The emphasis here on the term anophthalmos vera is necessary. Difficulty in distinguishing the several similar conditions has led to much confusion in the literature.
It is indeed rare to observe a case of true anophthalmos. Any interference in the growth of an embryo sufficient to produce complete suppression of the front of the forebrain is more likely to terminate development of the embryo; the embryo would be too abnormal to survive.
Allied aberrations which are often confounded with anophthalmos vera are distinguishable embryologically from it. These are congenitally cystic eyeball and microphthalmos.
Anophthalmos vera may be primary or secondary. The primary variety results from a total failure of one or both primary optic vesicles to bud out from the thalamencephalon. No trace of the ectodermal structures of the eye is formed, though there may have been differentiation of mesoderm so that an orbit is present. The autopsies in the
APPELBAUM MA, MARMELSTEIN M. CONGENITAL BILATERAL ANOPHTHALMOS VERA WITH UNILATERAL POLYDACTYLY AND CLEFT PALATE: REPORT OF A CASE. Arch Ophthalmol. 1943;29(2):258–265. doi:10.1001/archopht.1943.00880140104005
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