Congenital cystic eye: Report of two cases and review of the literature

A 13-month-old boy and a 2-week-old girl, who were considered to be anophth almic and who later each developed a cystic lesion in the left orbit with p rotrusion of the lower eyelid, were studied. The fellow eye in case 1 was s ubsequently found to be microphthalmic with cyst and was normal in case 2. Histopathologic study of each case revealed a cyst lined externally by dens e fibrous connective tissue to which skeletal muscle and adipose tissue wer e attached. The inner aspect of the cyst was lined by neuroglial tissue, po ssible immature retinal tissue, and cuboidal epithelium. No fully developed ocular structures or microphthalmos were identified. Fourteen cases of con genital cystic eye, including our cases, have been published in the English -language literature since 1964. We discuss and illustrate the findings in our cases and 10 others in which histopathologic findings were reported. Co ngenital cystic eye, microphthalmos with cyst, and microphthalmos with cyst ic teratoma should be suspected in patients with a small or unrecognizable eye and an orbital cystic mass that is detected by palpation or visualizati on. (C) 1999 by Elsevier Science Inc. All rights reserved.