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True anophthalmos is defined by Duke-Eider' as lacking any essential eye structures whatsoever. Because the presence of some amount of ocular tissue is a requisite stimulus for orbital development,2 the condition should be differentiated from cases of "clinical anophthalmos" (which may include extreme microphthalmos3), from congenital cystic eyeball, from microphthalmos with cyst, and from crypthophthalmos. Congenital anophthalmos traditionally has been difficult to diagnose: Sassani and Yanoff require histological sectioning to demonstrate absence of a rudimentary globe.4 In the living pediatric patient in whom blepharophimosis and a seemingly empty socket give rise to suspicion of congenital anophthalmos, definitive diagnosis heretofore has had to await surgical exploration under anesthesia.
We have recently confirmed the clinical diagnosis of true congenital anophthalmos in two infants using A- and B-scan ultrasonography.
CASE REPORTS
Patient No. 1
C.B., a female, was born in July 1 977, two weeks prematurely to a 24-year-old, G-2, P-2 black woman following a completely normal pregnancy. The child weighed...