Orthopaedic Manifestations of Thanatophoric Dwarfism: A Case Report

JBJS Case Connect. 2023 Jul 27;13(3). doi: 10.2106/JBJS.CC.23.00183. eCollection 2023 Jul 1.

Abstract

Case: We report the rare case of a 3-year-old male patient with thanatophoric dwarfism, a fatal skeletal dysplasia that arises from an autosomal dominant mutation in the fibroblast growth factor receptor 3 gene. The role of the orthopaedic surgeon in the in the management of this disease is discussed.

Conclusion: We advocate for the close monitoring of disease progression by the orthopaedic surgery team and offer a potential surgical intervention that may help prevent cardiorespiratory demise.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Humans
  • Male
  • Mutation
  • Orthopedics*
  • Receptor, Fibroblast Growth Factor, Type 3 / genetics
  • Thanatophoric Dysplasia* / genetics

Substances

  • Receptor, Fibroblast Growth Factor, Type 3

Supplementary concepts

  • Thanatophoric Dysplasia, Type I