Amelogenesis imperfecta.

This is a radiographic view
of amelogenesis imperfecta showing the altered
thickness and shape of the enamel crown that is
characteristic of this disease. Ìn this patient the
condition was inherited as an autosomal dominant
trait..
W podontia. This radiograph shows a
retained deciduous, mandibular
second molar. Note that the first
premolar is in place, full erupted, and
apparentl functional. There ma be
carious involvement of the distal
aspect of the crown of the deciduous
second molar.
W This photograph presents a clinical
view of a nine-ear-old bo who has
ectodermal dsplasia, a condition which
affects all ectodermall-derived
structures. Ìt is inherited as both an X-
linked and an autosomal recessive trait.
Note that the hair is sparse and there is
an absence of both the eebrows and
eelashes. Fingernails and toenails are
affected as well. What oral findings
might ou expect?
W podontia associated with ectodermal dsplasia. This photograph
shows an intraoral view that is characteristic of patients with
ectodermal dsplasia. Ìn this case onl the two maxillar canine teeth
have developed. The secondar dentition was also affected in this
patient, and there were no succedaneous replacements, even for
these two primar teeth. Because of the absence of teeth, alveolar
bone also fails to form. Patients with ectodermal dsplasia are best
treated through provision of full upper and full lower dentures, which
must be replaced at regular intervals as growth takes place.
W False anodontia. This frame shows an
interesting clinical phenomenon. This nine-
ear-old girl presented clinicall with an
"absence of teeth". Radiographs, however,
revealed the presence of teeth beneath the
existing gingiva. This condition was caused b
the overgrowth of gingiva as a result of a
phenomenon called gingival hperplasia
(increased cellular proliferation). Ìn this case,
the condition was triggered b the gradual and
continual eruption of the teeth. As the teeth
emerge from the alveolar process, the gingiva
overgrows and keeps pace with the rate of
eruption. Patients with this condition are best
treated b regular gingivoplast and
gingivectom to allow normal function and
aesthetics. Gingival overgrowth can also be
initiated in patients taking Dilantin (antiseizure
medication), Clcosporine
(immunosuppressive drug) or calcium channel
blocker, particularl Nifedipine (treatment of
angina and hpertension). Good oral hgiene
has been shown to reduce the severit of
drug-induced gingival hperplasia.
W $upernumerar teeth. This clinical view shows a supernumerar
tooth which has erupted in the mid-line between the left and right
maxillar central incisors. The mesiodens, as it is called, is one of the
more common forms of supernumerar teeth. Ìn this case the
supernumerar tooth was acceptable aestheticall, and therefore, no
treatment was provided to the patient. A less estheticall acceptable
example of a mesodens is shown here. Not all supernumerar teeth
resemble a normal tooth in either size or shape. $uch teeth are often
called accessor teeth.
W $upernumerar teeth. This is an
example of an impacted supernumerar
tooth (found in the maxilla between the
lateral incisor and canine) that was
found during a routine radiographic
examination. Multiple supernumerar
teeth, man of them impacted, are
characteristicall found in cleidocranial
dsostosis. Gardner's $ndrome is
another condition characterized b the
occurence of multiple, impacted
supernumerar teeth. This disease is of
interest to the dental profession,
because the presence of the
supernumerar teeth, and multiple
osteomas in the mandible and maxilla,
ma lead to its diagnosis.
W Dens-in-dente (Dens invaginatus). This radiograph show an example of what
happens when there is a distortion of the enamel organ. As a consequence, an
aberrant crown forms in which there is commonl a communication between the
surface of the crown and the pulp chamber. Carious involvement of this usuall deep
pit in the crown leads to bacterial invasion of the dental pulp. This radiograph
demonstrates bon tissue breakdown as a consequence of a severe inflammator
response in the pulp of this tooth. There is a large radiolucenc at the apex of this
tooth. Also note that this tooth has a widened pulp canal inconsistent with the
patient's age (compare this canal with that of the adjacent canine tooth). $econdar
dentin formation that would have resulted in normal narrowing of this canal did not
take place because of the death of the pulp.
W Dens-in-dente. This is another example of a "tooth within a tooth", but there is no
evidence of a connection between the surface of the crown and the dental pulp.
WGemination. This clinical view reveals the
presence of a rather large lateral incisor. Count
the teeth. ow man incisors are there? This
oversized tooth is a result of gemination or
twinning of the enamel organ during
development. This partial splitting results in a
larger template for the crown, and the clinical
crown becomes much larger than normal. $uch
large teeth, often with clefts in their surfaces,
should be considered twinned teeth if the proper
number of teeth are present
WGemination. This is a radiograph of another
patient showing that in a "twinned" tooth, the pulp
chamber is often divided into two chambers
within the single partiall divided crown of the
tooth. Notice that there is a single pulp canal.
