Background: Lesions of fibrous dysplasia involving the spine and
scoliosis are thought to be uncommon entities in patients with polyostotic
fibrous dysplasia and McCune-Albright syndrome. By examining bone scans of a
relatively large cohort of patients with these disorders, we sought to
determine the prevalence of spinal involvement and any association with
scoliosis.
Methods: Sixty-two patients with polyostotic fibrous dysplasia were
studied. There were twenty-three male and thirty-nine female patients, and
they had a mean age of twenty-five years (range, four to eighty years).
Technetium-99m-methylene diphosphonate (MDP) bone scans of the patients were
evaluated for evidence of increased uptake in the spine. The presence or
absence of scoliosis or a level pelvis and the distribution of other lesions
in the skeleton were noted.
Results: Thirty-nine (63%) of sixty-two patients were found to have
seventy-six lesions of fibrous dysplasia in the spine. Fifty-four lesions
(71%) demonstrated increased uptake in the posterior aspects of the spine.
Most lesions were located in the lumbar spine (thirty-two lesions) and the
thoracic spine (twenty-seven), with less frequent involvement in the sacrum
(ten) and cervical spine (six). Twenty-five (40%) of the sixty-two patients
had scoliosis; seventeen had a thoracolumbar curve; six, a lumbar curve; and
two, a thoracic curve. Seven patients had curves that could not be accurately
measured by bone scanning and, therefore, could not be classified. Thirty
patients (48%) had no evidence of scoliosis. Thus, the prevalence of scoliosis
in patients with polyostotic fibrous dysplasia was between 40% and 52%. There
was a strong correlation between spinal lesions and scoliosis (p < 0.001)
and pelvic asymmetry (p < 0.05). Back pain was an uncommon symptom. Two
patients had a neurologic abnormality; neither abnormality was related to the
location of the lesions or the curve.
Conclusions: Spinal lesions and scoliosis may be more common in
patients with polyostotic fibrous dysplasia than has been previously reported.
Since there is a strong correlation between a spinal lesion and scoliosis,
these patients should be screened clinically for scoliosis.
Level of Evidence: Prognostic study, Level II-1
(retrospective study). See Instructions to Authors for a complete description
of levels of evidence.