FIBROUS DYSPLASIA IMAGING CHARACTERISTICS H ZAGHOUANI BEN ALAYA

FIBROUS DYSPLASIA: IMAGING CHARACTERISTICS H. ZAGHOUANI BEN ALAYA, A. BEN ABDALLAH, S. YAHYAOUI, S. MAJDOUB, T. RZIGUA, L. BEN CHRIFA, H. AMARA, D. BEKIR, CH. KRAIEM Imaging department, Farhat Hached Hospital, Sousse, Tunisia

INTRODUCTION �Fibrous Dysplasia (FD) of bone is a rare non- inheritable congenital disease. �It is characterized by a focal proliferation of fibrous tissue in the bone marrow leading to osteolytic lesions deformities and fractures. �FD can be presented in a monostotic or polyostotic form. �The complications are represented mainly by bone deformities and nerve compression

Introduction �The imaging appearance is often characteristic and allows in combination with the clinic findings the diagnosis �Authors attempted to highlight the interest of imaging in the diagnosis of FD. �The literature is reviewed to delineate radiologic features of monostotic and polyostotic fibrous dysplasia.

PATIENTS AND METHODS �Retrospective study of 7 cases. �FD was monostotic in 4 cases and polystotic in 3 cases �It involves proximal femurs in 1 case, ribs in 2 cases � 4 reported cases were craniofacial form and the involved bones were maxilla (n= 1), sphenoid (n = 1), temporal (n= 1), and frontal bone (n= 2) �Only 2 cases showed involvement of spine.

CASE N° 1 � A 19 -year-old boy presented to his doctor because of shoulder pain of 1 year’s duration. b a (a) Well-defined lytic lesions of the humerus and both bones of the forearm, which at times appear multilocular and expansile with thinnes but unruptured cortical (b) CT showed in the medullary cavity replacement of cancellous bone with a homogeneous hyperdense beach responsible for thinning of the cortical

Case n° 1 MR coronal images: *The lesions has low signal intensity on T 1 -weighted MR image (a ), high weighted intensity on T 2 (b) * T 1 -weighted MR image with fat saturation and after administration of gadolinium (c ) show that there is mild to moderate heterogeneous enhancement of lesion. Polyostotic FD a e d b c *The same patient has cervical MRI for cervical pain that shows a lesion of the vertebral hemibody of D 1 which has low intensity signal on T 1 without cortical lysis or soft tissue abnormality on sagittal T 1 -weighted MR image (d). * T 1 -weighted MR image with fat saturation and after administration of gadolinium (e ) show that there is an intense enhancement of lesion.

CASE N° 2 � A 24 -year-old man who presents with a meningeal syndrome. A cerebro-medullar MRI was indicated Monostotic FD of spine c a b *Sagittal T 2 -weighted MR image (a ) shows a low signal lesion of the vertebral body of D 1 with an intense and homogene enhancement of the lesion on T 1 -weighted MR image (b ). *CT scan reveals increased bone density of D 1 (c ).

CASE N° 3 A 27 -years-old woman , consultsfor diplopia lasting for 6 months. the clinical examination found left convergent squint and paralysis of the V and VI cranial nerves. a b c Coronal MR images show a lesion interesting the left greater wing of sphenoid. This lesion has an intermediaire signal on T 1 (a) , a low signal on T 2 (b) and present an intense enhancement on contrast-enhanced T 1 -weighted MR image with fat-saturation (c ).

Case n° 3 Base of the skull (sphenoid) FD Axial and coronal CT scan show fibrous dysplasia involving body, the left greater wing of sphenoid bone (blue arrows). Note expanded left pterygoid process (arrowhead) and inflammatory changes in left sphenoid sinus (*).

CASE N° 4 A 35 -year old woman who consulted for left frontal and orbital swelling Cranio-facial FD a b c *Axial T 1 -weighted MR (a ) and coronal T 2 -weighted MR (b) images reveal expansile lesion, with low signal intensity involving rleft frontal and parietal bones * Axial T 1 -weighted MR image with fat saturation and after administration of gadolinium (c ) show that there is mild to moderate heterogeneous enhancement of lesion.

