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Copyright © 2011 The Korean Neurosurgical Society
J Korean Neurosurg Soc 50 : 139-142, 2011
Case Report
Cervical Fibrous Dysplasia Presenting as a Pathologic Fracture in an Older Patient Su Heon Lee, M.D., In Ho Han, M.D., Dong Wan Kang, M.D., Byung Kwan Choi, M.D. Department of Neurosurgery & Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea Vertebral involvement of fibrous dysplasia (FD) is rare, especially in the cervical spine. Moreover, cervical FD presenting as a pathologic fracture in older patients is extremely rare. We report a case of symptomatic cervical FD associated with pathologic fracture in a 63-year-old man. The patient presented with progressive weakness of the left arm and pain in the shoulder and arm. Radiologic studies revealed a collapsed and typical ‘ground glass’ radiolucency of C4. Multiple lytic lesions involved the odontoid process of C2 and the body, left pedicle, and posterior elements of C4. Combined anterior and posterior decompression and reconstruction were performed. Post-operatively, the histopathologic examination confirmed FD. On the post-operative follow-up examination, the neurologic deficits had completely resolved. Key Words : Fibrous dysplasia · Cervical spine · Elderly.
INTRODUCTION Fibrous dysplasia (FD), first described in 1983 by Lichtenstein8), is an uncommon benign fibro-osseous abnormality of the bone with an unknown etiology. The disorder involves a single bone (monostotic) or multiple bones (polyostotic)4). Polyostotic FD may be combined with cutaneous and/or endocrine abnormalities (McCune-Albright syndrome)1). The most common sites of the diseases are the long bones, craniofacial bones, ribs, and pelvis, whereas vertebral involvement is rare4). FD involving the cervical spine has rarely been reported2,3,5,9-14). Moreover, cervical FD presenting as a pathologic fracture is extremely rare in older patients12). We report a rare case of symptomatic cervical FD associated with a pathologic fracture in a 63-year-old man.
CASE REPORT History and examination A 63-year-old man presented with progressive weakness of the left arm and pain in the shoulder and arm. The symptoms had worsened over a period of 6 months. One week prior to admission, the weakness in the left hand was aggravated. He visitReceived : October 26, 2010 • Revised : January 25, 2011 Accepted : August 8, 2011 • Address for reprints : In Ho Han, M.D. Department of Neurosurgery, Pusan National University Hospital, 179 Gudeok-ro, Seo-gu, Busan 602-739, Korea Tel : +82-51-240-7257, Fax : +82-51-244-0282 E-mail :
[email protected] • •
ed a local clinic and was referred to our hospital for evaluation of a pathologic fracture in C4. There was no history of trauma. The physical examination showed slight deformities in his left extremities. There were no skin lesions, such as café-au-lait patches. In spite of the pathologic fracture, there was no neck pain or range of motion limitations. The neurologic examination showed tingling in both extremities and motor weakness of the left upper extremity (grade IV). Spurling and Hoffman’s signs were negative bilaterally. The white blood cell count, electrolytes, liver and thyroid function tests, and serum concentrations of parathyroid hormone, 25-hydroxyvitamin D, serum calcium, and phosphate were within normal limits.
Radiology Cervical spine radiographs showed that the C4 vertebral body was partially collapsed. Computed tomography (CT) demonstrated multiple lytic lesions involving the odontoid process of C2 and the body, left pedicle, and posterior elements of C4. These lesions displayed ‘ground glass’ radiolucencies (Fig. 1). On magnetic resonance imaging (MRI), the lesions were hypointense on both T1- and T2-weighted imaging, and were mildly enhanced following intravenous gadolinium. The collapsed vertebral body showed convexity due to an intra-body lesion, resulting in cord compression. The spinous process and lateral mass were expansile, but the cortex was preserved. The expanded lateral mass caused profound neural foraminal stenosis (Fig. 2). A 99 mTc methylene diphosphonate bone scan showed ab-
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normal uptake in the sternum, multiple spines, left scapula, extremities, ribs, and pelvis (Fig. 3). A bone mineral density was normal (Fig. 4). Therefore, osteoporotic compression fracture was excluded. Based on the radiologic findings, the lesions were thought to be polyostotic FD of the cervical spine causing a pathologic fracture.
