Case Of Polyostotic Fibrous Dysplasia Research Articles

Rare case of polyostotic fibrous dysplasia of bilateral tibia with pathological fracture and associated endocrinopathy managed conservatively

Rare case of polyostotic fibrous dysplasia of bilateral tibia with pathological fracture and associated endocrinopathy managed conservatively

18F-FDG PET findings of a case of polyostotic fibrous dysplasia

Fibrous dysplasia of bone (FDB) was a condition that the normal bone tissue was replaced by benign hyperplastic fibrous tissue; these patients may present with pain, swelling, or pathological fracture. The imaging findings of FDB on CT or MR images have been documented previously, however it was usually misdiagnosed as primary bone malignancy or metastatic tumor due to lack of specificity in routine radiographic examinations. Furthermore, the most common affected sites were extremities, pelvis, femur and craniofacial bones, and the spine is less involved. Here we reported a case with thoracic FD, she presented with chest and back pain for 9 years, the 18F-FDG PET/CT examination revealed abnormal hypermetabolic foci at 8th and 9th vertebrae, and the right ninth posterior ribs; Histopathological examination showed fibrous dysplasia, and she was discharged 1 week later, and was in good health during follow up.

A Novel Technique for Single-stage Correction of Severe Shepherd Crook Deformity of a Femur With the Large Cystic Lesion and Pathologic Fracture

Fibrous dysplasia with severe proximal femur deformity and multiple large cystic lesions associated with a pathologic fracture is an orthopedic challenge. Correction is often impossible with conventional fixation methods. We report a technique to address the deformity and fracture simultaneously in these scenarios. A 32-year-old man, a case of polyostotic fibrous dysplasia with severe shepherd crook deformity and the pathologic fracture was managed by curettage, multilevel osteotomy, tibial strut allograft, extramedullary fixed-angle implant, and augmentation with cortico-cancellous bone grafting in a single stage. The mechanical axis of the femur was corrected, with an increase in the neck-shaft angle from 70.6 to 121 degrees. Union was achieved with good outcomes in cosmetic and functional status without recurrence at 18 months follow-up. In proximal femur fibrous dysplasia, critical analysis of the bone stock for implant purchase especially in the proximal femur with large cystic lesions is essential. Augmentation of the head, neck, and calcar region with cortical strut allograft provide stable fixation and decreases the deformity recurrence. This technique is particularly useful in severe forms of the disease; wherein there would be an impaired implant purchase in the weak pathologic bone, deficient lateral wall, and a wide medullary canal.

Fibrous Dysplasia in Maxillary Bone: Case Report

Fibrous dysplasia is a benign lesion, congenital, recurrent character and etiology unknown, caused by a bone modeling disorder with constant alterations of the normal bone being replaced by immature fibrous tissue. The lesion may involve one or more bones being the maxilla the facial bone more affected. We report a case of polyostotic fibrous dysplasia in a male patient complaining of increased volume in the right genital region. In this case, Computed Tomography (CT) was the main radiographic examination to demonstrate the extension and radiodensity that the lesion assumes in the craniofacial bones, being therefore of fundamental importance in the surgical planning and in the longitudinal follow-up of the operated patient. The diagnosis was confirmed by anatopathology and the treatment of choice was osteoplasty. This article also aims to review the main clinical, radiological, histopathological, differential diagnosis and Fibrous dysplasia treatment.

Abstract #528 An Aggressive Case of Polyostotic Fibrous Dysplasia and Thyroid Cancer: A Possible Association

Abstract #528 An Aggressive Case of Polyostotic Fibrous Dysplasia and Thyroid Cancer: A Possible Association

Polyostotic Fibrous Dysplasia of Maxillofacial Region: A Case Report

Conditions in which normal bone is replaced with fibrous tissue containing abnormal bone or cementum constitute group of pathologies bone dysplasias. These pathologies should be differentiated from tumours, because the treatments of these pathologies are very different. Types of Fibrous dysplasia are monostotic, polyostotic or may occur as a component of McCune-Albright syndrome and Mazabraud syndrome. The most commonly affected bones are long bones, skull bones and ribs are. We present a case of polyostotic fibrous dysplasia in a 25 years old male patient.

