Aggressive Cherubism: Long-Term Clinicoradiologic Follow-Up and a New Class Proposal in Its Grading

Document Type : Original Article

Authors

Oral & Maxillofacial Surgery Department, Faculty of Dentistry, Mansoura University, Egypt

Abstract

Background: Cherubism is a rare autosomal dominant disorder characterized by fibrous tissue replacement of the bone at the mandibular rami and maxillary tuberosity.
Aim of the study: This study was conducted to assess the clinical and radio- graphical features of cherubism (familial and nonfamilial) during and at the end of the progressive period of the disease; and the relation between the grade and the clinical course of the disease.
Patients and methods: Five (3 males and 2 females) patients with age range of 4-14 years at the time of diagnosis with cherubism were included in this study. The patients were graded initially and at the end of the follow-up period according to the grading system developed by Raposo-Amaral and were followed up clinically and radiographically for an average of 10 years.
Results: Two patients who were diagnosed at early age and classified as grade VI cherubism, showed aggressive early manifestation of the disease. The patients who diagnosed at elder age and had the same classification ( grade I) showed mild course of the disease, one of these patients continued mild the whole period of follow-up with no changes in the grade and total improvement after puberty. The other patient became grade III with more enlargement of the mandible and involvement of the condyles.
Conclusion: The patients who initially present with minimal involvement should only be followed up regularly and those with severe function and/or esthetic affection should be strongly considered for surgery.

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Egyptian Dental Journal
Volume 68, Issue 2 - Serial Number 2
April Oral Surgery
April 2022
Pages 1357-1366

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