SCLEROCORNEA

In sclerocornea the normal translucent cornea is replaced by "scleral-like" tissue. Instead of a clearly demarcated cornea, white, feathery, often ill-defined and vascularized tissue develops in the peripheral cornea, appearing to blend with and extend from the sclera. The central cornea is usually clearer, but total replacement of the cornea with sclera may occur. The curvature of the cornea is often flatter, similar to the sclera.

Potentially coexisting abnormalities include a shallow anterior chamber, iris abnormalities, and microphthalmus. This condition is usually bilateral. In approximately 50% of reported cases a dominant or recessive inheritance has been described. Sclerocornea has been reported in association with numerous systemic abnormalities including limb deformities, craniofacial defects and genitourinary disorders. In generalized sclerocornea, early keratoplasty should be considered in an effort to provide vision.
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