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Scoliosis and spine involvement in fibrous dysplasia of bone

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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.

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Acknowledgments

The authors wish to thank Edward W. Bray (Shriners Hospitals, Greenville, South Carolina, USA), Ulrich G. Exner (Balgrist Orthopedic University Health Centre, Zurich, Switzerland), Pamela Gehron Robey (Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institute of Health, Bethesda, Maryland, USA), Franz Grill and Oswald Pinggera (Orthopadisches Spital Speising GmbH, Vienna, Austria), Roberto Lala (Division of Pediatric Endocrinology, Regina Margherita Hospital, Torino, Italia), Marco Massobrio (Department of Orthopedic Surgery, La Sapienza University, Rome, Italy), Slawomir Snela (Szpital Wojewodski, Rzeszow, Poland), Christos Zambakidis (P&A Kiriakou Children’s Hospital, Athens, Greece), and Paolo Bianco (Department of Experimental Medicine, La Sapienza University, Rome, Italy) for their adhesion and participation to the multicenter study on fibrous dysplasia promoted by the European Pediatric Orthopaedic Society in 1999.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery, University of Rome Tor Vergata, Viale Oxford 81, 00133, Rome, Italy

    Federico Mancini, Fernando De Maio & Ernesto Ippolito

  2. Department of Experimental Medicine, University of Rome La Sapienza, Viale Regina Elena 324, 00100, Rome, Italy

    Alessandro Corsi

  3. Department of Experimental Medicine, University of L’Aquila, Via Vetoio Coppito 2, 67100, L’Aquila, Italy

    Mara Riminucci

  4. Via Romoli 24 A-bis, 00040, Monteporzio Catone, Rome, Italy

    Federico Mancini

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  1. Federico Mancini
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  2. Alessandro Corsi
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  3. Fernando De Maio
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  4. Mara Riminucci
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  5. Ernesto Ippolito
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Correspondence to Federico Mancini.

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Mancini, F., Corsi, A., De Maio, F. et al. Scoliosis and spine involvement in fibrous dysplasia of bone. Eur Spine J 18, 196–202 (2009). https://doi.org/10.1007/s00586-008-0860-1

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  • DOI: https://doi.org/10.1007/s00586-008-0860-1

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