Radiographic Follow-Up of Fibrous Dysplasia in 138 Patients.

OBJECTIVE. The purpose of this study was to explore the temporal changes in fibrous dysplasia (FD) at radiographic follow-up. MATERIALS AND METHODS. A total of 138 patients with FD who had undergone extremity radiography at least twice with a minimum 12-month interval between examinations were enrolled in this study. FD was monostotic in 99 patients and polyostotic in 39 patients. Patients were also classified according to skeletal maturity as follows: Patients 16 years old or younger were classified in the skeletally immature group (n = 34), and patients 17 years old or older were classified in the skeletally mature group (n = 104). We compared the initial and follow-up radiographs for the following findings: lesion size, opacity, sclerotic rim, calcification, and trabeculation. RESULTS. Of the 138 patients, radiographic follow-up showed no change in lesion size in 101 patients (73.2%), progression in 31 (22.5%), and regression in six (4.3%). FD in immature bones progressed more often than FD in mature bones (23/34 [67.6%] vs 8/104 [7.7%], respectively; p = 0.000), and polyostotic FD had a greater chance of regressing than monostotic FD (4/39 [10.3%] vs 2/99 [2.0%]; p = 0.032). A temporal change in FD lesion opacity was noticed in a minority of patients (19/138, 13.8%). Variable changes were observed in the sclerotic rim, calcification, and trabeculation. CONCLUSION. The radiographic follow-up of FD showed that approximately a quarter of lesions changed in size over time. Regardless of the change in lesion size, opacity and several morphologic features of FD changed during the follow-up period, which might reflect the histopathologic evolution of FD.

Monostotic fibrous dysplasia at C7 treated with vertebroplasty: a case report and review of the literature.

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. CASE PRESENTATION: 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. CONCLUSION: VP can relieve pain while stabilizing the spine. Thus, the surgical treatment of MFD vertebral lesions by VP is a valuable option.

Ulnar focal cortical indentation: a progressive, deforming variant of focal fibrocartilaginous dysplasia.

BACKGROUND: Focal fibrocartilaginous dysplasia is a rare growth disturbance of bone resulting in deformity. In the ulna, focal fibrocartilaginous dysplasia is particularly rare, and the characteristic fibroligamentous tether can result in progressive deformity with progressive dislocation of the radial head. The fibroligamentous tether is similar in appearance and function to the Vickers ligament seen in Madelung deformity. The imaging features of ulnar focal fibrocartilaginous dysplasia include a unilateral angular deformity in the ulna with a radiolucent cortical defect and surrounding sclerosis, with secondary radial bowing or radial head dislocation. Focal fibrocartilaginous dysplasia of the ulna has been described using the term ulnar focal cortical indentation. OBJECTIVE: To review the clinical and radiologic features of four cases of ulnar focal cortical indentation occurring in pediatric patients. MATERIALS AND METHODS: We retrospectively reviewed picture archiving and communication system and electronic medical record imaging findings with surgical correlation of ulnar focal cortical indentation at two large specialty pediatric hospitals. RESULTS: Ulnar focal cortical indentation lesions typically arise in the ulnar metaphysis and result in angular forearm deformities with progressive radial deformity including radial head dislocation. Early surgical intervention prevents progression of the deformity and retains range of motion. CONCLUSION: Ulnar focal cortical indentation, although rare, is likely a progressive form of focal fibrocartilaginous dysplasia resulting in significant deformity and disability. Early recognition of the characteristic imaging features is important for early surgical intervention to preserve range of motion and prevent radial head dislocation.

Functional and radiological outcomes of a minimally invasive surgical approach to monostotic fibrous dysplasia.