W Fusion. Ìn this case, how man teeth are present? This is a
clinical example of fusion. Fusion occurs when neighboring
toothbuds fuse, which as in gemination also results in a single
larger-than-normal tooth. Counting the teeth in this mandibular
arch should have revealed the absence of a right lateral
incisor. The lateral incisor and the central incisor have fused to
make a single "macrodont" tooth. Fused teeth commonl have
a clinical crown that is fissured, as ou see here. The also
usuall have two pulp chambers and two pulp canals, but
these characteristics depend on the time at which fusion took
place. A second example of fusion is shown here.
W Dilaceration. This photograph, and frame #17
show examples of dilaceration, or curving of the
roots. This results from a distortion of ertwig's
root sheath during development. $uch teeth ma
present severe problems for either endodontic
treatment or extraction. The are usuall vital and
functional. $urprisingl, eruption does not seem to
be affected b the altered shape of the roots.
W Accessor roots. This is an example
of accessor root formation that can
occur as a result of an alteration in
root sheath development.
W Rootless tooth. A 31-ear-old male
patient complained of a molar that was
"loose". Radiographs revealed an
absence of roots. Because of the high
mobilit of the tooth, it was extracted.
For whatever reason, proliferation of
ertwig's root sheath abruptl ceased
before much of the roots were formed.
The cervical line of the tooth is clearl
evident.
W Rootless teeth. This is another example
of a patient who complained of "loose"
teeth. Radiographs revealed onl short
stubb roots. The cervical line of the
tooth is clearl evident, and the staining
is due to tetraccline. For whatever
reason, proliferation of ertwig's root
sheath abruptl ceased, but is not
known whether tetraccline was a
factor
W namel pearl. An enamel "pearl" is demonstrated in this photograph of
an extracted tooth. The "pearl" lies in the furca (groove) between the
mesial and distal roots of this maxillar molar. The differentiation of
ameloblasts, and the formation of small amounts of enamel below the
level of the cervical occurs with some regularit. The production of an
actual "enamel pearl" is not as commonl seen. Radiographicall, this
would have been recognized as a ver bright radiopacit overling the
furcal area of this tooth. The presence of these ectopic enamel
deposits can have significant consequences for the periodontal health
of these teeth. Notice that the palatal root of this tooth is affected b
dilaceration.
W utchinson's or "$crewdriver-shaped" incisors. This clinical
photograph presents an example of peg-shaped and misshapen
incisors in both the maxilla and the mandible that occur in
patients with congenital sphilis. Another anomal, "mulberr
molars," is shown in frame #22. There is narrowing of the incisal
third of the maxillar and mandibular incisors, a form which has
been described as screwdriver-shaped. The Treponemes (T.
pallidum) that cause sphilis cross the placenta, and the fate of
the infected fetus depends on the duration and the stage of the
mother's disease.
W Mulberr molars. This clinical photograph presents a
second example of the effects of congenital sphilis
on the developing dentition. The first anomal,
"screw-driver shaped" incisors is shown in the
previous frame. The blebbed surface of the mulberr
molar wears awa rather quickl, and carious
involvement of the multiple pits is not an uncommon
feature of these teeth.
W namel poplasia. poplasia commonl leads to an altered
tooth form as a result of a reduction in the quantit of the organic
matrix produced. Not uncommonl there is also a defect in the
mineralization process. poplastic and hpomineralization
defects usuall occur as a consequence of disease states.
Because of the pitting, and other structural defects, hpoplastic
enamel is often more susceptible to dental caries. This clinical
photograph shows severe hpoplasia in the incisal third of the
crowns with loss of some of the enamel.
W namel poplasia. Ìn this patient,
hpoplasia resulted in enamel pitting
that predisposed these teeth to carious
attack.
W namel pomineralization. This clinical view presents an
example of "snow-capped" teeth. This phenomenon is a form
of hpomineralization. Notice that all four incisor teeth are
affected with the central incisors showing the greatest defect.
Onl the maxillar teeth were involved in this patient. This
condition ma be due to a dietar deficienc, a sstemic
disease, or to an inherited defect in mineralization.
W namel hpoplasia associated with rickets. This is a case of
avitaminosis D that results in the clinical condition of Vitamin D-
dependent rickets. $evere hpoplastic defects are present in all
eight maxillar and mandibular permanent incisors. This gives
some idea of the time in which the nutritional deficienc took
place. Ìn some cases, vitamin C and vitamin A deficiencies can
lead to similar hpoplastic defects in enamel.
