2. • Waldron described fibro-osseous lesions as a group of
pathological changes within the jaw bones in which normal
bone is replaced by fibrous tissue, with or without
calcification.
• Fibro-osseous lesions refer to a collection of non-neoplastic
intraosseous lesions that replace normal bone and consist of a
cellular fibrous connective tissue within which non-functional
osseous structures form – Eversole
8. • Replacement of normal bone by a tissue composed of collagen fibres
and fibroblasts that contain varying amounts of mineralized
substances which may be bony or cementum like in appearance.
• All these lesions are grouped under same categories but a more
specific diagnosis is critical because the treatment of these pathosis
varies from none to surgical recontouring to complete removal.
9. FIBROUS DYSPLASIA
• Term coined by Louis Lichenstein in 1938
• 1st described by Von Recklinghausen in 1891
• Also known as- Fibrocystic disease
Osteitis fibrosa localista
Focal osteitis fibrosa
Fibro-osteodystrophy
• It is a hamartomatous lesion which can affect one or multiple bones in which there
is arrest of bone development in the woven bone stage with failure to mature to
lamellar bone.
10.
11. Fibrous dysplasia is normally subdivided into 3 different
forms:-
• Solitary or Monostotic fibrous dysplasia-affecting only one
bone (70-80%)
• Multifocal or Polyostotic fibrous dysplasia affecting multiple
bones(20-30%)
• Craniofacial fibrous dysplasia confined to bones of the
craniofacial complex
12. MONOSTOTIC FIBROUS DYSPLASIA
• 1st and 2nd decade of life
• Both males and females equally affected, mild female predominance
• Maxilla more commonly affected than mandible
• 1st clinical sign- Painless swelling & bulging of the jaw
• Swelling usually involves the labial or buccal plate, seldom the lingual aspect
• Swelling is unilateral slow growing with progressive enlargement
• As lesion grows, facial asymmetry is more evident, and may be patient’s chief
complaint
• Misalignment, tipping or displacement of the teeth due to progressive expansile
nature of the lesion & tenderness may ultimately develop
13. POLYOSTOTIC FIBROUS DYSPLASIA
• Unilateral or bilateral
• Simultaneous involvement of both jaws along with other bones
• “Hockey stick deformity” of femur seen with leg length
discrepency
• More commonly seen in females than males
• May be expansion or deformity of the jaws
• Eruption pattern of the teeth is disturbed due to loss of normal
support to the developing teeth and endocrine disturbances
• Well defined, generally unilateral tan macules on trunk, thighs,
oral mucosa (Café au lait pigmentations)
• Polyostotic fibrous dysplasia+ Café au lait pigmentations =
Jaffe’s type
• Polyostotic fibrous dysplasia+ Café au lait pigmentations +
Endocrine disturbances = Albright’s syndrome
14. CRANIOFACIAL FIBROUS DYSPLASIA
• Confined to bones of the craniofacial complex
• In its craniofacial form the maxilla, zygoma , sphenoid, frontal bones,
nasal bones and base of the skull can be involved.
• Involvement of orbital & periorbital bones Hypertelorism, cranial
asymmetry, facial deformity, visual impairment, exophthalmos, blindness
• Involvement of sphenoid wing & temporal bones Vestibular
dysfunction, tinnitus, hearing loss
• Involvement of cribriform plate Hyposmia or anosmia
15. Diagnosis
• Imaging studies including conventional radiography, CT scan
and MRI
• Nuclear Scintigraphy
• Biopsy
• Molecular or Mutational analysis of GNAS1 gene
• Lab studies to demonstrate endocrine disturbances
16. Lesion can be
When they are active, they are
often symptomatic in that the
patient may perceive throbbing or
discomfort
The lesions appear
hot on a bone scan
In a quiescent
phase they may be
totally asymptomatic.
