Clinical and radiographic analysis of osteochondromas and growth disturbance in hereditary multiple exostoses

D E Porter, M E Emerton, F Villanueva-Lopez, A H Simpson

Research output: Contribution to journalArticlepeer-review

Abstract

Hereditary multiple exostoses (HME) is traditionally described as a skeletal dysplasia. However, the discovery that the EXT family of tumour suppressor genes are responsible for HME suggests that it is more appropriate to classify HME as a familial neoplastic trait. In a clinical and radiographic analysis of paired bone length and exostoses number and dimensions in a HME cohort, the local presence of osteochondromas was consistently associated with growth disturbance. In particular, an inverse correlation between osteochondroma size and relative bone length (p<0.01) was found. These data suggest that the growth retardation in HME may result from the local effects of enlarging osteochondromas rather than a skeletal dysplasia effect. This study provides the first clinical rationale for ablation of rapidly enlarging exostoses to reduce growth disturbance.

Original languageEnglish
Pages (from-to)246-50
Number of pages5
JournalJournal of Pediatric Orthopaedics
Volume20
Issue number2
Publication statusPublished - 30 Mar 2000

Keywords

  • Adolescent
  • Adult
  • Bone Neoplasms
  • Child
  • Child, Preschool
  • Cohort Studies
  • Exostoses, Multiple Hereditary
  • Female
  • Genes, Tumor Suppressor
  • Growth Disorders
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Osteochondroma
  • Prognosis
  • Risk Assessment

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