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Semistructural allografting in bone defects after curettage.

BACKGROUND AND OBJECTIVES: A variety of aggressive benign bone tumors often require wide bone and soft tissue excision for adequate local control, but this creates a large defect and a seriously weakened extremity. Restoration of limb function presents a difficult problem. METHODS: The treatment of 104 patients with space occupying lesions of the long bone were analyzed. Deep-frozen (-70 degrees C) cortical strut allografts with or without allogeneic cancellous bone graft were implanted into the defects after extensive intralesional curettage. Thirty-six patients had fibrous dysplasias, 29 unicameral bone cysts, 22 giant cell tumors, 12 aneurysmal bone cysts, 3 benign fibrous histiocytomas, and 2 ossifying fibromas. Fifty-six patients had pathologic fracture. The average volume after curettage was 210 ml (range 60-460 ml). The average follow-up period was 50 months. RESULTS: At follow-up evaluation, the radiographs demonstrated complete incorporation of the allogeneic implant and new bone formation in the cavity in 83% of the patients (86/104). All fractures healed. There was no local recurrence or fracture of the cortical graft; neither were there other serious complications except one avascular necrosis of the femoral head. Good or excellent functional results were found in 97% (101/104) of the patients. CONCLUSIONS: For large osseous defects, the reconstructive technique using cortical stent allograft provides increased strength, easy fixation, remodeling of the cystic defect, and healing of the fracture and prevents deformity. However, remodeling occurs slowly and may never be complete.

Adolescent↗

Fibrous dysplasia associated with cortical bony destruction: CT and MR findings.

OBJECTIVE: Fibrous dysplasia is a benign disorder of bone that can cause cortical thinning as well as bony expansion. Full-thickness cortical bony destruction, however, typically characterizes more aggressive or malignant lesions of bone. We present three cases of fibrous dysplasia that illustrate this more aggressive and potentially confusing feature. MATERIALS AND METHODS: The findings on radiography and cross-sectional imaging studies are reviewed in three surgically proven cases of fibrous dysplasia that exhibited full-thickness cortical bony destruction. RESULTS: Well-defined cortical perforations without associated periosteal reaction were clearly seen on CT. This finding was suspected on plain radiography in only one of the three cases. A small associated soft tissue mass was detected by MR imaging in this case. Many cortical perforations were seen in the one case of polyostotic fibrous dysplasia. CONCLUSION: Full-thickness cortical bony destruction can be seen in fibrous dysplasia. This finding, which may only be evident on cross-sectional imaging studies, should not by itself alter a diagnostic impression of fibrous dysplasia supported by other radiographic and clinical features.

Adult↗

Evaluation of diffusion-weighted imaging for the differential diagnosis of poorly contrast-enhanced and T2-prolonged bone masses: Initial experience.

PURPOSE: To determine whether quantitative diffusion-weighted imaging (DWI) is useful for characterizing poorly contrast-enhanced and T2-prolonged bone masses. MATERIALS AND METHODS: We studied 20 bone masses that showed high signal intensity on T2-weighted images and poor enhancement on contrast-enhanced T1-weighted images. These included eight solitary bone cysts, five fibrous dysplasias, and seven chondrosarcomas. To analyze diffusion changes we calculated the apparent diffusion coefficient (ADC) for each lesion. RESULTS: The ADC values of the two types of benign lesions and chondrosarcomas were not significantly different. However, the mean ADC value of solitary bone cysts (mean +/-SD, 2.57 +/- 0.13 x 10(-3) mm(2)/second) was significantly higher than that of fibrous dysplasias and chondrosarcomas (2.0 +/- 0.21 x 10(-3) mm(2)/second and 2.29 +/- 0.14 x 10(-3) mm(2)/second, respectively, P < 0.05). None of the lesions with ADC values lower than 2.0 x 10(-3) mm(2)/second were chondrosarcomas. CONCLUSION: Although there was some overlapping in the ADC values of chondrosarcomas, solitary bone cyst, and fibrous dysplasia, quantitative DWI may aid in the differential diagnosis of poorly contrast-enhanced and T2-prolonged bone masses.

