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.

     

Fibrous dysplasia of the clivus

OBJECTIVE: Fibrous dysplasia is a developmental skeletal disorder that may lead to distortion, expansion, and weakening of the bone. Craniofacial involvement by this entity is well recognized and is known to cause neurovascular impingement and cosmetic deformity; fibrous dysplasia of the clivus, however, is unrecognized and seldom reported. Differentiating this entity from more aggressive disease processes affecting the clivus is central for the proper management of lesions in this area. We have studied fibrous dysplasia of the clivus with the goal of depicting its manifestations, outlining its management, and heightening awareness of this disease entity. METHODS: We retrospectively reviewed our database and identified patients with the diagnosis of fibrous dysplasia of the clivus. The demographic data, the clinical and radiological findings, and the management of these patients were reviewed. RESULTS: Eight patients who had experienced fibrous dysplasia of the clivus were identified. They either were asymptomatic (four patients) or presented with headache (four patients). Of the patients who presented with headache, one also had XIIth cranial nerve paralysis and another had dysphagia. The radiological findings for this entity were consistent, with typical findings of hypointensity on T1- and T2-weighted magnetic resonance imaging studies and ground-glass appearance on computed tomographic scans. Four of the patients in our series had pathological confirmation. Treatment was determined by the patient's symptoms. CONCLUSION: Fibrous dysplasia should be considered in the differential diagnosis of lesions affecting the clivus. Its clinical and radiological presentations permit the establishment of the diagnosis. Its management is usually conservative, unless the patient presents with nerve compression or extensive symptomatic involvement of the condyle.     

Diagnosis and treatment of an odontoid fracture in a patient with polyostotic fibrous dysplasia: case report

Fibrous dysplasia of the cervical spine is rare. No prior reports have discussed odontoid fractures in the setting of fibrous dysplasia. We describe a 26-year-old man who suffered a traumatic odontoid fracture in an area of preexisting fibrous dysplasia. The patient was treated conservatively in a sterno-occipito-mandibular immobilizer brace with a good result. A review of fibrous dysplasia is presented. Alternative methods of diagnosis and treatment options in our patient are discussed.     

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.     

Die atlantoaxiale Instabilität als seltene Komplikation der fibrösen Dysplasie

Fibrous dysplasia, in either monostotic or poliostotic form rarely involves the cervical spine. Most of these lesions remain asymptomatic and are incidental radiographic findings. A symptomatic case of polyostotic fibrous dysplasia involving the first, second and third cervical vertebra with potentially serious consequences is presented. A 53-year old woman who had shown cervical myelopathy for about 3 month was diagnosed as having cervical dysplasia on the basis of plain x-ray and CT scanning. In addition, atlanto-axial instability causing clinical symptoms was detected. A dorsal stabilisation was performed using the DCS System.     

An unusual presentation of Paget disease: pathological fracture of the odontoid

Paget disease of the cervical spine is a common disease in an uncommon location. The authors present an unusual case of a 58-year-old woman with a pathological fracture through the base of the odontoid vertebra secondary to Paget disease. The purpose of this case report is to describe the radiological features of Paget disease and their important application to treatment management.     

Odontoid metastasis: a potential lethal complication

Nearly one third of cervical spine metastasis has a primary breast malignancy. Patients with cervical metastasis have higher mortality due to advanced stage of the malignancy. Treatment is palliative to relieve pain, prevent pathological fracture, improve mobility and function, and prolong survival. We describe a 40-year-old woman with a history of breast cancer who presented with neck and shoulder pain of 1 week duration with no neurological deficit. Following clinical examination, radiographs taken of the cervical spine was normal. Radiographs repeated 3 weeks later revealed a large lytic lesion of the odontoid occupying 70–80% of the peg. Further investigation including magnetic resonance imaging and bone scan showed no further spinal lesions. She underwent cyclical radiotherapy with complete resolution of the odontoid peg lesion and clinically was asymptomatic at 2 years. Metastatic lesions of the odontoid are atypical, and this case reinforces the necessity of early detection to evade disastrous consequences.     

