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Disturbances in Development Continued (Dental & Facial)

Dentine Dysplasia Type 1- (Also called: Rootless teeth) has

shortening of root lengths with variable severity (can be very short or somewhat short) the shorter the root is the more probable that the tooth will be lost prematurely, Dentine dysplasia also contains Obliteration of

pulp chambers & peri-apical lesions,

appears normal. The only sign of Dentine Dysplasia type 1 is the loosening of teeth; upon preforming a radiograph the obliterated pulp, short roots &

Lesions can be seen.

however the outer view/Coronal view

Lesions can be seen. however the outer view/Coronal view Dentine Dysplasia Type 2- Has two different

Dentine Dysplasia Type 2- Has two different clinical appearances one for

primary teeth and a different set of clinical appearances for permanent teeth. In Primary teeth their appearance resembles the color of dentinogenisis imperfecta

appearances for permanent teeth. In Primary teeth their appearance resembles the color of dentinogenisis imperfecta
appearances for permanent teeth. In Primary teeth their appearance resembles the color of dentinogenisis imperfecta
appearances for permanent teeth. In Primary teeth their appearance resembles the color of dentinogenisis imperfecta

(Yellowish/Brownish color due to damaged dentine), as well as the presence of

obliterated roots (also present in dentinogenesis imperfecta). In Permanent Dentition

the root length is Normal and the Pulp is present and coronal pulp is enlarged and

extends radicularly resulting in a ‘flame shaped’ appearance, pulp channels are

narrow but not obliterated, but they may contain pulp stones.

Roots

Length/Morphology

Pulp Chambers

Dentine Dysplasia Type 1

Shortened/Rootless

Obliterated

Dentine Dysplasia Type 2

Normal Length

Present, Coronal is Flame shaped and Radicular is narrow

Regional Odontodysplasia- (also called Ghost teeth) in this condition ALL

OF THE TOOTH STRUCTURES in one area have abnormalities (dentine, pulp & Enamel

are all abnormal) the gingiva surrounding the teeth is also abnormal (thick gingiva).

Regional Odontodysplasia is considered idiopathic but the most likely theory is that

its caused by Ischemia or an abnormal blood flow to the affected region (teeth +

surrounding tissue). On a radiograph you can see very thin enamel and dentine, but

an enlarged pulp (giving it the ‘ghost tooth’ appearance). The teeth in the affected

area usually dont erupt because the erupting force is

abnormal and the overlying gingiva is very thick and calcified. **Note: Remember that ‘Turners tooth”
abnormal and the overlying gingiva is very thick and
calcified.
**Note: Remember that ‘Turners tooth” only affects
ONE SINGLE TOOTH while Regional Odontodysplasia
affects several teeth and the surrounding area.

Hypophosphatasia- is another cause for premature/early tooth loss, caused

by a decrease in Alkaline Phosphatase (an enzyme NOT THE SAME AS PHOSPHATE) resulting in abnormal cementum and abnormal alveolar bone. Abnormal cementum leads to failed attachment of PDL fibers, leading to abnormalities in tooth attachment to its sockets. Alkaline phosphatase is very important for bone formation and deposition meaning that abnormalities In it will cause a decrease in levels/formation of cementum and alveolar bone, even if calcium and phosphate levels are normal. Hypophosphatasia also leads to delayed eruption of the teeth because the eruption force (which depends on force originating from PDL fibers and tooth socket) is abnormal, this may also lead to spontaneous loss of BOTH DENTITIONS meaning that Hypophosphatasia is present for long periods of time, meaning that it is a GENETIC DESIEASE autosomal recessive.

Developmental Diseases in Soft Tissue-

Commisural Lip Pit- This is a very common developmental change that you

will see a lot in the oral diagnosis clinic, a pit appears in the corner of the mouth, possibly present bilaterally (on both sides). It is simply an invagination or an inwards depression in the epithelium of the mouth that is a BLIND TRACT meaning that it does not open into any other tracts or ducts, it may contain drainage from minor salivary glands. This developmental change is usually autosomal dominant (genetic/Familial), however this pit has no signifance.

glands. This developmental change is usually autosomal dominant (genetic/Familial), however this pit has no signifance.
glands. This developmental change is usually autosomal dominant (genetic/Familial), however this pit has no signifance.
glands. This developmental change is usually autosomal dominant (genetic/Familial), however this pit has no signifance.

