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The Long Bone Deformity of Osteogenesis Imperfecta III: Analysis of Structural Changes Carried Out with Scanning Electron Microscopic Morphometry

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Abstract

The wedges of the mid-diaphyseal osteotomies carried out to correct the femoral and/or tibial native deformity in type III osteogenesis imperfecta (OI III) were used to study the remodeling patterns and lamellar organization at the level of the major deformity. Histology and scanning electron microscopy (SEM) morphology showed abnormal cortical remodeling characterized by the failure to form a cylinder of compact bone with a regular marrow canal. Atypical, flattened, and large resorption lacunae with a wide resorption front on one side and systems of parallel lamellae on the opposite side were observed, resembling those formerly reported as drifting osteons. SEM morphometry documented a higher percentage of nonossified vascular/resorption area (44.3 %) in OI than in controls (13.6 %), a lower density of secondary osteons, and lower values for the parameters expressing the individual osteon size. The mean osteon total area, the mean central canal area, and the mean osteon bone area of two selected, randomized populations of secondary osteons were significantly higher (p < 0.001, p = 0.028, and p < 0.001, respectively) in control bones than in OI. The mean ossified matrix area was not significantly different, but the mean secondary osteon number and mean density were higher in controls (both p < 0.001). Osteon wedges were carried out to correct the native deformity of OI III and morphologic analysis suggested that the abnormal remodeling pattern (with “drifting osteons”) may result from the altered load and tensile stresses on the deformed tubular bones.

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Acknowledgments

This research was supported by funds of the Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Italy. The SEM study was carried out with the contribution of the Centro Grandi Strumenti of Insubria University (Varese, Italy). The authors acknowledge the contribution of the Scientific Committee of Fondazione Mario Boni in planning the study and drafting the article.

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Authors and Affiliations

  1. Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Brescia, Italy

    Ugo E. Pazzaglia & Luigi Magnano

  2. 2a Ortopedia e Traumatologia, Spedali Civili di Brescia, Brescia, Italy

    Ugo E. Pazzaglia & Luigi Magnano

  3. Department of Surgical and Morphologic Sciences, University of Insubria, Varese, Italy

    Terenzio Congiu

  4. Pediatric Orthopaedics, Spedali Civili di Brescia, Brescia, Italy

    Pier Carlo Brunelli

  5. Morbid Anatomy, Department of Clinical and Experimental Sciences, Università of Brescia, Brescia, Italy

    Anna Benetti

Authors
  1. Ugo E. Pazzaglia
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  2. Terenzio Congiu
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  3. Pier Carlo Brunelli
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  4. Luigi Magnano
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  5. Anna Benetti
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Correspondence to Ugo E. Pazzaglia or Luigi Magnano.

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The authors state they have no conflict of interest.

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Pazzaglia, U.E., Congiu, T., Brunelli, P.C. et al. The Long Bone Deformity of Osteogenesis Imperfecta III: Analysis of Structural Changes Carried Out with Scanning Electron Microscopic Morphometry. Calcif Tissue Int 93, 453–461 (2013). https://doi.org/10.1007/s00223-013-9771-1

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  • DOI: https://doi.org/10.1007/s00223-013-9771-1

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