DEFINITION

The condition to be described is that of an eye which has become enlarged under the influence of increased intra-ocular pressure. As the ability of the ocular tissues to stretch is practically limited to the period of childhood, and as glaucoma in the first few years of life is almost always associated with ocular distension, the term “Congenital Glaucoma” appears suitable for this condition. By congenital glaucoma we mean a state of raised tension due to an intra-uterine defect and manifest during the first few years of life.

The definition excludes from the scope of this work true cases of infantile staphyloma, which show evidence of perforation, in which the iris is incorporated with the cornea. Of course, the hypertension that is the cause of enlargement in congenital glaucoma may not be in evidence at the time of examination. The state of raised tension may have passed, but before doing so it sets up a series of degenerative changes and interferes with the nutrition of the various tissues to such an extent that ultimate blindness is almost inevitable.

Glaucoma in children, as in adults, may be primary or secondary. The term primary is reserved for cases of obscure origin, and simply implies ignorance as to the cause. It is hoped that in time the term may become obsolete. This study is mainly concerned with primary infantile glaucoma.

Some cases of glaucoma in children are obviously due to certain malformations, of which the most common are aniridia and microphthalmia. In aniridia, glaucoma is undoubtedly often caused by the union of the iris root and the posterior surface of the cornea. In microphthalmia the narrowness of the circumlental space plays a part. As the finer changes which may hinder the function of the drainage channels are beyond our knowledge at present, our conceptions are largely hypothetical.

TITLE

Of the many different names given in the past to the condition under discussion, “Hydrophthalmia” and “Buphthalmia” have been most widely used. A. Fuchs (1924) reserved the term “buphthalmos” for the condition in which an anterior staphyloma arises in infancy and “hydrophthalmos” for “primary infantile (congenital) glaucoma”.