Contributor: Gordon K. Klintworth
Sclerocornea (scleralization of the cornea) is a non-progressive developmental anomaly of the cornea in which all or part of the the cornea of one or both eyes is opaque with features of sclera. Sclerocornea is sporadic in half of the cases, but some cases are inherited with autosomal dominant or autosomal recessive modes of inheritance. Sclerocornea frequently occurs with cornea plana but may occur as an isolated developmental anomaly. Aniridia, anterior segment ocular dysgenesis, colobomas, cataract, glaucoma, and microphthalmos are associated. The corneal opacification may be diffuse or widespread and may be accompanied by a widespread malformation. The opaque corneal stroma contains coarse bundles of collagen fibers that are larger than normal. Most of Descemet membrane and corneal endothelium are absent. Sclerocornea may be part of a systemic syndrome involving the skull and facial bones, ears, brain, digits, and testes. It has been associated with osteogenesis imperfecta and blue sclera, trisomy 18 and an unbalanced chromosomal translocation (17p10q) [chromosome translocation syndrome]. It may also be part of the MIDAS syndrome.