Contributor: Gordon K. Klintworth
Peters anomaly is a central corneal leukoma without inflammation associated with defective underlying corneal endothelium and Descemet membrane. The lens may be normal, but is often cataractous and displaced anteriorly. It is often adherent to the to corneal defect on the posterior surface of the lens. A congenital corneal staphyloma may be present. The collagen fibers in the central deep stroma have a larger diameter than normal and are arranged in coarse bundles. Bowman layer is thickened. Iris strands extend from the iris border to the margin of the corneal opacity. Developmental glaucoma [glaucoma - secondary developmental] is commonly assocaited. Peters anomaly is commonly bilateral (80% of cases). Associated chromosomal aberrations include partial trisomy 16q, the 18-q syndrome [chromosome 18 - deletion long arm] and 13 deletion syndrome [chromosome 13 - deletion], and occasionally the Wolf-Hirschorn syndrome. Some cases of Peters anomaly result from a mutation in the CYP1B1 or PAX6 gene.