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CASE REPORT
Year : 2015  |  Volume : 15  |  Issue : 1  |  Page : 86-89

Fibrous dysplasia of femur, treated with fibular bone graft


1 Department of Orthopedics, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
2 Department of Physiology, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
3 Department of Pathology, Burdwan Medical College and Hospital, Burdwan, West Bengal, India

Date of Web Publication19-Jan-2015

Correspondence Address:
Arunima Chaudhuri
Krishnasayar South, Borehat, Burdwan - 713 102, West Bengal
India
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DOI: 10.4103/1319-6308.149545

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  Abstract 

Background: Fibrous dysplasia is a benign bony lesion managed surgically with curettage, bone grafting, and, in some cases, internal fixation. Aims: Study result of cortical bone grafting with fibular strut graft in patient of symptomatic dysplastic lesion without varus deformity of proximal femur. Case Report: A 25-year-old female presented with pain in left hip for 3 months which increased in intensity during walking. Clinically there was no swelling or deformity and tenderness. Range of hip joint movement was normal. The patients had no endocrine disturbances. X-rays of left proximal femur showed radiolucent cystic area involving trochanter, neck, and 30% head at postero-inferior aspect. No pathological fracture was seen. Image showed homogeneous loss of the normal trabecular pattern, with a ground-glass appearance caused by fibrous dysplasia. Patient underwent dual autogenous non-vascularized fibular strut grafts. The dual fibular graft was used as a bridge in the dysplastic lesion in femoral neck and it was securely anchored to the head of femur as well as lateral femoral cortex. The fibular graft was providing excellent structural support without any need for any form of internal fixation. At routine follow-up at 8 months, patient was found ambulating at her own without any substantial pain at the hip joint. Conclusion: For carefully selected patients (symptomatic dysplastic lesion without varus deformity of proximal femur) cortical bone grafting with fibular strut graft is an excellent procedure which provide strong structural support to biomechanically weakened bone.

  Abstract in Arabic 

خلل النسيج الليفي لعظم الفخذ و معالجتة بالطُّعم العظمي -
خلفية: خلل النسيج الليفي ورم عظمي حميد عولج بطرق جراحية مختلفة منها: الشظية العظمية ، و التثبيت الداخلي في بعض الحالات.
الأهداف: دراسة نتيجة الطعم العظمي القشري في المرضى الذين يعانون أعراض الخلل النسيبجي الليفي في عظم الفخذ دون تقوس وتشوهات في عظم الفخذ.
تقرير الحالة: امرأة بلغ من العمر 25 عاما جاءت تشكو الما في الفخذ منذ 3 أشهر وتزداد كثافة الألم أثناء المشي. بعد الكشف السريري لم يلاحظ تورم أو تشوه ، أو رقّة وكانت حركة مفصل الورك طبيعية وليس لدى المريضة أي اضطرابات في الغدد الصماء.
وقد أظهر التصوير الإشعاعي لعظم الفخذ باحة كيسية شملت المدور، والرقبة و30% من المنظر الخلفي السفلي، ولم يظهر كسر مرضي, كما أظهرت الصورة عدم التجانس في الطراز التربيق العادي، مع ظهور أرضية زجاجية سببها خلل النسيج الليفي لعظم الفخذ. وقد خضعت المريضة لطعم بدعامة مشبكية مزدوجة من غير أوعية دموية. وقد استخدمت الدعامة جسرا في الورم الليفي في عنق الفخذ وقد كانت راسية بشكل آمن في رأس عظم الفخذ والقشرة الجانبية على حد سواء. وقد حقق الطعم المشبكي دعما ممتازا، بدون الحاجة لأي نوع من أنواع التثبيت الداخلي. وبعد المتابعة الروتينية لمدة ثمانية أشهر استطاعت المريضة أن تعتمد على نفسها في المشي و الحركة دون أن تشعر بالألم في مفصل الورك.
الخلاصة: أظهرت الدراسة أن استخدام طعم العظم مع الدعامة المشبكية تعد طريقة ممتازة حيث إنها توفر دعما قويا لضعف العظام في بعض المرضي المصابين بخلل تانسيج الليفي.




