The cornea exhibits a variable degree of clouding: from a diffuse haze, to a "ground glass" appearance, with occasional focal gray spots.

[1] CHED exhibits autosomal recessive inheritance, with 80% of cases linked to mutations in SLC4A11 gene.

The SLC4A11 gene encodes solute carrier family 4, sodium borate transporter, member 11.

Multiple layers of basement membrane-like material appear to form on the posterior part of Descemet's membrane.

The corneal stroma becomes severely disorganised; the lamellar arrangement of the fibrils becomes disrupted.