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
HENKIND P, SIEGEL M, CARR RE. Mesodermal Dysgenesis of the Anterior Segment: Rieger's Anomaly. Arch Ophthalmol. 1965;73(6):810-817. doi:10.1001/archopht.1965.00970030812011