Unilateral microcornea and enophthalmos with bilateral anterior and posterior anomalies.

THE term microcornea should be reserved for the condition wherein an abnormally small cornea (diameter less than 11 mm.) occurs in an otherwise relatively normal eye (Mann, 1957). Friede (1922) suggested the term "anterior microphthalmos", because the whole of the anterior segment of the eye seems to be small. The rectus muscles are inserted abnormally far forwards, and the corneal curvature tends to be increased so that the corneal refraction is relatively great and the eye tends to be myopic. The total refraction may be almost emmetropic (Mann, 1957). Vision is usually unaffected, but the condition may occur coincidentally with buphthalmos (Fransois and Neetens, 1955); or buphthalmos may be present in the other eye (Srivastava, 1961). In some cases it is difficult to distinguish between anterior microcornea and microphthalmos. Associated ocular anomalies include colobomata, persistent pupillary membrane, mesoblastic tissue in the angle of the anterior chamber, microphakia, congenital cataract, microblepharon, and smallness of the orbit and extra-ocular muscles. Friedman and Wright (1952) reported a pedigree with eight cases among 37 individuals in five generations; Martin (1888) and Gatterbauer (1943) found it in three generations of one family; Friede (1922), Peter (1924), and Sommers (1949) suggested an occasional recessive transmission. The case here presented shows a combination of anterior and posterior segmental anomalies of congenital origin, with unilateral enophthalmos on the same side as the microcornea.