Congenital pseudarthrosis

Five to six percent of neurofibromatosis type 1 (NF1) patients have congenital tibial dysplasia, the lesion leading to congenital pseudarthrosis (CP). There is an equal sex distribution of NF1, but more boys than girls with NF1 have CP. Approximately 50% of patients with CP have NF1. Gait analysis in patients operated on for CP of the tibia showed that intramedullary rodding across the ankle joint resulted in a gait that was no more efficient than that of the amputee with a well functioning prosthesis. Three articles dealt with llizarov treatment of tibial CP, which is usually indicated after unsuccessful rodding and bone grafting. The union rate varied between 64% and 83%. None of these articles accounted for the functional outcome. Free vascularized fibular grafting seems at least as effective as llizarov treatment in obtaining union, and the functional outcome is good in most patients. This latter technique appears to be preferable in the rare CP of the upper extremity.