Journal of the Korean Academy of Pediatric Dentistry 2003;30(4):667-672.
Published online November 30, 2003.
RIEGER SYNDROME : A CASE REPORT
 
Correspondence:  , 
Abstract
Rieger syndrome is a rare, autosomal dominant genetic disorder characterized by malformation of the anterior chamber of the eye(goniodysgenesis) coincident with hypodontia. It may also be accompanied by a spectrum of dental, craniofacial and somatic anomalies. Mutations in paired-like homeodomain transcription factor2(PITX2) are associated with the syndrome, and its frequency in the general population has been estimated to be 1 : 200,000. In the present case, the patient, 4 year 7 month-old female, had posterior embryotoxon and polycoria. The maxilla was retrusive in cephalometric radiography. She had congenital missing on #52, #62 and some tooth germs of permanent tooth were not detected in panoramic radiography. The purpose of this paper is to report the dental and craniofacial findings and review the pertinent literature through this case.
Key Words: Rieger syndrome, Hypodontia, Polycoria, Posterior embryotoxon, Maxillary hypoplasia


ABOUT
BROWSE ARTICLES
EDITORIAL POLICY
FOR CONTRIBUTORS
Editorial Office
Seoul National University, Dental Hospital, B1-166 101, Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
Tel: +82-70-4145-8875    Fax: +82-2-745-8875    E-mail: info@kapd.org                

Copyright © 2022 by Korean Academy of Pediatric Dentistry.

Developed in M2PI

Close layer
prev next