Scoliosis and spine involvement in fibrous dysplasia of bone

Abstract

Few studies focused on the prevalence of scoliosis and involvement of the spine in patients with fibrous dysplasia (FD) of bone. We examined for FD involvement of the spine and scoliosis in 56 patients affected by FD of bone. Fifty patients were part of a cohort reported in a multicentric study on FD promoted by European Pediatric Orthopedic Society (EPOS) in 1999, and six were new patients. There were 30 females and 26 males (mean age 12.5 years; range 1-42 years). Twenty-three had monostotic FD, 9 polyostotic FD, and 24 McCune-Albright Syndrome (MAS). Scoliosis was observed in 11 cases of polyostotic FD and MAS (33.3%). In seven of the patients with scoliosis (63.3%) spine was involved by FD lesional tissue. FD lesions involved the thoracic or lumbar spine in all patients but one, where cervical spine was also affected. A correlation between scoliosis and either spinal (p \ 0.01) or pelvic lesions (p \ 0.05) and pelvic obliquity (p \ 0.01) was observed. Three of the 11 patients showed familiarity for scoliosis but in 2 of them spine was involved by FD. Scoliosis and spine involvement were never detected in monostotic FD. This study indicates that in FD patients with polyostotic disease (1) the prevalences of FD involvement of the spine and scoliosis are high enough to include spine in the clinico-radiographic survey of these patients, and (2) the involvement of the spine and pelvis by FD lesions and pelvic obliquity are important determinants in the occurrence of scoliosis.

Craniofacial fibrous dysplasia Report of a case with diverse radiological spectrum

“…The clinical features CFD encompass a broad clinical spectrum depending upon the location and extent of the lesions. These can vary from mild facial asymmetry or a painless "lump" to serious functional deficits owing to progressive expansion of (6). The ocular effects of FD are particularly worrisome.…”

A 33-year-old Man With Low Back Pain

“…Pathologic fracture of the spine is rare [24,33], and there has been only one case report of spinal cord compression from monostotic fibrous dysplasia [33]. Scoliosis has been shown to result from spine involvement of McCune-Albright syndrome in 41.6% of cases and from polyostotic fibrous dysplasia of the spine in 11.1% of cases, but monostotic fibrous dysplasia has not been associated with scoliosis [23]. Sarcomatous transformation of fibrous dysplasia is rare, with a lifetime risk of 0.5%, although this may be a highend estimate given many cases of fibrous dysplasia may remain asymptomatic; the most commonly developed sarcoma is osteosarcoma, followed by fibrosarcoma [10].…”

“…The mean age at presentation for all forms of fibrous dysplasia is 10 years, but the less severe, monostotic form can present later in adulthood [3,10]. Mancini et al reported monostotic fibrous dysplasia presented at a mean age of 16.9 years (range, 7-42 years) [23].…”

Pathologic fractures through benign bone lesions in children and adolescents

“…Vertebral involvement also may occur and result in scoliosis. 87 Patients with McCuneAlbright syndrome are the most severely affected compared with patients with fibrous dysplasia without endocrine abnormalities; they present at an earlier age, lesions tend to be larger, more persistent and cause more complications. 84 The ''café au lait'' skin pigmentations that are characteristic of this disease are distributed over the trunk or proximal parts of the extremities.…”