Polyostotic fibrous dysplasia involving the thoracic spine with myelopathy: case report and review of the literature

Background contextPolyostotic fibrous dysplasia (PFD) seldom involves the thoracic spine and usually presents with back pain.PurposeTo describe an extremely rare presentation of an uncommon disease.Study design/settingWe present a case report from a university hospital.MethodsWe report a case of symptomatic thoracic PFD associated with myelopathy and pathologic fracture. A thorough search of PubMed/MEDLINE was performed for the terms “polyostotic fibrous dysplasia,” “spine,” and “neurological deficit.”ResultsThe patient was treated by posterior laminectomy, vertebroplasty, and pedicle screw fixation and fusion. Satisfactory results were achieved, and there were no complications.ConclusionsIn the spine, PFD may lead to pathologic fracture and myelopathy even after adolescence. Vertebroplasty with or without decompression and fixation may be the appropriate option for cases with myelopathy.     

Monostotic fibrous dysplasia of the spine: report of a case involving a cervical vertebra

Monostotic fibrous dysplasia of the spine is a rare entity. Only 26 cases, of which 11 were located in the cervical spine, are to be found in the literature. We report a 56-year-old male patient with cervicobrachialgia of half year’s duration. Radiographs showed a diffuse destruction of the vertebral body and the spinous process of C₄. A biopsy of the spinous process confirmed histopathologically a fibrous dysplasia. Due to minor symptoms, no surgical treatment was performed or is planned unless in case of increasing pain, an acute instability or neurological symptoms.     

Surgery of massive fibrous dysplasia and osteoma of the midface

Since most bony lesions of the midface will stop growing or regress, it is generally not appreciated when fibrous dysplasia and osteoma occasionally continue to grow, causing functional and cosmetic problems. To demonstrate the potential of the disease processes and management options available to the surgeon, a series of patients with massive fibrous dysplasia and osteoma of the midface is reviewed. Most of the patients show a facial deformity alone or in combination with telecanthus, epiphora, exophthalmos, or nasal obstruction. Several types of radical and conservative surgical procedures are described to remove or control the bony lesions, to restore facial contour, and to reconstruct the sinuses, nasal passageways, lacrimal collecting system, and medial wall of the orbit.     

Aggressive aneurysmal bone cyst in association with polyostotic fibrous dysplasia: A case report

IntroductionAneurysmal bone cyst occurring in the setting of previously diagnosed fibrous dysplasia is rare. While both are benign processes, pain, compression of nearby structures and risk of fracture can require treatment.Presentation of caseIn this report, we describe a 56 year old male who developed an aggressive aneurysmal bone cyst secondary to fibrous dysplasia in the proximal tibia over a period of 8 months. He required an above knee amputation for disease and symptom control due to the aggressive nature of disease and medical comorbidities.DiscussionThe diagnosis of a secondary lesion can prove difficult. It is important to exclude a malignant disease process, particularly when imaging demonstrates an aggressive appearance. In this case, repeat imaging, CT guided biopsies and an open biopsy were performed to exclude malignancy prior to definitive surgical management.ConclusionIn order to exclude secondary lesions, we suggest further investigation for new onset pain in the setting of a benign lesion.     

Fibrous dysplasia of the spine with sarcomatous transformation: a case report and review of the literature

Summary A fibrosarcoma is reported in the spine of a 53-year-old man with polyostotic fibrous dysplasia. There was no history of endocrine disturbances and no previous irradiation. Malignant transformation in fibrous dysplasia is rare. A review of the literature reveals 101 cases of malignant degeneration occurring in fibrous dysplasia. We believe that this is the first report of sarcomatous change arising in an area of fibrous dysplasia in the spine.     

Ventral approaches to the upper cervical spine

The transoral approach to the upper cervical spine and the respective neuraxis has been recognized for almost 100 years, but it is still not in common use. Based on over 200 operations on patients with a variety of lesions and an age range from 2 1/2 to 83 years, we outline the pathological mechanism, surgical anatomy, indications for surgery, preoperative investigations, variations and extensions of the transoral approach to the upper cervical spine, neuraxis and skull base. The most common post-traumatic lesions and the most frequent tumors are discussed and the recommended surgical approach is described. Surgical techniques, possible complications and their management, as well as postoperative care, are listed.     

Halo instrumentation for the correction and immobilization of the cervical spine

An instrument set for application in patients requiring a correction treatment and/or a temporary fixation of the cervical spine is described. The halo is combined with a cast or a brace employing the 8 mm Hoffmann External Fixator system supplemented by three additional, newly designed parts. Applications of the instrument set in the treatment of traumatic fracture, fibrous dysplasia and ankylosing spondylitis are described.     

