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Mesodermal Dysgenesis of the Anterior Segment: Rieger's Anomaly
PAUL HENKIND, MD;
M. SIEGEL, PhD;
RONALD E. CARR, MD
Arch Ophthalmol. 1965;73(6):810-817.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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Mesodermal dysgenesis of the anterior segment, or Rieger's anomaly, is an uncommon heritable disorder.1 The developmental anomalies usually found in this condition include hypoplasia of the anterior stromal leaf of the iris, iridotrabecular adhesions or strands of noninflammatory nature, and posterior embryotoxon. These defects usually affect both eyes and are of themselves generally nonprogressive. On the other hand, secondary glaucoma (usually juvenile in onset) often occurs in this anomaly and if uncontrolled can lead to progressive ocular deterioration. While associated dental and skeletal anomalies have been reported,2-4 the pathology is most often confined to the anterior segment of the eye.
The pedigree to be reported demonstrates the broad range of expressivity associated with the disorder. It is also hoped that this presentation will clear up some of the confusion about Rieger's anomaly, particularly its relation to the so-called Axenfeld's syndrome.
Report of Cases
Pedigree S (Fig 1) comprises
. . . [Full Text PDF of this Article]
Author Affiliations
New York
From the Department of Ophthalmology, New York University Medical Center.
Footnotes
Submitted for publication Sept. 8, 1964.
Reprint requests to New York University Medical Center, 550 First Ave., New York 10016 (Dr. Henkind).
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