Cherubism: Cone-Beam Computed Tomography Findings in Two Siblings

Area of Science:

• Genetics
• Pediatric Dentistry
• Oral and Maxillofacial Surgery

Background:

• Cherubism is an uncommon, autosomal dominant, non-neoplastic, fibro-osseous condition affecting the jaws in children.
• It is characterized by bilateral, symmetrical enlargement of the jaw, leading to dental malocclusion and facial disfigurement.

Observation:

• This case report details two siblings presenting with painless, bilateral, symmetrical enlargement of the maxilla and mandible.
• The condition, though self-limiting, can cause significant functional issues such as phonation difficulties, airway compromise, visual disturbances, and psychological distress in severe forms.

Findings:

• Cherubism manifests as multilocular cystic lesions within the jawbones.
• The fibro-osseous nature of the lesions distinguishes it from other cystic jaw pathologies.

Implications:

• Early diagnosis and monitoring are crucial for managing cherubism.
• Surgical management is typically reserved for functional impairments or significant aesthetic concerns and is often postponed until skeletal maturity post-puberty.