Abstract:Objective To study the imaging and pathological characteristics of fibrous dysplasia of bone(FDB)in limbs of children, to improve knowledge and preoperative diagnosis of the disease.Methods From January 2011 to December 2016, the clinical features, imaging findings, pathologic features of 32 patients with limbs FDB confirmed by surgical pathologic in Children's Hospital of Nanjing Medical University were analyzed retrospectively. Of 32 patients, 19 patients were males, 13 patients were females. The average age was 9.2 years (2 to 14 years). X-ray examination was performed in 32 patients, CT scan and 3D reconstruction in 30 patients, and MRI examination in 14 patients. All imaging data, including shape, location, boundary, combined pathologic fracture or not and so on, were analyzed, those imaging features and pathology were compared and analyzed.Results Twenty-seven patients with single bone lesions including 15 lesions in the femur, 7 lesions in the tibia, 1 lesion in the fibula, 2 lesions in the humerus, and 2 lesions in the ulna. Four patients with multiple bone lesions including lesions were located in the femur and tibiofibula in 2 patients, femur and occipital bone in one patient, and ulna and radius in one patient. One patient with McCune-Albright syndrome was located in right femur, ilium and pubis. Pathologic fracture was seen in 11 patients. Histological examination revealed fibrous dysplasia was bone developmental anomaly characterized by replacement of normal bone and marrow bone by fibrous tissue. Imaging with ground-glass like changes, pathology of the mature fibrous tissue was leathery. Imaging showed cystic expansion translucent changes, pathological manifestations often dominated by fibrous tissue active growth, qualitative change mucoid degeneration and cystic changes. Imaging vegetable sponge like changes, the pathological manifestations for the hyperplasia of fibrous tissue and new bone trabecula distributed hybrid.Conclusions FDB of limbs usually involves femur and tibia. The images of FDB have some characteristic including ground-glass form images and expansive images. Pathological features showing normal bone marrow tissues are replaced by a large number of fibrous tissues. The different imaging changes have their corresponding pathological characteristics.
孙祥水, 侯华成, 王邦, 楼跃. 儿童四肢长骨骨纤维性结构不良影像学与病理学表现对照分析[J]. 中华解剖与临床杂志, 2018, 23(2): 99-103.
Sun Xiangshui, Hou Huacheng, Wang Bang, Lou Yue. Comparsion between imaging and pathological findings of fibrous dysplasia in limbs of children. Chinese Journal of Anatomy and Clinics, 2018, 23(2): 99-103.
Thomsen MD, Rejnmark L. Clinical and radiological observations in a case series of 26 patients with fibrous dysplasia[J]. Calcif Tissue Int, 2014, 94(4): 384-395. DOI:10.1007/s00223-013-9829-0
[2]
Rosario MS, Hayashi K, Yamamoto N, et al. Functional and radiological outcomes of a minimally invasive surgical approach to monostotic fibrous dysplasia[J]. World J Surg Oncol, 2017, 15(1):1.DOI 10.1186/s12957-016-1068-1
[3]
Leet AI, Boyce AM, Ibrahim KA, et al. Bone-grafting in polyostotic fibrous dysplasia[J]. J Bone Joint Surg Am, 2016, 98(3): 211-219. DOI:10.2106/JBJS.O.00547
[4]
Anitha N, Sankari L, Malathi L , et al. Fibrous dysplasia-recent concepts[J].J Pharm Bioallied Sci, 2015, 7(Suppl 1):S171-S172. DOI:10.4103/0975-7406.155892
[5]
Kushare IV, Colo D, Bakhshi H, et al. Fibrous dysplasia of the proximal femur: surgical management options and outcomes[J]. J Child Orthop, 2014, 8(6): 505-511.DOI: 10.1007/s11832-014-0625-9
[6]
Ippolito E, Bray EW, Corsi A, et al. Natural history and treatment of fibrous dysplasia of bone: a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society[J]. J Pediatr Orthop B, 2003, 12(3): 155-177. DOI:10.1097/01.bpb.0000064021.41829.94
[7]
DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment[J]. J Bone Joint Surg Am, 2005, 87(8): 1848-1864. DOI:10.2106/JBJS.D.02942
[8]
Han I, Choi ES, Kim HS. Monostotic fibrous dysplasia of the proximal femur: natural history and predisposing factors for disease progression[J]. Bone Joint J, 2014, 96-B(5): 673-676. DOI:10.1302/0301-620X.96B5.33281
[9]
Zhang X, Chen C, Duan H, et al. Radiographic classification and treatment of fibrous dysplasia of the proximal femur: 227 femurs with a mean follow-up of 6 years[J]. J Orthop Surg Res, 2015, 10: 171. DOI:10.1186/s13018-015-0313-6
[10]
Ippolito E, Farsetti P, Boyce AM, et al. Radiographic classification of coronal plane femoral deformities in polyostotic fibrous dysplasia[J]. Clin Orthop Relat Res, 2014, 472(5): 1558-1567. DOI:10.1007/s11999-013-3380-1
[11]
Benedetti Valentini M, Ippolito E, Catellani F, et al. Internal fixation after fracture or osteotomy of the femur in young children with polyostotic fibrous dysplasia[J]. J Pediatr Orthop B, 2015, 24(4): 291-295. DOI:10.1097/BPB.0000000000000192