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Poly-epiphyseal overgrowth: description of a previously unreported skeletal dysplasia

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Abstract

A skeletal dysplasia with previously unreported features is presented. Its evolution was characterized by growth abnormalities of bones without involvement of other organs. Advanced bone age, increased stature and irregular epiphyseal ossification with stippling of the main long bones were documented. Physeal overgrowth was massive in the left proximal humerus and femur. Furthermore, the hip joint appeared fused with an abundant mass of pathological calcific tissue extending from the femur to the ilium. Pathological epiphyses were characterized by anarchic cartilaginous proliferation with multiple ossification centres, while lamellar bone apposition and remodelling were normal. The observed bone changes were different from those in any previously reported syndrome, metabolic defect or bone dysplasia. However, they clearly indicated a defect of endochondral ossification with some resemblance to phenotypes observed in dysplasia epiphysealis hemimelica.

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References

  1. Silverman FN (1996) Reflections on epiphyseal dysplasias. AJR 167:835–842

    PubMed  CAS  Google Scholar 

  2. Fairbank TJ (1956) Dysplasia epiphysialis hemimelica (tarso-epiphysial aclasis). J Bone Joint Surg Br 38B:237–257

    Google Scholar 

  3. Azouz EM, Slomic AM, Marton D et al (1985) The variable manifestations of dysplasia epiphysealis hemimelica. Pediatr Radiol 15:44–49

    Article  PubMed  CAS  Google Scholar 

  4. Carlson DH, Wilkinson RH (1979) Variability of unilateral epiphyseal dysplasia (dysplasia epiphysealis hemimelica). Pediatr Radiol 133:369–373

    CAS  Google Scholar 

  5. Kettelkamp DB, Campbell CJ, Bonfiglio M (1966) Dysplasia epiphysealis hemimelica. A report of fifteen cases and a review of the literature. J Bone Joint Surg Am 48:746–766

    PubMed  CAS  Google Scholar 

  6. Merzoug V, Wicard P, Dubousset J et al (2002) Bilateral dysplasia epiphysealis hemimelica: report of two cases. Pediatr Radiol 32:431–434

    Article  PubMed  Google Scholar 

  7. Poznansky AK (1994) Punctate epiphyses: a radiological sign, not a disease. Pediatr Radiol 24:418–424

    Article  Google Scholar 

  8. Silverman FN (1989) Dysplasia epiphysealis hemimelica. Semin Roentgenol 24:247–258

    Article  Google Scholar 

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Acknowledgements

The authors are grateful to Prof. A. Benetti, Morbid Anatomy Department, University of Brescia, for processing tissue samples and for useful discussions during preparation of this article.

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Authors and Affiliations

  1. Orthopaedic Clinic, University of Brescia, Spedali Civili di Brescia, 25123, Brescia, Italy

    Ugo E. Pazzaglia & Giovanni Bonaspetti

  2. Department of Paediatric Radiology, Fondazione IRCCS Policlinico San Matteo, 27100, Pavia, Italy

    Giampiero Beluffi

  3. Paediatric Clinic, University of Pavia, Fondazione IRCCS Policlinico San Matteo, 27100, Pavia, Italy

    Antonietta Marchi, Mauro Bozzola & Salvatore Savasta

Authors
  1. Ugo E. Pazzaglia
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  2. Giampiero Beluffi
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  3. Antonietta Marchi
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  4. Mauro Bozzola
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  5. Salvatore Savasta
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  6. Giovanni Bonaspetti
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Correspondence to Ugo E. Pazzaglia.

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Pazzaglia, U.E., Beluffi, G., Marchi, A. et al. Poly-epiphyseal overgrowth: description of a previously unreported skeletal dysplasia. Pediatr Radiol 37, 1025–1030 (2007). https://doi.org/10.1007/s00247-007-0532-z

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  • DOI: https://doi.org/10.1007/s00247-007-0532-z

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