Sinonasal Manifestations of Severe Cherubism: A Case with 11-year Follow-up

Abstract

Cherubism, a type of fibroosteodysplasia, is a rare hereditary disease that causes variable degrees of facial deformity in children. Hypertrophy of the mandible is the most common symptom, but in severe cases, the disease affects the eyes, teeth, and sinonasal cavity. There have been few reports regarding sinonasal complications and no standard treatment has been established. This paper reports long-term treatment of severe cherubism that invaded the sinonasal cavity treated with consecutive endoscopic sinonasal surgeries.

Cherubism: Cone-Beam Computed Tomography Findings in Two Siblings

“…Cystic portions of the jawbone ossify as people age, resulting in patchy, atypical sclerosis. The tiny, compressed trabecular pattern has a typical (but nonspecific) ground-glass appearance [ 9 ].…”

“…Cystic portions of the jawbone ossify as people age, resulting in patchy, atypical sclerosis. The tiny, compressed trabecular pattern has a typical (but nonspecific) ground-glass appearance [9]. Cherubism-related syndromes include Ramon syndrome, Noonan syndrome, and neurofibromatosis type 1.…”