Bilateral microphthalmos without microcornea associated with unusual papillomacular retinal folds and high hyperopia

A 23-year-old man had bilateral microphthalmos without microcornea. The eyes measured about 15 mm in length by ultrasound while the corneas were 11 mm in diameter. A distinctive, elevated, funnel-shaped retinal fold stretching between the disk and the macula was present bilaterally. Other unusual aspects were the deep anterior chambers and uncompromised chamber angles. This combination of anomalies is believed to be the result of a failure of growth of the posterior outer coats of the eye. These were of insufficient size to accommodate the excess sensory retina which folded to conform to its outer structural shell. Other cases of high hyperopia may in some instances be cases of microphthalmos, unrecognized because of normal-sized corneas.