ABSTRACT

Congenital pseudoarthrosis of the tibia is a rare disorder with an incidence of 1 per 140,000 live births and associated with neurofibromatosis type-1 (NF-1) in 50% of the cases. The first descriptions of this disease were made by Hatzo¨cher in 1709 and Sir James Paget in 1891 (1-5). The name ‘‘congenital pseudoarthrosis of the tibia’’ does not reflect the facts that pseudoarthrosis of the tibia does not occur until the first years of life in 90% of cases (6-8) and appears with a pseudoarthrosis of the fibula in 60% of all cases. In addition, even an isolated fibula pseudoarthrosis could occur in 19% of patients (9).