ArticleJournal of clinical orthopaedics and trauma
A rare case of pycnodysostosis: Technical difficulties in managing long bone fractures.
Article in Journal of clinical orthopaedics and trauma. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 7 citations in OpenAlex.
- The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association · 2024Review
- Pathological Fractures in Patients Affected by Pycnodysostosis: A Case Series.Journal of clinical medicine · 2024Article
- Management of long bone fractures in patients with pycnodysostosis.BMJ case reports · 2023Article
- Review
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pycnodysostosis is a rare inherited disorder of autosomal recessive trait causing cathepsin K deficiency, leading to failure of osteoclastic activity. Brittle and sclerotic bones which are prone for frequent fractures is the characteristic feature of this congenital disorder. Despite good healing potential there are few issues in the management of fractures in pycnodysostosis patients. In this article we report the challenges faced in managing a fracture of the femoral shaft in a 12 year old girl with pycnodysostosis. For early rehabilitation and to avoid deformity and shortening, we opted for surgical fixation over conservative treatment. Narrow medullary canal ruled out the option for titanium elastic nail fixation. 4.5mm dynamic compression plate was used to fix the fracture. Sclerotic bone made drilling extremely difficult. Deformed femoral shaft allowed plating over the anterior surface only, instead of the routine lateral surface plating. Postoperative fracture healing was satisfactory. Implant was removed after 18 months.
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