Polyostotic fibrous dysplasia of the mandible and bony nasal septum with intracranial expansion

Fibrous dysplasia is an uncommon benign tumour in the nasal cavity. Due to adjacent vital soft tissue structures surgery is often the treatment of choice. We report a rare case of polyostotic fibrous dysplasia of the mandible and the ethmoid bone including nasal bony septum with intracranial expansion, and include a review of the literature. The tumour was successfully resected using a combined transnasal and craniotomy approach. Fibrous dysplasia is a rare benign tumour in the head and neck region, which often limits its growth during the follow-up period. Careful monitoring of the patient is necessary because in some rare cases there can be severe symptoms due to the growth of the tumour, which are indications of immediate surgery in the head and neck region.

Fibrous Dysplasia Of The Thyroid Cartilage – A Case Study And Review Of The Literature.

Fibrous dysplasia is a rare disorder of bone that is uncommon in the head and neck region. Herein, we present a rare case of polyostotic fibrous dysplasia involving the right temporal bone and thyroid cartilage in a 19-year-old male. This unique location necessitates close surveillance due to the proximity to the airway and the potential for malignant degeneration.

Polyostotic fibrous dysplasia with secondary aneurysmal bone cyst in tibia

Fibrous dysplasia is a benign tumor-like lesion of bone believed to be developmental in origin. Polyostotic fibrous dysplasia (FD) is a rare condition. Our case was further complicated by the presence of secondary aneurysmal bone cyst (ABC). This is the second reported case of polyostotic FD with secondary ABC.

A case of polyostotic fibrous dysplasia leading to multiple deformities

Abstract is not required for Clinical Images (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 5 No. 10, October 2014. ISSN – [0976-3198] Int J Case Rep Images 2014;5(10):734–736. www.ijcasereportsandimages.com Kutbay et al. 734 clinical images OPen access A case of polyostotic fibrous dysplasia leading to multiple deformities Nilufer Ozdemir Kutbay, Banu Sarer Yurekli, Fusun Saygili

Fibrous Dysplasia of the Thyroid Cartilage–A Case Study and Review of the Literature

Fibrous dysplasia is a rare disorder of bone that is uncommon in the head and neck region. Herein, we present a rare case of polyostotic fibrous dysplasia involving the right temporal bone and thyroid cartilage in a 19-year-old male. This unique location necessitates close surveillance due to the proximity to the airway and the potential for malignant degeneration.

Correction of Fibrous Dysplasia through Malarplasty without Internal Fixation

Fibrous dysplasia is a chronic developmental disease of the skeleton involving formation of immature bone. Involvement of facial bones can result in deformation of facial contour. Prominent cheek area is often treated with malarplasty, which has a variety of modifications, depending on the surgeon's preference. The authors report on a case of polyostotic fibrous dysplasia in which the patient's right cheek had a prominent appearance. The prominence was corrected with malarplasty without internal fixation. The soft nature of bone involved in fibrous dysplasia could provide greater flexibility for modification of the traditional surgery.

Polyostotic Fibrous Dysplasia with Epiphyseal Involvement in Long Bones: A Case Report

Fibrous dysplasia (FD) is an uncommon, but well-known benign skeletal disorder. In cases affecting long bones, FD is commonly recognized to locate in the diaphyses or the metaphyses and to spare the epiphyses. In this paper, we present a rare case of polyostotic FD in a 13-year-old girl with unilateral multiple epiphyseal lesions arising in the femur, the tibia, and the fibula with the growth plates.

