Contributor: Gordon K. Klintworth
In spherophakia both lenses are usually involved. Relative to the lens diameter the anteroposterior axis of the lens is increased. The anterior pole of the lens may be in apposition to the posterior surface of the cornea. Spherophakia can be transmitted as an autosomal recessive disorder. Sometimes spherophakia is an isolated anomaly. It may also accompany an abnormal anterior chamber angle, ectopia lentis and pupil, a persistent tunica vasculosa lentis and megalocornea. Such associations may be manifestations of Marfan syndrome, Weill-Marchesani syndrome and perhaps hyperlysinemia. An uncomplicated spherophakia is caused by an arrest in the lens development at about the fifth or sixth month of development when it is normally spherical.