Journal of South Asian Association of Pediatric Dentistry

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VOLUME 7 , ISSUE 1 ( January-April, 2024 ) > List of Articles

CASE REPORT

Axenfeld–Rieger Syndrome: A Case Report and Brief Review

Amina Sultan, Simran Uppal, Akanksha Juneja

Keywords : Axenfeld–Rieger syndrome, Case report, Everted umbilicus, Glaucoma, Hypodontia, Oligodontia

Citation Information : Sultan A, Uppal S, Juneja A. Axenfeld–Rieger Syndrome: A Case Report and Brief Review. J South Asian Assoc Pediatr Dent 2024; 7 (1):50-54.

DOI: 10.5005/jp-journals-10077-3291

License: CC BY-NC 4.0

Published Online: 27-04-2024

Copyright Statement:  Copyright © 2024; The Author(s).


Abstract

Axenfeld–Rieger Syndrome (ARS) is a rare autosomal dominant genetic disorder with an overall incidence of 1:200,000 that has mainly ocular, periumbilical, and dental abnormalities like oligodontia/hypodontia, microdontia, and delayed eruption of permanent teeth. Its management requires a multidisciplinary approach involving a team of dentists, ophthalmologists, cardiologists, and general physicians, along with regular follow-up. Pediatric dentists play a crucial role in such patients, as a timely diagnosis could prevent ocular complications such as glaucoma in later stages. This paper presents a case report with the classical signs and symptoms of ARS in a 12-year-old female patient demonstrating oligodontia, microdontia, and short roots.


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