Paramedian Lip Pits- If you draw a

midline through the lower lip you will see two pits surrounding the midline, these are called paramedian pits. Paramedian pits are very rare and may be associated with cleft pallet or Van Der Woude Syndrome.

be associated with cleft pallet or Van Der Woude Syndrome. Double Lip- is an excess fold

Double Lip- is an excess fold of tissue that is

present on the inner aspect of the upper lip especially visible when the patient tenses the upper lip (like smiling). Double lip may be associated with Ascehr Syndrome which is accompanied with drooping of the eyelids and non-toxic goiter (enlargement of the thyroid gland, without an increase in thyroid secretions).

thyroid gland, without an increase in thyroid secretions). Frenal Tag- A very common condition that must

Frenal Tag- A very common condition

that must be known to avoid confusion with a polyp or fibrous tissue enlargement, when in fact it is a small excess/projection from the maxillary labial frenum that is non-pathogenic and does not cause any problems, it can be Familial but it is composed of Normal Oral Mucosa.

that is non-pathogenic and does not cause any problems, it can be Familial but it is
that is non-pathogenic and does not cause any problems, it can be Familial but it is
that is non-pathogenic and does not cause any problems, it can be Familial but it is
that is non-pathogenic and does not cause any problems, it can be Familial but it is

Ankyloglossia- (Ankylo= tied / Glossia= related to the tongue) is a condition

when the tongue is either attached to the floor of the mouth ANTERIORLY or to the gingiva. The normal location of the lingual frenum (which attaches the tongue to the floor of the mouth is in the posterior part of the mouth) in Ankyloglossia the lingual frenum is located anteriorly at the tip of the tongue tying the tongue down to the floor of the mouth; limiting the movement of the tongue which may lead to complications such as problems, in speech, swallowing, gingival problems/gingival recession. Most forms of ankyloglossia prevent the patient from being able to pronounce the letter “R”.

the patient from being able to pronounce the letter “R”. Macroglossia- Is in an increase in

Macroglossia- Is in an increase in the size of

the tongue due to a congenital disorder such as Down syndrome (Primary cause) or tumors/cancer, lymphoangioma, hemangioma, neurofibroma, amyloidosis (protein deposition in tissues due to inflammation), acromegaly (hormone changes), cretinism (deposition in tissues).

(hormone changes), cretinism (deposition in tissues). Fordyce Granules- Usually present at the junction between

Fordyce Granules- Usually present at the junction between the upper

vermillion border and the buccal mucosa. Fordyce granules are Ectopic Sebaceous glands found intra-orally, but since there is no hair in the oral cavity Fordyce granules form in these sebaceous glands, they appear clinically as yellowish spots.

is no hair in the oral cavity Fordyce granules form in these sebaceous glands, they appear
is no hair in the oral cavity Fordyce granules form in these sebaceous glands, they appear
is no hair in the oral cavity Fordyce granules form in these sebaceous glands, they appear

Leukoedema- manifests as a whitish appearance on the buccal mucosa, the

whitish color on the buccal mucosa disappears UPON STRETCHING. It has a white milky appearance that disappears upon stretching giving a normal pink appearance. THIS CONDIIOTN IS NOT CAUSED BY EXCESS KERATIN it is caused by Intra-cellular edema in the prickle cell layer (intra-cellular edema CANNOT be removed) which leads to an increase in the thickness of the oral mucosa blocks the view of the blood vessels beneath (in normal condition blood vessels beneath oral mucosa give the mucosa its’ pink color).

beneath oral mucosa give the mucosa its’ pink color). *Increase in thickness of epithelium = whitish

*Increase in thickness of epithelium = whitish color

*Increase in Vascualrity with normal thickness = Pinkish color

White Sponge Nevus- Is a defect in keratin types K4 & K13 giving abnormal

structure or amount giving a whitish appearance of the buccal mucosa as well as abnormal keratinization of other mucosal surfaces (eyes, conjunctiva, nasal… may also appear whitish). White Sponge Nevus is autosomal Dominant found above and below the occlusal plane and extends posteriorly towards the retro-molar area to the alveolar mucosa (basically the white sponge nevus IS NOT CAUSED by keratinization of tissue in response to teeth biting down on the mucosa) - it is present from birth And does NOT DISSAPEAR UPON STRETCHING. Histologically you can find Hyperkeratosis (increase in keratin), acanthosis (increase in number of epithelial cells), and possibly edema of spinous layer.

(increase in keratin), acanthosis (increase in number of epithelial cells), and possibly edema of spinous layer.

Lingual Thyroid nodule- Forms when the thyroid gland does not go to its’

proper position during formation and attaches near foramen cecum (on the dorsum of the tongue). It is possible that the patient may be missing the actual thyroid gland completely and that the lingual thyroid nodule is the only thyroid tissue in the patients’ body. If the lingual thyroid does not interfere with any oral processes (mastication, speech, swallowing etc.) then there is no need for it to be removed; however of it does interfere it must be surgically removed (if this is the only thyroid tissue in the patient, removal may lead to shock).

thyroid tissue in the patient, removal may lead to shock). Oral Tonsil- are tonsils found sublingually

Oral Tonsil- are tonsils found sublingually on either side of the lingual frenum

or on the lateral sides of the tongue that contain lymphoid tissue, these tonsils act as normal tonsils and are part of the tonsil ring of waldeyer and may even enlarge during infection. These tonsils are common areas for ‘Oral lympho-epithelial cysts’

Retrocuspid papilla- Will be discussed in the lab.

Hemifacial Hypertrophy- (Hemi= half, Facial = Face half the face) One side of the face
Hemifacial Hypertrophy- (Hemi= half,
Facial = Face half the face) One side of the face is
Hypertrophied/larger than the other side the face,
skin, muscles, tongue, teeth, gingiva, soft tissue, and
the brain
etc. are hypertrophic on the affected side.