Teeth can be displaced
by the lesion
17. Radiographic features
• In one type, the lesion is generally a small
unilocular radiolucency or a larger multilocular
radiolucency , both containing a fine bony
trabeculae network
• .In 2nd type ,pattern is similar except that there
is increased trabeculation which renders the
lesion to appear more radio opaque and
mottled in appearance similar to pagets
disease
In 3rd type lesion is quite opaque with many
delicate trabeculae giving a ground glass or
Peaud orange appearance to the lesion
18.
19. • Mandibular involvement result in expansion of buccal & lingual cortical
plate & bulging of lower border of mandible
• Superior displacement of inferior alveolar canal can be seen with narrowing
of periodontal ligament space
• An important distinguishing feature of fibrous dysplasia is the poorly
defined clinical and radiographic margins of the lesion. The process
appears to blend imperceptibly into the surrounding normal bone without
evidence of a circumscribed border.
•
20.
21. On the basis of clinical behaviour and radiographic
findings, Kaban & colleagues classified-
22. Laboratory findings :-
• Serum level of calcium and phosphorus are not usually altered
in FD
• There may be some elevation of serum alkaline phosphatases
depending on extent of bone involvement .
24. Surgical treatment should be limited during an active phase
because the lesions are vascular and can bleed quite profusely.
Treatment is best reserved for quiescent periods, at which time
cosmetic recontouring is the normal treatment of choice.
Regrowth , however, can be expected following this treatment in
25 to 50% of cases, particularly if undertaken at a young age with
carcinomatous change to 3-7%
25. • Medications called bisphosphonates, including pamidronate
(Aredia) and alendronate (Fosamax), are used to inhibit bone
breakdown, preserve bone mass and even increase bone
density.
• Some studies indicate they may help relieve pain in children and
adolescents with severe fibrous dysplasia.
26. – Treatment plan depends on extent of involvement, functional disability, danger to
function, neurologic symptoms and esthetic considerations
– Treatment ranges from observation for minor lesions to radical resection
– Small lesions
• Biopsy for confirmation and follow up
– Lesions with functional or esthetic problems
• Osseous recontouring via transoral approach
• Resection with bony recontruction is often not advisable as complete excision
not possible and uncessary
• Recontouring generally undertaken after active growth stage and during phase of
stabilization
• It entails surgical reduction of lesion to an acceptable contour without any
attempts to remove entire lesion (surgical shave down procedure)
27. Cherubism
• Cherubism is the hereditary form of polyostotic fibrous
dysplasia that causes characteristic deformity in the lower
half of the face due to the degeneration of maxilla and
mandible bone tissue and its replacement with excessive
amounts of fibrous tissue.
• 1st described by Jones in 1933
• Maxilla and mandible
28. • “Cherubism” was coined to describe the rounded facial
appearance resulting from jaw hypertrophy that was
reminiscent of cherubs depicted throughout Renaissance art.
• Also called- Familial fibrous dysplasia of jaws
Disseminated juvenile fibrous dysplasia
29. Clinical features:-
•Bilateral swelling of the jaw
(on both sides in the same area,
although not necessarily to the same degree)
•More common in mandible than maxilla
•Upturned eyes (rim of sclera - the white of the eye - visible beneath the
iris)
•Inverted V-shaped palate arches
•Enlarged submandibular lymph nodes
•Painless
•Premature loss of primary teeth
•Failure of permanent teeth to erupt or random distribution on eruption.
•Rapid development in childhood, slowing during puberty, later
stabilization with possible regression.
•Sometimes, cherubism is associated with Noonan syndrome, which
includes a typical facial appearance with low set ears, sunken chest,
generalized mild low bone density, and subtle (usually) heart malformation
30. Radiological features:-
• Multilocular radiolucencies
• Note absence of solid bone
structure and enlarged jaw
region
Tiziani, V., et al. 1999. Figure 3. Ueki, Y., V. Tiziani, et al. 2001. Figure 1b.
