Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex

Abstract

Background:Owing to the overlapping clinical, radiographic and histopathological features among the diverse group of Fibro-osseous lesions, a precise and definitive diagnosis of Fibrous Dysplasia (FD) can be quite challenging. Moreover, FD itself may manifest with widely varying clinical presentations, radiographic appearances and histological pictures, depending upon the maturity of the lesion, and the relative quantum of its ‘fibrous’ and ‘osseous components’. Prompt and accurate diagnosis of Fibrous Dysplasia (FD) of the Craniomaxillofacial region is particularly important, as the condition is capable of causing considerable facial asymmetry or deformity leading even to marked disfigurement, which can have a profound psychosocial impact on the patient. Involvement of Maxillofacial bones by aggressive forms of FD, can produce serious functional debility as well, by compromising airway, breathing, vision, hearing, occlusion, mastication and mouth opening. Calvarial bone involvement can produce cranial asymmetry, and cranial base involvement can lead to persistent headaches, facial pain, numbness, and other neurological deficits owing to compression of cranial nerves.Aims and Objectives:To evaluate the importance of early and precise diagnosis, with prompt surgical management of these lesions, for a successful overall esthetic and functional outcome.Materials and Methods:A Case series of 15 patients, showcasing the principal variants of FD affecting the Craniomaxillofacial complex, namely, the Monostotic and Craniofacial forms have been described. Diversity in their Clinical, Radiographic and Histopathological presentations; their management modalities elucidating the various surgical approaches employed to access and excise these bone pathologies, have been provided along with a review of existing literature.Results:Various surgical approaches may be employed to access the lesions, depending upon their location, extent and involvement. Treatment protocols range from complete surgical excision to surgical shaving and recontouring, and must be decided upon on a case to case basis, with the aim to achieve the best possible esthetic and functional outcome with the least postoperative morbidity.Conclusion:Correlation of HPE with history, clinical features, biological behaviour, radiographic and CT appearance, laboratory findings, and intra-operative findings is imperative, so that they can be distinguished from other bony lesions and an appropriate, ideal and effective treatment modality can be instituted in time, so as to achieve the most favourable esthetic and functional outcome.

Comparison of the histopathological characteristics of diffuse sclerosing osteomyelitis of the mandible, chronic suppurative osteomyelitis, and craniofacial fibrous dysplasia

“…Subsequently, the fibrous tissue calcified to form elongated trabeculae of woven or lamellar bone and calcified spherules, similar to cementicles. 14,15 Therefore, in the CFD specimens, there are bundles of collagen fibers on the surface of bone tissue. However, there are no osteoblasts around them, and the formation of calcified spherules similar to cementicles in the bone marrow cavity is not observed.…”

“…The abnormal proliferation of fibroblasts in CFD bone tissue resulted in normal bone tissue being gradually replaced by fibrous tissue. Subsequently, the fibrous tissue calcified to form elongated trabeculae of woven or lamellar bone and calcified spherules, similar to cementicles 14,15 . Therefore, in the CFD specimens, there are bundles of collagen fibers on the surface of bone tissue.…”

“…The histopathological features of DSOM, CSO, and CFD are similar, and all three diseases present with abnormal osteosclerosis and osteolysis. 2,[12][13][14][15] According to the World Health Organization (WHO) 2017 Classification of Head and Neck Tumors, the lesion of CFD consists of woven bone with variable amounts of fibrous tissue. 16 But now, there are few reports on the histopathological characteristics of DSOM.…”

The Facial Osteoplasty for Polyostotic Fibrous Dysplasia in a Patient With McCune-Albright Syndrome: A Case Report

“…Given the possible complications, the diagnosis of fibrous dysplasia in the craniofacial region must be agile and efficient [6]. This is constructed through the association of clinical, imaging and histopathological examination [5].…”

An overview of juvenile ossifying fibroma

“…Histopathologically JOF is distinguished by the growth of tissue and the presence of mineralized elements, including woven bone trabeculae. [8][9][10] The lesion often displays a two phase pattern, with both fibrous regions. These histological features aid in distinguishing JOF from fibro conditions such as fibrous dysplasia and ossifying fibroma-treatment options for JOF range from approaches to aggressive surgical interventions.…”