Congenital corneal opacification secondary to Bowman's layer dysgenesis

Progressive, bilateral corneal clouding was noted at birth in an otherwise healthy infant. There was no evidence of an infectious or hereditary cause. A trial of corticosteroids was unsuccessful, and the opacification in both corneas increased during the course of a few months, necessitating penetrating keratoplasty. However, reopacification of both grafts ensued within two to four months, and a second graft was done on the right eye. Histologic examination of both the original corneal buttons and the failed graft from the right eye showed a thickening of Bowman's layer that was three to four times greater than that of normal controls. This was associated with an increased number of keratocytes producing pools of collagen bundles within Bowman's layer. This report documents a unique form of corneal opacification unassociated with other ocular or systemic diseases. This process may be interpreted as a phenomenon in which the cells that contribute to the intrauterine formation of Bowman's layer continue to proliferate in an abnormal and exuberant fashion beyond fetal life, leading to corneal opacification.