31. Cherubism presents several problems to affected
individuals
• Foremost, the facial appearance of those with cherubism
appears atypical. Affected children can feel different, which
is most negatively experienced through adolescence and
the teens. Observers also may generalize the different
appearance of the face of those with cherubism to
cognitive deficits.
• The loss of teeth and misplacement of teeth can result in
difficulty with eating.
• Cherubic lesions in the floor of the orbit can affect sight.
• Cherubic lesions in the back portion of the mandible can
impede the motion of the jaw
32. Treatment options
• Treatment options depend on the symptoms.
• If lesions are in the orbits an ophthalmologist should also monitor the
optic nerve.
• If the affected individual's appearance causes serious image problems,
surgery can be performed to remove the fibrous tissue. However, the
potential for spontaneous regression of the disease suggests that surgical
interventions should be delayed until after puberty.
• Malplaced teeth that hinder chewing and or cause serious image
problems can be adjusted through orthodontia or extraction.
• Missing teeth can be replaced with prostheses.
• Radiation is NEVER recommended!
• To date, there is no established medical intervention into the growth
phase although several scientific investigations have been conducted.
33. CEMENTO OSSEOUS DYSPLASIA
• It represents spectrum of dysmorphic bone and cementum in which
mesenchymal cells have lost their ability to maintain their structural
morphology and hence produce dysplastic bone.
• Most common fibro-osseous lesions seen clinically in tooth bearing area
Etiology
• Arise in close proximity to periodontal ligament so some believe it to
originate from periodontal ligament
• disorders in the metabolism of cells normally involved in the production of
bone and cementum matrices.
34. Types:-
There are three forms depending on the anatomic sites affected
1. Periapical cemento osseous dysplasia (anterior mandible)
2.Focal cemento osseous dysplasia (focal involvement other
than anterior mandible
3.Florid cemento osseous dysplasia (multifocal )
These forms are distinguished by there sites only but present
similar kind of histological features
35. PERIAPICAL CEMENTO OSSEOUS DYSPLAIA
Clinical features:-
– multiple teeth, mandibular anterior teeth
– involves the periapical bone at the apices of teeth with
vital pulp
• Middle-aged black women(70% )
• No tooth mobility or bony expansion.
• Less than 1.5cm in diameter
• Asymptomatic
36. Radiological features:-
• multiple round to ovoid, radiolucent lesions
• display patchy or mottled areas of radiopacity
• solid, radiopaque lesions with narrow, radiolucent
rims.
• Periodontal ligament is intact
40. FOCAL CEMENTO OSSEOUS DYSPLASIA
It exhibits single site of involvement . Before 1990 these were
misdiagnosed as a variant of ossifying Fibroma
end stage of an abnormal reaction of bone to injury.
Focal lesions that are radio logically and pathologically similar to PCD
but affecting site other than anterior mandible
Mean age 37.8 year in whites
Females are more affected 90%
Mandible more commonly affected
Body or posterior mandible
Generally asymptomatic smaller than 1.5cm.
Rarely produces expansion of bone
Development of idiopathic bone cavities
42. Differential diagnosis:-
Radiopaque mass
• Odontoma
• Osteoblastoma
• Ossifying fibroma
• The clinical and radiological features of it are very
characteristic but not diagnostic . Biopsy by curettage is
necessary .
43. FLORID CEMENTOOSSEOUS DYSPLASIA
• Exuberant variant of periapical cemental dysplasia.
• It is also known by other names like gigantiform cementoma
& familial multiple cementomas in 1992 WHO
• It is a wide spread form of PCD.
• Normal cancellous bone is replaced by dense acellular
cemento osseous tissue in background of fibrous tissue .
• Melrose first used the term florid to describe multiquadrant
radiopacity in the tooth bearing areas
44. Clinical features:-
• black women. Middle age.
• Bilateral fashion/ all four quadrants
• Idiopathic bone cavities (traumatic
bone cysts)
• Mild cortical expansion /
asymptomatic.
