This disorder is generally considered to consist of simple displacement of the pupil and dislocation of the lens (usually in opposite directions). However, other abnormalities are often present such as persistent pupillary membrane (87%), iridohyaloid adhesions, increased corneal thickness, enlarged corneal diameters, and axial myopia. The iris may transilluminate (67%) and the pupils dilate poorly. Iridodenesis is common (85%). The lens is often malformed and in some cases frankly microspherophakic. The lens displacement can progress and cataracts seem to form at a relatively young age. Visual acuity is highly variable, ranging from 20/20 to light perception depending upon the density of cataracts which often develop at a relatively young age. Prominent iris processes into the anterior chamber angle have been reported and glaucoma, both acute and chronic, is sometimes seen. Retinal detachment is a risk.
Studies in families with ectopia lentis et papillae have revealed that as many as 50% of individuals with dislocated lenses do not have ectopic pupils.