Purpose: To show a well-documented clinicopathologic correlation of lacrimal gland choristoma of the iris and ciliary body, to review the literature on the subject, and to recommend treatment of this condition.
Methods: A congenital iridociliary mass was removed by iridocyclectomy when a female infant was 19 months old because it had produced a large cyst, secondary glaucoma, a vascularized corneal pannus, focal cataract, and iris heterochromia secondary to pigment dispersion. The excised mass was studied by routine histopathologic methods.
Results: The mass proved histopathologically to be a lacrimal gland choristoma composed of mature lacrimal gland tissue associated with a large epithelial-lined cyst and several smaller cysts. Postoperatively, the glaucoma resolved and the child had visual acuity of 20/40 14 months after surgery.
Conclusions: Lacrimal gland choristoma of the anterior uvea has typical clinical features that should indicate that correct diagnosis. It is a fleshy, often cystic, lesion with an irregular, cerebriform surface that appears identical to the lacrimal gland in the orbit. Although the mass itself is generally stable, secondary cysts in the lesion can enlarge and secondary glaucoma and other complications can develop. Consequently, affected patients should be followed up at close intervals and surgical excision performed if such complications begin to develop.