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 Pediatrics Test 3: Club Foot/Hip/Spine
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The Journal of Bone and Joint Surgery (American) 86:531-537 (2004)
© 2004 The Journal of Bone and Joint Surgery, Inc.

Fibrous Dysplasia in the Spine: Prevalence of Lesions and Association with Scoliosis

Arabella I. Leet, MD1, Edward Magur, MD2, Janice S. Lee, MD, DDS3, Shlomo Wientroub, MD4, Pamela G. Robey, PhD5 and Michael T. Collins, MD5

1 Department of Orthopaedics, John Hopkins Medicine, 601 North Caroline Street, Room 5255, Baltimore, MD 21287. E-mail address: aleet1{at}jhmi.edu
2 Department of Orthopaedics, Georgetown University Hospital, 3800 Reservoir Road NW, Washington, DC 20007
3 Department of Oral and Maxillofacial Surgery, University of California-San Francisco, 521 Parnassus Avenue, C-522 San Francisco, CA 94143-0440
4 Department of Pediatric Orthopedic Surgery, Dana Children's Hospital, The Tel-Aviv Sourasky Medical Center, 6 Weizmann Street, Tel-Aviv, Israel 64239
5 Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, 30 Convent Drive, Building 30, MSC 4320, Bethesda, MD 20892

Investigation performed at the Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland

The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

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.


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