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 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
 ::  References
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CASE REPORT
Year : 1997  |  Volume : 43  |  Issue : 3  |  Page : 83-4

Two stage reconstruction for the Shepherd's crook deformity in a case of polyostotic fibrous dysplasia.


Department of Orthopaedics, Seth G.S. Medical College and King Edward Memorial Hospital, Parel, Mumbai.

Correspondence Address:
T V Nagda
Department of Orthopaedics, Seth G.S. Medical College and King Edward Memorial Hospital, Parel, Mumbai.

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Source of Support: None, Conflict of Interest: None


PMID: 0010740732

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 :: Abstract 

Polyostotic fibrous dysplasia leads to progressive and disabling deformity involving the proximal femur. Conventional methods of treatment have been ineffective in controlling this problem. Two stage reconstruction was carried out in a case of polyostotic fibrous dysplasia with bilateral shepherd's crook deformity. Bilateral subtrochanteric osteotomies with intramedullary fixation in the first stage and intertrochanteric osteotomies in the second stage with nail plate fixation was done to provide definitive control of the deformity. Bone graft was not used.


Keywords: Adult, Bone Nails, Case Report, Female, Femur, surgery,Fibrous Dysplasia of Bone, surgery,Human, Orthopedic Fixation Devices, Osteotomy,


How to cite this article:
Nagda T V, Singh H, Kandoi M, Samant A, Patel B R. Two stage reconstruction for the Shepherd's crook deformity in a case of polyostotic fibrous dysplasia. J Postgrad Med 1997;43:83

How to cite this URL:
Nagda T V, Singh H, Kandoi M, Samant A, Patel B R. Two stage reconstruction for the Shepherd's crook deformity in a case of polyostotic fibrous dysplasia. J Postgrad Med [serial online] 1997 [cited 2019 Dec 11];43:83. Available from: http://www.jpgmonline.com/text.asp?1997/43/3/83/396





  ::   Introduction Top


Numerous authors while describing the natural history of fibrous dysplasia have emphasized on morbidity associated with the polyostotic form in terms of multiple deformities and fractures[5],[6]. Lesions in the proximal femur are particularly troublesome. The forces of weight and muscle pull in mechanically weakened bone in this area produce an unrelenting propensity for fractures and varus deformity[3]. Treatment of curettage and grafting controls the deformity only temporarily[2],[4]. We present results of treatment of a case of fibrous dysplasia with bilateral hip deformity using two stage reconstruction.


  ::   Case report Top


A 22-year-old female presented with pain in both hips and limp. On examination findings were suggestive of varus deformity of both the hips with the high riding trochanters touching the pelvis. She walked with marked waddling gait and Trendelenberg’s test was positive bilaterally. X-rays showed fibrous dysplasia involving both femora with the typical Shepherd’s Crook deformity [Figure - 1]. The neck-shaft angle was 65 degrees on the left and 60 degrees on the right side. The skeletal survey showed involvement of both tibiae and proximal humerus. Preoperative planning included paper tracings from the radiographs from which location of angulation and size of each closed wedge osteotomy were determined.

At the first stage subtrochantric osteotomy was carried out at the site of maximum angulation and was fixed with intramedullary nail [Figure - 2]. After a period of one year on union of the osteotomy a second stage intertrochanteric osteotomy was carried out fixed with sliding nail plate device. Bone grafting was not done at any stage.

At two year follow up after the second osteotomy the patient was completely relieved of her pain and limp. She was able to perform the activities of daily living normally. The Trendelenberg’s test became negative. Radiographs showed consolidation of the proximal femora with maintenance of the neck shaft angle (Fig.3).


  ::   Discussion Top


Fibrous dysplasia is a benign pathological condition that affects skeletal development. The lesion with its associated endocrine manifestations and skin pigmentation was described initially by Albright et al in 1937. Lichtenstein coined the term fibrous dysplasia in 1938 and Jaffe detailed its pathological features in 1942.

Although histologically the lesions of fibrous dysplasia are benign fibro-osseous replacement of bone, clinically they cause considerable morbidity. The forces of weight and muscle pull on mechanically weakened proximal femur causes relentless progression of the varies deformity in spite of the treatment. Harris et al[5] have noted the propensity for progression of this disease by appearance of new lesions, extension of existing lesions and increasing deformity, even after puberty.

Conventional methods of surgical management which aim at the elimination of the diseased bone viz., curettage with bone grafting or excision of the entire trochanter have proved ineffective in treating the polyostotic lesions[4],[5]. This has been ascribed to reabsorption of the graft and subsequent recurrence of the disease due to technical difficulty in totally eradicating the disease[6].

The internal fixation with the osteotomy has the advantage of avoiding prolonged immobilization and preventing the recurrence of the deformity. Multiple osteotomies with intramedullary fixation[2] have been utilised in the past but carry the risk of varus angulation in cases involving the neck. As noted by Harris[5], the femoral neck is usually involved in the lesions of proximal femur while the femoral head is generally spared. These findings suggest the feasibility and importance of stabilization of the femoral neck by a device that traverses the involved neck and has a firm purchase in the normal head[1].

The ability to stabilize the neck with correction of the polysegmental deformity makes the two stage osteotomy with internal fixation particularly applicable to the treatment of the shepherd’s crook deformity in polyostotic fibrous dysplasia.

 
 :: References Top

1. Freeman BH, Bray EW, Meyer LC. Multiple Osteotomies with Zickel Nail Fixation for Polyostotic Fibrous Dysplasia Involving the Proximal Part of Femur. J Bone and Joint Surg 1987; 69A:691-698.  Back to cited text no. 1    
2.Breck LW. Treatment of Fibrous Dysplasia of bone by Total Femoral Plating and Hip Nailing. Clin Orthop 1972; 82:82-86.  Back to cited text no. 2    
3.Onnoly JF. Shepherd’s Crook Deformity in Polyostotic Fibrous Dysplasia Treated by Osteotomy and Zickel Nail Fixation. Clin Orthop 1977; 123:22-24.  Back to cited text no. 3    
4.Funk FJ Jr., Wells RE. Hip Problems in Fibrous Dysplasia. Clin Orthop 1973; 90:77-82.  Back to cited text no. 4    
5.Harris WH, Dudley HR Jr., Barry RJ. The Natural History of Fibrous Dysplasia - An Orthopaedic, Pathological and Radiographic Study. J Bone and Joint Surg March 1962; 44A:207-233.  Back to cited text no. 5    
6.Stephenson RB, London MD, Hankin FH. Fibrous Dysplasia. J Bone and Joint Surg 1987; 69A:400-409.  Back to cited text no. 6    
7.Stewart MJ, Gilmer WS, Edmonson AS. Fibrous Dysplasia of Bone. J Bone and Joint Surg 1962; 44B:302-318.   Back to cited text no. 7    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

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2 A two-stage surgery for severe femoral neck deformity due to fibrous dysplasia: A case report
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