Bilateral, congenital, dermis-like choristomas overlying corneal staphylomas
A. Bernuy, F. Contreras, A. E. Maumenee and F. E. O'Donnell Jr
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.
