PRESENTATION

ON
FIBROUS DYSPLASIA
Made by Dr. Waqas Iqbal
MDS in Oral Pathology
 LEARNING OBJECTIVE
AT THE END OF PRESENTATION YOU HAVE TO LEARN ABOUT
• Fibro-osseous lesions of Jaws
• Classification of Fibro-osseous lesions
• Fibrous dysplasia
Causes
Pathology
• Types of Fibrous dysplasia
Monostotic Fibrous dysplasia
 Polystotic Fibrous dysplasia
Assessment of students
 MCQ’s
 AETIOLOGY AND BEHAVIOUR
• Non hereditary caused by mutation in somatic cell.
• There is replacement of bone by benign connective
matrix.
• New mineralization in form of woven bone or
cementum like round acellular basophilic structures.
• Most expand during the period of active skeletal
growth and become inactive in adult life.
• It can be present in any part of facial skeleton.
Mostly arise from maxilla and mandible i.e fibrous
dysplasia cemento-ossifying fibroma and its sub-
types.
 FIBRO-OSSEOUS LESIONS OF JAWS

• A group of lesions in which

normal bone is replaced initially by
fibrous connective tissue.
• It is benign, self limiting but not
encapsulated lesion.
CLASSIFICATION OF FIBRO-OSSEOUS
LESIONS
a) non neoplastic lesions.
1. Fibrous dysplasia
 Monostotic
 Polyastotic
2. Peripheral cemental dysplasia
3. Florid cemento-osseous dysplasia
4. Focal cemento-osseous dyplasia
b) Fibro-osseous neoplasms
 Ossifying/cemento-ossifying fibroma
 FIBROUS DYSPLASIA

• Is an idiopathic
• Benign
• Non-encapsulated
• Severe deformity causes asymmetry
 Causes
• May be due to localized
infection or trauma
• Localized changed in bone
metabolism.
 PATHOLOGY
• Fibrous dysplasia arises from medullary cavity of
bone and expands outwards towards the surrounding
cortical bone.
•The lesion arises by replacing the normal bone by
mesenchymal cells resembling fibroblast.
•These cells deposits the abnormal connective tissue.
•This is followed by deposition of immature woven
bone never undergoes maturation.Hence disorder is
termed as dysplasia.
•There is gradual expansion, distortion and weakness
of bone. These surrounding structure is compressed
by expansile lesions.
Types of Fibrous dysplasia
 Monostotic Fibrous dysplasia

• Clinical features:
 It is commonest accounts 80-85% of fibrous dysplasia.
 Involve single bone, rib or jaws.
 More common in maxilla.
 Second to third decade of life.
 Female
Displacement of teeth
Tenderness.
Intact mucosa over the lesions.
Not well circumscribed.
In children any teeth involved by the lesions may fail to erupt.
Fibrous dysplasia affecting the right
side of mandible
 Histological Features

• Histologically, the lesion is fibrous with

proliferating fibroblast in a compact stroma of
interlacing collagen fibers.
•Irregular trabeculae which are coarse woven
bone are scattered throughout with no definite
pattern. Some may be C-shaped or chinese
character-shaped.
• On maturation the lesional tissue may show
lamellae bone.
 Radiographic Features
• Shape and Borders:
Poorly defined

• Internal structure
Highly variable, mixed lucent and opaque,
Early lesions may be more lucent.
Trabecules are shorter, Thinner and irregularly aligned.

