Aniridia

Aniridia (congenital absence of the iris) can be inherited as a dominant trait and can be associated with congenital glaucoma. The lens can be subluxated or dislocated from birth. This might be suspected if the iris is seen to be tremulous. This strange wobbling movement of the iris used to be seen in the old days after cataract surgery without an implant, but it is now still seen after injuries to the eye and signifies serious damage. Congenital subluxation of the lens is seen as part of Marfan's syndrome (congenital heart disease, tall stature, long fingers, high arched palate). Congenital glaucoma has already been discussed in the chapter on glaucoma; it can be inherited in a dominant manner and is the result of persistent embryonic tissue in the angle of the anterior chamber. When the intraocular pressure is raised in early infancy, the eye becomes enlarged, producing buphthalmos ("bull's eye"). This enlargement with raised pressure does not occur in adults.

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