Visual loss associated with fibrous dysplasia of the anterior skull base. Case report and review of the literature

The authors present a case of visual loss associated with fibrous dysplasia of the anterior skull base and the surgical management of this case. Preoperative computerized tomography scanning in this patient demonstrated a patent optic foramen and a rapidly growing cystic mass within the orbit, which was responsible for the patient's visual loss. A literature review revealed that this case is typical, in that cystic mass lesions of various types are frequently responsible for visual loss associated with fibrous dysplasia. The authors did not find significant evidence in the literature to support the notion that visual loss associated with fibrous dysplasia is the result of progressive optic canal stenosis, thus raising questions about the value of prophylactic optic canal decompression. Instead, as demonstrated by this case and those uncovered in the literature review, most instances of visual loss result from the rapid growth of mass lesions of cystic fibrous dysplasia, mucoceles, or hemorrhage. Findings of the literature review and the present case of fibrous dysplasia of the anterior skull base support a role for extensive surgical resection in these cases and indicate a need for additional prospective analysis of a larger number of patients with this disease.     

Fibrous Dysplasia of the Maxilla: Diagnostic Reliability of the Study Image. Literature Review

Objective Fibrous dysplasia (FD) is a benign bone disorder in facial bones. This study evaluates the possibility of diagnosing fibrous dysplasia on imaging alone, without biopsy of the lesion, which is often burdensome for the patient. Materials and Methods The authors bring their experience of four cases of bone lesions of the maxillofacial region and present a review of published studies. The imaging techniques evaluated are computed tomography (CT) and magnetic resonance imaging (MRI) with and without contrast. Results The literature review demonstrates that it is impossible to make diagnosis of fibrous dysplasia exclusively by imaging. Radiographic images often show a ground-glass appearance, which is characteristic but not pathognomonic of fibrous dysplasia. Conclusion Although CT and MRI images may in many cases suggest a diagnosis of fibrous dysplasia, histological examination or follow-up imaging should follow.     

Monostotic fibrous dysplasia of the lumbar spine: case report and review of the literature

Fibrous dysplasia has been frequently reported to involve the spine in the polyostotic form, but only rarely has monostotic fibrous dysplasia been noted. In the only previously reported case involving the lumbar spine, the disease was confined to the transverse process. The present case demonstrates monostotic fibrous dysplasia involving the vertebral body in addition to the posterior elements. The plain radiographic, computerized tomography, and histologic examinations are presented.     

Monostotic fibrous dysplasia of the lumbar spine: case report and review of the literature

Monostotic fibrous dysplasia of the spine is extremely rare. We present a 57-year-old man who complained of persistent low back pain with monostotic fibrous dysplasia of the lumbar spine. Computed tomography revealed a lytic expansile lesion and marginal sclerosis in the L2 posterior element, although a bone scan did not reveal increased uptake in the lesion. The patient underwent total excision of the tumor via a posterior approach. Two years later, he is asymptomatic with no recurrence of the lesion, as confirmed by imaging.     

经眶外侧颅内外联合径路治疗颅眶骨纤维异常增殖症

目的 探讨颅眶骨纤维异常增殖症手术治疗的径路. 方法 对累及额骨、眼眶、蝶骨、颞骨的复杂颅面骨纤维异常增殖症患者,应用经眶外侧颅内外联合径路进行手术治疗,包括眶外侧及颞部开窗,病灶的部分或大部切除,视神经减压,颅眶骨外形重建等. 结果 自2005年以来,于临床治疗8例患者,术后随访9个月至3年,无明显并发症发生,复杂颅眶部畸形得以矫正,视力障碍有所改善.头颅CT示骨瓣愈合良好,未见病变复发. 结论 经眶外侧颅内外联合径路可作为治疗颅眶部骨纤维异常增殖症的首选方法.     

