title: Congenital pseudarthrosis of the tibia: an atypical proximal location.
abstract: Proximal location of congenital pseudarthrosis of the tibia (CPT) is uncommon, and its management challenging, risking to end in amputation. We here report a case of proximal CPT managed in a limb-sparing perspective and followed up until the end of growth. A 17-year-old girl presented with type-1 neurofibromatosis and proximal CPT. Initial X-ray showed severe pseudarthrosis of the tibia with bone atrophy, 12-cm shortening and femorotibial and femoropatellar dislocation. Inter-tibiofibular graft and fibular tibialization were performed. At end of follow-up (age 33 years), fusion had been obtained. For orthoprosthetic and cosmetic reasons, a Boyd amputation of the tarsus was performed when the patient was 22 years of age. The functional result was very good, with 0-100 degrees knee mobility. CPT, when proximal, completely disorganizes the knee joint, which is otherwise usually unaffected by this pathology. To achieve a good result, a limb-sparing treatment should combine correction of the tibial axis and of the dislocation of the knee, fibula osteosynthesis and bone graft.
  
  
  METHODS
  Level IV retrospective
authors.affiliation: Service d'orthopédie traumatologie, hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Khiami
authors.forename: V
authors.initials: V
authors.lastname: Rampal
authors.forename: R
authors.initials: R
authors.lastname: Seringe
authors.forename: P
authors.initials: P
authors.lastname: Wicart
mesh_terms: D000328:Adult; D000671:Amputation, Surgical; D001174:Arthrodesis / Q000379:methods; D016025:Bone Transplantation / Q000379:methods; D005260:Female; D006801:Humans; D009456:Neurofibromatosis 1 / Q000601:surgery*; D011183:Postoperative Complications; D011542:Pseudarthrosis / Q000151:congenital* / Q000601:surgery; D020127:Recovery of Function; D013977:Tibia / Q000002:abnormalities*