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KMID : 1144020120080020113
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Journal of Korean Association for Disability and Oral Health
2012 Volume.8 No. 2 p.113 ~ p.117
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AXENFELD-RIEGER SYNDROME WITH DENTAL PROBLEM : A CASE REPORT
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Seong Min-Kyoung
Shin Teo-Jeon
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Lee Sang-Hoon
Kim Chong-Chul
Hyun Hong-Keun
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Abstract
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Axenfeld-Rieger syndrome (ARS) is a rare disorder with variable morphology characterized by malformations of the anterior segment of the eye such as iris hypoplasia, iridocorneal adhesions, corectopia and polycoria. Craniofacial and dental anomalies are also regularly reported in connection with ARS. Dental features include hypodontia in primary and permanent dentition, microdontia, short roots and abnormally shaped teeth. The patient in this case is a 14-year-old girl who has visited Seoul National Dental Hospital in 2002 for the first time. A panorama was taken on the day of her first visit, and total absence of maxillary anterior dentition and mandibular premolars were observed. The patient was on a treatment schedule by the department of ophthalmology at Seoul National Hospital for a morphological dyplasia of the pupil and she was diagnosed with ARS. Periodic panoramic observations were performed followingly, and she was finally diagnosed of congenital missing of maxillary anteriors and mandibular second premolars. In 2009, composite resin build-ups of maxillary primary central incisors were performed, and she has been on her regular follow-ups until now.
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KEYWORD
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Axenfeld-Rieger Syndrome, Dental Anomalies, Tooth agenesia
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