Management of femoral fractures and pseudofractures in adult hypophosphatasia

Ten pseudofractures and six complete fractures occurred in five patients with adult hypophosphatasia. The four pseudofractures that were treated non-operatively progressed to complete fracture. The six pseudofractures and six complete fractures that were treated by internal fixation united. New fractures occurred in four patients after the removal of the internal fixation. We recommend that complete fractures, symptomatic pseudofractures, and progressive asymptomatic pseudofractures in patients with adult hypophosphatasia be treated with load-sharing internal-fixation devices, such as rigid intramedullary nails. These should not be removed unless it is absolutely essential. If removal is necessary, however, they should be replaced, preferably during the same anesthetic session. In our opinion, the guidelines developed from this study may be applied reasonably to patients with osteomalacia from causes other than hypophosphatasia, especially if medical therapy has been unsuccessful.