Disturbed remodeling and delayed fracture healing in pediatric pycnodysostosis patients.
case_series · Level IV
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- Record sourced from PubMed, PMID 31048950.
- Also identified by DOI 10.1016/j.jor.2019.03.022 and PMC identifier 6484229.
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Abstract
Pycnodysostosis is an autosomal recessive disease caused by a gene mutation leading cathepsin K deficiency. Pathological fractures of the long bones are common, but guidelines on fracture treatment in these patients are still lacking. We have treated 5 fractures in 2 pediatric pycnodysostosis patients. We hypothesize that pycnodysostosis patients have an incomplete remodeling process in fracture healing because of cathepsin K deficiency. Therefore, to minimize the role of endochondral bone formation (indirect) after a fracture, it seems prudent to strive for direct bone healing (intramembranous) instead of indirect bone healing. Open reduction with internal fixation should be the goal.