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Case Reports: Rare disease
Management of long bone fractures in patients with pycnodysostosis
  1. Ajay Sharma1,
  2. Samarth Mittal1,
  3. Vivek Trikha1 and
  4. Pulak Vatsya2
  1. 1Orthopaedics, All India Institute of Medical Sciences, New Delhi, Delhi, India
  2. 2Orthopedic Surgery, Indraprastha Apollo Hospital, New Delhi, India
  1. Correspondence to Dr Pulak Vatsya; Pulakvatsya7{at}gmail.com

Abstract

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.

  • Orthopaedics
  • Congenital disorders
  • Medical education

http://dx.doi.org/10.1136/bcr-2022-252667

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    Footnotes

    • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical

      images, investigation results, drawing original diagrams and algorithms, and critical revision for

      important intellectual content: AS, SM, PV. The following authors gave final approval of the manuscript:

      VT, SM.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.