Done by: Zahra Juma Ahmed Alaswad

Uni num: 20102050079

2014-2015

CHERUBISM
:Synonym
Familial fibrous dysplasia is a synonym for
.cherubism
:Disease mechanism
Cherbisum is rare, inherited autosomal dominate
disease that cause bilateral enlargement of the
jaws, giving the child a cherubic facial
.appearance ,these lesion regress with age
:Clinical feature
Cherubism develops in early childhood between
2 and 6 years of age . the most common
presenting sign is a painless, firm , bilateral
enlargement of the lower face. Profound
swelling of the maxilla may result in stretching of
the skin of the check which cause an "eyes
. raised to heaven" appearance

:Imaging feature
Location: this lesion is bilateral and
effects both jaws. When present in only
one jaw , the mandible is the most
common location . the epicenter is
always in the posterior aspect of the jaw ,
in the ramus of the mandible or the
tuberosity of the maxilla. The lesion
grows in an anterior direction and in
sever case can extend almost to the
.midline
Periphery: the usually is well defined and
.in some instance corticated
Effects of surrounding structure:
expansion of the maxilla and mandible
can result in sever enlargement of the
. jaw
Maxillary lesions enlarge into maxillary
.sinuses
The degree of displacement can be
sever, and the tooth buds are destroyed
. with some lesions

Axial CT of the left mandiblur

condoyle you can see the
sever expansion and the wispy
., ill-defined septa

Panoramic image shows four lesion in the maxilla and

mandible .the epicenter of the lesion are in the maxillary
tuberosity and mandibular ramus ,also note the anterior
.displacement of the unerupted maxillary first molars

:Differential diagnosis
central giant cell granuloma-1
multiple odontogenic-2
keratocysts in basal cell nevus
syndrome
:Management
Treatment can be delayed
because the cytslike lesions
usually become static and fill
in with granular bone during
adolescence and at the end of
skeletal growth. After skeletal
growth has stopped ,
conservative surgical
.procedure maybe done

Portion of the posteroanterior

skull view shows expansion
of the mandible

PAGET'S DISEASE
:Synonym
Osteitis deformans is a synonym for
.paget's disease
:Disease mechanism
Paget's disease is a skeletal disorder
and essentially a disease involving
osteoclasts resulting in abnormal
resorption and apposition of poorquality osseous tissue in one or more
. bones
is seen most frequently in great Britain
and Australia and less often in north
.America
this disease is an autosomal
dominant trait with gentic
heterogeneity and may involve
paramyxoviral infection, but the
etiology for the disease remains
.unclear

axial CT images of a case of pagets

disease involving all of the cranial
bones as well as the maxilla and
mandible .note increase in bone
density and dimension between the
internal and outer cortex of the skull

:Clinical features
Paget's disease is primarily a disease of late
. middle and long age
.Men is approximately twice than women
the resulting of poor quality of bone
formation is bowing of the legs , curvature
of the spine , and enlargement of the skull
and the jaws . Separation and movement of
. the teeth
Patients with this disease may also have illdefined neurologic pain as the result of
bone impingement of foramina and nerve
.canals
elevated levels of serum alkaline phosphate
.and hydroxylproline in urine
Imaging feature
Location: is bilateral ,occurs most often in
the pelvis femur, skull , vertebrae and
. infrequently in the jaws
Note it affects the maxilla about twice than
.mandible

Coronal CT image demonstrates

enlargement of the mandibular
ramus

:Internal structure
Paget's disease has three
:radiographic stages
an early radiolucent resorptive-1
stage
a granular or ground-glass-2
appearance

Edentulous mandible involved

with Paget's disease

denser more radiopaque-3

appositional late stage creating
. cotton wool appearance
But these stages are less apparent
.in the jaws
Occlusal film show the loss of
normal outer cortex and the liner
alignment of trabeculae

: Effects on surrounding structures

prominent pagetoid skull bones may
swell to three of four times their
. normal thickness
In enlarged jaws, the outer cortex
.may be thinned but remains intact
The lamina dura may become less
evident and may be altered into the
.abnormal bone pattern
hypercementosis often develops on
few or most of the teeth in the
involved jaw. This hypercementosis
may be exuberant and irregular ,
which is characteristic of Paget's
. disease

The expansion of the mandible

and the maintence of a the
outer cortical plate

Multiple radiopaque masses in the mandible that have a

cotton-wool appearance

:Differential diagnosis
Paget's disease may appear similar to
.fibrous dysplasia
the enlargement of involved bone)
,elevation of serum alkaline phosphate
..(aid in dif
:Management
Paget's disease usually is managed
medically at the present time, using
calcitonin, sodium etidronate, or
bisphosphonates more recently.
surgery may be required to correct
deformities of the long bone and the
. fractures

Periapical images of Paget's

disease showing exuberant
irregular hypercementosis of the
.root