[Congenital pseudarthrosis of the tibia : A\xa0rare often underestimated disorder].

Abstract

Congenital pseudarthrosis of the tibia (CPT) is a\xa0rare disease that has challenged trauma and orthopedic surgeons for a\xa0long time. Clinically CPT mostly presents with progressive varus and antecurvation malformation of the tibia in infancy and childhood. In many cases CPT is associated with neurofibromatosis or fibrous dysplasia. Without knowledge of the clinical picture a\xa0discontinuity of the tibia and/or fibula visible on an X‑ray can be misinterpreted as a\xa0simple fracture. Histopathological investigations have revealed that a\xa0pathological alteration of the periosteum in the region of the pseudarthrosis, a\xa0hamartoma, may be essentially responsible for this disorder. Consequently, to treat the CPT a\xa0resection of the hamartoma must also be carried out. Multimodal treatment approaches combining pharmacological and surgical treatment, such as the cross-union technique of Paley, seem to have improved the prognosis of CPT.