Subtrochanteric fracture managed by intramedullary nail in a patient with pycnodysostosis

A rare case of a 54-year-old man with pycnodysostosis having subtrochanteric fracture of right femur is being reported. This patient had this fracture after a trivial fall while walking. He had typical facial features, short stature, open fontenella, short stubby hands, high arched furrowed palate and radiological features including open cranial sutures, obtuse angle of mandible, dysplasia of lower end of radius and ulna, notching of lumbar vertebra and generalized skeletal sclerosis. The fracture was treated with an intramedullary nail; the surgery was quite a tedious procedure where we faced per-operative difficulty in reaming. The fracture united in 12 weeks. Extramedullary osteosynthesis might offer a better alternative in such patients as marrow is narrow but healing is normal. (C) 2003 Elsevier SAS. All rights reserved.