An extensive type of polyostotic fibrous dysplasia

Abstract

The case describes an 11-year-old girl affected by an unusually extensive type of polyostotic fibrous dysplasia. The cranium and face and both femurs, tibias, and fibulas were extensively and almost symmetrically involved. Tubulation deformities were noted in the metacarpals and middle phalanges of both hands. These findings appear to represent a very severe manifestation of the disease, as polyostotic fibrous dysplasia is known to be predominantly unilateral. The patient had endocrine dysfunction consistent with McCune-Albright syndrome. Radiological work-up included plain radiography of the skeleton and CT, MR imaging, and MR angiography of the cranium.

Imaging of McCune-Albright syndrome using bone single photon emission computed tomography

“…The lumbar uptakes were in the facet joints, and were not degenerative nor stress changes at the site of scoliosis. Symmetric conditions have also been described [13]. In our case the left ankle was also involved, a ®nding not previously reported [8].…”

“…In our case all the lesions could be con®rmed by conventional X-ray techniques. MRI will be helpful in selected conditions [4,13].…”

Benign Bone Tumors

“…[5][6][7] The skull and face commonly are involved. This process can involve a single bone (monostotic form) or multiple bones (polyostotic form).…”