Computed Tomography in Craniofacial Fibrous Dysplasia: A Case Series with Review of Literature and Classification Update

Abstract

Introduction:Fibrous dysplasia (FD) is a fibroosseous lesion of the osseous structures of the body. It is not a commonly reported lesion yet it is considered as an important lesion which can affect the maxillofacial region as well. As a result, it can cause deformity of the jaw bones which can further lead to severe facial asymmetry. Craniofacial fibrous dysplasia (CFD) is one of the subtypes of FD that can affect the bones of the craniofacial complex, including the mandible and maxilla. It can also present as facial asymmetry and can be investigated with the help of Maxillofacial Radiology and Imaging. The radiographic findings may vary according to the extent and degree of the disease. Although conventional radiographs provide a good clue regarding the lesion, advanced maxillofacial imaging is capable of providing detailed extent of the disease. Furthermore the classification of CFD is not very clear in the literature. Case Report:This particular paper attempts to document and report the CT appearance of CFD with an attempt to propose a better classification system for the same. Four different patients are reported which presented with FD with involvement of bones of craniofacial region. Working diagnosis of CFD was made with the help of clinical features as well as with radiographic assessment. Advanced imaging included CT scan of the lesions. The article highlights the importance of computed tomography in diagnosis as well as assessment of extent of the disease. Conclusion:It can be concluded that the Dental professionals must be aware regarding the different radiographic appearances of CFD. Advanced imaging modality like CT can provide with exact diagnosis as well as extent of the lesions like FD. Further collaboration of researchers is required to incorporate this proposed change in classification of CFD.

First two cases of craniomaxillofacial fibrous dysplasia from Nepal – case series

“…However, the diagnostic gold standard for any disease is histopathology, but in the case of craniomaxillofacial fibrous dysplasia, many authors have claimed that radiography and CT play a remarkable role in diagnosis, treatment planning, and in follow-up of the case. 3 Radiographically, ground glass appearance is seen with no visible trabecular pattern. Grossly the lesion is usually well circumscribed, dense with tan-gray color, and may vary in consistency from fibrous to gritty due to the presence of bone.…”

“…A lot of controversies exist whether to classify the craniomaxillofacial fibrous dysplasia into monostotic or polyostotic form. 3 , 11 , 12 In a recent review, the authors proposed that craniofacial fibrous dysplasia involving the bones attached to each other and present in the same anatomic area, separated only by sutures, can be considered as monostotic only if no other bone in the body is involved. On the other hand, two separate anatomic areas within the craniofacial skeleton showing involvement must be categorized as polyostotic craniofacial dysplasia.…”

“…On the other hand, two separate anatomic areas within the craniofacial skeleton showing involvement must be categorized as polyostotic craniofacial dysplasia. 3 …”

“…A third form known as craniofacial fibrous dysplasia is considered a separate entity by some authors; however, others believe it to be a form of polyostotic form of fibrous dysplasia involving multiple cranial bones. 3 Here, we are presenting two cases of craniomaxillofacial form of fibrous dysplasia, which are most likely the first two cases to be reported and published from Nepal.…”

“… 2 This mutation results in proliferation and differentiation of preosteoblasts that eventually affects the bone, replacing its marrow by fibrous tissue and randomly arranged woven bone. 3 The consequences can range from asymptomatic defects to severe bone deformities and increased fragility involving either one bone (monostotic) or multiple bones (polyostotic). Monostotic form is more common compared to polyostotic form.…”