Congenital aphakia is a rare anomaly. Despite its rarity, it appears reasonable to subdivide the condition into two groups: (a) primary congenital aphakia, in which no lens "anlage" has developed, and (b ) secondary congenital aphakia, in which a lens has developed to some degree, but has been resorbed or extruded through a corneal perforation before or during birth.
That primary congenital aphakia can occur when the optic cup has invaginated and partially developed, has been shown by examination of a human embryo at the 13 mm. stage1 in which bilateral aphakia was present, and from a number of histologically studied cases2-6 in which primary aphakia could be demonstrated. All these eyes showed severe congenital deformities.
The first sign of lens development is a thickening of the surface ectoderm in the area of contact between the ectoderm and the primary optic vesicle. The ectodermal cells (lens placode) acquire
MANSCHOT WA. Primary Congenital Aphakia. Arch Ophthalmol. 1963;69(5):571-577. doi:10.1001/archopht.1963.00960040577007