Bilateral Microcornea with Cornea Plana in a Family

ABSTRACT

Microcornea is a condition characterized by a small cornea in an otherwise normal eye and can be defined when corneal diameter is less than 11.00mm. Microcornea may occur as an isolated abnormality or accompany other anterior segment anomalies like aniridia and anterior chamber cleavage abnormalities. Microcornea is part of nanophthalmos, a condition in which a reduction in global dimensions is the only structural abnormality, as well as microphthalmos, a term generally, used to signify a small, abnormal globe. Microcornea is a rare anomaly and its etiology is probably a primary aberration of the growth of the ectoderm of the optic cup. Both autosomal dominant and autosomal recessive patterns of inheritance occur, but it may appear sporadically. Cornea plana is usually seen in association with microcornea wherein the corneal curvature is less than normal. It is often associated with diffuse opacities of the cornea stroma. Cornea plana might be caused by a deep developmental arrest in the fourth month, at which time the cornea begins to increase its curvature relative to the sclera. The authors experienced a case of bilateral microcornea with cornea plana in a family which seemed to transmit as the dominant trait.