Bilateral corneal dermis-like choristomas; an X chromosome-linked disorder
H. W. Topilow, R. C. Cykiert, K. Goldman, E. Palmer and P. Henkind
A male child was born with bilateral corneal opacification. His maternal
uncle and a male first cousin of the patient's mother also had been born
with identical bilateral corneal opacification that was pathologically
proven to result from corneal dermoids. Histopathological examination of
tissue removed from the present patient at the time of lamellar
keratoplasty revealed thickened, keratinized epithelium, absent Bowman's
membrane, and dense, irregular bands of highly vascularized collagenous
connective tissue. No adnexal appendages were identified. The remainder of
the ocular and general physical examination results was normal. In view of
these clinical and histopathological similarities, the diagnosis of corneal
dermis-like choristomas was made. To our knowledge, this report is the
first to describe a family with X chromosome-linked recessive transmission
of this condition.