Clinicoradiologic perspective of a severe case of polyostotic fibrous dysplasia

Fibrous dysplasia (FD) of bone is a congenital non-heritable disorder that was first reliably reported by von Recklinghausen, when he described patients with pathologic condition of bone characterized by deformity and fibrotic changes that he termed as osteitis fibrosa generalisata. FD may involve one bone (monostotic) or multiple bones (polyostotic) and occurs throughout the skeleton with predilection for long bones, ribs, and cranio-facial bones. Seventy percent of the lesions are monostotic and asymptomatic, and identified incidentally. The polyostotic form of disease is often deforming and devastating, with multiple skeletal complications like repeated fractures, limb length discrepancies, and bone pains. The bone lesion of unknown origin is characterized by slow progressive replacement of normal bone by abnormal proliferative, isomorphic fibrous tissue. This case report documents a 40-year-old male with severe polyostotic FD that involved most of the skeleton, including long bones of all extremities, pelvis, facial bones, and skull base. Initial evaluation consisted of physical examination, plain radiographs, which was followed by computed tomography scan, Single-photon emission computed tomography scan, and biochemical and hematological examination. This paper stresses on the clinical implications and management of this rare debilitating disease.

An extensive hemimelic polyostotic fibrous dysplasia: a case report

We report a new case of polyostotic fibrous dysplasia (FD). A 26-year-old woman was referred to our department complaining of pain in her left arm. She had suffered for tow fractures in left leg and arm previously. Plain radiographs showed osteolytics lesions at the left humerus and radius. Histological examination of the surgical specimens showed FD. She has beneficed with zoledronic acid perfusion.

An interesting case of polyostotic fibrous dysplasia: The "pirate sign" evaluated with Tc-99m methylene diphosphonate single-photon emission computed tomography/computerized tomography

Polyostotic fibrous dysplasia is a rare progressive benign disorder of the bone. Bone scintigraphy is extremely useful in the initial evaluation for identifying the extent of disease. We report a case presenting with pathological fracture of the shaft of the right femur. After treatment of the fracture, bone scintigraphy revealed involvement of multiple bones including the skull and facial bones. The utility of single-photon emission computed tomography/computerized tomography in the evaluation of the extent of skull base involvement is highlighted.

Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune–Albright syndrome

We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.

Displasia ósea fibrosa en un varón joven

Fibrous dysplasia of bone is a skeletal disease due to a specific mutation in the GNAS1 gene with a broad spectrum of clinical presentations ranging from isolated monostotic and polyostotic forms to associations with other extra-skeletal manifestations (McCune-Albright syndrome). The main complications are bone pain, deformities and fractures and sometimes cranial nerve compression and the development of bone sarcomas. Traumatologic surgery represents the principal approach in complicated lesions. Currently, the efficacy of pharmacological therapies is fairly limited. Cyclic administration of intravenous biphosphonates is the most effective approach to achieve symptomatic improvement and to lower the risk of fractures, although more evidence from well-designed randomized clinical trials evaluating the safety and efficacy of these treatments is still required. We present a case of polyostotic fibrous dysplasia of bone in a 19-year-old male.

Restrictive lung disease and cor pulmonale secondary to polyostotic fibrous dysplasia

Polyostotic fibrous dysplasia is a rare benign pathological condition of bone in which proliferation of fibrous and osteoid elements results in expansile deformities of the skeleton. We present a case of polyostotic fibrous dysplasia in a young man in whom the severe deformities of the chest wall and spine produced restrictive lung disease, cor pulmonale and respiratory failure.

Fibrous dysplasia localized to spine: a diagnostic dilemma

Fibrous dysplasia of the spine is uncommon, especially in monostotic form. Isolated vertebral involvement in polyostotic form is very rare. We report a case of polyostotic fibrous dysplasia with lesions localized to dorso-lumbar spine in a 45-year-old rheumatoid arthritis patient. No associated appendicular lesions, cutaneous manifestations or endocrinopathies were seen. The extreme rarity of this type of lesion can pose a diagnostic dilemma, and biopsy is required for diagnosis. The association with rheumatoid arthritis in our case seems to be a chance occurrence.