Keywords: Fibrous dysplasia, fibular strut graft, operative outcome


How to cite this article:
Ghosh S, Chaudhuri A, Datta S, Sirdar BK. Fibrous dysplasia of femur, treated with fibular bone graft. Saudi J Sports Med 2015;15:86-9

How to cite this URL:
Ghosh S, Chaudhuri A, Datta S, Sirdar BK. Fibrous dysplasia of femur, treated with fibular bone graft. Saudi J Sports Med [serial online] 2015 [cited 2019 Jun 18];15:86-9. Available from: http://www.sjosm.org/text.asp?2015/15/1/86/149545


  Introduction Top


Fibrous dysplasia is a benign bony lesion resulting from congenital dysplasia of bone. The hallmark is replacement of normal bone and marrow by fibrous tissue and small, woven spicules of bone. In some patients it involves a single bone (monostotic fibrous dysplasia), but in others it involves multiple bones (polyostotic fibrous dysplasia). The most common sites of occurrence are the proximal femur, tibia, humerus, ribs and cranio-facial bones (apleys). Fibrous dysplasia can occur in the epiphysis, metaphysis, or diaphysis. [1],[2],[3],[4]

Fibrous dysplasia primarily affecting adolescents and young adults, it accounts for 7% of benign bone tumors. Many of the asymptomatic lesions are found incidentally; the remainder present with symptoms of swelling, deformity, or pain. Fibrous dysplasia has been associated with multiple endocrine and nonendocrine disorders and with McCune-Albright and Mazabraud's syndrome. The etiology remains unclear, but molecular biology suggests a mutation in the G (s) alpha subunit and activation of c-fos and other proto-oncogenes. Most cases do not require intervention, but those that do usually are managed surgically with curettage, bone grafting, and, in some cases, internal fixation. When some intervention is necessary but surgery is not practical, treatment is with bisphosphonates. [1],[2],[3],[4]


  Case report Top


A 25-year-old female attended Department of Orthopedics of Burdwan Medical College complaining of pain in left hip for 3 months which increased in intensity during walking. Clinically there was no swelling or deformity and tenderness. Range of hip joint movement was normal.

The patient was advised detailed radiologic investigation (X-ray), CT scan, MR scan, hematological and biochemical investigation. The patients had no endocrine disturbances. X-rays of left proximal femur showed radiolucent cystic area involving trochanter, neck, and 30% head at postero-inferior aspect [Figure 1]. No any pathological fracture was seen. Image showed homogeneous loss of the normal trabecular pattern, with a ground-glass appearance caused by fibrous dysplasia. CT scan showed a bony mass with relative homogeneous appearance of the lesion. MR scan showed low signal within of left proximal femur.
Figure 1: Anteroposterior and lateral view showing radiolucent cystic lesion involving trochanter and neck of femur

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Operative procedure

The patient was placed in supine position in a fracture table without applying any traction under image-intensifier. Operation was performed under spinal anaesthesia. A long segment (6″) of fibula was harvested through a minimally invasive double incision technique from ipsilateral (left) lower limb. Surgical approach was through lateral approach to the proximal femur: A longitudinal incision given approximately 8 cm, beginning over the tip of the greater trochanter and extending down the lateral side of the thigh over the lateral aspect of the femur. A cortical fenestration was made wide enough to visualize whole lesion in the lateral aspect of trochanter using osteotome. Lesion was removed by curettage and removed material sent for histopathological examination. Two parallel guide pins were inserted into the neck through lateral side of femoral cortex and reamed by DHS triple reamer. Cavity was irrigated with normal saline and harvested non-vascularized fibular strut graft was inserted in reamed portion and cavity filled with cancellous bone graft. Finally the wound was closed in layers after applying suction drain.

Post-operative care

Following surgery patient was advised to avoid weight bearing and active static quadriceps and hamstring exercises. After 2 weeks of operation stitches were removed. Partial weight bearing was allowed after 8 week and full weight bearing given after 12 week. X-rays were taken at 6-week intervals. The patient had significant relief of her left hip pain postoperatively. Fibular cortical graft was to be incorporated with femoral neck after about 1 year of operation with no residual pain, deformity, and functional restriction of left hip.


  Discussion Top


Fibrous dysplasia is a benign bone lesion characterized by replacement of bone and bonemarrow with fibrous tissue with small woven spicules of bone which accounts for approximately 7% of all benign bone tumors. Majority of lesions ceased to progress following adolescense with the exception of McCune Albright syndrome. Conservative treatment is recommended for dysplastic lesions involving upper extremity and adolescents. [1],[2],[3],[4] Surgery is recommended for fibrous dysplasia of femoral neck with progressive or severe deformity, prevention of pathological fracture, persistent pain, and for confirmation of diagnosis by biopsy. The surgical treatment options include curettage with cancellous and cortical bone grafting, and internal fixation by either intramedullary device or osteotomy and nail-plate fixation. [1],[2],[3],[4]