Apperance
Ground Glass
orange Peel
Cotton Wool
Thumb Whorl

• Effect on adjacent structures

Maxillary lesions expand into maxillary sinus
Teeth displacement
Periodontal ligament appear narrowed
Superior displacement of mandibular canal
 expansive radiopacity in the left
maxillary sinus in fibrous dysplasia
 Treatment

• Block – Resection
• Conservative removal
 Differential Diagnosis

• Eosinophilic granuloma
• Aneurysmal bone cyst
• Giant cell granuloma
• Osteoma
• Osteosarcoma
• Osteogenesis imperfecta
 Polystotic Fibrous Dysplasia
• The distribution of lesions in very variable. They
frequently occur in the bones of one limb, Especially
lower but skull, verteberae, ribs are also involved.
• Two types of polyostotic fibrous dysplasia are
described jaffe’s type – involving a variable number of
bones, associated with pigmented lesions of the skin
or “cafe-au-lait” spots. Albright’s syndrome-more
severe lesions involving almost all skeletal bones
associated with pigmented lesions of skin and also
endocrine disturbances of varying types.
Skin pigmentation in Albright’s
syndrome
 Polyostotic form
(i) Jaffe-Lichtenstein syndrome
 Clinical Features
• Thickening of long bones
• Aching recurrent bone pain
• Facial skull bones are involved frequently
leading to asymmetry.
• Clavicles, pelvic bones, scapulae, long bones,
metacarpals and metatarsals are involved.
• Spontaneous fracture.
• Pituitary, thyroid, parathyroid and ovarian
glands are involved.
FIBROUS DYSPLASIA
 ORAL MANIFESTATION

• Expansion and deformity of the

jaws.
• Eruption pattern of the teeth is
disturbed.
 Radiographic Findings
• Medullary portions of the bone present irregular
trabeculations, often a multilocular cystic
appearance.
• Thinned and expanded cortical bone.

Lab Findings
• Increased serum alkaline phosphatase level.
• Premature secretion of pituitary follicle
stimulating hormone.
Late stage of fibrous dysplasia
 Histological Features
• The lesions show replacement of normal bone by fibrous
tissue containing islands and trabeculae of metaplastic bone.
• Richly cellular
• Show whorled pattern
• Thick Collegen bundles
• Immature woven bone
• Spheriodal areas of calcification present
• Margins of the lesions fuses with surrounding normal bone
• As the lesions matures there is remodelling of the woven
bone to lamellar bone
FIBROUS DYSPLASIA
 Treatment and Prognosis

• Mild cases can be surgically treated

• Severe cases with radiation
therapy
• Side effect osteosarcomas
 MCQ’s

1. FIBROUS DYSPLASIA IS A FIBROSSEOUS DISEASE

CHARACTERISZED BY FIBROUS TISSUE REPLEACED WITH
a) METAPLASTIC BONE FORMATION
b) MALIGNANT BONE FORMATION
c) DYSPLASTIC BONE FORMATION
d) DYSTROPHIC BONE FORMATION
e) NORMAL BONE FORMATION
Key A
2. ‘’CAFÉ AU LAIT” SPOT IS A FEATURE OF
TYPE OF FIBROUS DYSPLASIA.
a) POLYSTOTIC FIBROUS DYSPLASIA
b) MONOSTOTIC FIBROUS DYSPLASIA
c) CHERRUBISM
d) PERIAPICAL CEMENTAL DYSPLASIA
e) BOTH A AND B
Key A
3. FIBROUS DYSPLASIA OF MULTIPLE BONES,
PIGMENTED LESION OF THE SKIN AND
ENDOCRINE DISTURBANCE IS SHOWN IN
a) JAFFE’s TYPE
b) ALBERT’s SYNDROME
c) APERT’S SYNDROME
d) ALBRIGHT’S SYNDROME
e) DOWN SYNDROME
Key D
4. GROUND GLASS/ PEAUD’ ORANGE IS A
RADIOLOGICAL FEATURE OF
a) FIBROUS DYSPLASIA
b) CLEIDO CRANIAL DYSPLASIA
c) OSTEITIS DEFORMANS
d) ECTODERMAL DYSPLASIA
e) MARFAN’S SYNDROME
Key A
5. TYPICAL HISTOLOGICAL FEATURE OF
FIBROUS DYSPLASIA
a) JIGSAW- PUZZLE APPEARANCE
b) “C” SHAPED TRABECULAE
c) STARRY SKY APPEARANCE
d) CART AND WHEEL APPEARANCE
e) NON OF THE ABOVE
Key B
THANK YOU