BACKGROUND: Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). PATIENTS AND METHODS: Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies-plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating-were included. There were nine men and three women, with median age of 38 years. Mean follow-up was 88 weeks. Five cases involved the proximal femur, two each involved the femoral and tibial diaphyses, and one each involved the distal humerus, radial diaphysis, and proximal tibia. All cases were reviewed for functional and radiological outcomes. RESULTS: Median time to full activity was 1 day (range 1 to 3) for the plain open biopsy group, while the prophylactic bridge-plating and plain ATP reconstruction groups had longer median recovery times (59 days, range 3 to 143, and 52 days, range 11 to 192, respectively). Musculoskeletal Tumor Society scores at last follow-up were excellent for all the cases (mean 29.6, range 25 to 30). Radiological analysis using Gaski et al.'s criteria showed plain open biopsy resulted in partial resolution of proximal femoral lesions, while ATP reconstruction and prophylactic plating resulted in no change and progression in this lesion site, respectively. For femoral diaphyseal lesions, prophylactic plating resulted in partial resolution, while ATP reconstruction resulted in no change. In the tibial diaphysis, prophylactic plating resulted in partial resolution, while plain open biopsy resulted in no change. For the lesions involving the distal humerus and the proximal tibia, plain open biopsy resulted in partial resolution, while for the radial diaphyseal lesion, ATP reconstruction resulted in no change. Radiological progression was limited in 11 (92%) cases, and none had postoperative complications. CONCLUSION: Plain open biopsies for asymptomatic lesions; prophylactic bridge plating for symptomatic, large diaphyseal lytic lesions; and plain ATP reconstructions for both small and large nondiaphyseal symptomatic lytic lesions may be acceptable alternatives to curettage-incorporating procedures for MFD.

En bloc resection and bone graft: does it alter the natural history of monostotic expansile fibrous dysplasia in children?

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.

Treatment of craniofacial fibrous dysplasia: a case report.

The concept of "fibro-osseous lesions" of bone has evolved over the last several years and now includes two mayor entities: the fibrous dysplasia (FD) and the cement-ossifying fibroma (COF). Fibrous dysplasia is considered to be a developmental, tumor-like (hamartomatous), fibro-osseous disease of unknown etiology. There is a maxillary predominance when craniofacial FD occurs in the jaws and the maxillary sinus is commonly involved. Differentiation of OF from FD is important because of differences in treatment and behaviour. This article report a case of 60-year-old female with a history of painless unilateral palatal swelling.

Focal fibrocartilaginous dysplasia in the upper limb: case report and review of the literature.

Focal fibrocartilaginous dysplasia (FFCD) is an uncommon, benign bone lesion that causes angular deformities of the long bones in young children. Most deformities were seen around the knee. Diagnostic criteria are based on clinical and radiological signs: unilateral angular deformity in a long bone of a young child, associated with on X-ray a typical lucent bony defect with surrounding sclerosis at the concavity of the deformity with a cortical defect. The etiology is not understood. There were 16 cases described previously involving the upper extremity: 9 cases with FFCD of the ulna, 3 of the humerus, 2 of the radius and 2 of the phalanx. We report a case of cubitus varus deformity in a young girl of 2 years and 2 months where the deformity worsened very quickly. We believe that debridement of the fibrous tether at very young age will prevent further deformity and can correct spontaneously by remaining growth.

Surgical treatment of craniomaxillofacial fibrous dysplasia: functionally or aesthetically?

BACKGROUND: Fibrous dysplasia (FD) is a tumor-like growth that consists of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. The most commonly affected bones are facial bones, causing a number of facial cosmetic and functional problems. METHODS: From December 2008 to July 2012, 10 patients with craniomaxillofacial fibrous dysplasia were treated by conservative resection and local recontouring. The patients were followed up yearly, with an average of 3 years; the longest follow-up period was 5 years. RESULTS: All the 10 patients received appropriate treatment and histopathological examinations were performed to confirm the diagnosis of FD. Four patients with zygoma involved had received partial zygoma osteoectomy and 2 patients received mandibular partial osteoectomy. Average time of follow-up was 3 years, with a range from 1 to 5 years, and all patients obtained satisfactory aesthetic and functional results. CONCLUSION: In most patients, a conservative surgery will achieve good functional and aesthetic results. For patients with mild symptoms, the aesthetic effect should be given priority while for the heavier patients the restoration of function and aesthetic effects should all be taken into account.

Frequency of spontaneous osteosarcomatous transformation of craniofacial fibrous dysplasia: a systematic review.

