Paget's bone disease : A series of 40 cases

Transcription

1 Paget's bone disease : A series of 40 cases Poster No.: C-1835 Congress: ECR 2014 Type: Scientific Exhibit Authors: H. Tayari 1, Y. Hentati 2, F. Frikha 1, K. Chermi 1, I. Kobbi 1, E. Keywords: DOI: Daoud 1, Z. Mnif 1, H. Derbel 1 ; 1 Sfax/TN, 2 TN Musculoskeletal bone, CT, Localisation, Pathology /ecr2014/C-1835 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 19

2 Aims and objectives Paget's disease of the bone (PBD), also known as osteitis deformans, is a chronic bone disorder characterised by excessive abnormal bone remodelling. It constitutes the second bone disorder after osteoporosis in terms of frequency. Page 2 of 19

3 Methods and materials Our retrospective study concerns 40 cases of PBD gathered in the department of internal medecine of Hedi Cheker Hospital, Sfax, over a period of 16 years (1996 to 2011). We tried to identify the demographic and clinical aspects, the imaging means and therapeutic modalities. Page 3 of 19

4 Results Results Our serie includes 22 women (55%) and 18 men (45%). Mean age was /- 9.7 years (with extremities between 52 and 86 years). The pain was the most common functional sign, observed in 33 patients (82.5%). It interested the lumbar spine in 18 cases (45%), the thoracic spine in 4 cases (10%), cervical spine in 10 cases (25%), the hip in 19 cases (45%), the shoulder in 15 patients (38%) and the knee in 10 patients (25%). Deformations were noted in 5 cases (12.5%): typical curve of the left leg in 1 case, the right leg in 2 cases and the right arm in 2 cases. An increase in the volume of the skull was noted in one patient. Other rare physical signs were observed: deafness (3 cases), vasomotor symptoms (2 cases), signs of heart failure (1 case) and Table of spinal cord compression (1 case). The radiological abormalities (picture1-6) were: cottony skull (8 cases), thickening of the cranial vault (16 cases), vertebral condensation (11 cases), hemipelvic condensation (13 cases), femur lesions (7 cases), tibia lesions (4 cases) and humerous lesions (3 cases). In our series, we found 11 cases of hip pagetic location. Two patients showed a pathological fracture of the left forearm (1 case) and left leg (1 case). The bone scan was performed in 6 patients. It was a CT of lumbar spine in 4 cases and skull in 2 cases. It demonstrated signs of Paget's disease. Four patients underwent a biopsy which revealed fairly significant Paget modifications, without histological evidence of malignancy. In our series 27 patients (67.5%) have received special anti-osteoclastic treatment of PD. The most used molecular was pamidronate (in 22 patients). Our patients were followed with a mean of months. DISCUSSION: Generalities: Paget's disease of the bone is a common, non-inflammatory, metabolic, skeletal disorder characterized by an increase in osteoclast-mediated bone resorption and compensatory excessive osteoblast activation. Page 4 of 19

5 It can affect up to 4% of individuals over 40 and up to 11% over the age of 80. There may a slight male predilection. A pronounced geographical variation is well documented. The disease is often an incidental finding on a radiological examination requested for an unrelated indication. Polyostotic disease is more common than the monostotic one. The most frequent sites of involvement are spine, pelvis (often asymmetric), skull and proximal long bones. Three phases are detected in PBD: 1. "Hot" or "active" phase (osteolytic dominant, with fibrous replacement of marrow and vascular hypertrophy); 2. "Mixed" phase (disorganized lamellar bone deposition plus osteolysis; rapid bone turnover); 3. "Cold" phase (osteoblastic dominant; pagetoid woven bone replaced by islands of lamellar bone, but haversian systems and remodeling along lines of stress do not occur). Radiography: Radiological signs characteristic of Paget's disease Shape abnormalities Density anomalies Structural abnormalities Bone's hypertrophy Heterogeneous condensation of the bone Thickening of cortical Fibrillar structure of the bone Plain films: Lysis begins typically at an epiphysis and advances slowly at rate of a few mm per year to involve entire bone. Classically, it described as a "candle flame" or "blade of grass" lesion, with lucent osteolytic advancing edge, and sharp transition between normal and involved bone. Pagetoid (woven) bone forms behind advancing osteolysis, with coarsened trabeculae and overall decreased cortical density. Spine: Classic lesion is the "picture frame" appearance of the vertebral body, with initial widening of the peripheral cortex. This progesses to a dense sclerotic body with continued endosteal progression, and eventually even the posterior elements are involved. Skull: It commonly involves the cranial vault. It begins with well demarcated geographic appearance ("osteoporosis circumscripta") in lytic phase (particularly frontal bones), Page 5 of 19

