[Congenital cystic eye]

Three patients with congenital cystic eye are described. Clinical manifestations of the condition were absence of the eyeball with massive cystic lesions occupying the orbit and protrusion of eyelids. Contralateral eye may be involved. This abnormality can be associated with systemic developmental abnormalities. Histopathologically there was no ocular structure in the orbit. The cyst wall consisted of two layers. The outer layer consisted of fibrous connective tissue and the inner layer of neuroectodermal tissue with a high level of antibodies to neuronspecific enolase. The cyst contained light yellowish fluid similar to cerebrospinal fluid. Etiology, differential diagnosis. and treatment of the condition are discussed.