital anomalies. Generally, the jaw cysts are multiple odontogenic keratocysts, affecting any area of maxilla and mandible. Multiple odontogenic keratocysts of this syndrome are more recurrent than the keratocysts of non-syndrome, thus they are treated aggressively for complete removal. We report a case of multiple jaw cysts associated with nevoid basal cell carcinoma syndrome. In clinical and radiological examinations, frontal bossing, hypertelorism, mild mental retardation and two odontogenic keratocysts in both the maxilla and mandible were observed. Two cysts were treated by marsupialization. For the management of eruption of unerupted teeth, periodic recall check and orthodontic treatment are required.

Key Words: Nevoid basal cell carcinoma syndrome, Multiple odontogenic keratocysts, Marsupialization