Monostotic fibrous dysplasia of the cranial vault: a case report

Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault. Although there have been several reports on magnetic resonance imaging (MRI) findings of fibrous dysplasia involving the skull base, cases occurring in the cranial vault have seldom been reported. We describe here a rare case of monostotic fibrous dysplasia that occurred in the parietal bone and discuss the characteristics of the MRI findings. A 47-year-old female was admitted to our hospital with a complaint of vertigo. A computed tomography (CT) scan did not reveal any intraparenchymal lesions in either the infra- or supratentorium, and her vertigo improved immediately without any treatments. However, a solitary osteolytic lesion was found incidentally in the left parietal bone. MRI showed that the lesion demonstrated hypointensity on T1-weighted images and hyperintensity on T2-weighted images, and was enhanced heterogeneously following injection of Gadolinium-DTPA. Removal of the parietal bone containing the lesion was performed according to the patient's wishes. The histopathological findings of the removed tissues corresponded to fibrous dysplasia. Although it is well known that craniofacial fibrous dysplasia demonstrates iso- or hypointensity on T1- as well as T2-weighted images, in the present case, the lesion showed apparent hyperintensity on T2-weighted images. These findings suggest that fibrous dysplasia can display various MR intensities depending on its origin.     

Polyostotic Fibrous Dysplasia with Craniofacial Localization Presenting with Frontal Lobe Compression in a 14-year-old Girl

Summary  We describe a rare case of polyostotic fibrous dysplasia with craniofacial localization associated with involvement of the spine and extremities. A 14-year-old girl presented with progressive headache, left frontal swelling, exophthalmos of the left eye, deformity and palpable mass in the left frontal area. Cranial computerized tomography revealed extensive involvement of all cranial bones except right frontal and right parietal bones. Most of the facial bones were invaded. Especially, there was the involvement of ethmoidal air sinuses and frontal sinus also. Computerized tomography showed left frontal lobe compression. In addition, the spine and bones of the extremities were involved in the patient. Craniofacial approach was planned. Cranial surgery was performed by an extradural frontal approach. Firstly, the frontal cyst was excised. For left frontal lobe decompression, we removed successfully all the abnormal bones causing mass effect and frontal deformity. Secondly, facial surgery was performed with external approach. An attempt to remove all of the involved bone is necessary, as the lesion may recur and grow if a portion of dysplastic bone is left in place. However, the present case suggests that removal of all the involved bones in the polyostotic fibrous dysplasia may be possible in spite of extensive involvement. In this situation, it may be removal of only abnormal bones responsible for compression of affected neural elements is indicated.     

Frontal bone hyperostotic mass associated with fibrous dysplasia in a male patient with systemic lupus erythematosus (SLE)

We present a rare clinical report of a 45-year-old man affected by Systemic Lupus Erythematosus (SLE) with a unilateral mass of the left frontal bone, diagnosed as a possible recurrence of fibrous dysplasia. This patient was evaluated with computed tomography (CT scan) and was treated with resection of the mass and reconstruction with splitting a calvarian bone graft. The pathological evidence was suggestive for a bone cavernous haemangioma.No previously described cases of bone dysplasia associated with this systemic syndrome were reported so far. Patient's disease was under stringent control at the time of hospitalization, and the outcome has been successful, even though a mild increase of inflammatory indexes was reported after surgery. This laboratory evidence was transient and not related to further clinical complications.     

Bisphosphonates and bone remodeling: effectiveness in Paget's disease, fibrous dysplasia and osteoporosis

Paget's disease of bone, bone fibrous dysplasia and osteoporosis are characterized by abnormal bone remodeling, and frequently imply intervention of orthopedists. Recent significant medical therapeutics improvements have been obtained with the use of bisphosphonates. Theses drugs decrease bone remodeling. In Paget's disease, bisphosphonates relieve bone pain, allow lytic areas refilling and decrease complications risk with normal bone formation. In fibrous dysplasia, bone pain is alleviated, osteolytic areas are refilled and cortices are widened. In postmenopausal osteoporosis, bisphosphonates increase bone mineral density, and reduce very significantly the subsequent risk of vertebral, femoral and lower forearm fractures. Bisphosphonates are a breakthrough in Paget's disease of bone, fibrous dysplasia of bone and osteoporosis.     

Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports

Two cases of fibrous dysplasia in the skull base bone appeared hypointense on T1- and T2-weighted magnetic resonance imaging, with accumulation of [11C]methyl-L-methionine ([11C]Met) on positron emission tomography (PET). Fibrous dysplasia is a benign bone disorder which is identified by its distinctive radiography, computed tomography, and bone scintigraphy findings. [11C]Met PET may indicate the presence of viable tumor-like cells in fibrous dysplasia.     

Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Fibrous dysplasia of the cranium is a relatively uncommon disorder that affects primarily the anterior cranial region; its occurrence in the cranial base in combination with aneurysmal bone cyst (ABC) constitutes an extremely rare condition, only two cases of which have been reported previously in the literature. It is important to recognize and treat these cases properly because of the special location in the cranial base and the possibility of neural structure impingement. CLINICAL PRESENTATION: We report the case of a 19-year-old man with a slowly enlarging mass of the occiput, with computed tomographic and magnetic resonance imaging revealing involvement of petrous and basisphenoid bone and growing ABC. INTERVENTION: Open biopsy confirmed the diagnosis of fibrous dysplasia. Partial excision of the lesion and removal of the ABC were performed in a second stage after embolization. CONCLUSION: ABC associated with fibrous dysplasia of the cranial base may enlarge rapidly after puberty and require excision. This is facilitated by preoperative embolization.     

C2/C3 pathologic fractures from polyostotic fibrous dysplasia of the cervical spine treated with percutaneous vertebroplasty

We will discuss a potential role of percutaneous vertebroplasty (PVP) in the management of patients with severe fibrous dysplasia of the spine with multiple cervical lesions and C2–C3 pathologic fractures that may not be a good surgical candidate. Polyostotic fibrous dysplasia involvement of the cervical spine is rare. Review of literature indicates only few reported cases of surgical management with one case of mortality indicating increased risks associated with surgical intervention. While PVP is commonly used for the treatment of osteoporotic thoracolumbar vertebral compression fractures, its role in vertebral stabilization for fibrous dysplasia has not been reported. A 35-year-old man with McCune–Albright syndrome and severe polyostotic fibrous dysplasia of C2 and C3 vertebrae presented with severe neck pain, radiculopathy, quadriparesis and myelopathy. The lesion had pathologic fractures, and there was an os odontoideum with cervical cord atrophy at the C1 level. After discussing need for aggressive surgical management and potential complications, we offered PVP due to surgical risks involved. PVP was performed with a posterolateral transpedicular approach without complication. The patient had remarkable improvement in clinical relief of neck pain and improvement of myelopathic symptoms at 1-year follow-up. We present a case that illustrates a potential use of PVP in the management of a patient with symptomatic spinal fibrous dysplasia with associated pathologic fractures who was poor surgical candidate.

     

Polyostotic fibrous dysplasia and acromegaly

A review of the recent orthopaedic literature reveals little information on the endocrinopathic associations of polyostotic fibrous dysplasia. This article illustrates the need for endocrinologic evaluations at both initial diagnosis and at follow-up examinations. Two patients with polyostotic fibrous dysplasia and acromegaly are presented. In both cases, diagnosis was made by endocrinologic and radiographic evaluations, including computed tomography (CT) scan of the skull. One patient had pituitary macroadenoma, and the other had pituitary microadenoma. Irradiation therapy was performed successfully in both patients without any complications.     

Fibrous dysplasia of the clivus with a second T8 bone lesion: case report

BACKGROUND: We present a rare case of a probably poliostotic fibrous dysplasia of the clivus in a young woman with a clival and vertebral involvement. We also compare our case with data reported in tables that include all cases of clival fibrous dysplasia present in literature. CASE DESCRIPTION: Thirty-year-old women presented with headache. We reviewed radiological and clinical literature before surgery. It was possible to perform a preoperative diagnosis of fibrous dysplasia of the clivus so it was possible to adopt a minimally invasive surgical approach on the clivus. The diagnosis of fibrous dysplasia of the clivus was confirmed after the operation. As the patient was asymptomatic, we did not operate on the vertebral lesion which was stable after 1 year. CONCLUSION: We draw the conclusion that it is possible to obtain a correct preoperative diagnosis of fibrous dysplasia based on magnetic resonance imaging and computed tomographic scans; this makes it possible to avoid major surgical operations. The reported case is particularly interesting because apart from the clival fibrous dysplasia, a vertebral involvement of the same type can be assumed; this association has never been reported in literature.     

