Management of long bone fractures in patients with pycnodysostosis.


Journal

BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291

Informations de publication

Date de publication:
18 Sep 2023
Historique:
pmc-release: 18 09 2025
medline: 20 9 2023
pubmed: 19 9 2023
entrez: 18 9 2023
Statut: epublish

Résumé

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.

Identifiants

pubmed: 37723084
pii: 16/9/e252667
doi: 10.1136/bcr-2022-252667
pmc: PMC10510937
pii:
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Ajay Sharma (A)

Orthopaedics, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Samarth Mittal (S)

Orthopaedics, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Vivek Trikha (V)

Orthopaedics, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Pulak Vatsya (P)

Orthopedic Surgery, Indraprastha Apollo Hospital, New Delhi, India Pulakvatsya7@gmail.com.

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