Abstract

STUDY DESIGN: A case report.
PURPOSE:To report ocular, dental and facial abnormalities in Axenfeld Rieger
Syndrome(ARS).
Method: A 13-year-old male was brought to OPD with complaints of diminution of vision in both eyes since childhood,born out of 1st degree consanguinous marriage.General examination showed telecanthus,a broad,flat nasal bridge.Dental abnormalities also seen.Redundant umbilical skin,hypospadias noted. Ocular examination:BCVA BE:6/18.BE-microcornea,corectopia and posterior
embryotoxon.IOP:BE-normal,Gonio:BE open angles with iris processes.BE-normal fundus.
RESULT: Based on complete examination a provisional diagnosis of
ARS was made.Patient was prescribed spectacles for better vision.
CONCLUSION: ARS is an uncommon autosomal dominant disorder.Risk of glaucomatous vision loss is more.Therefore,the importance of early diagnosis, follow-up and adequate treatment becomes evident in order to preserve the visual function of patients and thus avoid an unfavorable evolution.