RIEGER'S SYNDROME

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Abstract
Three generations of a family with Rieger's syndrome are presented. Ocular and dental abnormalities constitute the major manifestations. Hypodontia, microdontia, enamel hypoplasia, missing teeth, peg-shaped teeth, and malocclusion are frequent oral findings. Hypoplasia of the maxilla causes the mandible to appear prominent, giving the patient a prognathic appearance. Aniridia, hypoplastic iris, and glaucoma are the major eye problems. The propositus also has short stature and decreased growth hormone levels which have not been reported before in this syndrome. The importance of early diagnosis is stressed in order to preserve the remaining dentition by proper dental care and to be aware of the possibility of the presence of glaucoma, which should be treated as soon as discovered.

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