Cystic fibrous dysplasia in the long bone

Prominent osteolysis associated with "ground glass" density of fibrous dysplasia may indicate cystic change or sarcomatous transformation. This complication has been reported only sporadically in the long bones. This article presents clinical, radiographic, and pathologic findings, and outcome of simple curettage and bone graft observed in a series of 8 patients with prominent cystic fibrous dysplasia of the long bone. Magnetic resonance imaging features provide a basis for separation of benign cystic change from malignant transformation. However, biopsy is necessary to distinguish nonspecific cystic degeneration from secondary aneurysmal bone cyst. Simple curettage with allo-chip-bone graft is an effective treatment for cystic fibrous dysplasia.     

Chronic idiopathic hyperphosphatasia and fibrous dysplasia in the same child

A generalized skeletal dysplasia with features of chronic idiopathic hyperphosphatasia and fibrous dysplasia of the mandible were observed in a 6-year-old child. The abnormal development of the bones resulted from enhanced remodeling and the failure of mature bone to form. The occurrence of the two lesions in the same child and a review of the literature support the hypothesis that pathogenesis of fibrous dysplasia and idiopathic hyperphosphatasia reflect an underlying common defect in the control of bone cell activity.     

Management of fibrous dysplasia. A report on 36 cases

A consensus on the clinical course of fibrous dysplasia has not yet emerged in the literature. We retrospectively evaluated 36 patients who were diagnosed with fibrous dysplasia in our institution and were followed for a mean duration of 56.5 months (range 7-210 months). Their mean age was 25.8 years (range 5-67 years); 46.7% were male. The most frequent presenting complaints were pain (66% of patients) and pathological fracture (20%). Osteosarcoma developed in one patient 20 years after he had undergone radiation therapy for fibrous dysplasia in the tibia. Mazabraud syndrome was encountered in two patients, and aneurysmal bone cyst associated with fibrous dysplasia was seen in one patient. Fibrous dysplasia is generally considered a static disease, but with long-term follow-up it is found to have a more dynamic nature. For this reason, patients with fibrous dysplasia should be followed carefully over the long-term.     

同种异体骨移植修复骨纤维异样增殖症术后骨缺损

目的观察同种异体皮质骨移植可吸收钉固定修复骨纤维异样增殖症刮除术后皮质骨缺损的疗效。方法自2004年3月至2007年4月我科共收治骨纤维异样增殖症24例,其中股骨11例,肱骨5例,合并桡骨2例,股骨颈及转子间1例,胫骨6例（合并腓骨1例）,单纯腓骨1例。24例中6例出现病理骨折并骨愈合。所有患者入院后行病灶刮除,松质骨打压植骨后运用同种异体皮质骨移植可吸收螺钉固定修复皮质骨缺损。术后1、2、3、6、9、12个月对患者进行门诊随访,此后每年随访一次,观察患者的移植骨愈合、并发症及患肢功能恢复情况。结果术后患者切口均Ⅰ/甲级愈合。术后1年,24例患者植入的骨粒及皮质骨块与正常骨质均基本融合;术后1年3例失访,随访的21例术后2～5年移植骨吸收重建,骨缺损修复,病灶消失,其中14例骨干病灶中有6例髓腔再通。3例残留手术区域隐痛,与天气变化有关。所有患者截止随访末期功能均良好,无复发。1例患者术后3年邻近腓骨出现新病灶,5年后因摔伤导致腓骨病理性骨折,但胫骨无骨折。结论同种异体皮质骨移植可吸收钉固定修复骨纤维异样增殖症刮除术后皮质骨缺损疗效可靠。