Ring dermoid syndrome. A new syndrome of autosomal dominantly inherited, bilateral, annular limbal dermoids with corneal and conjunctival extension
J. Mattos, F. Contreras and F. E. O'Donnell Jr
A new hereditary syndrome of bilateral ocular dermoids is described in five
patients from three generations of a single family. There were no
associated extraocular anomalies. The choristomas involved the limbus for
360 degrees, extended anteriorly onto the cornea, and extended posteriorly
about 5 mm within the conjunctiva for 360 degrees. Additional clinical
findings of diagnostic value were conjunctival plaques of keratinization,
hairs, and corneal lipid deposition. An irregular corneal astigmatism,
amblyopia, and concomitant strabismus were secondary features. The unique
bilateral, annular configuration of the dermoids is pathognomonic of this
syndrome. Early surgical intervention is indicated to try to improve the
visual prognosis and cosmesis. At surgery, the conjunctival portion of one
case was easily resected in toto, but the limbal portion required partial
excision. Histopathological examination confirmed the clinical diagnosis of
dermoid choristoma.
