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CASE REPORT Table of Contents   
Year : 2004  |  Volume : 38  |  Issue : 2  |  Page : 134-136
Fracture neck of femur with avascular necrosis of head in fibrous dysplasia


Department of Orthopaedics and Traumatology, St. Johns Medical College Hospital, Bangalore, India

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How to cite this article:
Amaravati RS, Thomas K T, Phaneesha M S, Jadhav R, Mallikarjunswami B, Isaac T. Fracture neck of femur with avascular necrosis of head in fibrous dysplasia. Indian J Orthop 2004;38:134-6

How to cite this URL:
Amaravati RS, Thomas K T, Phaneesha M S, Jadhav R, Mallikarjunswami B, Isaac T. Fracture neck of femur with avascular necrosis of head in fibrous dysplasia. Indian J Orthop [serial online] 2004 [cited 2019 Dec 16];38:134-6. Available from: http://www.ijoonline.com/text.asp?2004/38/2/134/40931

   Introduction Top


Fibrous dysplasia of bone probably represents fibrous metaplasia of the primitive mesenchymal cells in the skeleton. The disease in some begins right from the childhood and is usually self-limiting, but may also progress beyond puberty compromising the structural integrity of the affected bones, leading to progressive deformities and pathological fractures [1] . Deformities are a result of micro fractures in the metaplastic weak bone during normal weight bearing [2] . Bone heals readily after fracture but with a deformity like Shepherd's crook deformity of the femur, Harrisons groove following the rib fractures and protrusio acetabuli [3] . Fibrous dysplasia of bone is an enigma with no known cure. In case of fibrous dysplasia of the neck of femur with fracture the usual from of treatment described in the literature are closed reduction and cast application, curettage and bone grafting, internal fixation, multiple osteotomies, radical resection and amputation. However, because of poor bone quality incidence of refractures and failure of the implant are high [4],[5] . The purpose of this paper is to evaluate the outcome of primary arthroplasty of the hip following avascular necrosis of the head due to pathological fracture of the neck in fibrous dysplasia.


   Case report Top


A 20-year-old male had a five month history of left hip pain and inability to walk. A review of the patient's medical history revealed that he had sustained injury from a fall after being thrown away on coming into contact with high-tension electric wire. He was apparently fine till he sustained the injury. Physical examination revealed one inch shortening of left leg, which was abducted and externally related with limited range of motion of hip joint. The left hip joint was tender with cafe-eu-lait spots on the chest and back.

Radiographically there was thinning and expansion of cortex of the left femur with radiolucent and partly sclerotic lesions of various sizes with a typical ground glass appearance in the left femur, left tibia and left fibula. There was intracapsular fracture neck of left femur with a sclerosed head [Figure 1]a,b. Further evaluation was carried out to assess the vascular status of head of femur. Bone scan revealed no uptake in the head of femur but increased uptake in the left femur and tibia. Computerised axial tomography aided biopsy from the head and neck of the left femur confirmed the histological findings consistent with fibrous dysplasia [Figure 1]c. All haematological parameters were normal except for high alkaline phosphatase levels. The patient was not compliant for salvaging the head of femur by osteosynthesis with fibular graft or to do total joint replacement. A primary bipolar hemireplacement arthroplasty of the left hip was performed [Figure 2]a. After 6 weeks of non-weight bearing, patient stated partial weight bearing and crutch walking. The patient was allowed to walk independently four months after surgery. At the latest follow up 28 months after surgery the patient was free of pain and functionally independent. The X-rays showed mild sclerosis of the trochanter with sinking of the prosthesis at the calcar [Figure 2]b.


   Discussion Top


Lichtenstein is credited with having coined the term fibrous dysplasia in 1938 and in 1942, he and Jaffe reviewed all known cases of this entity [6],[7] . Fibrous dysplasia is divided in to three clinical forms, monostotic, polyostotic and endocrinopathic ( McCune-Albright syndrome More Details). Polystotic lesions are large, difficult to treat with unpredictable results [1] .

Fractures in the bones affected by fibrous dysplasia do not need surgery and generally heal without difficulty, but the callus that forms is dysplastic and is prone to repeated microfractures and deformity [6],[9] . The indications for surgical intervention are persistence of pain, progression of lesion and failure of union [10] . In some instances vascular status of the head of femur in a pathological fracture neck of femur cannot be assessed as the tumorous tissue and the fracture tissue obscure the findings [11] . Prediction of vascular status of head of femur can be done by using electrochemically generated hydrogen clearance method or by the hydrogen wash out technique [12],[13] . Surgical treatment of fibrous dysplasia is challenging. Curettage and bone grafting used to replace the dysplastic bone is associated with high failure rates [14] . Internal splintage with metal implants has been used to prevent deformity but they are prone to fatigue failure.

