developmental disturbances

Question 2

4 features of gardners syndrome

Answer 2

(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,

Question 3

congenital syphilis

Answer 3

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.

Question 4

dentinogenesis imperfecta

Answer 4

. 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.

Question 5

numeration of Developmental nomalies of tongue

Answer 5

Aglossia
♦ Microglossia
♦ Macroglossia
♦ Ankyloglossia or tongue tie
♦ Cleft tongue
♦ Fissured tongue
♦ Median rhomboid glossitis
♦ Geographic tongue
♦ Hairy tongue
♦ Lingual thyroid nodule
♦ Lingual varices

Question 6

amelogenesis imperfecta

Answer 6

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