Operation
A
B
Fig. 1. Pre-operative CT scans. A : The axial image shows lytic, expansile lesions of the C3 body and spinous process, and a left lateral mass. The overlying cortex is thinned and fractured. B : The sagittal image shows a collapsed vertebral body of C4 with minor retropulsion.
A
B
Fig. 2. Pre-operative MR imagings. A : sagittal T2-weighted MR imaging shows a C4 burst fracture, in which a hypointense lesion replaced the cancellous bone of the C4 body with spinal cord compression. B : The expanded lateral mass caused profound neural foraminal stenosis.
An anterior approach to C4 was performed. The involved bone was soft and white. Corpectomy of C4 was performed and the involved bone was easily removed with curettage and rongeurs. Because the entire iliac bone was also involved with FD, a human allograft block was used for reconstruction of the corpectomy site. Additional internal fixation was performed by a plate and screws at C3-5. Then, the patient was turned to the prone position for posterior stabilization. Posterior reinforcement was performed because FD partially involved the C3 and C4 vertebral bodies and posterior elements of C4; an allograft strut was used. A left posterior foraminotomy was performed for foraminal stenosis due to the hypertrophied lateral mass. An additional lateral mass screwing and fusion with allograft were performed at C3-4-5 (Fig. 5). The histopathologic examination demonstrated fibrous tissue within medullary bone and irregularly-shaped bony trabeculae embedded in the fibrous stroma, which was consistent with FD (Fig. 6).
Post-operative course There were no post-operative complications. The patient’s pain, tingling, and weakness improved rapidly. After 7 days, the patient was discharged wearing a Philadelphia collar. Eight months postoperatively, the graft fusion was confirmed without FD invasion.
DISCUSSION FD is a benign intramedullary fibro-osseous lesion, representReference : L1-L4
BMD (g/cm2)
2
1.30
1
1.18
0
1.06
-1
0.94
-2
0.82
-3
0.70
-4
0.58
Fig. 3. Skeletal scintigraphy shows abnormal uptake in the sternum, multiple spines, left scapula, extremities, ribs, and pelvis.
YA T-Score
1.42
20
30
40
50
60 70 Age (years)
80
90
-5 100
Fig. 4. Dual energy X-ray absorptiometry shows normal bone mineral densitometry on lumbar spine.
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Fig. 5. Sagittal reconstruction CT image after 8 months shows the graft fusion without FD invasion. FD : fibrous dysplasia.
ing approximately 2.5% of all bone neoplasms and 7% of benign neoplasms6). FD can present in a single bone (monostotic) or multiple bones (polyostotic). Monostotic FD is more frequent (70%) than polyostotic FD (27%). McCune-Albright syndrome (3%) is a type of FD in which polyostotic lesions are accompanied by café-au-lait spots, precocious sexual development, and other endocrine abnormalities. This syndrome appears to be occur more frequently in females2,4). The most common sites for FD include the ribs, femur, tibia, and humerus. Spinal involvement is unusual and occurs in 7-24% of patients with polyostotic FD, but is rare with monostotic FD2,4,12,13). Cervical involvement with FD is rare and approximately 30 cases of cervical FD have been reported, with 12 cases of monostotic FD2). The disease can affect every level and location of the cervical spine, and occurs more often in males than in females12). Most patients are young adults, ranging in age from 11-56 years12,14). In FD, dysplastic areas rarely expand after puberty because FD usually tends to be exhausted after cessation of bone growth12). Therefore, most pathologic fractures in FD occur in weight-bearing long bone at a young age4). Like our case, cervical FDs presenting as a pathologic fracture are extremely rare in older patients. The possible explanation may be that unlike monostotic FD, polyostotic lesions often continue to enlarge after skeletal maturity, with an increase in pathologic fracture4). The majority of patients with FD are asymptomatic and the diagnosis is incidental. The most common symptom of FD is localized pain4,11). In some patients, FD is first diagnosed at the time of a pathologic fracture9-11). Myelopathy or radicular sensory and/or motor loss as in our case has rarely been reported. Neurologic impairment due to cervical FD has been reported in only 6 cases5). Radiologically, FD typically exhibits ‘ground glass’ radiolu-
Fig. 6. Histopathologic finding shows fibrous tissue within medullary bone with irregularly-shaped bony trabeculae embedded in the fibrous stroma (H&E ×100).
