ABSTRACT

Congenital pseudarthrosis of the tibia (CPT) refers to nonunion of a tibial fracture that develops spontaneously or after trivial trauma in a dysplastic bone segment of the tibial diaphysis. The pseudarthrosis usually develops during the first two years of life; however, there are reports of cases in which fractures developed before birth and reports of late-onset pseudarthrosis. The etiological nature of this condition is unclear; however, there is a strong association between CPT and neurofibromatosis Type I (NF-I). CPT develops in about 5.7% of patients with NF-I. On the other hand, 40% of patients with CPT were found to have NF-I. Electron microscopy and histopathological studies showed that the main pathology of CPT is hyperplasia of fibroblasts with the formation of dense fibrous tissue. This invasive fibromatosis is located in the periosteum and between broken bone ends and surrounds the tibia causing compression, osteolysis, and persistence of pseudarthrosis (1-3).