Osteosynthesis using cortical autografts and allografts has been employed with varied success [15],[16],[17] . Unlike conventional grafts vascularized grafts are unaffected by the dysplasia of humerus and radius [18] . No evidence is available as to the use of vascularized fibular graft in osteosynthesis of fracture neck of femur with avascular necrosis of the head in fibrous dysplasia. Total hip arthroplasty was done in a case of polyostotic fibrous dysplasia with degenerative disease of hip joint due to multiple osteotomies and bone grafting [19] . Bipolar hemireplacement arthoplasty can be considered as an alternative to total hip replacement surgery in younger individuals especially in third world countries.

 
   References Top

1.Funk FJ Jr, Wells RE. Hip problems in Fibrous Dysplasia, Clin Orthop. 1973; 90: 77-82.  Back to cited text no. 1  [PUBMED]  
2.Connolly JF. Shepard's Crook Deformities of Polyostotic fibrous Dys­plasia treated by Osteotomy and Zickel Nail Fixation. Clin Orthop. 1977; 123: 22-24  Back to cited text no. 2    
3.Stewart MJ, Climer WS, Edmonson AS. Fibrous dysplasia of bone. JBone Joint Surg (Br). 1962 ; 44-2: 302-308  Back to cited text no. 3    
4.Breck LW. Treatment of fibrous dysplasia of bone by total femoral plating and hip nailing. A case report. Clin Orthop. 1972; 82: 82-83  Back to cited text no. 4    
5.Freeman BH, Bray EW, Meyer IC. Multiple Osteotomies with Zickel nail fixation for polystotic fibrous dysplasia involving proximal part of femur. J Bone Joint Surg (Am). 1987; 69: 691.  Back to cited text no. 5    
6.Lichtenstein L. Polyostotic Fibrous Dysplasia. Arch Surg. 1938; 36: 874-898.  Back to cited text no. 6    
7.Lichtenstein L, Jaffe HL. Fibrous dysplasia of bone. A condition affecting one, several or many bones. The graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism of still other Extra-skeletal Abnormalities. Arch Pathol. 1942; 33: 777-816.  Back to cited text no. 7    
8.Grabias SC, Campbell CJ. Fibrous dysplasia. Orthop Clin North Am. 1977; 8: 771-783.  Back to cited text no. 8    
9.Enneking WF. Clinical musculoskeletal pathology Third Revised ed. Gainesville: University of Florida Press. 1990:266-7.  Back to cited text no. 9    
10.Harries WH, Dudley HR Jr, Barary RJ. The natural history of fibrous dysplasia. An orthopaedic, pathological and roentgenographic study. JBone Joint Sur (Am).. 1962; 44: 207-233.  Back to cited text no. 10    
11.Hiroyuki T, Katsuro T, Matsumoto J, et al. Shepherds crook defor­mity with intracapsular femoral neck fracture in fibrous dysplasia. Clin Orthop. 1995, 310: 160-164.  Back to cited text no. 11    
12.Matsumoto J, Tsuji S, Miyamori K, et al. Precition of avascular necrosis in the femoral head following fracture dislocation using the electro chemically generated Hydrogen clearance method. J Jpn Orthop Assoc. 1988; 62: 747.  Back to cited text no. 12    
13.Whiteside LA, Lange TR, Capello FR, et al. The effects of surgical procedures on the blood supply to the femoral head. J Bone Joint Surg (Am). 1983; 65: 1127.  Back to cited text no. 13    
14.Guille JT, Kumar SJ, MacEwen GD. Fibrous Dysplasia of the proxi­mal part of femur. Long term results of curettage and hone grafting and mechanical realignment. J Bone Joint Surg (Am). 1998; 80: 648-658.  Back to cited text no. 14    
15.Bryant DD III rd RF, Tang D. Fibular Strut grafting for Fibrous dysplasis of femoral neck. J Nat Med Assoc. 1992; 84: 893-7.  Back to cited text no. 15    
16.Enneking WF, Gearen PF. Fibrous dysplasia of femoral neck treat­ment by cortical bone grafting. J Bone Joint Surg (Am). 1986; 68:1415-­22.  Back to cited text no. 16    
17.Shin HN, Chen YJ, Huang TJ, et al. Treatment of Fibrous dysplasia involving the proximal femur. Orthopaedics. 1998; 21:1263-6.  Back to cited text no. 17    
18.Kumta SM, Leung PC, Griffith JF, et al. Vascularized bone grafting for Fibrous dysplasia of upper limb. J Bone Joint Surg (Br). 2000; 82: 409-412.  Back to cited text no. 18    
19.Matlen JA, Hupfer TA, Caste ME, et al. Total hip arthoplasty and polyostotic Fibrous dysplasia: a case report. Bull Hosp Jt Dis Orthop Inst. 1987; 47: 58-66.  Back to cited text no. 19    

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Correspondence Address:
Rajkumar S Amaravati
Department of Orthopaedics and Traumatology, St. Johns Medical College Hospital, Bangalore – 560 034
India
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Source of Support: None, Conflict of Interest: None


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