Treatment approach with clinical follow-up in monostotic fibrous dysplasia: a case series

Abstract

Background: Long bones are one of the most common sites involved in fibrous dysplasia. In some cases, there is no deformity, but patients suffer sustained or intermittent dull pain.

Case presentation: Since 2021, a retrospective case series of seven East Asian patients with fibrous dysplasia of a long bone without severe deformity were reviewed. These patients include three male and four female patients, with an average age of 32.7 years (range 4-70 years). Fibrous dysplasia was diagnosed at a total of three different sites in these seven cases including five femurs (71.4%), one humerus (14.3%), and one fibular bone (14.3%). All patients received treatment with allogenic bone grafting or cortical strut grafting with or without compression locking and screw fixation. The radiological and clinicopathologic presentation was analyzed by the surgeon. There were no cases with polyostotic forms or fibrous dysplasia in combination with extraskeletal disease. The presenting complaint was pain in all cases and localized swelling in 1 (14.3%) of the cases.

Conclusions: Autogenous fibular cortical strut grafting and compression hip screw fixation achieved good postoperative function and provided an early return to work for adult patients with fibrous dysplasia of the femoral neck with mild but prolonged symptoms. However, total hip replacement may be a suitable method for fibrous dysplasia of the femoral neck accompanied by pathological fracture.

Keywords: Allogenic bone grafting; Clinicopathologic presentation; Fibrous dysplasia; Pathological fracture; Radiological presentation; Surgical treatment.

Fig. 1

The radiographic results of Patient 1. A The preoperative anteroposterior X-ray showed a ground-glass appearance (blue asterisk) of the left proximal femur bone. B The preoperative transverse computed tomography image showed the appearance of a lesion site in the marrow cavity of the proximal femur bone (blue asterisk). C The preoperative transverse T2-weighted (magnetic resonance imaging) image showed high intensity in the medullary space of the left proximal femur bone (blue asterisk)

Fig. 2

The result of histological examination of our seven cases. A–H Hematoxylin and eosin staining, magnification 40×. All pathological examinations of our cases showed the typical appearance of fibrous dysplasia. The lesion contained some irregular immature bone trabeculae scattered throughout fibrous tissue. B and D Fibrous dysplasia accompanied by bone cyst. Solid areas and cystic spaces filled with blood, with cellular septa, and hyperplastic fibrous cells. G and H The first result (F) of fibular fibrous dysplasia and final result of recurrent fibular fibrous dysplasia showed the same appearance of irregular trabecular bone and hyperplastic fibrous tissue with a woven bone formation

Fig. 3

The therapeutic strategies fibrous dysplasia in our cases. A and F The postoperative anteroposterior X-rays showed the radiographically apparent refilling of bone grafts in ground-glass and partial radiolucent sites. B, D, and E The postoperative anteroposterior X-rays showed curettage and bone allograft along with internal fixation in these lesion sites. C The postoperative anteroposterior X-rays showed the total hip replacement had been performed in her right hip joint

Fig. 4

The postoperative X-rays of our cases with removal of internal fixation. A The postoperative anteroposterior X-ray showed bone fusion (yellow asterisk) in a previous lesion site with a radiographically ground-glass appearance for Patient 2. B and C The postoperative anteroposterior X-ray showed removal of internal fixations and bone fusion (yellow asterisk) in previous lesion sites with a radiographically ground-glass appearance for Patient 4 and Patient 5, respectively. D For Patient 7, a strong autogenous fibular bone fused to allogenic fibular bone (yellow asterisk) was observed, and the internal fixation was removed by the surgeon

Fig. 5

Fibrous dysplasia accompanied with bone cyst in Patient 2. A Preoperative radiographic image showed multiple lesion sites with a ground-glass appearance (yellow arrows) in the femur bone for Patient 2. B–D Preoperative axial computed tomography images showed the appearance of lesion sites in the marrow cavity of the proximal femur bone (yellow asterisks). E Preoperative magnetic resonance imaging. The preoperative sagittal T2-weighted image showed multiple high intensities in the medullary space of the femur bone (yellow arrows)

Fig. 6

The radiographic images of the neck of the right femur in Patient 3. A Preoperative anteroposterior X-ray showed a typical ground-glass appearance (blue asterisk) and pathological bone fractures of the neck of the right femur. B and C Preoperative computed tomography image showing pathological bone fractures (red arrows) and cystic lesion of the site (yellow asterisks)

Fig. 7

Fibrous dysplasia accompanied with bone cyst in Patient 4. A Preoperative radiographic image showing a typical ground-glass appearance of the proximal femur bone (black asterisk). B and C Axial computed tomography showed a typical proximal femur lesion with a cyst in the right proximal femur and femoral neck (black asterisks). D and E Preoperative magnetic resonance imaging. Coronal and sagittal T2-weighted images showed the right proximal femur, and a femoral one showed a proximal femoral lesion with a dominant cystic cavity without any adjacent soft-tissue edema (black asterisks)

Fig. 8

The radiographic images of fibrous dysplasia in Patient 5. A Preoperative anteroposterior X-ray showed a typical ground-glass appearance (black asterisk). B Preoperative coronal magnetic resonance imaging. The preoperative sagittal T2-weighted image showed multiple high intensities in the medullary space of humeri bone and a cystic lesion of the site (black asterisk)

Fig. 9

The radiographic images of fibrous dysplasia in Patient 6. A Preoperative anteroposterior X-ray showed a typical ground-glass appearance (black asterisk). B Preoperative coronal magnetic resonance imaging. The preoperative sagittal T2-weighted image showed high intensities in the cystic lesion site of the femur bone (black asterisk)

Fig. 10

A therapeutic strategy of recurrent fibular fibrous dysplasia. A The first preoperative anteroposterior X-ray showed a ground-glass appearance and partial radiolucent lesions with clear borders around soft tissue (black asterisk). B The first postoperative anteroposterior X-ray showed a radiographically apparent bone graft refilling of ground-glass and partial radiolucent sites (yellow arrows) in X-ray images compared with preoperative X-ray images, as shown in Fig. 10 A. C The postoperative anteroposterior X-ray showed recurrent fibular fibrous dysplasia on follow-up (black asterisk). D Radical resection of the lesion and a fibularis longus bone allograft combined with fibular locking plate and screws were used for reconstruction of the left fibula. D A strong autogenous fibular bone fused to allogenic fibular bone was observed (red arrows), and the internal fixation was removed by a surgeon