• Few which are large are directly
connected with buccal and lingual
cortical plate
• Susceptible to the development of
osteomyelitis.
45. Radiological features:-
• multiquadrant lobular or irregular radiopaque masses in tooth bearing parts of jaw,
continuous with tooth apices
• Cyst like radiolucent areas
• Periphery is well defined
• Large FOD can displace the ID Canal in downward direction & floor of maxillary
sinus in upward direction .
• CT can be helpful to differentiate florid cement-osseous dysplasia from lesions that
exhibit a similar sclerotic appearance on conventional radiographs
46. Differential diagnosis:-
• Pagets disease – deformities of multiple bone,extensive expansion of jaw,
alkaline phosphatase determination needed, hypercementosis, over 40,
teeth migration,diminution of lamina dura
• Gardner syndrome – other radiograph to search for osteomas
• Calcifying odontogenic cyst – calcifications observed at cyst wall, thick
granular yellow fluid
• Osteitis- radiopacity not surrounded by radiolucency
• Cement-ossifying fibroma- more apparent bucco-lingual expansion, single
site, well circumbscribed.
48. OSSIFYING FIBROMA
• Benign neoplasm arising from undifferentiated cells of the
periodontal ligament tissues
• Referred to as osteofibroma, fibro-osteoma, and benign fibro-
osseous lesion of periodontal ligament origin
Etiology
• Chromosomal translocations
• localized dysplastic process in which bone metabolism has been
altered.
49. Clinical findings:-
• slow- growing, expansile lesion that
replaces normal bone as it enlarges
• body of the mandible molar region
/craniofacial bones
• Female predominance 5:1.
• Ages of 20 and 40.
• Pain, paresthesia, bone perforation, and
mucosal ulceration are rare.
• Multiple ossifying fibromas are usually
sporadic, although a familial tendency.
• most important distinguishing
feature of ossifying fibroma is the
well-circumscribed, sharply defined
border between the lesion and adjacent
normal bone
50. Radiographic findings:-
• Well defined unilocular or
multilocular radiolucencies .
• Mixed radiolucent-radiopaque stage
• Fully mature, long-standing lesions
appear as dense, radiopaque masses
surrounded by a thin, well-defined,
regular, radiolucent rim.
• As lesions enlarge, they may displace
adjacent teeth and, less commonly,
cause resorption of tooth roots.
51. Differentiation from fibrous dysplasia
The ossifying fibroma is felt to be a true neoplasm and occurs at a later
age than does fibrous dysplasia, being most common later in the third and
early in the fourth decades.
When it grows beyond the confines of the jaw, it maintains a thin capsule
of new subperiosteal cortical bone, an important feature that distinguishes
it from fibrous dysplasia.
ossifying fibromas are usually received as intact well-defined lesions, but
fibrous dysplasias are almost always fragmented specimens
52. Differential diagnosis:-
• Osteoblastoma – painful, long bone, vertebrae,expansion
• Calcifying epithelial odontogenic tumor- 40 years,does not
produce large dense masses of calcified tissues
• Calcifying odontogenic cyst – thick granular yellow fluid
• Cementoblastoma- obscure the apical half of tooth root
• Florid cemento-osseus dysplasia- multiple quadrant lesions,
osteomyleitis
53. Treatment and prognosis
• Enucleation of tumour mass done
• Surgical resection and bone grafting done for large lesions
• Good prognosis with no evidence of malignant change
54. Juvenile aggressive Ossifying fibroma
• 1st described in 1952 as a variant of ossifying fibroma
• Young children & adolescents
• “ An actively growing lesion mainly affecting individuals below the age of
15 years, which is composed of a cell-rich fibrous tissue containing bands
of cellular osteoid without osteoblastic rimming together with trabeculae
of more typical woven bone.” - WHO
• Well demarcated lesion
• 2 variants: Trabecular
Psammomatous
• More aggressive than the common ossifying fibroma