title: Fibrous dysplasia of the proximal femur: surgical management options and outcomes.
abstract: OBJECTIVE
  The management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.
  
  
  METHODS
  This is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.
  
  
  RESULTS
  Group 1: shepherd crook deformity-included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.
  
  
  CONCLUSIONS
  Shepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.
authors.affiliation: Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA.
authors.forename: Indranil V
authors.initials: IV
authors.lastname: Kushare
authors.forename: Dino
authors.initials: D
authors.lastname: Colo
authors.forename: Hooman
authors.initials: H
authors.lastname: Bakhshi
authors.forename: John P
authors.initials: JP
authors.lastname: Dormans