What causes secondary congenital glaucoma in patients with Axenfeld-Rieger syndrome?

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

Axenfeld-Rieger syndrome

On postulated pathophysiologic mechanisms that have been abandoned, Axenfeld-Rieger syndrome also was known as anterior chamber cleavage syndrome and mesodermal dysgenesis of the cornea and iris. The basic pathology is the developmental arrest of the neural crest cells during gestation. Because these cells are the origin of facial bones and teeth, abnormalities of these structures are associated with ocular abnormalities.

In Axenfeld anomaly, a prominent, anteriorly (centrally) displaced Schwalbe line (posterior embryotoxon) and iris strands are present that reach to the angle.

When Axenfeld anomaly is associated with glaucoma, it is termed Axenfeld syndrome.

In Rieger anomaly, with or without posterior embryotoxon or iris strands, corectopia (displacement of pupil) is present due to iridocorneal adhesions that are associated with membrane covering angle; polycoria (multiple pupils) due to iris atrophy and hole formation; and ectropion uvea.

Small-sized teeth (microdontia) and decreased number of teeth (hypodontia) of anterior maxillary incisors are observed.

When the whole anomaly is associated with glaucoma, it is termed Rieger syndrome.

Lately, Axenfeld-Rieger syndrome has been used for all types of clinical presentations.


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