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Radiographic Classification of Coronal Plane Femoral Deformities in Polyostotic Fibrous Dysplasia

  • Clinical Research
  • Published:
Clinical Orthopaedics and Related Research®

Abstract

Background

Fibrous dysplasia of bone is a skeletal dysplasia with a propensity to affect the femur in its polyostotic form, leading to deformity, fracture, and pain. The proximal femur is most commonly involved with a tendency to distal progression, thereby producing the typical shepherd’s crook deformity. However, there are few data on the spectrum and progression of femoral deformities in polyostotic fibrous dysplasia.

Questions/purposes

The purposes of this study were (1) to develop a radiographic classification for polyostotic fibrous dysplasia; (2) to test this classification’s intra- and interobserver reliability; and (3) to characterize the radiographic progression of polyostotic fibrous dysplasia in a population of patients with the condition who were treated with a variety of approaches at several centers.

Methods

We retrospectively reviewed radiographs of 127 femurs from 84 adult patients affected by polyostotic fibrous dysplasia. Fifty-nine femurs had undergone one or more operations. The radiographs were evaluated in the coronal plane for neck-shaft angle and angular deformities along the whole femoral shaft down to the distal epiphysis. Four observers evaluated each film two times at intervals; intra- and interobserver reliability testing was performed using the kappa statistic. Eighty-nine femurs (70%) were available for followup to evaluate for progression at a mean of 10 years (range, 6–20 years).

Results

Six reproducible patterns of deformity were identified in both untreated and operated femurs: type 1 (24%), normal neck-shaft angle with altered shape of the proximal femur; type 2 (6%), isolated coxa valga with neck-shaft angle > 140°; type 3 (7%), isolated coxa vara with neck-shaft angle < 120°; type 4 (20%), lateral bowing of the proximal half of the femur associated with normal neck-shaft angle; type 5 (14%), like in type 4 but associated with coxa valga; and type 6 (29%), like in type 4 but associated with coxa vara. Interobserver and intraoberver kappa values were excellent, ranging from 0.83 to 0.87. In 46 of the 89 femurs (52%) for which longitudinal radiographic documentation was available, there was progressive worsening of the original deformity, although the pattern remained the same; types 1 and 2 tended not to progress, whereas types 3 to 6 did.

Conclusions

A reproducible radiographic classification of polyostotic fibrous dysplasia-associated femoral deformities is proposed, which can serve as a tool for assessing and treating these deformities. After reviewing the radiographs of 127 femurs, we identified six reproducible patterns of femoral deformities.

Level of Evidence

Level III, diagnostic study. See Guidelines for Authors for a complete description of levels of evidence.

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Acknowledgments

We thank Prof Paolo Bianco, Department of Molecular Medicine of the University of Rome La Sapienza, Rome, Italy, and Prof Mara Riminucci for their scientific support; Roberto Lala MD, head of the Department of Pediatric Endocrinology of Regina Margherita Hospital Turin, Turin, Italy, who provided several cases of McCune-Albright syndrome; C. Chebli MD, and M. Kelly RN, MS, from the National Institutes of Health, Bethesda, MD, USA, for case review; and Matteo Benedetti Valentini MD, from the Department of Orthopaedic Surgery of the University of Tor Vergata, Rome, Italy, who provided drawings of the six types of the proposed classification.

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Authors and Affiliations

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

    Ernesto Ippolito MD, Pasquale Farsetti MD & Fernando De Maio MD

  2. National Institutes of Health, Bethesda, MD, USA

    Alison M. Boyce MD & Michael T. Collins MD

  3. Department of Molecular Medicine, University of Rome La Sapienza, Rome, Italy

    Alessandro Corsi MD

Authors
  1. Ernesto Ippolito MD
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  2. Pasquale Farsetti MD
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  3. Alison M. Boyce MD
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  4. Alessandro Corsi MD
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  5. Fernando De Maio MD
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  6. Michael T. Collins MD
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Corresponding author

Correspondence to Ernesto Ippolito MD.

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Each author certifies that he or she, or a member of his or her immediate family, has no funding or commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article.

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Clinical Orthopaedics and Related Research neither advocates nor endorses the use of any treatment, drug, or device. Readers are encouraged to always seek additional information, including FDA-approval status, of any drug or device prior to clinical use.

Each author certifies that his or her institution approved the human protocol for this investigation, that all investigations were conducted in conformity with ethical principles of research, and that informed consent for participation in the study was obtained.

This work was performed at the University of Rome Tor Vergata, Rome, Italy.

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Ippolito, E., Farsetti, P., Boyce, A.M. et al. Radiographic Classification of Coronal Plane Femoral Deformities in Polyostotic Fibrous Dysplasia. Clin Orthop Relat Res 472, 1558–1567 (2014). https://doi.org/10.1007/s11999-013-3380-1

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  • DOI: https://doi.org/10.1007/s11999-013-3380-1

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