Some cases show autosomal dominant inheritance, some autosomal recessive inheritance and some sporadic.
Clinical features:
(1) Scleral tissue involves the cornea, ranging from peripheral to diffuse involvement. With diffuse involvement the cornea is white, flattened and opaque.
(2) The limbus is abnormal and may show blood vessels associated with the encroaching scleral tissue.
(3) The condition is usually bilateral.
(4) The condition is noninflammatory.
(5) Accompanying ocular findings may include cornea plana, strabismus, nystagmus, anterior chamber abnormalities, glaucoma and microphthalmos.
(6) Sclerocornea may be accompanied by non-ocular congenital malformations.
A patient with severe compromise of vision will often undergo corneal transplant.
Glaucoma is a common complication and may be difficult to control.
Differential diagnosis of corneal whitening: congenital glaucoma, dermoid, Peter's anomaly and response to corneal ulceration
