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Management of long bone fractures in patients with pycnodysostosis.

Ajay SharmaSamarth MittalVivek TrikhaPulak Vatsya
Published in: BMJ case reports (2023)
Pycnodysostosis is a rare genetic condition that leads to generalised bony sclerosis and increased fracture risk. Orthopaedic specialists play a crucial role in managing affected children due to their susceptibility to frequent fractures. We had a case of a middle childhood female patient with pycnodysostosis and a femur fracture. Initially, an attempt using the Titanium Elastic Nailing System was made, but the sclerotic metaphyseal bone made it challenging. So, we opted for a 4.5 mm locked compressive plate, with multiple drill bits as a backup due to potential drill breakage. Though elastic nailing is preferred for paediatric long bone fractures, surgeons must be prepared for extremely sclerotic cortices and a narrow medullary canal when dealing with patients with pycnodysostosis. Open fixation and multiple drill bits in the toolkit are essential to overcome the potential obstacles during the procedure.
Keyphrases
  • bone mineral density
  • minimally invasive
  • soft tissue
  • postmenopausal women
  • bone loss
  • bone regeneration
  • emergency department
  • intensive care unit
  • body composition
  • case report
  • human health