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January 1978

Bilateral Optic System Aplasia With Relatively Normal Eyes

Author Affiliations

From the Laboratory of Ophthalmic Pathology (Dr Yanoff), Departments of Ophthalmology (Drs Allman and Yanoff) and Pathology (Dr Yanoff), Hospital of the University of Pennsylvania, and the Scheie Eye Institute (Drs Allman and Yanoff), Philadelphia, and the Departments of Pathologic Anatomy (Drs Rorke and Yanoff) and Ophthalmology (Dr Yanoff), the Children's Hospital of Philadelphia.

Arch Ophthalmol. 1978;96(1):97-101. doi:10.1001/archopht.1978.03910050053013

• An infant with extensive CNS malformations and aplasia of the optic system in association with grossly normal-appearing eyes was studied. The neural malformations included partial agenesis of the medulla, pons, and cerebellum and striking maldevelopment of the telencephalon and diencephalon, with lissencephaly, complete arrhinencephaly, and agenesis of the optic system. Because the optic vesicles are outgrowths from the diencephalon, the absence of some structures derived from them is not surprising.