cency, reflecting random woven bone formation4). The adjacent disc height is frequently decreased. A CT scan is the best diagnostic tool to demonstrate the radiographic characteristics of FD. A CT scan confirms decreased cancellous bone and cortical thinning, ballooning, or collapse of vertebrae. MRI is a sensitive tool to demonstrate the extent of tumor involvement, extent of neural compression, and differential diagnosis. FD appears as a homogeneous hypo-intense lesion on T1-weighted imaging unless there is a pathologic fracture. On T2-weighted imaging, the lesion is heterogeneous depending on the amount of fibroosseous tissue, cellularity, cystic alterations, hemorrhage, and cartilaginous tissue. FD is homogeneously enhanced after intravenous gadolinium. The differential diagnosis of FD includes hemangiomas, giant cell tumors, aneurysmal bone cysts, Paget disease, and osteoblastomas. In older patients, multiple myeloma and metastatic carcinoma should also be considered4,9,11). In most cases of FD involving the non-axial skeleton, conservative management is recommended, even following pathologic fractures4). The healing potential for all fracture sites has been reported to be 94%7). In vertebral FD, conservative management has also been recommended, in particular with polyostotic FD. Two cases of odontoid fractures (type II) complicating polyostotic FD, which were successfully managed with braces, have been reported11,13). However, surgical treatment is indicated for persistent pain, neurologic deficits, vertebral collapse, instability, and/or cord compression. In previous reports, different surgical methods have been performed, such as laminectomies, corpectomies, local curettage, and arthrodesis2,9,14). Due to the different expansion properties of FD, there is no gold standard for operative treatment. When FD is restricted to the anterior or posterior element, one approach may be sufficient. However, vertebral FD commonly involves both anterior and posterior
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elements, and a combined anterior and posterior approach may be needed, as in our case. In our case, there was potential instability resulting from vertebral collapse and a pathologic fracture. Neurologic deficits also existed due to the collapsed vertebral body and the hypertrophied lateral mass on the left side. Therefore, an anterior corpectomy with anterior plating and an additional posterior laminoforamintomy with posterior reinforcement were performed. In our case, posterior instrumentation was sufficient; however, we reasoned that anterior instrumentation was not appropriate due to partial involvement of the C3 and C4 vertebral bodies, the use of an allograft with a low fusion rate, and the posterior laminoforaminotomy. Five cases of combined anterior and posterior approaches have been reported in patients with cervical FD10,14). In all cases, a staged combined approach was performed during a 1 week to 10 month interval. In the current case, a single-stage combined approach was performed. There were no complications associated with the surgery. In patients who are poor surgical candidates, percutaneous vertebroplasty may be used as an alternative for symptomatic FD associated with pathologic fractures3).
CONCLUSION We report a case of symptomatic cervical FD associated with a pathologic fracture in a 63-year-old man. Cervical FD presenting as a pathologic fracture is extremely rare in older patients. The current case is the first report of cervical FD presenting as a pathologic fracture in the 7th decade of life. Although most pathologic fractures in FD occur in weight-bearing long bone at a young age, it could also occur in older patients with cervical FD. References
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1. Albright F, Butler AM, Hampton AO, Smith P : Syndrome characterized
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