Ramon Syndrome- A Rare Form of Cherubism

Abstract

Cherubism is an inherited, autosomal dominant disorder that affects the jaws of children. The disease is usually obvious as a painless bilateral swelling in which bone is replaced with fibrous tissue. Affected children appear normal at birth. Swelling of the jaws usually occurs between 2 and 7 years of age and relapses as age progresses leaving a few facial deformities and malocclusion. The disease is microscopically indistinguishable from other giant cell lesions. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred to as a rare case of possible Ramon syndrome with extraordinary tissue enlargement over the teeth. Here, we present a case of Ramon syndrome in a 6-year-old girl describing the clinical and radiographic features successfully treated with a brief review of literature.

Keywords: Cherubism; Ramon syndrome; fibro-osseous lesions.