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CRANIOFACIAL DYOSTOSIS MANDIBULOFACIAL DYOSTOSIS
Also called Cruzon syndrome(mcq) Also called Treacher Collin syndrome
Autosomal dominant craniofacial disorder Autosomal dominant
Characterized by the premature closure of cranial An autosomal dominant disorder characterized by the
bone sutures (craniosynostosis) abnormal development of structures derived from
the first and second branchial arches
MUTATION: fibroblast growth factor(mcq) GENE: long arm of chromosome 5 (5q32-q33).
Hypoplastic Maxilla • Short upper lip • Widely Zygomas are hypoplastic, resulting in depressed
spaced eyes (hypertelorism) • Shallow orbits, cheeks. • Downward-sloping lower eyelids • Notched
protruding eyeballs (ocular proptosis) • Poor lower eyelid (coloboma) • The ears exhibit a spectrum
vision and hearing deficits • Short head of abnormalities, including hypoplastic ear lobes,
(brachycephaly) • Calcified stylohyoid ligaments • malformed pinnae, absent ear canals, and defective
Dental arch width is reduced • Crowding of the middle-ear structures with varying degrees of hearing
maxillary teeth • Unilateral or bilateral posterior loss. • The mandible exhibits condylar and coronoid
crossbite hypoplasia’s as a retruded chin
The skull typically exhibits increased digital Absent
markings (beaten metal pattern) (mcq)
As uhs exam
Ossifying fibroma Cemento osseous Fibrous Dysplasia
dysplasia
Clinical features mandible posterior Periapical ---- Nonexpansile Mono ostotic (MFD)—no
to the canines and Nonsymptomatic gender predilection
only occasionally in Anterior mandible Juvenile MFD
the maxilla and Middle-aged women
Vital teeth
Adult MFD
other locations. It
Focal—posterior mandible Poly ostotic (PFD)--
occurs twice as
Florid ---involves multipe females
often in women and
primarily in the 20- quadrants Buccal /lingual cortical
to 30-year age expansion
group. The lesion is MFD..maxilla>>mandible
usually painless and PFD…craniofacial bones,
grows slowly, femur, tibia, pelvis,
exhibiting marked
buccal and lingual
bony expansion
Radiographic early stages the Periapical & focal--- EARLY LESIONS Radiolucent
features lesions are small completely radiolucent to becoming radiopaque as
and usually densely radiopaque with a more bone
completely thin peripheral radiolucent formation occurs
radiolucent As rim. MATURE LESIONS
they enlarge, Florid-- Mixed lucent and Orange peel (peau
increased amounts opaque lesions d'orange) appearance
of irregularly “Cotton ball” appearance Ground glass appearance
shaped
radiopacities
As uhs exam
RADIOGRAPHIC FEATURES HISTOPATHOLOGICAL FEATURES
EWING SARCOMA bone appears “moth-eaten,” Individual cells may be of two types:
simulating an osteomyelitis with (1) small and round, with darkly
indistinct margins. The staining nuclei and a visibly delineated
periosteum often has a lamellar cytoplasm, and (2) larger, with a finely
layering, referred to as an granular nucleus and a faint ill-defined
“onionskin” reaction. cytoplasm. Both cell types are
undifferentiated cells.
OSTEOSARCOMA The “classic” sunburst or sun ray contain normal or abnormal osteoid or
appearance bone that is closely associated with the
Codman’s triangle, a triangular malignant connective tissue cells to
distinguish them from other forms of
elevation of the periosteum
sarcoma
Symmetrical widening of the
periodontal ligament space
around a tooth or several teeth.
“Spiking” resorption of roots as
a result of the tapered
narrowing of the root
As UHS exam
RADIOGRAPIC FEATURES HISTOPATHALOGICAL FEATURES
Acute suppurative Little radiographic changes unless The bone shows a loss of the osteocytes from their
OM the disease has developed for 1-2 lacunae,
week, an ill-defined radiolucency, peripheral resorption, and bacterial colonization
Individual trabeculae become The periphery of the bone and the haversian
fuzzy and indistinct------MOTH canals contain necrotic debris and an acute
EATEN APPEARENCE inflammatory infiltrate consisting of
polymorphonuclear leukocytes.
