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Cherubism: Report of a case.

Vikas Elias Kuruvilla¹, Varghese Mani, N Bilahari

¹Department of Oral and Maxillofacial Surgery, PSM College of Dental Science, Akkikkavu, Thrissur, India.

Contemporary Clinical Dentistry

|October 15, 2013

Summary

Cherubism, a rare jaw disorder, causes progressive swelling in children. This case highlights clinical and radiographic features of cherubism in a 4-year-old, offering therapeutic insights.

Keywords:

Cherubism mandible pathogenesis

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Area of Science:

Pediatric Dentistry
Oral and Maxillofacial Surgery
Genetics

Background:

Cherubism is a rare, autosomal dominant genetic disorder characterized by bilateral, symmetrical enlargement of the jaw.
The condition typically presents within the first few years of life and progresses until puberty, often showing spontaneous remission thereafter.
Histologically, cherubism involves the replacement of normal bone with fibrovascular tissue containing numerous multinucleated giant cells.

Observation:

A 4-year-old child presented with significant, progressive swelling on both sides of the mandible.
Clinical examination revealed characteristic facial disfigurement associated with cherubism.
Radiographic imaging demonstrated the typical ill-defined, radiolucent lesions replacing the mandibular bone.

Findings:

The case presented confirms the typical clinical and radiographic manifestations of cherubism in a young child.
The observed jaw enlargement was consistent with the progressive nature of the disorder.
Histopathological examination, though not detailed in the abstract, is characteristic for cherubism.

Implications:

Early diagnosis and monitoring of cherubism are crucial for managing potential complications such as malocclusion and airway obstruction.
Understanding the natural course of cherubism aids in providing appropriate supportive care and anticipating remission.
This case underscores the importance of a multidisciplinary approach involving dentists, surgeons, and geneticists for optimal patient management.