Year : 2017

Volume : Volume 7

Issue : Issue 3

Address for correspondence :
Gauri Madan
GauriMadan@gmail.com

Abstract

Background: Axenfeld-Rieger syndrome is a rare genetic condition characterized by ocular, dental, craniofacial, periumbilical  and other variable systemic abnormalities. A young female presented for replacement of missing teeth. She had negligible previous dental attendance, impaired vision consequential to pediatric glaucoma and displayed craniofacial anomalies, dental anomalies, oligodontia and periumbilical redundant skin. A diagnosis of Axenfeld-Rieger syndrome was made. Unilateral Stahl ear deformity and mandibular condyle hypoplasia were noted. Dental prostheses and genetic counseling were provided. A missed case of Axenfeld-Rieger syndrome was diagnosed prosthodontically treated and novel findings were noted.

Keywords: Axenfeld-Rieger Syndrome, oligodontia, Stahl ear, condyle hypoplasia, PITX2.