This condition can lead to Mental retardation and a seizure in 15-20% of the cases, Hemi facial hypertrophy is one of the few conditions that displays TRUE MACRODONTIA.

Passive Hemifacial Atrophy- (Parry-Romberg Syndrome) this condition is

almost the exact opposite of hemi facial hypertrophy, where the child is born with a normally proportional face but after a certain age (around 6 years) one side of the face begins to completely degenerate (including skin, muscle and bone). This condition usually affects the left side of the face more than the right and can lead to problems in the brain (due to cramping of space- BRAIN DOES NOT SHRINK) leading to seizures and epilepsy ON THE CONTRALATERAL SIDE of the body (the side opposite to the atrophied side has seizures). The cause of hemi facial atrophy is not completely understood but it is possibly related to Sympathetic Innervation. Teeth still being developed when atrophy begins (permanent teeth are still developing at the age of 6 years) may have delayed development or eruption.

Cleft Lip and Pallate- we won’t go into too much detail because we will

discuss this later, just know that the mildest form of Cleft palate is the bifid uvula, the most sever form is when the cleft palate perforates the whole bone and communicates with the nasal cavity. Cleft lip and palate are commonly associated with supernumerary teeth or hypodontia.

communicates with the nasal cavity. Cleft lip and palate are commonly associated with supernumerary teeth or
communicates with the nasal cavity. Cleft lip and palate are commonly associated with supernumerary teeth or
communicates with the nasal cavity. Cleft lip and palate are commonly associated with supernumerary teeth or
communicates with the nasal cavity. Cleft lip and palate are commonly associated with supernumerary teeth or

Stafne bone defect - (lingual mandibular salivary gland depression) a defect

(lingual mandibular salivary gland depression) a defect found on the lingual aspect of the mandible that

found on the lingual aspect of the mandible that appears as a depression caused by the salivary glands. In the radiograph you can see the Molar area of the mandible, below the mandible you have the IV canal beneath that there is a radiolucent rounded area, that can be confused with a cavity inside the bone IT IS NOT A CAVITY IT IS A DEPRESSION/CONCAVITY, this was caused by the lingual salivary glands pushing against the mandible during formation forming the depression, this depression may be confused with bony diseases or metastatic cancer, but initially when we see a radiolucent area beneath the IV canal we should think about stafne bone defect. When this area is biopsied you will find Salivary gland tissue (usually sub mandibular but rarely sub lingual)

Focal Osteoporotic Bone Marrow Defect- is presented on a radiograph as

a lesion/ finding in the bone that contains BONE MARROW TISSUE, in this case the bone marrow is hyper-plastic and active (in anemic females this marrow will be activated to produce more RBCs). Appears as an ill-defined relatively radiolucent on the radiograph, once biopsied you will find bone marrow and lipid cells. This condition may occur

radiolucent on the radiograph, once biopsied you will find bone marrow and lipid cells. This condition
radiolucent on the radiograph, once biopsied you will find bone marrow and lipid cells. This condition
radiolucent on the radiograph, once biopsied you will find bone marrow and lipid cells. This condition
radiolucent on the radiograph, once biopsied you will find bone marrow and lipid cells. This condition

in edentulous areas after tooth extraction, present as FOCAL osteoporotic, still it SHOULD NOT be confused with tumors.

Cleidocranial Dysplasia- Is a genetic disease that has

Abnormal growth of bone the most alarming of which is ABSENCE OF CALVICLES leading to approximation of shoulders and hypermobility of the shoulders, The skull is also abnormally wide (due to large parietal bones) this is due to DELAYED CLOSURE OF FONTANELS or sutures. The Maxilla appears hypoplastic and retruded the patient also has A ‘V’ Shaped Pallet this condition is also accompanied by multiple supernumerary impacted teeth as well as abnormal/lack of cellular cementum formation.

as well as abnormal/lack of cellular cementum formation. Crouzon Syndrome- Is a condition where the cranial

Crouzon Syndrome- Is a condition

where the cranial sutures close prematurely leaving no room for the brain to expand/develop, leading to an abnormally shaped skull, Exopthalmic eyes (bulging eyes), affected vision (damaged optic nerve), Nose appears beak like, maxilla is depressed along with the sinus area, short upper lip and lower lip appears protruded.

Nose appears beak like, maxilla is depressed along with the sinus area, short upper lip and
Nose appears beak like, maxilla is depressed along with the sinus area, short upper lip and
Nose appears beak like, maxilla is depressed along with the sinus area, short upper lip and
Nose appears beak like, maxilla is depressed along with the sinus area, short upper lip and

Treacher Collins syndrome- appears to be involved with Paternal age (the

older the father is the more likely it is to occur), in this condition there is a defect in the 1 st and 2 nd branchial arches which are responsible for formation of the face defected face. In treacher Collins syndrome THE MANDIBLE IS RETRUDED AND HYPOPLASTIC (underdeveloped) as well as abnormal ears, the TMJ and its joints are also hypoplastic and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them.

and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done

Done By: Ali Hassan Al-Qudsi

and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done
and the maxilla is also hypoplastic, the eyes are depressed and have depression beneath them. Done

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