• The Journal of the Korean bone and joint tumor society
• /
• v.2 no.1
• /
• pp.78-87
• /
• 1996

The results of treatment of eighteen lesions of fibrous dysplasia which of them seventeen lesions were treated with surgery were reviewed at the Department of Orthopedic Surgery, College of Medicine, Hanyang University Hospital. We studied to evaluate the functional clinical results and the recurrence according to the type of disease, grafted bone, methods of treatment and location of lesion. We treated sixteen patients(five males and eleven females) and their mean age was 22.6 years. There was no association with skin pigmentation or dysfunction of endocrine system. Twelve patients had a monostotic pattern and four patients had a polyostotic pattern. Twelve lesions were treated with curettage and bone grafting and four lesions in the proximal femur were treated by internal fixation with curettage and bone grafting. One lesion was treated by en-bloc resection. There were eleven satisfactory results in twelve monostotic lesions and there were four satisfactory results in five polyostotic lesions, but the recurrence were four cases, respectively. The two unsatisfactory results were seen in two patients treated by autografting, but there were three recurrence of four lesions in autografting only, one of five in autografting and allografting, four of eight in allgrafting or xenografting. Four of six lesions in upper extremity were recurred after curettage and bone grafting and five of them showed satisfactory results. In pelvic and lower extremity lesions, the recurrence were occurred in two lesions after curettage and bone grafting and in two lesions after internal fixation and bone grafting. The recurrence does not always provide an unsatisfactory functional results and the recurrence showed higher rate in radiologic pattern of ground glass appearance with-out marginal sclerotic rim, but the recurrence according to grafted bone showed similar rates. Curettage and bone grafting is adequate for a symptomatic lesion and firm internal fixation is needed for a lesion in proximal femur. In use of grafted bone, xenograft or allograft may be preferable to autograft because of the disadvantage of autografting like a increased blood loss, prolonged operation times, etc.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.35 no.5
• /
• pp.304-309
• /
• 2009

Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.13 no.2
• /
• pp.120-125
• /
• 2013

Purpose: McCune-Albright syndrome (MAS) is caused by activating mutations in the GNAS gene, resulting in peripheral precocious puberty, caf$\acute{e}$-au-lait spots, and polyostotic fibrous dysplasia (POFD). The aim of the present study was to describe the diverse clinical and endocrine characteristics of patients with MAS. Methods: Seven patients with MAS were included in this study and medical charts were reviewed retrospectively for following parameters: patient's sex and age at diagnosis, POFD, ovarian cysts, and precocious puberty. Results: The mean age at diagnosis was $5.8{\pm}4.2$ years. One patient was male (14%) and the other six patients were female (86%). Peripheral precocious puberty was associated with 6 patients (86%). Five patients manifested premature menarche as early as 2 to 5 years of age. Letrozole was administered to 4 patients, tamoxifen to one patient and GnRH agonist to one patient. Five females developed ovarian cysts. Thyroid function tests were performed in all patients and one patient showed hyperthyroidism (14%) and has been treated with methimazole. One patient presented with pseudohypoparathyroisdism, phosphaturia, calciuria suggesting hypophosphatemic rickets. Six patients (86%) revealed POFD. One patient had symptoms of optic nerve compression and secondary esotropia and 2 patients had bone pain. Conclusion: This study described clinical characteristics and endocrine complications of patients with MAS. Careful physical examinations with history taking and serial endocrine function tests should be needed to detect complications such as endocrinologic hyperfunction and POFD.

