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Pediatric Radiology

, Volume 37, Issue 10, pp 1025–1030 | Cite as

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

  • Ugo E. Pazzaglia
  • Giampiero Beluffi
  • Antonietta Marchi
  • Mauro Bozzola
  • Salvatore Savasta
  • Giovanni Bonaspetti
Case Report

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.

Keywords

Epiphyseal growth Skeletal dysplasia Growth abnormalities Physeal overgrowth 

Notes

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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Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Ugo E. Pazzaglia
    • 1
  • Giampiero Beluffi
    • 2
  • Antonietta Marchi
    • 3
  • Mauro Bozzola
    • 3
  • Salvatore Savasta
    • 3
  • Giovanni Bonaspetti
    • 1
  1. 1.Orthopaedic ClinicUniversity of BresciaBresciaItaly
  2. 2.Department of Paediatric RadiologyFondazione IRCCS Policlinico San MatteoPaviaItaly
  3. 3.Paediatric Clinic, University of PaviaFondazione IRCCS Policlinico San MatteoPaviaItaly

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