FIBROUS DYSPLASIA IN THE MAXILLO-MANDIBULAR REGION – case report

Abstract

Craniofacial fibrous dysplasia is 1 of 3 types of fibrous dysplasia that can affect the bones of the craniofacial complex, including the mandible and maxilla.Fibrous displasia is a skeletal developmental disorder of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation.It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology.Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours.The aim of this article is to represent a rare case of bilateral fibrous dysplasia of the upper and lower jaws, in combination with Intellectual disability (previously called mental retardation). The clinical diagnostic approach including imaging studies: Orthopantomogram (OPG) and 3D tomography is described. Histological examination was also essential for obtaining a definitive diagnosis.

Fibrous dysplasia: a case series of five cases

“…4,9 It occurs as a result of mutation in the gene that encodes the sub-unit of a stimulatory G protein (GS alpha) located on chromosome 20. 2,3,8,10 There is substitution of cysteine by arginine.…”

“…1,2 It is a skeletal developmental disorder of bone forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. 3,4 Reed defined fibrous dysplasia as an arrest of bone maturation. 3 It is a rare disorder characterized by deformities of bone, fractures, nerve compressions and bone pain.…”

“…3,4 Reed defined fibrous dysplasia as an arrest of bone maturation. 3 It is a rare disorder characterized by deformities of bone, fractures, nerve compressions and bone pain. 5 It can affect a single bone, or even a small segment or the entire skeleton diffusely.…”

“…1 Fibrous dysplasia as a separate entity was first described by Lichenstein and Jaffe and hence is also called as Lichtenstein Jaffe disease. [1][2][3]8,9 It is associated with many endocrinological diseases, most common being Albright syndrome. 7,8 There is no familial or hereditary or congenital basis to the disease.…”

Aneurysmal bone cyst of the maxillary sinus secondary to fibrous dysplasia: Report of a rare case and review of the literature

“…It results from abnormal fibroblast development [1]. Aneurysmal Bone Cysts (ABC) are relatively rare, benign vascular lesions and also may exist secondary to the pathological bone lesions [2].…”