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FIBROUS DYSPLASIA: IMAGING CHARACTERISTICS FIBROUS DYSPLASIA: IMAGING CHARACTERISTICS

FIBROUS DYSPLASIA: IMAGING CHARACTERISTICS - PowerPoint Presentation

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FIBROUS DYSPLASIA: IMAGING CHARACTERISTICS - PPT Presentation

H ZAGHOUANI BEN ALAYA ABEN ABDALLAHS YAHYAOUI S MAJDOUBT RZIGUA L BEN CHRIFAH AMARA D BEKIR CH KRAIEM Imaging department Farhat Hached Hospital Sousse ID: 908453

dysplasia fibrous lesion bone fibrous dysplasia bone lesion weighted polyostotic monostotic show signal bones lesions left image form cases

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Slide1

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

Slide2

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

Slide3

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.

Introduction

Slide4

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.

Slide5

CASE N°1

A 19-

year

-

old boy presented to his doctor because of shoulder pain of 1 year’s duration.

(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

a

b

Slide6

Case n° 1

MR coronal images:

*

The

lesions

has

low

signal

intensity

on T1-

weighted

MR image (a ),

high weighted intensity on T2

(b)

*

T1-weighted MR image with fat saturation and after administration of gadolinium

(c )

show that there is mild to moderate heterogeneous enhancement of lesion

.

a

b

c

*The same patient has cervical MRI for cervical pain that shows a lesion of the vertebral hemibody of D1which has low intensity signal on T1 without cortical lysis or soft tissue abnormality on sagittal T1-weighted MR image (d).* T1-weighted MR image with fat saturation and after administration of gadolinium (e ) show that there is an intense enhancement of lesion.

d

Polyostotic FD

e

Slide7

CASE N°2

A 24-

year

-

old man who presents

with

a

meningeal

syndrome. A

cerebro

-

medullar MRI was indicated

*Sagittal T2-weighted MR image

(a ) shows a low signal

lesion

of the

vertebral

body of D1

with

an intense and homogene enhancement of the lesion on T1-weighted MR image

(b ).*CT scan reveals increased

bone density of D1 (c ).

a

bcMonostoticFD of spine

Slide8

CASE N°3

Coronal MR images show a lesion interesting the left

greater

wing

of

sphenoid

. This

lesion

has an intermediaire signal on T1 (a) , a low signal on T2

(b) and present an intense enhancement on contrast-enhanced T1-weighted MR image with fat-saturation (c ).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

Slide9

A

xial

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 (

*

).

Base of the

skull

(sphenoid) FDCase n° 3

Slide10

CASE N°4

*

Axial T1-weighted MR

(a )

and coronal T2-weighted MR

(b)

images reveal

expansile

lesion, with low signal intensity involving

rleft

frontal and parietal bones

*

Axial T1-weighted MR image with fat saturation and after administration of gadolinium (c ) show that there is mild to moderate heterogeneous enhancement of lesion.a

b

c

Cranio

-facial FD

A 35-

year

old

woman

who

consulted for left

frontal and orbital swelling

Slide11

CASEN°5

A 12-

years

old boy who presented a ptosis

of the left upper eyelid

*

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 T1 (c) and T2 (b) .*T1-weighted MR image after administration of gadolinium (d ) show that there is intense and heterogeneous enhancement of the lesion.

Cranio

-facial FD

a

b

c

d

Slide12

DISCUSSION

Fibrous dysplasia (FD) is a congenital

and

noninherited

benign bone diseaseFD

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

Slide13

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 McCune-Albright syndrome (MAS) or Mazabraud syndrome

Discussion

Slide14

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 humerusSolitary involvement of other bones is unusualFD of the spine is rare. It most commonly involves the body and adjacent pedicle without particular predilection for a part of the spinal column

Slide15

The

skull

and facial

bones are the affected sites in 10–25%

All bones of the skull and the face may be affectedIt 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

Slide16

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

Monostotic Fibrous Dysplasia

Slide17

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 asymmetricIt tends

to involve larger segments of bone and is frequently associated with fractures and severe deformities.

Slide18

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

Polyostotic

Fibrous

Dysplasia

Slide19

Syndromes

Associated

with

Fibrous

Dysplasia

1-McCune-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 soft-tissue myxomas

Slide20

Classically, fibrous dysplasia lesions are

intramedullary

,

expansile

, and well defined lesion with thick sclerotic bordersAlthough 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

Imaging findings: radiograh and CT

Slide21

Three types of lesions are distinguished depending on the

degree

of hazy density :

*The ground

-glass pattern

*The

homogeneously

dense pattern:

i

ncreased bone density compared with adjacent normal bone

*The cystic variety: At times, a mubtilocubar, cystic lesion with well-defined margins can be seenOccasionally, calcified cartilaginous and osseous foci may be present within the lesion

Slide22

MRI

findings

The MRI characteristics of FD

are variable

Typically showing

signal

intensity that is intermediate to low on T1-

weighted

images

Intermediate

to

high signal on T2-weighted imagesThese high signal intensities on T2- weighted images correspond to nonmineralized areas and regions of cystic changeFibrous dysplasia reveals varying degrees of enhancement after gadolinium infusion.

Slide23

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 materialMRI

findings

Slide24

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 massesThe most common malignancies include osteosarcoma, fibrosarcoma, and malignant fibrous histiocytoma.

Slide25

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.