Pyknodysostosis with long bone fractures 2 case reports

Our experience with two cases of long bone fractures in pyknodysostosis patients treated with internal fixation is discussed in this article. Pyknodysostosis is a rare disorder of osteoclast dysfunction presenting with abnormally dense bones, prone to pathological fracture, often multiple. Fracture healing in these patients may be normal or delayed. In our case report, fracture shaft of femur was treated with plating and fracture non union tibia was managed by plating with bone grafting. Nailing was not preferred because of the deformed long bones, narrow medullary canal and sclerosed fracture ends. Peroperatively, nibbling, drilling and bone grafting were difficult. Patients were followed up for one year. Fracture shaft of femur showed good union at 3 months whereas healing in fracture non union tibia progressed slowly and united at 6 months. Fracture management in pyknodysostosis patients is a challenge. It requires good pre operative planning to counter the intra operative difficulties.