Adolescent↗

Hydroxyapatite cranioplasty in fibrous dysplasia of the skull.

We describe the case of a woman who had complained of worsening headache during the previous year. Analysis of computed tomography imaging and biopsy led to a diagnosis of fibrous dysplasia of the skull. A symptomatic frontal bone lesion was removed, and the resulting defect was repaired with hydroxyapatite cement. Hydroxyapatite cement has several advantages over hydroxyapatite ceramic blocks and granules, and it is particularly well suited for calvarial reconstruction.

Adult↗

Computed tomography of fibrous dysplasia.

Skeletal fibrous dysplasia produces changes that are usually readily recognized on plain radiographs. Occasionally, routine radiography may not demonstrate the characteristic appearance of the disease. The density of abnormal bone in craniofacial fibrous dysplasia may preclude adequate assessment of areas where soft-tissue impingement may occur. Computed tomography (CT) is useful in demonstrating the amorphous "ground-glass" texture of the lesion and in defining the extent of craniofacial disease including impingement upon orbital structures. CT was useful in five patients with fibrous dysplasia in whom the nature or extent of involvement was not entirely clear.

Adult↗

Reversible visual loss caused by fibrous dysplasia.

Fibrous dysplasia is a developmental anomaly of bone, often affecting the facial bones. We treated a patient who had fibrous dysplasia involving the right maxillary and sphenoid bones. The patient had a sudden loss of visual acuity to R.E.: counting fingers. Visual fields demonstrated a central scotoma, and retrobulbar neuritis was diagnosed. When vision failed to improve spontaneously, however, radiologic studies were performed. These showed compromise of the optic canal by bony proliferation and an apparent mucocele at the orbital apex. Surgical exploration disclosed fibrous dysplastic bone and a cystic structure overlying the optic nerve. Successful surgical excision of the cyst and debulking of the fibrous dysplasia resulted in visual acuity returning to R.E.: 20/25.

Adult↗

Monostotic fibrous dysplasia of the lumbar spine: case report and review of the literature.

Fibrous dysplasia has been frequently reported to involve the spine in the polyostotic form, but only rarely has monostotic fibrous dysplasia been noted. In the only previously reported case involving the lumbar spine, the disease was confined to the transverse process. The present case demonstrates monostotic fibrous dysplasia involving the vertebral body in addition to the posterior elements. The plain radiographic, computerized tomography, and histologic examinations are presented.

Adolescent↗

Breast Cancer in a Patient with McCune-Albright Syndrome.

We treated a patient with breast cancer associated with McCune-Albright syndrome. A 40-year-old woman with a history of precocious puberty visited our hospital complaining of a mass in the upper lateral quadrant of the right breast. Although bone scintigraphy revealed multiple high uptake of 99mTc, plain X-ray demonstrated ground-glass appearance, suggesting fibrous dysplasia rather than bone metastases. Serum levels of tumor markers and alkaline phosphatase were within the normal range. Breast cancer associated with McCune-Albright syndrome was diagnosed, and she subsequently underwent breast conserving surgery, excision of abdominal wall myxoma and bone biopsy of the left clavicula. The bone lesion was histologically confirmed as fibrous dysplasia. Although McCune-Albright syndrome isa rare clinical entity, it should be considered as a possible differential diagnosis of bone metastasis in patients with breast cancer. As recent molecular studies have suggested genetic mutations in McCune-Albright syndrome, this syndromemay possibly predispose patients to breast cancer.

Journal Article↗

Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment.