Chronic Suppurative A patchy, ragged, Soft tissue component consists of chronically or
OM and ill-defined subacutely inflamed fibrous connective tissue
radiolucency that filling the intertrabecular areas of the bone
often contains Scattered sequestra and pockets of abscess
central formation are seen
radiopaque
sequestra
Focal Sclerosing OM Localized, usually uniform zone of Dense bony trabeculae with little interstitial
increased radiodensity-- Adjacent marrow, Empty lacunae
to the apex of a tooth ---Exhibits a Multiple reversal lines giving pagetoid appearance
thickened periodontal ligament Connective tissue between trabeculae is fibrotic and
space infilterated by small number of lymphocytes
Diffuse Sclerosing Diffuse, patchy sclerosis of bone Dense, irregular bony trabeculae, some of which are
OM (cotton wool appearance)---May be bordered by an active layer of osteoblasts
extensive or bilateral Focal areas of osteoclastic activity
May show mosaic pattern
The soft tissue is fibrous with small capillaries &
focal collections of lymphocytes and plasma cells If
the adjacent inflammatory process extends into the
sclerotic bone, then necrosis often occurs. The
necrotic bone separates from the adjacent vital
tissue and becomes surrounded by subacutely
inflamed granulation tissue. Secondary bacterial
colonization often is visible.
Chronic OM with multiple thin layers of new bone, Parallel rows of highly cellular and reactive woven
Proliferative referred to as an “onion skin” bone in which the individual trabeculae are
periostitis appearance. The trabecular bone frequently oriented perpendicular to the surface.
will also exhibit the characteristic The trabeculae sometimes form an interconnecting
diffuse mottling of a chronic meshwork of bone or are scattered more widely,
osteomyelitis resembling the pattern seen in immature fibrous
dysplasia
Between the cellular trabeculae, relatively
uninflamed fibrous connective tissue is evident.
AMELOGENESIS DENTINOGENESIS DENTINE DYSPLASIA
IMPERFECTA IMPERFECTA
CLINICAL HYPOPLASTIC TYPE I (ass with Type I (radicular DD): All teeth in
FEATURES Generalized or localized Osteogenesis both dentitions are affected. The color
• Pitted - • imperfecta), Type II of the teeth is usually within the
Smooth - • Rough - (Hereditary oplascent normal range
teeth). Type III Type II (Coronal DD): Both the
• The enamel is
(Brandywine type) primary and permanent dentitions are
thinner than normal in affected.
focal or Clinical features: In all
three subtypes of DI, Clinically the deciduous teeth exhibit a
generalized areas bluish-gray, brownish, or yellowish
HYPOMINERALIZED teeth of both dentitions color and have the same translucent,
Enamel matrix is laid are affected with opalescent appearance that is seen in
down appropriately but variable clinical DI.
no significant appearances. The teeth the permanent teeth have a normal
mineralization occurs. are opalescent with the clinical appearance.
The enamel is of normal color ranging from
thickness but softer than bluish-gray to brown
normal and can be to yellowish. The
easily removed with a dentin is abnormally
blunt instrument soft, providing
Opaque white to inadequate structural
yellow-brown, support to the
• soft rough overlying enamel.
enamel surface, ---- Although the enamel is
Wear off easily normal, it fractures or
• dental chips away easily,
sensitivity and open bite exposing the occlusal
common and incisal dentin The
HYPOMATURATION exposed soft dentin
TYPE often undergoes rapid
The enamel is of normal and severe functional
thickness but not of attrition.
normal hardness and
translucency enamel can
be pierced with the
point of a dental
explorer with firm
pressure and can be
chipped away from the
underlying normal
dentin; mildest form of
the hypomaturation type
---enamel of normal
hardness and has white
opaque flecks in the
incisal areas of the teeth
(snow-capped teeth)
RADIOGRAPHIC In the smooth The teeth in DI DD 1: roots of the teeth are usually
FEATURES hypoplastic type, the subtypes I and II are short, blunt, bulged, conic, or absent.
