J Korean Ophthalmol Soc.
1987 Oct;28(5):1143-1153.
Two Cases of Rieger's Syndrome
- Affiliations
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- 1Department of Ophthalmology, Chonnam University Medical School, Kwangju, Korea.
Abstract
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The Rieger's syndrome is characterized by the ocular anomalies include posterior embryotoxon(a prominent Schwalbe's line), tissue strands across the anterior chamber angle and variable degrees of iridic distortion and systemic defects most often involve the teeth and facial bones. These disorders are typically bilateral, are usually diagnosed at birth or in early childhood, and most have genetic basis of autosomal dominant inheritance. A large number of patients have glaucoma due to developmental defects of the anterior chamber angle structures. The authors experienced two cases of Rieger's syndrome which were occurred in a 13-year-old female in one family and in a 21 year-old male in the other family who had characteristic ocular and other systemic abnormalities associated with developmental glaucoma.