We reviewed the long-term outcomes of treatment of fibrous dysplasia of the proximal part of the femur in twenty-two patients (twenty-seven femora). There were fifteen male patients and seven female patients. Patients who had monostotic disease had no involvement of the calcar femorale, fewer microfractures, less deformity, and stronger bone that could support internal fixation. Patients who had polyostotic disease had frequent involvement of the calcar femorale; more microfractures; severe deformity, including shepherd's crook deformity; and, in many instances, bone that could not support internal fixation. Twenty-two of the twenty-seven femora had a microfracture at the time of the initial presentation. At least one osteotomy was performed in four femora that had monostotic disease and in nine femora that had polyostotic disease. Curettage and cancellous or cortical bone-grafting did not appear to have any advantage compared with osteotomy alone in the treatment of symptomatic lesions, as all grafts resorbed with persistence of the lesion. At the time of the latest follow-up evaluation, no lesion had been eradicated or had decreased in size. A satisfactory clinical result was achieved in twenty patients (twenty-four femora): nine who had monostotic disease and eleven who had polyostotic disease. Two patients who had polyostotic disease and an endocrinopathy (one of whom had bilateral involvement) had an unsatisfactory result. All three femora in these two patients had a neck-shaft angle of less than 90 degrees at the time of the most recent follow-up evaluation. Varus deformity of the proximal part of the femur is best treated with valgus osteotomy and internal fixation early in the course of the disease. If the calcar of the femoral neck is involved or if the quality of the bone is such that internal fixation is not possible, a medial displacement valgus osteotomy can provide a more mechanically favorable position for healing of the microfracture.

Adolescent↗

Fibrous dysplasia.

DEFINITION: Fibrous dysplasia (FD) of bone is a non-inheritable congenital disorder affecting both genders. It is characterized by expanding fibrous lesions, which contain bone-forming mesenchymal cells. PATHOPHYSIOLOGY: FD is caused by a somatic activating mutation of the alpha subunit of the Gs protein (Gsalpha). Bone mesenchymal cells produce a matrix of randomly distributed collagen fibres and islands of woven bone. Osteoclasts are responsible for the spread of the lesions. CLINICAL FINDINGS: The symptoms are bone pain, fracture, bone deformities and neurological deficits. Spontaneous regression of lesions does not occur. TREATMENT: Recently, an observational study of treatment with bisphosphonate has yielded promising results. There was a decreased intensity of bone pain, a decrease in biochemical markers of bone turnover and a radiographically apparent 'refilling of osteolytic sites' in about half of the patients. CONCLUSIONS: Very little is known about the effects of bisphosphonate treatment in children and adolescents with FD. Most patients report decreased bone pain after the first pamidronate infusion, which, in our view, justifies the use of this drug in severely affected patients. The many unanswered questions regarding this form of treatment can only be addressed when a large number of patients is treated in a standardized fashion, and data on the outcome are collected.

Adolescent↗

The benign fibroosseous lesion: its association with paranasal sinus mucoceles and its MR appearance.

Although fibrous dysplasia and ossifying fibroma of the facial bones may, with some difficulty, be distinguishable pathologically, they are inseparable radiographically. Based on a study of 30 patients, there was no good or reliable imaging correlation with the histology and the degree of ossification or the bone(s) involved. Therefore, "benign fibroosseous lesion" has been proposed by several noted bone authorities as the term for these entities. Eleven patients had MRI and the overall signal intensities were low on both T1-weighted and T2-weighted sequences. Areas of high signal intensity on T2-weighted imaging correlated with either cysts within the lesion or the presence of mucocele, especially in the frontoethmoid region. Four such mucoceles are presented. The coexistence of mucoceles and benign fibroosseous lesions may be more common than previously reported. It is both realistic and appropriate for the radiologist to diagnose these entities as benign fibroosseous lesions and leave the final histologic diagnosis to the pathologist who can diagnose most such cases, albeit with some difficulty. Sites of high signal intensity on T2-weighted imaging in the frontoethmoid area should raise the possibility of a coexistent mucocele.

Adolescent↗