Scientific knowledge of congenital tibial pseudarthrosis

  Congenital pseudarthrosis of the tibia (CPT) is a relatively rare disease in pediatric orthopedics that is very difficult to treat and has a poor prognosis. The incidence is 1/140,000 to 1/190,000. The proportion of males and females is equal, with the left side being slightly more involved than the right, and bilateral involvement is very rare. The etiology is unknown, with approximately 50% of children suffering from neurofibromatosis (NF) and others with genetic and mechanical factors. Clinical experience in the last decade has shown that the use of llizarov technique, selection of the appropriate age for surgery and careful brace protection can achieve satisfactory clinical results, with a clinical bone healing rate of 75.5%.  Children with congenital tibial pseudarthrosis, commonly known as “glass man”, have typical clinical manifestations: most children are born without obvious deformity formation or only the lower middle third of the tibia is bent forward, and may have signs of neurofibromatosis (such as skin milk and coffee spots, neurofibromatosis, axillary or inguinal spots, etc.). Fracture can occur after minor trauma, followed by bone discontinuity, progressive forward angular deformity, calf shortening, soft tissue contracture, horseshoe inversion or valgus deformity of the foot, difficulty in bearing weight on the affected limb, the majority of unilateral, rare bilateral, some can be manifested as a congenital tibial pseudarthrosis on one side and congenital tibial curvature on the other side. The fracture end is thin and sclerotic, and the medullary cavity is partially or completely occluded. Some fracture ends may also have cystic changes, often involving the fibula, and the fibula is bent, thin, or also has a pseudarthrosis.