What causes secondary congenital glaucoma in patients with Peters anomaly?

Updated: Jul 27, 2020
  • Author: Inci Irak Dersu, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

Peters anomaly

Peters anomaly is characterized by bilateral congenital central corneal opacity, which is associated with iridocorneal adhesions toward the defective area.

The peripheral cornea is clear, and the total cornea is of normal size in most cases.

Peters anomaly results from the absence or thinning of endothelium, Descemet membrane, posterior corneal stroma, and sometimes Bowman layer.

Large fibroblastic cells fill into this space and adhere to the counterpart iris section.

Several mechanisms have been proposed, as follows: anoxia, infection (eg, rubella), and a mechanism that is similar to Axenfeld-Rieger anomaly.

Most cases of Peters anomaly have a sporadic origin. A mutation in the PAX6 gene on chromosome 11 has been identified.

The mechanism of glaucoma is reported as a problem in differentiation from neural crest cells, causing trabecular meshwork anomalies, such as absence of the Schlemm canal.


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