OBJECTIVE: A systematic review was undertaken to examine the spontaneous of craniofacial fibrous dysplasia (FD) into osteosarcoma (OS). STUDY DESIGN: Five databases were searched, and data were evaluated in 2 subsets: (1) frequency analysis involving only cross-sectional studies that specifically examined patients for osteosarcomatous transformation, thereby allowing a calculation of the frequency of transformation of craniofacial FD, and (2) case analysis of case reports and case series of FD that underwent osteosarcomatous transformation. Quality assessment of the studies in the frequency and case analyses was performed. RESULTS: Seven cross-sectional studies involving 779 patients in the frequency analysis documented transformation of craniofacial FD into OS in 13 patients, for a frequency of 1.67%. Of the 18 separate cases of osteosarcomatous transformation in the case analysis, 11 (61.1%) occurred in females, 11 (61.1%) developed from monostotic FD, and 7 (38.9%) arose in the mandible, with 5 in the maxilla (27.8%) and 6 (33.3%) in other craniofacial sites. The mean age of the patients at the time of malignant diagnosis was 36.4 years, with an average of 11.3 years from FD diagnosis to the appearance of OS. The quality of the articles ranged from fair to excellent. CONCLUSIONS: The risk of osteosarcomatous transformation of craniofacial FD is low, but the possibility of transformation should be taken into consideration by clinicians, as this will guide treatment and follow-up regimens.

Focal fibrocartilaginous dysplasia in the ulna: report on 3 cases.

Focal fibrocartilaginous dysplasia is an uncommon, benign bone lesion that causes deformity in young children. It is most commonly encountered in the proximal tibia, with few cases documented in the upper extremity. We report 3 cases affecting the ulna. In each case, the fibrous tissue appeared to anchor itself in the ulnar diaphysis, behaving as a tether that retards growth. All 3 patients had excision of the fibrous tissue. There was concern for radial head subluxation before surgical intervention, but all patients maintained a congruent radiocapitellar articulation. The visible deformity improved in all 3 patients, although the limbs remained shorter than the contralateral side. In our limited series, we believe that early excision of the fibrous tissue tether can prevent radiocapitellar joint dislocation in patients with focal fibrocartilaginous dysplasia of the ulna.

Monostotic fibrous dysplasia of the mandible.

Fibrous dysplasia is a benign fibro-osseous disease that affects 1 or more bones. Deformities leading to aesthetic and functional disorders are observed in almost all cases. Plastic surgery is often recommended when the jaws are involved. Monostotic fibrous dysplasia of the mandible is an unusual manifestation of the disease that is usually benign, occurs in young individuals, and is managed by conservative curettage or debridement, such as surgical sculpting. The authors report a case of a 15-year-old patient with a large monostotic fibrous dysplasia located in the right mandible, which was treated by contouring bone. The lesion did not recur on follow-up for 4 years after the surgical procedure.

Treatment of fibrous dysplasia of the proximal femur by means of pedicled iliac crest bone graft: a case report.

In this report, we present a case of treatment of fibrous dysplasia (FD) of the proximal femur with the pedicled iliac crest bone graft. An 18-year-old patient presented with hip pain and polyostotic dysplasia with involvement of the proximal femur and a history of pathological fracture. The patient was operated on using vascularized bone graft from the iliac crest and osteosynthesis with Dynamic Hip Screw (DHS®). With vascularized bone graft, we found an improvement on X-ray with no reabsorption, and with osteosynthesis, we controlled the pain and prevented pathological fracture and progression of the deformity. Several other studies where the pedicled iliac crest bone graft has been successfully used for the management of defects in the proximal femur (osteonecrosis of the femoral head and pseudarthrosis of the femoral head) can be found in the medical literature. However, the pedicled iliac crest bone graft in a patient with FD of the proximal femur is unique.

Decortication of fibrous dysplasia of the maxillary sinus.

We present 3 cases of fibrous dysplasia of the maxillary sinus that were treated by decortication through the anterior wall of the maxilla. The thickened anterior wall of the maxilla and the zygoma were contoured into normal shape with a bur, and a 2 × 2-cm window was made below the infraorbital foramen using a reciprocating saw. The removed piece of bone was 3 to 5 mm thick. All abnormal fibrous tissues in the maxillary sinus were extirpated by drilling through the window until the sinus cavity was clear. The preserved bone plate was restored in place to close the window of the maxillary anterior wall and fixed with miniplates. Three patients were treated by means of such internal decompression method. No overgrowth or disfigurement of the facial bone was appreciated 14 to 51 months postoperatively. We contend that internal decortication of fibrous dysplasia through a window in the anterior wall is effective to minimize external expansion of fibrous dysplasia.

