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Topic:

Femoral Shaft Fracture

Issue:

Thigh

Category:

Trauma

Title:

Femur Fractures in 5 Individuals With Pantothenate Kinase-associated Neurodegeneration: The Role of Dystonia and Suggested Management

Author:

Behrndt, Laken MD; Gregory, Allison MD; Wakeman, Katrina BS; Freed, Alison BA; Wilson, Jenny L. MD; Spaull, Robert MBBS; Kurian, Manju A. PhD; Mordekar, Santosh MD; Fernandes, James A. MCh Orth; Hayflick, Susan J. MD; Hogarth, Penelope MD; Yang, Scott MD

Journal:

Journal of Pediatric Orthopaedics

Date:

January 2024

Reference:

44(1): p e61-e68, DOI: 10.1097/BPO.0000000000002555

Level Of Evidence:

IV

# of Patients:

5

Study Type:

Retrospective case review

Location:

Not specified

Summary:

This study focuses on the management of femur fractures in patients with Pantothenate kinase-associated neurodegeneration (PKAN), a rare neurodegenerative disorder that leads to dystonia, osteopenia, and a higher risk of atraumatic fractures.

Methods:

Patients: 5 non-ambulatory patients aged 10 to 20 years with PKAN. Data Collection: Information on fracture presentation, surgical treatment, and postoperative complications.

Exclusions:

Not specified

Results:

Fractures: 4 out of 5 patients experienced atraumatic femur fractures linked to dystonia episodes; 1 patient also had a contralateral acetabular fracture. Complications: Post-surgery, 4 of 5 patients had orthopedic complications, with 3 requiring surgical revision due to dystonia. Hospitalization: 4 patients needed extended hospitalization for dystonia management.

Conclusions:

Rigid intramedullary rods with interlocking screws are recommended for minimizing complications due to postoperative dystonia. Comprehensive, multidisciplinary postoperative care, including sedation and pain management, is critical for reducing the risk of further injuries and managing refractory dystonia effectively.

Relevance:

Limitations:

Perspective:

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