developmental disturbances Flashcards
(23 cards)
ghost teeth
Regional odontodysplasia
localised non hereditary developmental abnormality of structure of teeth
small irregular crowns yellow to brown with rough surface
thin enamel and dentin
enlarged pulp with open apices
4 features of gardners syndrome
(1) multiple polyposis of the large intestine;
(2)osteomas of the bones, including long bones, skull, and jaws;
(3)multiple epidermoid or sebaceous cysts of the skin, particularly on
the scalp and back;
(4) occasional occurrence of desmoid tumors; and
(5) the impacted supernumerary and permanent teeth.
autosomal
dominant pattern of inheritance,
congenital syphilis
Congenital syphilis is transmitted to the offspring only by an infected
mother and is not inherited
Persons with congenital
syphilis manifest a great variety of lesions, including frontal bossae
(Olympian brow frontal bossing of forehead), short maxilla, high
palatal arch, saddle nose (degradation of nasal cartilage), mulberry
molars (extra cusps on first molars)
Hutchinson triad: hypoplasia of the incisor (screw
driver–shaped permanent upper central incisors) and molar teeth
(mulbery molars), eighth nerve deafness, and interstitial keratitis.
dentinogenesis imperfecta
. Dentinogenesis imperfecta is an inherited disorder
of dentin formation which affects deciduous and
permanent dentition.
Classification
♦ Sheild’s Classification
* Dentinogenesis imperfecta Type I: Dentinogenesis
imperfecta without osteogenesis imperfecta.
* Dentinogenesis imperfecta Type II: Dentinogenesis
imperfecta with osteogenesis imperfect
* Dentinogenesis imperfecta Type III: It is a racial isolate
in Maryland and is known as Brandywine type.
♦ Extensive studies have shown that dentinogenesis imperfecta is clearly a disorder from osteogenesis imperfect, so
the following revised classification is given:
* Dentinogenesis imperfecta1: Dentinogenesis
imperfecta without osteogenesis imperfecta (opalescent
dentine). This corresponds to dentinogenesis imperfect
Type II of Sheild’s classification.
* Dentinogenesis imperfecta 2: Brandywine type
dentinogenesis imperfecta: This corresponds
to dentinogenesis imperfect Type II of Sheild’s
classification.
There is no substitute in present classification for the category
which is designated as Dentinogenesis imperfect Type I of
Sheild’s classification.
etiology
♦ Gene affected is present on chromosome 4 and it codes for
DSPP (Dentine sialoprotein and phosphoprotein)
Clinical Features
♦ On eruption the teeth exhibit a normal contour and have
opalescent amber like appearance.
♦ Few days after the eruption the teeth may achieve the
normal color. Finally the teeth become gray or brownish
in color with bluish reflection from enamel.
♦ In some cases the affected teeth may exhibit hypomineralized areas on surface of enamel.
♦ Teeth are not particularly sensitive even when most of the
surface enamel is lost.
♦ The dentin is soft and easily penetrable in dentinogenesis
imperfecta, these teeth are not caries prone.
histopathology
♦ Histopathologically the enamel appears normal in
Dentinogenesis imperfecta. Mantle dentin is also nearly
normal.
♦ Dentinal tubules are less in number per square unit area
of dentin as compared to normal dentin. The tubules are
often distorted, irregular in shape, widely spaced and
larger in size.
♦ Pulp chamber and root canal are often obliterated by the
abnormal dentin deposition.
♦ DEJ appears smooth or flattened instead of being scalloped.
♦ Large area of a tubular dentin is present.
numeration of Developmental nomalies of tongue
Aglossia
♦ Microglossia
♦ Macroglossia
♦ Ankyloglossia or tongue tie
♦ Cleft tongue
♦ Fissured tongue
♦ Median rhomboid glossitis
♦ Geographic tongue
♦ Hairy tongue
♦ Lingual thyroid nodule
♦ Lingual varices
amelogenesis imperfecta
Amelogenesis imperfecta is also known as hereditary
enamel dysplasia or hereditary brown enamel.
Amelogenesis imperfecta is a heterogeneous group
of hereditary disorders of enamel formation affecting
both deciduous or permanent teeth
Clinical Features
♦ Color of teeth is mostly chalky white but sometimes it can
be yellow or even dark brown.
♦ Contact points in proximal surfaces are mostly open
while the occlusal surfaces and incisal edges are severely
abraded.
♦ Enamel may have a cheesy consistency which is easily
removable from tooth surface.
♦ Amelogenesis imperfecta does not increase the susceptibility of teeth to dental caries.
♦ In amelogenesis imperfecta the enamel is of near normal
hardness and has some white opaque flecks at incisal areas of
teeth. These types of teeth are known as snow capped teeth
shell teeth
Dentinogeneisis imperfecta type 3 = brandywime isolate
severe form of DGI
extremely thin dentin
presence of unusual pulp enlargement with multiple pulp exposures
seen in deciduous teeth
thistle tube shaped or flame shaped pulp chambers seen in
dentin dysplasia type 2 or mild form of DGI or coronal dentin dysplasia
enlarged pulp chambers and apical extension
DD = pulpal dysplasia (also has thistle tube )
rootless teeth
DD 1
loss of organisation of root dentin
shortening of roots
prone to fractures
HP shows central portion has whorled dentin appearance is called as “stream flowing arund boulders”
hypoplastic AI
inadequate deposition of enamel matrix
generalised pattern has pinpoint pits brown scattered across
localised one has pits in a linear manner like middle third
cheesy enamel is associated with
hypocalcification type of AI
snow capped apearance of teeth seen in
hypomaturation AI
Appear like denture dipped in white paint
surface enamel is mottled and agar brown, enamel fracrures
pigmented hypocalified AI
DD is fluorosis
amelogenesis imperfecta with taurodontism is seen in whcih syndrome
tricho dento osseous syndrome
trichodento osseous syndrome
kinky steel wool texture of hair and later straight.
taurodontism
AI
osteosclerosis
brittle nails
enamel renal syndrome
generalised delayed eruption and impaction of teeth with hypoplastic AI
kidney symptoms include nephrocalcinosis and renal failure
treacher collins syndrome
mandibulofacial dysostosis
defects of structures derived from first and second branchial arches
coloboma
deformed pinna
hypoplasia of condyles
hypoplastic mandible
apert syndrome
acrocephalosyndactyly
cloverleaf skull/ tower skull
ocular proptosis and middle face hypoplasia
downward slanting of palpebral fissures
syndactyly of hand
abnormal shape of maxilla
pseudoclefts
crouzon syndrome
craniofacial dysostosis
premature closing of cranial sutures
short head
boat shaped head
triangle shaped head
cloverleaf skull
shallow orbits
increased digital markings/ beaten metal pattern
underdev maxilla
crowded maxillarys
PARRY ROMBERG SYNDROME
HEMIFACIAL ATROPHY
atrophic changes on one side
autoimmune \disease
linear scleroderma
en coup de sabre (strike of sword)
enopthalmos
mouth an nose devaited
unilateral atrophy of tongue
STAFNE DEFECT/ BONE CYST / STATIC BONE DEFECT
asymptomatic radiolucency near the angle of mandible and molar
well circumscribed with sclerotic border
that contains submandibular gland/ muscle / fat
EAGLE SYNDROME /STYLOHYOID SYNDROME
facial pain dysphagia otalgia headache
named after dr eagle
mineralisation of stylohyoid ligament
