Henry Ford Hospital Medical Journal

Article Title

Hereditary Sclerocornea


A rare case of total hereditary sclerocornea is presented along with clinical history, histopathology, and a review of the possible pathogenesis and current literature on the subject. It is quite possible that imperfect cleavage of the anterior chamber angle may be the predisposing factor in scleralization of the cornea due to a failure of neural crest cell differentiation. If surgical intervention to correct for the corneal opacification is indicated, corneal transplantation should be performed as soon as possible after birth, similar to the indications for removal of dense congenital cataracts to avoid deprivation amblyopia.