Guille et al. in 1998 [5] reviewed the long-term outcomes of treatment of fibrous dysplasia of the proximal part of the femur in 22 patients. Patients who had monostotic disease had no involvement of the calcar femorale, fewer microfractures, less deformity, and stronger bone that could support internal fixation. Patients who had polyostotic disease had frequent involvement of the calcar femorale; more microfractures; severe deformity, including shepherd's crook deformity; and, in many instances, bone that could not support internal fixation. Twenty-two of the 27 femora had a microfracture at the time of the initial presentation. At least one osteotomy was performed in four femora that had monostotic disease and in nine femora that had polyostotic disease. Curettage and cancellous or cortical bone-grafting did not appear to have any advantage compared with osteotomy alone in the treatment of symptomatic lesions, as all grafts resorbed with persistence of the lesion. At the time of the latest follow-up evaluation, no lesion had been eradicated or had decreased in size. A satisfactory clinical result was achieved in twenty patients. Two patients who had polyostotic disease and an endocrinopathy (one of whom had bilateral involvement) had an unsatisfactory result. All three femora in these two patients had a neck-shaft angle of less than 90 degrees at the time of the most recent follow-up evaluation. Varus deformity of the proximal part of the femur is best treated with valgus osteotomy and internal fixation early in the course of the disease. Results showed that if the calcar of the femoral neck is involved or if the quality of the bone is such that internal fixation is not possible, a medial displacement valgus osteotomy can provide a more mechanically favorable position for healing of the microfracture.

Children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively by Durand et al. in 2007. [6] The study cohort included 22 children. Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail. In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities.

Li et al. in 2009 [7] conducted a study to find an effective method of surgical treatment of fibrous dysplasia of bone involving the proximal femur. From January 2001 to January 2006, 57 patients with fibrous dysplasia of bone involving the proximal femur were treated. There were 29 males and 28 females, aging 8-50 years (mean 22 years). Thirty-five patients were involved one bone and 22 patients were involved more than two bones. The choice of the various operative procedures depended on the quality of the bone and the extent of the lesion. When the quality of the bone was good, then curettage and bone-grafting was performed. When the quality of the bone was poor, curettage and bone-grafting combined with internal fixation was performed. Medial displacement valgus or valgus osteotomies were used to treat fibrous dysplasia of bone involving the proximal part of the femur with coax varus. No infections and recurrent fracture and progression of the deformity occurred in all patients.

Yang et al. in 2010 [8] conducted a retrospective study on 13 patients to investigate the effectiveness of valgus osteotomy combined with intramedullary nail in treatment of Shepherd's crook deformity of fibrous dysplasia. The four-step procedure was performed orderly as valgus osteotomy, curettage lesion, massive impaction allograft, and insert intramedullary nail with neck cross pinning. Results showed that valgus osteotomy can correct Shepherd's crook deformity, prevent recurrent fracture, and restore alignment, thus improve functioning of limb. The intramedullary nail with neck cross pinning should be the first consideration of internal fixation. Massive impaction allograft is the key technique to improve full incorporation of allograft and to prevent pathological fracture.

In the case reported here, patient underwent dual autogenous non-vascularized fibular strut grafts. The dual fibular graft was used as a bridge in the dysplastic lesion in femoral neck and it was securely anchored to the head of femur as well as lateral femoral cortex [Figure 2]. The fibular graft was providing excellent structural support without any need for any form of internal fixation [Figure 3]. At routine follow-up at 8 months, patient was found ambulating at her own without any substantial pain at the hip joint.
Figure 2: Immediate postoperative X-Ray showing internal fixation with dual fibular strut graft

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Figure 3: X- Ray taken 1 year after operation showing incorporation of fibular bone graft

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  Conclusion Top


For carefully selected patients (symptomatic dysplastic lesion without varus deformity of proximal femur) cortical bone grafting with fibular strut graft is an excellent procedure which provide strong structural support to biomechanically weakened bone.

 
  References Top

1.
Kusano T, Hirabayashi S, Eguchi T, Sugawara Y. Treatment strategies for fibrous dysplasia. J Craniofac Surg 2009;20:768-70.  Back to cited text no. 1
    
2.
DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64.  Back to cited text no. 2
    
3.
Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD. Fibrous dysplasia. J Am Acad Orthop Surg 2004;12:305-13.  Back to cited text no. 3
    
4.
Chapurlat RD, Orcel P. Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol 2008;22:55-69.  Back to cited text no. 4
    
5.
Guille JT, Kumar SJ, MacEwen GD. Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment. J Bone Joint Surg Am 1998;80:648-58.  Back to cited text no. 5
    
6.
Durand S, Hamcha H, Pannier S, Padovani JP, Finidori G, Glorion C. Fibrous dysplasia of the proximal femur in children and teenagers: Surgical results in 22 cases. Rev Chir Orthop Reparatrice Appar Mot 2007;93:17-22.  Back to cited text no. 6
    
7.
Li T, Xing B, Duan H, Liu Y, Pei F, Tu C. Surgical treatment for fibrous dysplasia of bone involving proximal femur. Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2009;23:261-4.  Back to cited text no. 7
    
8.
Yang L, Jing Y, Hong D, Chong-Qi T. 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 2010;130:497-502.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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