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anterior chamber cleavage syndrome


Definition: a congenital disorder originating from faulty separation of embryonic structures; it results in bilateral central corneal opacities, with an anterior ring attachment of the iridic pupillary border and anterior polar cataracts; associated with short-limbed dwarfism; these are autosomal dominant and autosomal recessive forms, caused by mutation in the PAX6 gene, the PITX2 gene, the CYP1B1, or the FOXC1 gene.

See: iridocorneal endothelial syndrome

Synonym(s): Peters anomaly

Further information

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.