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Axenfeld-Rieger 증후군과 연관된 치과적 이상

Other Titles
 Dental anomalies associated with Axenfeld-Rieger syndrome 
 김기림  ;  이두영  ;  김승혜  ;  이상휘  ;  최병재  ;  이제호 
 Journal of Korean Association for Disability and Oral Health, Vol.6(2) : 94-98, 2010 
Journal Title
Journal of Korean Association for Disability and Oral Health(대한장애인치과학회지)
Issue Date
Axenfeld-Rieger syndrome ; Dental anomalies ; Glaucoma ; Congenital missing teeth
Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities
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2. College of Dentistry (치과대학) > Dept. of Oral and Maxillofacial Surgery (구강악안면외과학교실) > 1. Journal Papers
2. College of Dentistry (치과대학) > Dept. of Pediatric Dentistry (소아치과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Seung Hye(김승혜)
Lee, Sang Hwy(이상휘) ORCID logo https://orcid.org/0000-0002-9438-2489
Lee, Jae Ho(이제호) ORCID logo https://orcid.org/0000-0002-1556-3485
Choi, Byung Jai(최병재)
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