Malignant transformation of monostotic fibrous dysplasia into angiosarcoma in the proximal tibia.

The majority of bone angiosarcomas are primary tumors while secondary angiosarcomas arise after radiation therapy or bone infarctus. This article presents a case of malignant transformation of monostotic fibrous dysplasia into angiosarcoma. An 80-year-old female presented with pain on right cruris. Radiological examination revealed a lesion with lytic areas and destruction of cortical bone on right tibia. Gross and histopathological examination showed two areas with an abrupt transition. The solid component was composed of curved, immature bony trabeculae in a fibroblastic stroma. The other component involved epitheloid cells forming slit-like vascular spaces. The diagnosis of angiosarcoma and fibrous dysplasia was given. Malignant transformation of fibrous dysplasia into angiosarcoma is extremely rare; as this is the sixth case in the existing literature. Prognosis of fibrous dysplasia is generally good and less than 1% of the patients develop a malignant tumor. Therefore, patients with fibrous dysplasia should be offered a life-long follow-up.

Fibrous dysplasia limited to an ossicle.

Fibrous dysplasia is an unusual pathologic condition caused by abnormal bone metabolism. Temporal bone involvement is often seen, but it is uncommon to find fibrous dysplasia limited to the middle ear, especially originating in and confined to a single ossicle. Here we report a case of osteofibrous dysplasia limited exclusively to an ossicle (malleus) causing gradual conductive hearing loss, which recovered after eventual complete removal of the dysplastic area. The lesion showed firm attachment to adjacent structures and initial removal was not possible. This report provides information to help other otologic surgeons facing similar conditions.

Monostotic fibrous dysplasia of the clivus: image-guided endoscopic endonasal transsphenoidal surgery.

INTRODUCTION: Fibrous dysplasia is a non-heritable, congenital disease involving the bones. Its diagnosis relies on image examination and pathology. Treatment is usually conservative, but in cases of cranial nerve impingement or extension to the condyles, surgery should be considered. In this paper, we describe a singular case of monostotic fibrous dysplasia of the clivus in a child. CASE REPORT: A 14-year-old boy presented with chronic headache and diplopia. A neurological examination revealed palsy of the right VIth cranial nerve. The radiological findings were consistent with fibrous dysplasia, with a hypointense and isointense clival lesion on T (1)- and T (2)-weighted images, respectively. Surgical treatment was indicated by cranial nerve impairment. An endoscopic endonasal approach with an image-guided system was used. The patient was discharged four days after surgery and his diplopia improved postoperatively. DISCUSSION: A variety of surgical approaches have been used in the treatment of midline extra-axial cranial base tumors. Fibrous dysplasia of the clivus without expansion to the condylus can be removed with a transsphenoidal endonasal endoscopic approach. Preoperative imaging reveals the individual patient's anatomy and improves the intraoperative orientation. CONCLUSION: Image-guided transsphenoidal endoscopic surgery is recommended for its safety and minimal invasiveness.

Monostotic fibrous dysplasia of the distal phalanx: case report.

Monostotic fibrous dysplasia in the finger is rare. We are aware of only 4 cases, none of which involved the distal phalanx. We present a case of a 16-year-old boy with a fibrous dysplasia of the left long finger distal phalanx. Treatment consisted of curettage and bone grafting.

Malignant transformation of monostotic fibrous dysplasia in the mandible.

Malignant transformation of fibrous dysplasia (FD), which was first introduced by Coley and Steward is very rare. We present a case of malignant transformation of monostotic mandibular FD after 2 surgical excisions, 20 and 15 years ago, respectively. The treatment choice of FD is usually conservative. With the advanced surgical techniques, radical excisions followed by immediate reconstruction have been reported. However, the decision criteria are still controversial and we might think that radical treatment is a good method for recurrent lesions based on the recent literatures. The case discussed here is of sarcomatous transformation of a recurrent FD.