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- DOI 10.18231/j.jds.96348.1759140597
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CrossMark
Cherubism: A rare hereditary skeletal dysplasia manifesting as bilateral maxillofacial swellings – A case report and review of the literature
- Author Details:
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Naga Supriya Alapati *
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Chinmayee Mannava
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Ravikanth Manyam
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Swetha Pasupuleti
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Jyothi Sangineedy
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Kishore Moturi
Naga Supriya Alapati *
Chinmayee Mannava
Ravikanth Manyam
Swetha Pasupuleti
Kishore Moturi
Background: Cherubism is a rare, hereditary skeletal dysplasia, predominantly affecting children. It is characterized by painless, bilateral, symmetrical bony swellings localized to the maxillofacial region, often resulting in a distinctive "cherubic" appearance. The condition typically regresses spontaneously after puberty. Early diagnosis is essential for appropriate management and differentiation from other similar disorders.
Case Description: We report a case of an 11-year-old female child presenting with unilateral bony swelling in the maxillofacial region, which later evolved into bilateral lesions. Radiographic examination revealed bilateral multilocular cystic spaces, a hallmark feature of cherubism. Histopathology confirmed the diagnosis, showing perivascular eosinophilic cuffing around giant cells. The therapeutic approach for this patient was tailored to her specific functional and aesthetic needs, focusing on conservative management as spontaneous regression was expected.
Conclusion: Cherubism is a condition that requires a comprehensive approach for diagnosis, including clinical, radiological, and histopathological evaluation. Clinician awareness is crucial for distinguishing cherubism from other similar disorders. Early intervention can help manage functional and aesthetic concerns while monitoring for spontaneous regression during puberty.
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