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The paper is concerned with roentgenological characterization of mono- and poly-osseous fibrous dysplasia in cranial facial bones of 60 persons of both sexes and at a different age, of them 14 persons were under 14. Mono-osseous lesions after radical operations were shown to be capable of continued growth of rearranged osseous tissue in those cases when the periosteum was not completely removed.
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Fibrous dysplasia is a nonneoplastic developmental disease of osseous tissue. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. In this series of 16 patients with fibrous dysplasia of the craniomaxillofacial bones, the disease was generally monostotic and most commonly maxillary in location. Two patients demonstrated typical symptoms of the McCune Albright syndrome. Marked deformity or functional disturbances were the major indications for treatment. Total excision of the involved bone was the most successful form of treatment but produced the greatest functional and cosmetic deficits and long-term postoperative complications. A conservative therapeutic approach with a modest reduction in the bulk of these lesions may be sufficient to relieve signs and symptoms effectively. Periodic follow-up is indicated to detect recurrences or malignant changes in the early stages.
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A case of polyostotic fibrous dysplasia with increased bone density on radiographic presentation but a normal histologic appearance of dense trabecular bone is presented. Peculiar to this case of fibrous dysplasia was that treatment took place relatively late in the patient's life.
Fibrous dysplasia accounts for approximately 2% of bone tumors. The ribs, proximal femurs and cranio-facial bones represent the majority of bone lesions. Surgery is the mainstay of treatment but the technique is controversial: conservative surgery or removal of dysplastic lesions followed by implantation of autogenous bone graft. The aim of this study was to assess the indications of each method. The medical records of 25 patients with fibrous dysplasia of the cranio-facial bones treated between January 1, 1980 and December 31, 1994 at the Department of Maxillofacial Surgery, Centre Hospitalier Universitaire de Bordeaux, France, were reviewed. Fourteen (56%) patients were women and 11 (44%) men. The median age at the time of diagnosis was 23 years (ranging from 8 to 56 years). The mean follow-up was 8 years. Two patients were unavailable for follow-up after treatment. The primary sites of the tumors were the mandible (n = 19 [76%]), maxilla (n = 1 [4%]) and skull (n = 5 [20%]). For mandibular lesions, the primary treatment always included a correction of deformations and asymmetry, which was the only treatment in 14 cases. Two patients required subsequent surgery to reduce further bone enlargement (1 and 2 years later in the first case and 11 years later in the second) without further problems. In 3 cases a segmental mandibulectomy followed by implantation of autogenous bone graft was required, and no further recurrence was observed. Therefore, the success rate of conservative surgery was 74% initially, and up to 86% after subsequent surgery. Skull lesions, although often very extensive, were remarkably stable and asymptomatic. They were successfully treated 4 times by conservative surgery, mainly for cosmetic reasons. One patient, with an ethmoidal tumor producing a mass effect along the course of the optic nerve, underwent a combined cranio-facial resection. As for the only maxillary tumor, three curettages were performed throughout an 11-year period and there was no evidence of further recurrence 4 years after the last intervention. In all cases, conservative surgery may be recommended as primary treatment of fibrous of the craniofacial bones, providing essential structures like the optic nerve are not at risk. Cosmetic results and local control proved excellent, and a further removal of the tumor remained feasible in the event of a recurrence. Success or failure did not correlate with tumor size, which justifies the use of this technique.
A 25 year old patient presented with a post-traumatic soft tissue swelling of the occiput, overlying an area of bone expansion and destruction. At operation an aneurysmal bone cyst was found. 11 years previously a similar parietal lesion had been found in conjunction with fibrous dysplasia. The differential diagnosis and treatment of this entity is discussed.
Fibrous dysplasia is a benign disease with unknown etiology. Skull attempt may cause neurological disorders. The diagnostic can be made through radiological examination. A case of a patient with right temporal bone involvement with facial palsy, right parietal lobe infarctions and elevated anticardiolipin antibody titers is presented. Cerebral angiography showed occlusion of several cerebral arteries. Post mortem examination displayed recent myocardial infarction, ischemic cerebral softening, and generalized arteriosclerosis. The simultaneous occurrence of fibrous dysplasia and a probable anticardiolipin syndrome is commented.
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When fibrous dysplasia affects the temporal bone, it most often presents with conductive hearing loss attributable to stenosis of the external auditory canal. Sensorineural hearing loss has usually been attributed to involvement of the otic capsule. We present a patient with bilateral fibrous dysplasia of the temporal bones who complained of unilateral hearing loss, facial tingling, and facial twitching. The audiogram showed severe sensorineural hearing loss. The hearing markedly improved and facial twitching and tingling ceased after decompression of the internal auditory canal via a middle fossa approach. This is the only case of which we are aware showing reversal of sensorineural hearing loss caused by fibrous dysplasia.
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The authors present the case of a child with a predominantly unilateral craniofacial fibrous dysplasia and a substantial redirection of internal carotid blood flow to the dysplastic bone. The case illustrates the advantages of using multiple imaging modalities in the evaluation of this disorder. CT showed the diagnostic findings of fibrous dysplasia and demonstrated the extent of bony involvement. MRI showed the extent and vascularity of the intradiploic fibrous mass and best demonstrated the distortion of underlying cerebral structures. Tc-99m HM-PAO brain scintigraphy demonstrated adequate ipsilateral cerebral perfusion, thereby excluding any significant cerebral "steal."
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