Determinants of impaired quality of life in patients with fibrous dysplasia.

BACKGROUND: Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. Data were compared with those of the general Dutch population. RESULTS: Out of 138 patients from a cohort of 255 patients with fibrous dysplasia that were sent questionnaires assessing quality of life and pain, the response rate was 70.3%, with 97 patients, predominantly female (65%), completing the questionnaires. Monostotic fibrous dysplasia was predominant (n = 62, 64%). Fibrous dysplasia patients had significantly lower quality of life outcome scores than the general Dutch population for all tested domains of the Short Form-36 except for the "Mental health" and the "Role emotional" domains. More severe forms of fibrous dysplasia, had the more severe Short-Form-36 quality of life outcomes, but there was no significant difference in Brief Pain Inventory domains between different subtypes of fibrous dysplasia. Quality of life was lower in patients with higher disease burden, as reflected by high skeletal burden scores (p = 0.003) and high levels of P1NP (p = 0.002). CONCLUSION: We demonstrate impairments in all domains of quality of life, except for 'Mental health' and 'Role emotional' domains, across the wide spectrum of fibrous dysplasia including its milder forms. We identified high skeletal burden scores, reflecting disease severity, as the most consistent predictor of impaired quality of life. Our findings hold significant clinical implications as they draw attention to the clinically unmet need to address quality of life issues in the management of patients with all subtypes of fibrous dysplasia, including its milder forms.

Treatment of fibrous dysplasia of the proximal femur by means of pedicled iliac crest bone graft: a case report.

In this report, we present a case of treatment of fibrous dysplasia (FD) of the proximal femur with the pedicled iliac crest bone graft. An 18-year-old patient presented with hip pain and polyostotic dysplasia with involvement of the proximal femur and a history of pathological fracture. The patient was operated on using vascularized bone graft from the iliac crest and osteosynthesis with Dynamic Hip Screw (DHS®). With vascularized bone graft, we found an improvement on X-ray with no reabsorption, and with osteosynthesis, we controlled the pain and prevented pathological fracture and progression of the deformity. Several other studies where the pedicled iliac crest bone graft has been successfully used for the management of defects in the proximal femur (osteonecrosis of the femoral head and pseudarthrosis of the femoral head) can be found in the medical literature. However, the pedicled iliac crest bone graft in a patient with FD of the proximal femur is unique.

Displasia fibrosa da mandíbula / Fibrous dysplasia of mandible

A displasia fibrosa da mandíbula é uma patologia óssea benigna que representa cerca de 2% de todos os tumores ósseos. Relatamos o caso de uma paciente de 11 anos de idade, com queixa de dor em região mandibular esquerda há 1 semana, com história de abaulamento da mesma região há 3 meses. Após realização de exames de imagem, foi diagnosticado displasia fibrosa da mandíbula. O objetivo deste relato é revisar as principais características clínicas, radiológicas e possíveis tratamentos para o caso, dando a devida importância para o seguimento ambulatorial pós-tratamento

Fibrous dysplasia is a benign bone disease which represents about 2% of all bone tumors. Here we report the case of an 11-year-old who complained of pain at the left mandible area for a week, with history of arching in this area for 3 months. Imaging examinations led to the diagnosis of fibrous dysplasia of the mandible. The aim of this work is to review the main clinical and radiological characteristics and possible treatments for the case, giving due importance to ambulatory follow-up care

Role of surgical management in temporal bone fibrous dysplasia.

CONCLUSION: Surgical treatment of fibrous dysplasia of the temporal bone (TBFD) revealed relatively good outcomes. Also, active surgical intervention is necessary to prevent the formation of secondary cholesteatoma or other complications. In particular, FD involving the inner ear may require greater caution when deciding on the resection area and timing of surgery. OBJECTIVES: The purpose of this study was to analyze clinical manifestations of TBFD and its surgical outcomes. PATIENTS AND METHODS: Nine patients diagnosed with TBFD after surgery at three tertiary referral centers were included. Pre- and postoperative clinical, audiological, and radiological findings were analyzed retrospectively. RESULTS: Most patients (88.9%) showed FD involving the mastoid and adjacent area. Radiological patterns consisted of six pagetoid and three sclerotic forms; seven patients were monostotic, whereas two patients were polyostotic. Canalplasty (CP) was performed in six cases and intact canal wall mastoidectomy was performed in three of them; hearing gain was achieved in five and one patient maintained preoperative normal hearing. Two patients underwent revision CP and showed no recurrence. In addition, one case had secondary cholesteatoma formed by an obstruction of the external auditory canal; another with a fast-growing cystic mass in the petrous bone underwent transmastoid labyrinthectomy and had no recurrence for 11 months.

Monostotic fibrous dysplasia: a 23-year follow-up of a patient with spontaneous bone remodeling.

