Fibrous dysplasia of the proximal femur: surgical management options and outcomes

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• 作者：Indranil V. Kushare (1)
  Dino Colo (1)
  Hooman Bakhshi (1)
  John P. Dormans (1)
  
• 关键词：Fibrous dysplasia ; Shepherd crook deformity ; McCune ; Albright syndrome
• 刊名：Journal of Children's Orthopaedics
• 出版年：2014
• 出版时间：December 2014
• 年：2014
• 卷：8
• 期：6
• 页码：505-511
• 全文大小：355 KB
• 参考文献：1. DiCaprio MR, Enneking WF (2005) Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 87(8):1848-864 CrossRef
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  3. Ippolito E et al (2014) Radiographic classification of coronal plane femoral deformities in polyostotic fibrous dysplasia. Clin Orthop Relat Res 472(5):1558-567 CrossRef
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  13. Yang L et al (2010) Valgus osteotomy combined with intramedullary nail for Shepherd’s crook deformity in fibrous dysplasia: 14 femurs with a minimum of 4?years follow-up. Arch Orthop Trauma Surg 130(4):497-02 CrossRef
  14. Li W et al (2013) Valgus osteotomy in combination with dynamic hip screw fixation for fibrous dysplasia with shepherd’s crook deformity. Arch Orthop Trauma Surg 133(2):147-52 CrossRef
  15. Grabias SL, Campbell CJ (1977) Fibrous dysplasia. Orthop Clin North Am 8(4):771-83
  
• 作者单位：Indranil V. Kushare (1)
  Dino Colo (1)
  Hooman Bakhshi (1)
  John P. Dormans (1)
  
  1. Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA
  
• ISSN：1863-2548

文摘

Purpose 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. Conclusion 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.