Monostotic fibrous dysplasia of a cervical vertebra

Summary Fibrous dysplasia has been frequently reported to involve the spine in the polyostotic form, but only rarely in the monostotic form of the disease. The present case demonstrates monostotic fibrous dysplasia of the fourth cervical vertebra. We present the plain radiographic, CT-scan, scintigraphy and histologic examinations. C3–C4 posterior and anterior arthrodesis was performed. The post-operative follow-up was satisfactory.     

Rheumatoid arthritis: evaluation and surgical management of the cervical spine

BACKGROUND CONTEXT: Rheumatoid arthritis is a debilitating polyarthropathic degenerative condition. Eighty-six percent of patients with rheumatoid arthritis have cervical spine involvement. Often these lesions are clinically asymptomatic or symptoms are erroneously attributed to peripheral manifestation of the patient's rheumatoid disease. Because these lesions are common and missed diagnosis can result in death, early recognition is vital. PURPOSE: The purpose of this literature review is to identify common lesions present in the rheumatoid neck and review diagnostic methods as well as treatment options for those requiring surgical intervention. STUDY DESIGN: A review of the English medical literature with focus on more recent studies on the presentation, diagnosis, management, surgical treatment and clinical outcomes of rheumatoid arthritis of the cervical spine. METHODS: A comprehensive literature review of the English medical literature obtained through Medline up to November 2003 was performed identifying relevant and more recent articles that addressed the presentation, evaluation, surgical management and outcomes of rheumatoid patients with cervical spine involvement. RESULTS: If left untreated, a large percentage of rheumatoid patients with cervical spine involvement progress toward complex instability patterns resulting in significant morbidity and mortality. Once myelopathy occurs, prognosis for neurologic recovery and long-term survival is poor. In properly selected patients, anterior and/or posterior cervical procedures can prevent neurologic injuries and preserve remaining function. CONCLUSION: Cervical spine involvement in the rheumatoid patient is common and progressive. Early diagnosis and treatment is imperative; however, surgical intervention should be considered carefully because associated morbidity and mortality is high.     

Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report

IntroductionFibrous dysplasia is tumor like lesions of bone which develop as substitution of bone by an expansion of fibrous connective tissue mixed with hard trabeculae. Chondrosarcomas is one of common malignant primary bone tumor derived from heterogenous group of neoplasm producing chondroid matrix. Chondrosarcoma arising in fibrous dysplasia, especially in monostotic fibrous dysplasia is a very rare case.Case reportA 54-year-old male presented with chief complaint of pain on left thigh. Patient with history of pathological fracture on left femoral diaphysis 3 years ago due to fibrous dysplasia and had underwent curettage, open reduction, and internal fixation at other hospital. Plain radiography revealed expansive lytic lesion, interrupted periosteal reaction with plate and screw attached to the lesion, and soft tissue mass. MRI T2FS sequence showed hyperintense mass extending from subtrochanteric to distal of left femoral diaphysis. Histopathological result from biopsy suggested chondrosarcoma.ConclusionsMalignant transformation of monostotic type was less frequently compared to polyostotic type. Among all malignant transformation cases, alteration to chondrosarcoma was more scarce than other malignancy such as osteosarcoma and fibrosarcoma. Wide surgical margin and reconstruction in chondrosarcoma provide good local control and functional outcome.     

Symptomatic polyostotic fibrous dysplasia of the thoracic spine

We present a case of polyostotic fibrous dysplasia with limited involvement in thoracic spine and adjacent ribs. The patient underwent posterior instrumentation performed between Th3 and Th11 with pedicle screw system, followed by costotransversectomy of 7th and 8th costovertebral junctions and posterior spinal fusion for costal lesions. In the same operation, curettage was done for the lesion in Th6 vertebra and bone grafting and anterior total corpectomy were performed for Th7 and Th8 vertebrae. 360 degrees spinal fusion was done using titanium mesh as strut graft and autogenous rib grafts. Fibrous dysplasia occurs rarely in axial bones than peripheral bones. The cystic lesions in segments of the whole spine should be evaluated for the possibility of fibrous dysplasia with detailed radiographical examination and biopsy.     

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.