Bilateral, congenital, dermis-like choristomas overlying corneal staphylomas

Two unrelated patients with diffuse, bilateral, congenital tumefactions overlying the corneas were seen. The tumors were protuberant, fleshy masses that partially concealed underlying corneal staphylomas. Spontaneous rupture was suspected in one eye. Histopathologic study of three specimens disclosed dermis-like tumors. In one specimen, an apocrine gland was identified, thus supporting the diagnosis of choristoma. An evisceration specimen from a ruptured globe confirmed that the ectatic cornea was an anterior staphyloma. The posterior pole seemed grossly normal. This disorder may have a genetic basis or it may result from a developmental or inflammatory insult in utero. Preoperative diagnosis is important because the associated anterior staphylomas complicate management.