CASEN° 5 A 12 -years old boy who presented a ptosis of the left upper eyelid Cranio-facial FD a b c *Axial CT scan (a) show thickening and increased fronto-orbital left bone density. *Coronal MR images show a lesion interesting left roof of orbit. This lesion has a low signal intensity on T 1 (c) and T 2 (b). *T 1 -weighted MR image after administration of gadolinium (d ) show that there is intense and heterogeneous enhancement of the lesion. d

DISCUSSION �Fibrous dysplasia (FD) is a congenital and noninherited benign bone disease �FD has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. �It affects both sexes with a slight female predominance and is diagnosed between 5 and 30 years on average

Discussion �It affects both sexes with a slight female predominance and is diagnosed between 5 and 30 years on average �The lesions grow with the child, stabilize after puberty and appear exceptionally in adulthood �FD can affect one bone (monostotic form) or multiple bones (polyostotic form), and the latter may form part of the Mc. Cune-Albright syndrome (MAS) or Mazabraud syndrome

Monostotic Fibrous Dysplasia �The monostotic form of FD comprises approximately 80% of all cases �It is seen in patients between 10 and 70 years old. �The most common sites of involvement include the rib, femur, tibia, mandible, skull, and humerus �Solitary involvement of other bones is unusual �FD of the spine is rare. It most commonly involves the body and adjacent pedicle without particular predilection for a part of the spinal column

Monostotic Fibrous Dysplasia �The skull and facial bones are the affected sites in 10– 25% �All bones of the skull and the face may be affected �It concerns mainly the ethmoid (72%), sphenoid (43%), the frontal bone (33%), maxilla (24%) and less frequently the temporal, parietal, occipital or mandible bone.

Monostotic Fibrous Dysplasia �Uncomplicated monostotic lesions are generally asymptomatic and usually do not cause significant deformity. �As a rule, monostotic fibrous dysplasia does not convert to the polyostotic form �lesions do not increase in size over time, and the disease becomes inactive at puberty

Polyostotic Fibrous Dysplasia �The polyostotic form of FD may involve many or few bones, most commonly the skull and facial bones, pelvis, spine, and shoulder. �Polyostotic fibrous dysplasia is often unilateral, and may be bilateral, always asymmetric �It tends to involve larger segments of bone and is frequently associated with fractures and severe deformities.

Polyostotic Fibrous Dysplasia �Involvement of the skull may cause cranial nerve dysfunction with visual and hearing impairment �Although the manifestations of polyostotic fibrous dysplasia may be severe, it does not spread or proliferate and generally becomes quiescent at puberty, but existing deformities may progress

Syndromes Associated with Fibrous Dysplasia 1 -Mc. Cune-Albright syndrome is an endocrinopathy occurring mainly in girls, consisting of the triad of precocious puberty, polyostotic FD, and characteristic cutaneous pigmentation referred to as “café au lait” spots 2 -Mazabraud syndrome is the rare combination of fibrous dysplasia and softtissue myxomas

Imaging findings: radiograh and CT �Classically, fibrous dysplasia lesions are intramedullary, expansile, and well defined lesion with thick sclerotic borders �Although endosteal scalloping may be present, a smooth cortical contour is always maintained �Lesions show varying degrees of hazy density with a ground-glass quality, although some may appear almost completely radiolucent or sclerotic

�Three types of lesions are distinguished depending on the degree of hazy density : *The ground-glass pattern *The homogeneously dense pattern: increased bone density compared with adjacent normal bone *The cystic variety: At times, a mubtilocubar, cystic lesion with well-defined margins can be seen § Occasionally, calcified cartilaginous and osseous foci may be present within the lesion

MRI findings �The MRI characteristics of FD are variable �Typically showing signal intensity that is intermediate to low on T 1 - weighted images �Intermediate to high signal on T 2 -weighted images �These high signal intensities on T 2 - weighted images correspond to nonmineralized areas and regions of cystic change �Fibrous dysplasia reveals varying degrees of enhancement after gadolinium infusion.

MRI findings �The fibrous tissues in FD are well vascularized and often show numerous small vessels in the center and large peripheral sinusoids. These histologic features explain why fibrous dysplasia enhances intensely after the injection of contrast material

Complications of Fibrous Dysplasia 1 -Pathologic fracture � These fractures generally heal normally, but additional fractures may subsequently occur at the same site 2 -Malignant degeneration of FD �Complicates less than 1% of all cases �Radiographic findings include cortical destruction and associated soft-tissue masses �The most common malignancies include osteosarcoma, fibrosarcoma, and malignant fibrous histiocytoma.

CONCLUSION �Fibrous dysplasia is a common benign bone disease existing in monostotic and polyostotic forms �Complications, and associations of fibrous dysplasia is important to ensure the accurate diagnosis and appropriate management of this disease �The imaging features of fibrous dysplasia are characteristic, although not specific, and depend on the underlying histopathology of a given lesion.