Axenfeld–Rieger Syndrome: A Case Report and Brief Review

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 multidiscip...

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Bibliographic Details
Main Authors: Amina Sultan, Simran Uppal, Akanksha Juneja
Format: Article
Language:English
Published: Jaypee Brothers Medical Publisher 2024-04-01
Series:Journal of South Asian Association of Pediatric Dentistry
Subjects:
Online Access:https://www.jsaapd.com/doi/JSAAPD/pdf/10.5005/jp-journals-10077-3291
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Summary: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.
ISSN:2582-1024