W namel fluorosis (mottled enamel). Low-grade pitting, followed b
pigmentation of the pitted areas can be seen in teeth when exposure
levels of fluoride exceed 1-2 parts per million in the drinking water, or if
inappropriate fluoride supplementation is carried out during earl infanc
and childhood. The degree of fluorosis seen in this patient suggests that
fluoride levels in the drinking water exceeded 5 parts per million. The high
levels of fluoride incorporated into the hdroxapatite crstals of the
enamel renders these teeth extremel resistant to carious attack. Ìn fact,
the beneficial effects of fluoride were recognized after epidemiological
studies of communities in which fluorosis was endemic revealed a
significant reduction in the prevalence of dental caries. A second example
of fluorosis is shown here.
W Amelogenesis imperfecta. This patient demonstrates an inherited
form of enamel hpomineralization in which tooth form is reasonabl
good. An geneticall inherited form of hpomineralization or
hpoplasia is called amelogenesis imperfecta, and there are at least
13 distinct inheritance patterns that have been characterized,
including both autosomal and sex-linked recessive and dominant
forms.. The thinness of the enamel and its poor attachment to the
underling dentin lead to the rapid loss of the enamel from the
surface of the crown. The brown areas seen in this photograph
represent discolored areas of exposed dentin. A second patient is
shown here.
W Amelogenesis imperfecta. This is
different patient presenting a more
severe tpe of amelogenesis imperfecta.
Ìn this case there is both a hpoplasia
and a hpomineralization of the enamel.
W Amelogenesis imperfecta. This is a
radiographic view of amelogenesis
imperfecta showing the altered thickness
and shape of the enamel crown that is
characteristic of this disease. Ìn this patient
the condition was inherited as an autosomal
dominant trait..
W Dentinogenesis imperfecta. Dentinogenesis imperfecta is often associated with
a sstemic condition known as osteogenesis imperfecta in which bone is
affected, as well as teeth. Ìn the latter condition, a peculiar blue coloration of
the sclera of the ee ma sometimes be seen. Dentinogenesis imperfecta is
characterized b the presence of a pearl-gra coloration of the teeth and an
earl loss of the enamel, particularl from incisal and occlusal surfaces. The
loss of the enamel is the result of an abnormal dentinoenamel junction. Ìn
addition, the dentin is excessivel "soft" due to the high amount of interglobular
dentin, which is hpomineralized, and thus support for the enamel is poor. The
peculiar coloration of the tooth is a result of the obliteration the pulp chamber,
which normall gives a pinkish coloration to the dentin. A second view is
shown in Frame #34 presents a radiograph of these teeth showing several
distinctive features.
W Dentinogenesis imperfecta. Dentinogenesis imperfecta is
characterized b the presence of a pearl-gra coloration of the teeth
and an earl loss of the enamel, particularl from incisal and occlusal
surfaces. The loss of the enamel is the result of an abnormal
dentinoenamel junction. Ìn addition, the dentin is excessivel "soft" due
to the high amount of interglobular dentin, which is hpomineralized,
and thus support for the enamel is poor. The premature loss of enamel
exposes the dentin to occlusal forces that can lead to severe attrition.
As in this patient, full crown coverage is often used to preserve
occlusal height. A another view is shown in frame #33. Frame #35
presents a radiograph of these teeth showing several distinctive
features.
W Dentinogenesis imperfecta. This is a radiograph of the previous patient
showing several distinctive features of dentinogenesis imperfecta. Clinical
photographs are shown in frame #33 and frame #34. The teeth have a
"lollipop" look, i.e. a bulbous crown that appears to be attached to a narrow
root. There is a marked reduction in the size of the pulp chambers and pulp
canals, with an absence of the canals in at least one of these teeth. Ìt is not
uncommon for these teeth to be painful, but the cause of the pain is not
full understood. Ìn contrast, it is uncommon for patients who have
amelogenesis imperfecta to experience pain, even in teeth that have been
denuded of their enamel. A closer look is shown in this radiograph.
W Dentinal dsplasia. This is a rare phenomenon in
which there is abnormal dentin development and
aberrant root formation. The dentin is poorl formed
with large amounts of interglobular dentin present,
and pulp chambers and pulp canals are virtuall
nonexistent. These teeth quickl become mobile, and
are commonl extracted because the cannot
withstand the forces of occlusion.
W percementosis. This radiograph reveals
an excessive radiopacit surrounding the
root. owever, careful inspection will
reveal the presence of a radiolucenc (the
periodontal ligament space) and a
radiodense line (the lamina dura).
W Concrescence. This frames shows the clinical features of the
condition known as concrescence wherein hpercementosis
has resulted in the union of two or more teeth. The
histological appearance is shown in frame #38. The cause of
this condition is not understood, but it presents significant
problems for extraction of these teeth. Normal function,
however, seems to be unaffected.
W Concrescence. This frames
shows the histological
features of the condition
known as concrescence
wherein hpercementosis
has resulted in the union of
two or more teeth. The
clinical appearance is
shown in frame #37. Note
that there is an absence of
the intervening alveolar
bone.

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