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June 1980

Ring Dermoid Syndrome: A New Syndrome of Autosomal Dominantly Inherited, Bilateral, Annular Limbal Dermoids With Corneal and Conjunctival Extension

Author Affiliations

From the Ophthalmology Service, Hospital Santo Toribio de Mogrovejo, Frederico Villareal University, Lima, Peru (Drs Mattos and Contreras); and the Wilmer Ophthalmological Institute, The Johns Hopkins Medical Institutions, Baltimore (Dr O'Donnell). Dr O'Donnell is now with The Bethesda Eye Institute, St Louis.

Arch Ophthalmol. 1980;98(6):1059-1061. doi:10.1001/archopht.1980.01020031049007

• 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°, extended anteriorly onto the cornea, and extended posteriorly about 5 mm within the conjunctiva for 360°. 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.

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