Version 1
: Received: 12 June 2023 / Approved: 13 June 2023 / Online: 13 June 2023 (15:46:01 CEST)
How to cite:
Assimi, S.; Cherkaoui, A. Ramon Syndrome: A Rare Case of Cherubism with Gingival Fibromatosis in an Adult Female. Preprints2023, 2023060953. https://doi.org/10.20944/preprints202306.0953.v1
Assimi, S.; Cherkaoui, A. Ramon Syndrome: A Rare Case of Cherubism with Gingival Fibromatosis in an Adult Female. Preprints 2023, 2023060953. https://doi.org/10.20944/preprints202306.0953.v1
Assimi, S.; Cherkaoui, A. Ramon Syndrome: A Rare Case of Cherubism with Gingival Fibromatosis in an Adult Female. Preprints2023, 2023060953. https://doi.org/10.20944/preprints202306.0953.v1
APA Style
Assimi, S., & Cherkaoui, A. (2023). Ramon Syndrome: A Rare Case of Cherubism with Gingival Fibromatosis in an Adult Female. Preprints. https://doi.org/10.20944/preprints202306.0953.v1
Chicago/Turabian Style
Assimi, S. and Amine Cherkaoui. 2023 "Ramon Syndrome: A Rare Case of Cherubism with Gingival Fibromatosis in an Adult Female" Preprints. https://doi.org/10.20944/preprints202306.0953.v1
Abstract
Abstract: Cherubism is a rare genetic disorder characterized by painless bilateral expansion of the mandible and maxilla. We present an unusual case of a 50-year-old female patient with Ramon syndrome, a rare variant of cherubism, accompanied by gingival fibromatosis. The patient exhibited symptoms of short stature, mental retardation, rheumatoid arthritis, epilepsy, hirsutism, and gingival enlargement. Clinical examination revealed generalized gingival inflammation with plaque and attachment loss. Radiographically, bilateral defined multilocular radiolucencies were observed in the mandibular body and ramus. The patient underwent a non-surgical phase, including oral hygiene motivation and periodontal debridement, followed by a surgical phase involving gingivoplasty and internal bevel gingivectomy with open flap debridement. Histopathological analysis confirmed the presence of fibroblasts, multinucleated giant cells, and fibrous connective tissue. The patient's son also presented with bilateral facial swelling and similar radiographic findings, indicating familial inheritance of cherubism. The case demonstrates the clinical, radiographic, and histopathological features of cherubism associated with gingival fibromatosis and highlights the challenges in diagnosis and management. Regular follow-up and maintenance are essential for long-term stability and prevention of recurrence. This report contributes to the limited literature on cherubism in older patients and emphasizes the importance of early identification and intervention in affected individuals.
Medicine and Pharmacology, Dentistry and Oral Surgery
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
