Navigation-guided nasal endoscopic surgery for acute vision loss caused by fibrous dysplasia: a case report and review of literatures

Background Bone fibrous dysplasia is a benign disease of bone tissue dysplasia. Vision impairment is the commonest neurological complication of craniofacial fibrous dysplasia. Most of the vision loss caused by craniofacial fibrous dysplasia is usually a gradual process. Very few present with acute visual impairment as described in our case. Case presentation We report a patient with fibrous dysplasia presenting rapidly progressive visual loss in the left eye secondary to bone cyst formation. Transnasal endoscopic surgery guided by navigation with drainage and curettage of this bone cyst and orbital decompression resulted in progressive improvement in visual acuity that returned to normal 1 month post-operatively. Conclusions In cases with acute visual loss due to fibrous dysplasia, emergency surgical treatment should be considered to preserve vision. In the surgical approach, navigation-guided nasal endoscopic surgery may be preferred because of its advantages.

New technologies in oral radiology as a diagnostic aid for monostotic fibrous dysplasia: a review

Monostotic fibrous dysplasia is a benign asymptomatic lesion that affects only one bone, which is replaced by amorphous connective tissue. Clinically there is an increase in the volume of the affected area, which is observed by imaging as a radiopaque area with diffuse non-corticalized limits capable of expanding to neighboring structures, and it is histologically evidenced as “resembling Chinese characters”. The lesion is seen as a radiopaque image with diffuse borders in conventional or digital radiography, while cone beam computed tomography identifies the exact location and extension of an isodense, mixed or hyperdense image of non-corticalized edges. Magnetic resonance imaging is also used when the lesion involves soft tissues or nerves, and bone scintigraphy is performed in order to systemically observe bone quality. The objective of this article was to describe the new technologies in oral radiology for the diagnosis of monostotic fibrous dysplasia and the importance of the current imaging methods in achieving an adequate diagnosis. These techniques range from conventional radiography to bone scans, which provide images of higher quality, clarity and better precision with less invasive techniques to the patient. This review of the literature helps to expand the knowledge of dental professionals in relation to the clinical and imaging characteristics of monostotic fibrous dysplasia.

Monostotic Fibrous Dysplasia in the Femur Strongly Expressing RANKL With Concomitant Osteoporotic Vertebral Compression Fracture: A Case Report

Background/Aim: This study aimed to present a rare case of fibrous dysplasia (FD) in a healthy young adult man with a concomitant osteoporotic vertebral compression fracture. FD is a benign lesion of the bone characterized by replacement of the medullary component with fibro-osseous tissue that contains abnormally arranged trabeculae of immature woven bone. Recently it has been reported that several bone tumors including FD express the receptor activator of nuclear factor-kappa B (RANK) and its ligand (RANKL). Therefore, we hypothesized that FD contributed to osteoporosis, linked by the RANK-RANKL pathway of osteoclastogenesis. Case Report: We report the case of a healthy man with monostotic femoral fibrous dysplasia (FD) with concomitant 7th thoracic vertebra compression fracture due to osteoporosis [young adult mean (YAM) was 79% in bone mineral density (BMD)]. After curettage of the FD, artificial bone grafting in the cavity, and administration of alendronate sodium, BMD improved considerably within 9 months. FD is a benign bone condition in which abnormal fibrous tissue replaces normal bone. The axis of the receptor activator of nuclear factor-kappa B (RANK) and its ligand (RANKL) has been implicated in osteoporosis pathogenesis. RANKL immunohistochemical staining was performed, and strong staining of stromal cells was observed compared to other FD cases that showed weak to moderate staining. Conclusion: The presence of FD might have contributed to the low BMD due to the RANK-RANKL axis acting as osteoclastogenesis stimulator.