Fibrous dysplasia is a benign fibro-osseous disease that affects one or more bones. Although its etiology has been defined, the mechanism of spontaneous resolution is still unclear. There is strong evidence indicating the occurrence of stabilization when bone maturation is completed. Deformities that lead to esthetic and functional disorders are observed in almost all cases. Plastic surgery is often recommended when the maxilla and mandible are involved. In the case of mild deformities, careful follow-up during skeletal growth is recommended. We describe here the 23-year follow-up of a patient with monostotic fibrous dysplasia whose disease had stabilized by 13 years of follow-up.

Fibrous dysplasia of the first cervical vertebra.

STUDY DESIGN: Case report. OBJECTIVE: To describe a case of monostotic fibrous dysplasia of the first cervical vertebra. SUMMARY OF BACKGROUND DATA: Fibrous dysplasia is a disorder of bone development of unknown etiology in which cancellous bone is replaced with fibrous tissue, resulting in expansion of the bone. The disease presents in either a monostotic or polyostotic form. Although fibrous dysplasia can involve any bone in the body, vertebral involvement is quite unusual. METHODS: A case of monostotic fibrous dysplasia of the first cervical vertebra is presented. RESULTS: A 53 year-old woman presented with a 1-month history of occipito-cervicalgia associated with great restriction of neck movements. Computed tomography and magnetic resonance imaging revealed an expansile, destructive lesion involving the right lateral mass of the first cervical vertebra, without involvement of the spinal cord. She was submitted to surgery and the lesion was removed. Microscopic examination was consistent with the diagnosis of fibrous dysplasia. Four years after surgery there was no clinical or imagiological recurrence. CONCLUSION: Although there are descriptions of vertebral fibrous dysplasia, this is the 12th case of monostotic fibrous dysplasia of the cervical spine described in the literature. Computed tomography and magnetic resonance imaging were essential for making the preoperative diagnosis.

Hallazgos radiológicos en la displasia fibrosa monostótica / Neuroradiological findings in monostotic fibrous dysplasia

No disponible

No disponible

Fibrous dysplasia.

Fibrous dysplasia is a developmental disorder of bone that can present in a monostotic or polyostotic form. Primarily affecting adolescents and young adults, it accounts for 7% of benign bone tumors. Many of the asymptomatic lesions are found incidentally; the remainder present with symptoms of swelling, deformity, or pain. Fibrous dysplasia has been associated with multiple endocrine and nonendocrine disorders and with McCune-Albright and Mazabraud's syndromes. The etiology remains unclear, but molecular biology suggests a mutation in the G(s)alpha subunit and activation of c-fos and other proto-oncogenes. Fibrous dysplasia has a characteristic radiographic appearance. Most cases do not require intervention, but those that do usually are managed surgically with curettage, bone grafting, and, in some cases, internal fixation. When some intervention is necessary but surgery is not practical, treatment is with bisphosphonates. The prognosis generally is good, although poor outcomes are more frequent in younger patients and in those with polyostotic forms of the disease. The risk of malignant transformation is low.

Fibrous dysplasia in the maxilla: possible mechanism of bone remodeling by calcitonin treatment.

The long-term follow-up case of monostotic fibrous dysplasia of the maxilla in a 10-year-old girl is described with her endocrinologic data and therapeutic consequence of calcitonin administration in association with surgical interventions. The fibrous dysplasia tends to become more quiescent or static after skeletal growth ceases, but the causative has been still unknown to date. In this case reported changes of calciotropic hormones in the serum were well corresponding to the ceasing of the tumor growth at the puberty and reflected to the calcitonin administration. Although calcitonin has been applied to the fibrous dysplasia associated with McCune Albright syndrome, no histologic changes have been described after the calcitonin treatment. This report might be the first description of bone remodeling after the calcitonin treatment for the fibrous dysplasia of the maxilla in immature people. Data of this case may provide a clue to the pathogenesis of fibrous dysplasia. Surgical intervention can be performed after the local bone calcification by a calcitonin treatment, because of alleviation of vigorous hemorrhage by the bone remodeling.

Dental management of fibrous dysplasia.

This report describes two patients with monostotic fibrous dysplasia affecting maxillary alveolar processes. The long-term care for patient ML between ages 6 and 28 years included the transplantation of a premolar and a third molar tooth into the affected area, but the transplanted tooth 14 had to be extracted 6 years later. The patient JW, who presented at age 5 with an asymptomatic swelling in the left deciduous molar and first permanent molar region, was followed for 15 years. Teeth 24, 25, and 26 were developmentally absent. The roots of the deciduous teeth of both patients resorbed normally, and were replaced by tissue with the same radiographic appearance as fibrous dysplasia.