enamel layer is similar and exhibit characteristic W-shaped roots. -
conspicuously thin and bulb-shaped crowns deciduous dentition, -----total
its radiodensity is greater with constricted obliteration of the pulpal chambers and
than the adjacent dentin; cementoenamel canals. permanent teeth ------pulpal
in the hypocalcified type, junctions and thin obliteration; OR , thin crescent shaped
the enamel layer appears roots.Depending on the or chevron-shaped remnants of the pulp
wispy or absent and is age of the patient, the chambers are often present. Periapical
usually less radiodense teeth will exhibit radiolucencies --abscesses,
than the adjacent dentin; varying stages of granulomas, or cysts may be present in
in the hypomaturation obliteration of the pulp the absence of dental caries
type, the radiodensity of chambers and pulp DD2: deciduous teeth of DD type II
the enamel is almost canals The cementum, exhibit obliterated pulpal chambers
equal to that of normal periodontal ligament, and canals that are similar to those
dentin. and supporting
alveolar bone appear seen in DD type I and in DI. pulpal
normal. The teeth in obliteration roots of the deciduous
DI type III may be and permanent dentitions are of
similar to those seen in normal shape and length.
types I and II, or they pulp chambers in the permanent teeth
may exhibit extremely are -----a thistle or flame shape to the
large pulpal chambers root portion of the pulp.
surrounded by a thin Pulpal calcifications (pulp stones) -
shell of dentin. coronal pulp chambers and the root
pulp canals are narrowed
A radiograph of the lower incisors revealed a single radiolucent lesion at the apex of a tooth.
A. What is differential diagnosis? Periapical cyst…. Periapical cyst, granuloma, periapical cemento osseous
dysplaisa
DEFINITION: A mathematical model used to determine the impact of ingested foods on the pH of dental plaque
and subsequent caries formation. Decalcification of teeth occurs when the pH in the oral cavity is less than 5.5.
SIGNIGICANCE:The significance of a Stephan curve shows that the plaque pH curve will drop a lot and quickly
after the consumption of fermentable carbohydrates. And then it will drop below what we call a critical pH, in which
case the demineralization of the tooth enamel minerals will occur.
FACTORS AFFECTING CURVE:
• The time taken for these changes to occur varies between individuals and also varies according to the nature
of the challenge.
• The initial sharp drop depends upon the speed with which plaque microbes are able to metabolise sugar.
Large molecules, like starch for example, diffuse into the plaque more slowly and take longer to be broken
down resulting in a less steep drop in pH.
As uhs exam
Den’s Invaginatus Den’s Evaginatus
Definition: Deep enamel-lined pit that extends for Definition: characterized by a cusp like supernumerary focal
varying depths into the underlying dentin enamel protrusion on the occlusal or lingual surface of the crown
Pathogenesis: Invagination is an end result of a speedy Pathogenesis: caused by abnormal proliferation of the inner
and aggressive proliferation of a part of inner enamel enamel epithelium into the Stellate reticulum
epithelium invading the dental papilla
Synonym: Also called as “Tooth in tooth” Synonym: “TUBERCULATE CUSP” or “ACCESSORY
TUBERCLE” OR “LEONG’S PREMOLAR
Site: Maxillary lateral incisors Site: Primarily affects premolar teeth
Clinical features
granulomatous hyperplastic gingivitis that Observed in adults. initial signs and
appears to originate in the interdental symptoms -----localized to the nasal region
papilla. Such lesions are deep red, nodular, and include nasal stuffiness or epistaxis.
and friable and have been referred to as Pain may accompany the nasal symptoms.
“strawberry gingivitis,” ulcers and Swelling of the soft palate or posterior hard
perforations of the palate and swelling palate
and desquamation of the lips. may precede the formation of a deep,
necrotic ulceration, which usually occupies a
midline position. -----ulceration enlarges and
destroys the palatal tissues,
which typically creates an oronasal fistula
Histopathology
the tissues feature a prominent vasculitis a mixed infiltrate of a variety of inflammatory
with zones of necrosis and fibrinoid deposits cells, often arranged around blood vessels
in a general background of granulomas. (angiocentric)
Eosinophils and multinucleated giant cells are Necrosis is often present in some areas of the
commonly encountered. lesion, secondary to infiltration of the blood
vessels by the tumor cells.
Large, angular, lymphocytic cells with an
atypical appearance are usually identified as a
component of the cellular infiltrate
Investigation
CT chest /Xray chest Biopsy
Tissue biopsy Immunohistochemistry…T cell markers
Lab test: C ANCA (high levels of positive
antineutrophil cytoplasmic antibody ) Urine
analysis…hematuria or proteinuria
As uhs exam