Anterolateral Tibial Bowing and Congenital Pseudoarthrosis of the Tibia: Current Concept Review and Future Directions.

Abstract

Congenital pseudarthrosis of the tibia (CPT) is a rare condition closely associated with neurofibromatosis type I. Affected children are born with anterolateral bowing of the tibia which progresses to pathologic fracture, pseudarthrosis, and high risk of refracture even after initial union has been attained. There is currently no consensus on the classification of this disease or consensus on its treatment. The purpose of this review is to (1) review the clinical presentation, etiology, epidemiology, classification, and natural history of congenital pseudarthrosis of the tibia and (2) review the existing trends in treatment of congenital pseudarthrosis of the tibia and its associated complications. Current treatment protocols focus primarily on combining intramedullary fixation with external or internal fixation to achieve union rates between 74 and 100%. Intramedullary devices should be retained as long as possible to prevent refracture. Cross-union techniques, though technically difficult, have a reported union rate of 100% and no refractures at mid- to long-term follow-up. Vascularized fibular grafting and induced membrane technique can be successful, but at the cost of numerous surgical procedures. Growth modulation is a promising new approach to preventing fracture altogether, though further study with larger patient series is necessary. The primary consideration in treatment of CPT is expected union rate and refracture risk. Combined intramedullary and external or internal fixation, especially with cross-union techniques, show most promise. Perhaps most exciting is further research on preventing fracture through guided growth, which may reduce the morbidity of multiple surgical procedures which have been the mainstay of treatment for CPT thus far.