Congenital cystic eye: recurrence after initial surgical removal

Congenital cystic eye arises from failure of invagination of the primary optic vesicle. The cyst lacks the usual intraocular structures and contains only abortive derivatives of the anterior and posterior portions of the primary optic vesicle. Because the cystic eye usually occupies a large portion of the orbit and distends the upper eyelid, surgical excision is often carried out. We report a case of congenital cystic eye in which an initial attempt at excision was followed in three months by recurrence of the cyst in the orbit. A second excision appears to have eliminated the cyst since no recurrence has become evident in the ensuing two years. The pathology of the cyst from both excision specimens suggests that the pigmented cells from the posterior portion of the cyst were not included in the first resection. Based on this experience, we conclude that every effort should be made to excise all of the congenital cystic eye when surgical removal is undertaken.