Fracture management in pycnodysostosis: 27 years of follow-up.

Abstract

Pycnodysostosis is a rare hereditary disease, characterized by systemic bone sclerosis, which is often brought to the orthopedic surgeon's attention because of repeated fractures. The operative treatment of the patient with a fracture is a real challenge for the orthopedic surgeon because of the unusual problems imposed by the hard but-brittle bone characteristics of the disease. We report a 27-year follow-up of a patient treated for fractures of both femurs and tibia with intramedullary nailing. According to our experience and literature review, we recommend the use of an internal fixation, preferably intramedullary nailing as the treatment of choice for these rare cases. Once the fractures are healed, the removal of the hardware is not recommended, as these keep the bones from further fractures.