• Journal of the Korean Orthopaedic Association
• /
• v.54 no.6
• /
• pp.519-527
• /
• 2019

Purpose: To evaluate the treatment result in polyostotic fibrous dysplasia classified according to the involvement of the femoral head. Materials and Methods: Twenty-three patients from March 1987 to March 2014 were reviewed retrospectively. Patients with no involvement of the physeal scar in the femoral head were classified as Type I, and those with involvement of the physeal scar were classified as Type II. A plain radiograph was used to measure the femoral neck shaft angle, articulo-trochanteric distance (ATD), and anterior bowing through the lateral view. A teleoroentgenogram of the lower limb was used to measure the leg length discrepancy and lower extremity mechanical axis. The pre- and postoperative femoral neck-shaft angle and ATD were compared to assess the degree of correction of the deformity. Results: Among a total of 46 cases (23 patients), 28 cases (23 patients) had lesions in the proximal femur. Type I were 16/28 cases (15/23 patients) and Type II were 12/28 cases (9/23 patients). The preoperative proximal femoral neck-shaft angle was 116.8° in Type I and 95.3° in Type II. The ATD was 12.08 mm in Type I and -5.54 mm in Type II. The deformity correction showed significant improvement immediately after surgery, the deformity correction was lost in Type II (neck shaft angle Type I: 133.8°-130.8°, Type II: 128.6°-116.9°, and ATD Type I: 17.66-15.72 mm, Type II: 7.44-4.16 mm). The extent of anterior bowing was 12.74° in Type I and 20.19° in Type II. The mean differences of 12 mm between the 9 patients who showed a leg length discrepancy and the lower extremity mechanical axis showed 4 cases of lateral deviation and 7 cases of medial deviation. Conclusion: In polyostotic fibrous dysplasia, when the femur head is involved, the femur neck shaft angle, ATD, and anterior bowing of the femur had more deformity, and the postoperative correction of deformity was lost, suggesting that the involvement of the femoral head was an important factor in the prognosis of the disease.

• The Journal of the Korean bone and joint tumor society
• /
• v.13 no.1
• /
• pp.22-30
• /
• 2007

Purpose: We describe clinical, radiographic, MRI and pathologic findings as well as final outcome after simple curettage and bone graft of cystic fibrous dysplasia (FD) in the long bone, which has been rarely documented in the literature. Materials and Methods: Clinical records, radiographs, MRI and histologic slides of 11 patients with cystic FD in the long bone were retrospectively analyzed. Results: Six patients complained pain for several months, 4 patients presented pain after trivial injury event, and 1 patient suffered pathologic fracture. The mode of involvement was monostotic in 10 patients and polyostotic in l patient. The femur was affected in 7 patients, the humerus in 3, and the radius in 1. Radiography showed prominent, expansive lysis associated with ground-glass density of FD. MRI revealed 2 different signals of FD and cyst. Microscopic examination revealed classic findings of FD and non-specific cystic degeneration. The final outcome was satisfactory in every patient. Local recurrence was not observed. Conclusion: Cystic FD in the long bone seems not as rare as the scarcity of reported cases would indicate. MRI features provide a basis for differential diagnosis between benign cystic change and malignant transformation. Cystic FD would be an indication for surgery and simple curettage with allo-chip-bone graft is effective.

• The Journal of the Korean bone and joint tumor society
• /
• v.11 no.2
• /
• pp.111-117
• /
• 2005

Purpose: We evaluate the results of treatment of pathologic femur fractures secondary to bone tumors in children. Materials and Methods: Between January 1995 and June 2004, 18 patients(20 cases) were evaluated. Their mean age of the first episode of fracture was 10.2 years and mean follow-up period is 42.5 months. Primary bone tumors, the location of fracture, time to union and complications were evauated. Results: Fractures occurred at proximal portion in 14 cases, shaft 3 cases and distal portion 3 cases. The bone tumors causing pathologic fracture were fibrous dysplasia(9 c ases), simple bone cyst(4 cases), aneurysmal bone cyst(4 cases), nonossifying fibroma(2 cases) and eosinophilic granuloma(1 case). In the treatment for fractures, cast was in 11 cases, internal fixation 8 cases and external fixation in 1 case. In the treatment for tumors, observation was in 11 cases, curettage & bone graft in 8 cases and resection in 1 case. In polyostotic fibrous dysplasia, all cases were treated by cast initially but deformity developed in all cases. Fracture prevention and deformity correction were obtained with intramedullary nailing. Conclusion: Adequate choice of treatment of bone tumor and fracture will result in good prognosis.