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May 1963

Primary Congenital Aphakia

Author Affiliations

Rotterdam, Netherlands
From the Central Pathological Laboratory, Municipal Hospitals, Rotterdam.

Arch Ophthalmol. 1963;69(5):571-577. doi:10.1001/archopht.1963.00960040577007

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.

Embryology  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

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