Total Reconstruction and Rehabilitation with Vascularized Fibula Graft and Osseointegrated Teeth Implantation after Segmental Mandibulectomy for Fibrous Dysplasia

Fibrous dysplasia is a benign fibro-osseous disease that often occurs in young children and adolescents. It is characterized by a loss of normal bone production and abnormal proliferation of fibrous connective tissue with poorly formed trabeculae of woven bone. Lichtenstein1 was the first to suggest the term “fibrous dysplasia” and noted that the lesion can occur not only in multiple bones (polyostotic) but also in a single bone (monostotic). Subsequently, this terminology was approved universally.2 Fibrous dysplasia accounts for 2.5 percent of all bony tumors and 7 percent of all benign neoplasms of bone.3 In general, the more extendsive the disease, the earlier the onset of symptoms. The monostotic form of the disease is much more common (70 percent) than the polyostotic variety (20 percent). The rest of fibrous dysplasia cases are syndromic, like Albright syndrome and cherubism.4,5 Although fibrous dysplasia may occur in any bone of the body, the jaws and skull are among the most commonly affected bones.6,7 The maxilla is more commonly affected than the mandible.6,8 However, maxillary lesions not uncommonly involve adjacent bones separated by sutures (i.e., zygoma, sphenoid, occiput), so in this sense, they are not strictly monostotic. Clinically, craniofacial fibrous dysplasia usually presents as a slowly enlarging, painless, nonmobile, nontender, and hard swelling. The onset is often gradual, so that the patient only seeks treatment when severe facial deformity or functional disturbance is present. Lesions located at the jaws are more ossified, and expansion of the buccal cortical plate is more frequent than expansion on the lingual side.9,10 Proliferation of the tumor may cause interference in tooth eruption, displacement, or missing teeth at the site of the lesion, leading to malocclusion.6,7 Macroscopically, the lesion appears grayish white or grayish red and is highly vascular,10 with a gritty texture due to the presence of newly formed bone spicules.7 Under the microscope, the lesion is essentially fibrous, consisting of proliferating fibroblasts in a compact stroma of interlacing collagen fibers. Occasional foci of calcification or cartilaginous tissue may be found. There is no sharp demarcation between the dysplastic bone and the adjacent normal bones. Some lesions of the jaw may undergo a progressive maturation in which the proportion of fibrous tissue diminishes with increasing age while calcification increases.10