Bilateral congenital ocular cysts.

This report presents an unusual case of bilateral congenital ocular cysts. These are in the spectrum of congenital cystic eye and microphthalmos with cysts. Diagnostic evaluation, including orbital B-scan ultrasonography, computed tomography and magnetic resonance imaging is described. Pathologic evaluation of the orbital cysts was consistent with congenital cystic eye. An associated microphthalmic eye was not detected with serial sectioning of both specimens. Examination of the orbit at exploration as well as pre- and post-operative MRI scans failed to demonstrate the presence of a microphthalmic eye in either orbit. Failure to detect a microphthalmic eye clinically, by diagnostic imaging studies, or serial microscopic sections of the cysts supports the diagnosis of congenital cystic eye. This child has associated mild facial clefting (median cleft lip and cleft palate) and basal cephalocele. Histopathologically documented cases of ocular abnormalities associated with basal encephalocele have not been reported. To the authors' knowledge, MRI findings of congenital ocular cysts have been reported once previously in a case of microphthalmos with cyst, however, histopathologic correlation has not been reported previously.