Abstract
Pycnodysostosis is a relatively rare disease characterized by systemic osteosclerosis and susceptibility to bone fracture. We performed intramedullary nail fixation in 2 patiants with fracture of long bone, and obtained good results.
Patient 1: A 17-year-old male suddenly had severe pain in the left leg during a running high jump in physical training at school on November 11, 1991. X-ray examination showed fracture of the left fibula and tibia with an open wound (about 1 cm) on the anterior surface of the left leg. After immediate, washing and debridement, wire traction was initiated and intramedullary nail fixation was performed, on November 26.
Patient 2: A 43-year-old female underwent internal fixation using a D. C. plate due to left femoral subtrochanteric fracture in 1981, and nail removal in 1983. Intramedullary nail fixation was made due to fracture of the right femur in 1987. On November 29, 1989, she fell down in the bathroom and visited our hospital because of pain in the left thigh. Re-fracture was found at the same site of the left femur, intramedullary nail protruded at the proximal site in the right femur, with bone fusion failure. On December 15, 1989, horizontal type intramedullary nail fixation in the bilateral femurs was performed, and complete fusion was gained.
Intramedullary nail fixation for pycnodysostosis allows motions of the joint in the early stage, and complications such as joint contracture can be prevented. In addition, horizontal fixation enhances fixation force, and we do not remove nails because of possible re-fracture.
