Cherubism.
Cherubism is a rare inherited fibro-osseous disorder that affects the jaws, producing a characteristic facial appearance. Patients are normal at birth; however, by their second or third year of life they develop a fullness of the lower one half of their face and retraction of the lower eyelids that exposes a rim of sclera below the iris. The facial appearance is similar to a Renaissance cherub with its gaze toward heaven. A case of cherubism in a 19-year-old male, complicated by severe orbital compression resulting in diplopia, is presented. The patient was diagnosed at three years of age when he developed the characteristic cherub facies and severe dental anomalies that required surgical correction. At age 14, he underwent facial contouring surgery of his jaws. Orbital compression later developed due to lesions growing in the floor of his orbits. This was corrected by way of a subciliary approach to the orbit. The patient's facial appearance and visual function were markedly improved by the surgery. A review of the literature detailing this rare disease is discussed.
Marck
P A
PA
Department of Surgery, University of Alberta, Walter MacKenzie Health Sciences Centre, Edmonton, Canada.
Kudryk
W H
WH
D000328:Adult; D002636:Cherubism / Q000145:classification / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed