BACKGROUND: Monostotic fibrous dysplasia (MFD) involving the spine is rare, and the treatment options are controversial. Surgery is needed when patients suffer from persistent pain, spinal cord compression/injury, and vertebral collapse/instability. Treatment methods include biopsy/observation, corpectomy with instrumented fusion, posterior fusion, vertebroplasty (VP), curettage and bone graft, and complete removal of the vertebra with a combined anterior and posterior fusion procedure.
METHODS: The patient was a 56-year-old woman with a 2-year history of neck pain. No obvious abnormalities were detected on neurological or physical examination, and laboratory findings were all within normal limits. An imaging examination suggested a C7 vertebral bone tumor. The patient refused to continue conservative observation treatment and requested surgery. Open VP of the C7 vertebral body was carried out, and her postoperative neck pain was completely relieved. The postoperative pathological results supported the diagnosis of fibrous dysplasia, and the patient was ultimately diagnosed with MFD. At the 12-month follow-up visit, the patient reported no clinical symptoms, and no signs of tumor recurrence were detected.
CONCLUSIONS: VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option.

Children with fibrous dysplasia (FD) chronically suffer from pain, pathological fractures, and limb deformities. The most effective methods for managing the associated pathological fractures remain controversial. The purpose of this study was to evaluate the clinical results of the treatment of diaphyseal pathological fractures in children with monostotic fibrous dysplasia (MFD) using cortical strut allografts and internal plating.We retrospectively analyzed outcomes in nine children (5 boys, 4 girls) with diaphyseal pathological fractures due to MFD, who were treated with cortical strut allografts and internal plating (6 femoral fractures and 3 humeral fractures) between July 2007 and November 2012. The median age of patients in our study was 10 years (range 6-14 years). The fracture healing time, pain, extremity function, refracture, graft resorption, and complications were recorded to evaluate treatment effects.The median time of follow-up was 69 months (range 60-75 months). All patients had good postoperative fracture healing with a median healing time of 14 weeks (range 12-16 weeks). None experienced refracture, graft resorption, nerve injury, or limitation of extremity function or other complications. The fixation remained stable in all patients, with no evidence of loosening screws after surgery.In pediatric patients, the described surgical approach is an effective and reliable treatment method for diaphyseal pathological fractures caused by MFD. Cortical strut allografts, which act as biological bone plates, can provide good mechanical support while increasing the rate of fracture union.

BACKGROUND: The purposes of this study were to analyze the prevalence of various craniofacial bones involved in patients with craniofacial fibrous dysplasia (CFD) and to demonstrate the most common form and bone involvement in patients with CFD for surgeons.
METHODS: To address the research purpose, the authors designed and performed a systematic review with meta-analysis. A comprehensive electronic search without date was performed in August 2013. Data extracted from the previously published literature were analyzed with STATA 11.0 software.
RESULTS: Relevant data were extracted from 18 studies (487 total participants) and revealed that in CFD patients, the prevalence of the monostotic type was 56% (95% confidence intervals [CI]: 0.42-0.70; P = 0.000) and polyostotic type was 47% (95% CI: 0.31-0.63; P = 0.000); McCune-Albright syndrome was a relatively rare form (7%; 95% CI: 0.02-0.12; P = 0.006). Subgroup analyses indicated that the maxilla was most commonly involved (28%; 95% CI: 0.42-0.70; P = 0.000) in monostotic CFD, followed by the orbital (27%; 95% CI: -0.23-0.76; P = 0.298), mandibular (25%; 95% CI: 0.16-0.35; P = 0.000), frontal (22%; 95% CI: 0.09-0.34; P = 0.001), and temporal bones (12%; 95% CI: 0.03-0.21; P = 0.012). The prevalence of maxilla involvement in polyostotic CFD patients was as high as 30% (95% CI: 0.18-0.42; P = 0.000).
CONCLUSIONS: This meta-analysis found that monostotic and polyostotic forms of CFD shared similar prevalence rates. Furthermore, the maxilla was found to be the most commonly involved bone in both monostotic and polyostotic CFD.

Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss. A hard external canal tumor contributing to canal stenosis and a near-absent tympanic membrane were found. Canaloplasty and type I tympanoplasty were performed, but the symptoms recurred after 5 years. She received canal wall down tympanomastoidectomy with ossciculoplasty at the second time, and secondary cholesteatoma in the middle ear was diagnosed. Fifteen years later, left otorrhea recurred again and transcanal endoscopic surgery was performed for middle ear clearance. Currently, revision surgeries provide a stable auditory condition, but her monostotic temporal fibrous dysplasia is still in place.

OBJECTIVE: Fibrous dysplasia (FD) is a benign bone lesion manifested by local pain, swelling and deformity change. We report a case of monostotic fibrous dysplasia of the first thoracic vertebrae that treated by radical removal and reconstruction.
METHODS: A 29-year-old man with monostotic fibrous dysplasia of the first thoracic vertebrae was admitted to our department because of persistent, dull back pain for 3 months. Radical removal of the first thoracic vertebrae and reconstruction were performed in a combined posterior-anterior approach. This patient experienced complete pain relief without any complication.
CONCLUSIONS: This report presents a rare case of monostotic fibrous dysplasia of the first thoracic vertebrae, with symptoms of chronic back pain that was successfully treated with radical excision and reconstruction, providing a good option to the patient.

Defects of the lateral malleolus and distal fibula occur occasionally, mainly because of severe trauma or wide resection of fibular neoplasms. These bony defects should be reconstructed to avoid persistent pain and to prevent an abnormal gait induced by ankle instability. Various methods of repair have been developed, including allografting, autologous iliac crest transplantation, scapular apophysis transplantation, and arthrodesis and prosthetic reconstruction. A reverse transfer of the proximal vascularized fibula is also effective. Its morphologic advantage is apparent, and the surgery is simple and direct, with no need for vascular anastomosis. We treated recurrent fibrous dysplasia in the distal fibula of a young male with wide resection and reconstruction using reverse transfer of the proximal, vascularized fibula. At the 6-year follow-up examination, the patient had an American Orthopaedic Foot and Ankle Society ankle-hindfoot score of 100, without any apparent complications. Additionally, we critically reviewed other methods for reconstructing the lateral malleolus and distal fibula and have concluded that reverse transfer of the proximal vascularized fibula compares favorably and could be the treatment of choice.

Malignant transformation in fibrous dysplasia (FD) is uncommon. The purpose of this study was to investigate clinical and imaging features, and outcomes of malignant transformation in monostotic FD.Data for 10 pathologically confirmed malignant transformations in monostotic FD from January 2005 to December 2013 were retrospectively reviewed. Patient data were recorded, and radiographs (n = 10), computed tomography (CT) (n = 5), magnetic resonance (MR) (n = 4), and bone scintigrams (n = 10) were evaluated for lesion location, margin, cortical destruction, marrow involvement, periosteal reaction, and soft tissue mass by 2 musculoskeletal radiologists with agreement by consensus. Clinical features, management, and prognosis were also analyzed for each of the 10 cases.There were 8 male and 2 female patients (mean age 46.5 ± 15.9 years). The affected sites were the femur (n = 4), humerus (n = 2), tibia (n = 3), and ilium (n = 1). Five cases had received previous surgery and 5 cases had no history of surgery. No patients had been given prior irradiation treatment. For the 5 cases with surgery, radiographs and CT showed purely osteolytic lesions with poor margination in the curettage area (n = 5), cortical destruction (n = 5), obvious soft tissue mass (n = 1), and mineralization (n = 2). For the 5 cases without surgery, radiographs and CT identified poorly marginated, osteolytic lesions within or near the area with \"ground-glass\" opacity (n = 4), cortical erosion (n = 4), and mineralization (n = 2). Magnetic resonance imaging (MRI) also identified lesions with heterogeneous signal intensity and pronounced enhancement. Bone scintigraphy revealed eccentric increased uptake of radionuclide in monostotic lesion (n = 10). Pathology reports revealed osteosarcoma (n = 7), fibrosarcoma (n = 2), and malignant fibrous histiocytoma (MFH) (n = 1). At the end of the study, 1 patient died from tumors, 1 patient was alive with lung metastasis, 1 patient experienced recurrence, and 7 patients were alive without recurrence.Patients with FD and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation. The radiographic feature of FD-related malignancies is poorly marginated, mineralized, and osteolytic lesions with cortical destruction. Further investigations are needed to explore the pathogenesis of malignancies in FD and to establish optimal therapeutic strategies.

The surgical treatment of fibrous dysplasia remains a challenge for the pediatric orthopedist because of its high recurrence rate. Although a few successful treatments have been reported by using en bloc resection and bone graft in adults, this has not been reproduced in children. In this report, the authors present two children (2.5 and 6 years old) with monostotic expansile fibrous dysplasia involving the ulna and fibula, respectively, who underwent en bloc resection and autograft to replace the involved bones. Good bone union and functional recovery were obtained postoperatively. However, during a follow-up period of 8 and 5 years, respectively, the lesions recurred completely, and the deformities remained progressing over time. En bloc resection and bone graft cannot prevent recurrence in skeletally immature patients with monostotic expansile fibrous dysplasia, and cannot alter for the natural history of the disease. A combination of other management should be considered in children with fibrous dysplasia.

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BACKGROUND: The clinical characteristics of craniomaxillofacial fibrous dysplasia (FD) have not been clearly identified. The objective of this meta-analysis is to assess the predominance of the monostotic form of FD using an evidence-based review. Furthermore, we examined the laterality and sex dominance of FD in patients from international study populations.
METHODS: We performed a systematic search of PubMed, Embase, Cochrane Central Register of Systematic Reviews, Cochrane Central Register of Controlled Trials and EBSCO for trials published through August 2013. Data extracted from the literature were analysed with Review manager 5.0.24.
RESULTS: The results of this study showed that unilateral FD occurred more frequently than bilateral FD (RR, 12.37; 95% CI, 2.92-61.24; P = 0.008, N = 263 patients). For unilateral FD, there was no significant difference between cases involving the left or right side of the face (RR, 0.98; 95% CI, 0.66-1.44; P = 0.91; N = 201 patients). There were no significant sex-dependent differences for monostotic and polyostotic forms of craniomaxillofacial FD.
CONCLUSIONS: There is a significantly higher percentage of the unilateral form than the bilateral form in the craniomaxillofacial FD studies analysed, and an almost equal distribution of left- and right-sided unilateral FD. These proportions were maintained among males and females and there were nearly equal frequencies of monostotic and polyostotic FD.