Open AccessAxenfeld-Rieger Syndrome in Monozygotic Twin Brothers: Case Report
Author(s) -
Betül İlkay Sezgin Akçay
Publication year - 2021
Publication title -
i̇stanbul kuzey klinikleri
Language(s) - Uncategorized
Resource type - Journals
ISSN - 2148-4902
DOI - 10.14744/nci.2021.89577
Subject(s) - medicine , glaucoma , dysgenesis , iris (biosensor) , pathognomonic , hypospadias , monozygotic twin , hypoplasia , intraocular pressure , ophthalmology , dental anomalies , surgery , anatomy , disease , orthodontics , computer security , biology , computer science , biometrics , genetics
Axenfeld-Rieger syndrome (ARS) is a genetic disease affecting multiple organ systems. In the eye, it is associated with anterior segment dysgenesis with a high risk for glaucoma. Dental anomalies, cardiovascular malformations, hypospadias, and craniofacial abnormalities are other associated systemic conditions. Five years old monozygotic twin brothers with ARS were referred to Umraniye Training and Research Hospital, ophthalmology clinic for iris abnormalities. At presentation, pathognomonic components of ARS were found in both patients, including iris anomaly (corectopia, iris hypoplasia, and iris strands in Scwalbe's ring), oligodontia, hypodontia, hypospadias, and periumbilical skin fold. Intraocular pressure was within normal ranges in both of the patients. Patients were followed up in the glaucoma unit.