These are caused by failure of the embryonic fissure to close during the second month of development in utero. The iris defect is also known as a "keyhole" pupil. Here, there is not only an iris defect, but also missing tissue in the lens (yellow arrow) and zonules. This can cause difficulties during cataract surgery and capsular tension rings may be required. Some associations to look out for include retinal coloboma, microphthalmia, amblyopia, strabismus, glaucoma and CHARGE syndrome. Treatment can be considered for symptomatic glare and can include blue blocking spectacles and cosmetic contact lenses.
Uploaded on: 09/18/2020