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Cherubism: a radiological and clinical presentation

O T Yücel¹, E Genç, S Kaya

¹Department of Otorhinolaryngology, Hacettepe University Faculty of Medicine, Ankara.

The Turkish Journal of Pediatrics

|October 9, 1998

Summary

Cherubism, a rare hereditary fibrous dysplasia of the jaws, typically self-limits and rarely requires surgery. Early recognition by pediatricians and otolaryngologists is crucial for managing this condition.

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Area of Science:

Oral and Maxillofacial Surgery
Pediatric Dentistry
Medical Genetics

Background:

Cherubism is an autosomal dominant genetic disorder characterized by fibrous dysplasia of the jawbones.
It presents as bilateral, symmetrical enlargement of the mandible and maxilla, often with a cherubic facial appearance.
While typically hereditary, sporadic cases can also occur.

Observation:

The condition is usually apparent between 2 and 7 years of age and has a self-limiting course.
Clinical presentation and radiographic findings are highly characteristic.
Surgical intervention is generally reserved for cases with severe functional impairment (respiration, deglutition, vision) or significant cosmetic concerns impacting psychiatric well-being.

Findings:

This report details a case of cherubism, including clinical presentation and radiological evaluation.
The study emphasizes the typical diagnostic features of the disease.
Discussion focuses on the clinical outcome and management strategies.

Implications:

Pediatricians and pediatric otolaryngologists must be aware of cherubism for timely diagnosis and appropriate management.
Understanding the self-limiting nature of cherubism guides conservative treatment approaches.
Early identification prevents unnecessary interventions and addresses potential psychosocial impacts.

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