Congenital Pseudarthrosis of Tibia and Fibula: An Introduction

Management of congenital pseudarthrosis of the tibia (CPT) is most challenging. Almost half of the cases are associated with neurofibromatosis type 1. The problems include a recalcitrant pseudarthrosis of the tibia, deformity, limb length discrepancy, concomitant affected fibula in many cases, ankle valgus, and a notorious tendency for refracture after having healed. These problems are due to biological causes in the form of unhealthy avascular and scarred soft tissue environment with very poor potential for healing, mechanical causes due to difficulty in obtaining stable fixation of the distal fragment which is often thin, short, and osteoporotic, as well as other associated causes including deformities of the fibula, ankle valgus, and limb length discrepancy. The goal in treating this condition is not only to obtain union but equally important to maintain union and address all associated deformities in order to achieve a functional limb. There is no universally accepted classification of this disease as the radiological types described in the various classifications often change during treatment and with progression of the disease. As there is no standard and generally accepted treatment for this condition, multiple surgeries and techniques have been described in the management of this condition, and often the end result is a frustrated adolescent who had numerous surgeries, several refractures, and a limb that may be nonfunctional. It is imperative, as soon as the diagnosis of CPT is established, to have a transparent and detailed discussion with the patient and the family regarding all the potential problems of this condition and the expected numerous surgeries including the possibility of an amputation. Poor prognostic factors include first surgery at a very young age, residual ankle valgus, and fibular pseudarthrosis.