6 followed by focal radiodensities with pagetoid bone formation, and finally a thickened radiodense "cotton wool" appearance in the quiescent phase. Basilar invagination can be seen due to insufficiency of the pagetoid bone; subsequent narrowing of neural foramina and foramen magnum can occur. Pelvis:Paget disease of the pelvis usually manifests with cortical thickening and sclerosis of the iliopectineal and ischiopubic lines, with subsequent trabecular thickening and acetabular protrusion. These findings are often asymmetric and for some reason, may be more commonly seen on the right side. Enlargement of the pubic rami and ischium are also often seen. Long bones:in the long bones, osteolysis begins as a subchondral area. The advancing wedge of osteolysis often demonstrates a characteristic sharp radiolucent margin without sclerosis likened to a blade of grass or flame. In rare cases, the disease is isolated to the diaphysis, most commonly in the tibia, rather than subchondral bone, which can cause diagnostic confusion. Computer Tomography: CT facilitates the diagnosis of pagetic abnormalities in bone like those detected by radiography. Because CT images generally provide superior cortical and trabecular detail in a cross-sectional display, CT conspicuously exhibits the classic findings of Paget disease that include osteolysis, trabecular coarsening, cortical thickening, and osseous expansion. In addition, CT commonly is helpful in the workup of suspected complications including fractures, spinal stenosis, and secondary neoplasms. MRI: (Picture 7-8-9) The MRI signal intensity characteristics in Paget disease are variable, reflecting the natural course of the disease process in different phases. Because Paget disease can be confined to one bone or to a portion of one bone, diagnosis may be challenging. Three major patterns of involvement are recognized. The most common pattern is dominant signal intensity in pagetic bone similar to that of fat; this pattern of involvement presumably corresponds to long-standing disease and is noted in most patients. The second most common pattern probably corresponds to the early mixed active phase when involved bone shows heterogeneous, relatively low T1 signal intensity and high T2 signal intensity. This pattern of signal intensity alteration, also referred to as the "speckled" appearance, probably corresponds to the presence of granulation tissue, Page 6 of 19

7 hypervascularity, and edema seen in active disease when abnormal, disorderly bone mineralization is present. The least common pattern of signal intensity changes is seen in the late blastic inactive phase when pagetic bone shows low signal intensity on both T1- and T2-weighted images, suggesting the presence of compact bone or fibrous tissue. The preservation of fatty marrow signal in pagetic bone generally excludes diagnosis of superimposed sarcoma. In previous studies, investigators have reported increased enhancement in pagetic bone after the IV administration of contrast material, which is indicative of hyperemia in active disease or a complication by secondary disease processes. Page 7 of 19

8 Images for this section: Fig. 1: Picture n 1: Thickened calvarium with "cotton wool" appearance Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 8 of 19

9 Fig. 2: Picture n 2: L5 Pagetic vertebra with fibrillar appearance Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 9 of 19

10 Fig. 3: Picture n 3: Hypertrophy and condensation of the right hemipelvis with a straight Pagetic hip disease Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 10 of 19

11 Fig. 4: Picture n 4: Incurvation of the femur with a fibrillar structure and dedifferentiation cortico-medullary Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 11 of 19

12 Fig. 5: Picture n 5-6: Bilateral Pagetic hip disease with pinching of hip joints. Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 12 of 19

13 Fig. 6: Picture n 5-6: Bilateral Pagetic hip disease with pinching of hip joints. Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 13 of 19

14 Fig. 7: Picture n : Axial T1 and T2- weighted : MR images of head exhibit abnormal heterogeneous, decreased signal intensity in expanded calvaria. Several areas of low signal intensity representing focal osteosclerosis are seen. Axial CT image: shows marked calvarial thickening and sclerosis. Daphne J. Theodorou I, Stavroula J. Theodorou, Yousuke Kakitsubata. Imaging of Paget Disease of Bone and Its musculoskeletal Complications: Review. AJR Page 14 of 19

15 Fig. 8: Picture n : Axial T1 and T2- weighted : MR images of head exhibit abnormal heterogeneous, decreased signal intensity in expanded calvaria. Several areas of low signal intensity representing focal osteosclerosis are seen. Axial CT image: shows marked calvarial thickening and sclerosis. Daphne J. Theodorou I, Stavroula J. Theodorou, Yousuke Kakitsubata. Imaging of Paget Disease of Bone and Its musculoskeletal Complications: Review. AJR Page 15 of 19

16 Fig. 9: Picture n : Axial T1 and T2- weighted : MR images of head exhibit abnormal heterogeneous, decreased signal intensity in expanded calvaria. Several areas of low signal intensity representing focal osteosclerosis are seen. Axial CT image: shows marked calvarial thickening and sclerosis. Daphne J. Theodorou I, Stavroula J. Theodorou, Yousuke Kakitsubata. Imaging of Paget Disease of Bone and Its musculoskeletal Complications: Review. AJR Page 16 of 19

17 Conclusion Paget's disease of bone is a chronic osseous disease characterized by bony enlargement or deformity and bone fragility. It may involve one or more regions of the skeleton and commonly presents as pain. Various imaging appearances are possible and often suggest the phase of cellular activity at the time of imaging. Most cases are diagnosed by conventional radiography. CT and MRI help delineate pagetic bone changes and have prove extremely useful in the diagnosis of sarcomatous transformation, which constitutes the most dreaded complication of the disease. Page 17 of 19

18 Personal information Radiology department, Faculty of medecine of Sfax, Habib Bourguiba Hospital, Sfax - Sfax/TN Page 18 of 19

19 References M.C. Boissier. Décision en rhumatologie. Vigot Edt. Y. Pawlotsky.Rhumathologie. Ed Ellipse, Paris Robert JUVIN. La maladie de Paget. Corpus Médical. Faculté de Médecine de Grenoble. Juillet Daphne J. Theodorou I, Stavroula J. Theodorou, Yousuke Kakitsubata. Imaging of Paget Disease of Bone and Its musculoskeletal Complications: Review. AJR Mirra JM, Brien EW, Tehranzadeh J. Paget's disease of bone: review with emphasis on radiologic features. Skeletal Radiol Cooper C, Dennison E, Schafheutle K, Kellingray S, Guyer P, Barker D. Epidemiology of Paget's disease of bone. Bone Smith R. Paget's disease of bone: past and present. Bone Cundy T. Is Paget's disease of bone disappearing? Skeletal Radiol Murali Sundaram. Imaging of Paget's Disease and Fibrous Dysplasia of Bone. Journal of bone and mineral research Page 19 of 19