Monostotic fibrous dysplasia with epiphyseal involvement

Clinical Cases 27.08.2002
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 16 years, female
Authors: A. López-Medina, L. Oleaga, M. González de Garay
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Details
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AI Report

Clinical History

The patient presented with a five-week history of increasing pain in the right elbow. Physical examination showed tumefaction of the proximal end of the ulna. Plain radiographs, CT and MRI were performed.

Imaging Findings

The patient presented with a five-week history of increasing pain in the right elbow. Physical examination showed tumefaction of the proximal end of the ulna. The patient had no fever, chills, weight loss or prior trauma. Laboratory tests were unremarkable. Plain radiographs (Fig. 1), CT (Fig. 2) and MRI (Fig. 3) were performed. Final diagnosis was established by trephine biopsy.

Discussion

Skeletal fibrous dysplasia is a developmental anomaly, typically encountered in adolescents and young adults, in which normal bone marrow is replaced by fibro-osseous tissue (1).

Radiographs show a well-defined medullary lesion, usually with thick sclerotic borders. It can be moderately expansive but the cortex is intact and there is usually no periosteal new bone formation. Depending on the amount of woven bone present and the extent to which it is mineralised, the lesion may have a radiolucent, ground-glass, or even sclerotic appearance. The disease may affect a single bone (monostotic fibrous dysplasia), or many bones (polyostotic fibrous dysplasia). Any bone in the skeleton may be affected and lesions may involve only a small segment of a bone or extend along most or all of a long bone. In the long bones, it usually arises at the metaphysis, but can occur at any location, including the epiphysis before and after closure of the growth plate (2,3).

The present case showed an extensive proximal ulnar epiphyseal involvement, with dysplastic fibro-osseous tissue reaching the joint surface over a wide area. The epiphyseal involvement was associated with multiple erosions of the articular cortex and some well-defined radiolucent foci in subchondral bone, which were evident on CT scans but were unapparent on plain radiography. To our knowledge, these findings have not been previously described in association with fibrous dysplasia. We speculate that these findings might represent osteonecrotic changes secondary to increasing marrow pressure caused by the growth of the dysplastic fibrous tissue. A superimposed septic arthritis may be ruled out in the absence of joint space narrowing and disease in the other side of the joint.

Differential Diagnosis List

Monostotic fibrous dysplasia

Final Diagnosis

Monostotic fibrous dysplasia

Liscense

Figures

Conventional X-ray of the right elbow; AP and lateral views

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Conventional X-ray of the right elbow; AP and lateral views
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Conventional X-ray of the right elbow; AP and lateral views

Computed tomography; coronal series

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Computed tomography; coronal series
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Computed tomography; coronal series

Magnetic resonance; sagittal images

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Magnetic resonance; sagittal images
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Magnetic resonance; sagittal images