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Dent 355

Oral Pathology 1 Developmental Disturbances Dr. Rima Safadi

Developmental Disturbances of the Oral Region


Teeth Soft tissues Bone

Developmental Disturbances of the Oral Region


Teeth
Size Number Eruption Shape Structure

TEETH Disturbances in size


Microdontia Generalized
True Relative

Localized
Peg laterals Maxillay third molars Supernumerary

TEETH Disturbances in size


Macrodontia Generalized
True Relative

Localized/regional
Hemifacial hypertrophy

Rhizomegaly=radiculomegaly
Mandibular canine

TEETH Disturbances in NUMBER


Anodontia: No deciduous, no permanent Ectodermal dysplasia Hypohidrotic X linked recessive affecting males Autosomal recessive

Hypodontia
Ectodermal dysplasia

Transmembrane protein in
Keratinocytes Teeth (partial, rarely complete/total anodontia) Sweat glands Hair follicles

Ectodermal dysplasia

Ectodermal dysplasia

TEETH Disturbances in NUMBER Hypodontia


Familial tendency
Third molars maxillary laterals Second premolars

In deciduous: maxillary laterals

TEETH Disturbances in NUMBER


Supernumerary Teeth Single or multiple Erupted impacted
Maxilla >>>>mandible Mesiodense> Paramolars (fourth molar)> Maxillary lateral incisor In mandible:

Shape: conical or like normal counterpart In deciduous teeth: maxillary lateral incisor

most common: premolars Also fourth molars and incisors

SUPERNUMERARY

TEETH Disturbances in NUMBER Supernumerary Teeth


Multiple and impacted in: Cleidocranial dysplasia Gardner syndrome

Disturbances in Eruption
Premature eruption:
One or 2 teeth usually deciduous mandibular central incisors May be familial Not supernumerary
Natal teeth: present at birth Neonatal teeth: present in first 30 days of life Permanent teeth: think of endocrine disorder if generalized

Natal/neonatal teeth

Disturbances in Eruption
Delayed eruption:
Cleidocranial dysplasia, cretinism Gingival fibromatosis

Impacted teeth:
Physical barrier Crowding, supernumerary teeth, odontogenic cyst and tumors e.g: third molars, max. canines

Disturbances in Eruption
Eruption sequestrum: A small spicule of calcified tissue that is extruded through the alveolar mucosa that overlies an erupting molar. Requires no treatment

TEETH Disturbances in Shape


Dilaceration: Causes
Trauma during tooth development Continued root formation idiopathic

Complications :

TEETH Disturbances in Shape


Taurodontism:
Elongated crown and apically placed root furcation in molars Molars, premolars occasionally Permanent, deciduous occasionally Cause: Invagination of root sheath during development Associated with:
Amelogenesis imperfecta Down syndrome Klinefelter syndrome

Complications

TEETH Disturbances in Shape


Dense Invaginatus (dense in dente) Affect pulp champer and sometimes shape of root Mainly maxillary lateral incisor Lingual pitdilated odontome Pulpal involvement and periapical pathosis

Dense in Dente

Disturbances in Shape
Supernumerary cusp: Cusp of Carabelli mesiolingual surface of upper 6s Causes no problems, requires no treatment

Talon cusp
Lingual aspect of maxillary centrals Cingulum portion Pits on lateral aspects May interfere with occlusion Contain prominent pulp horn

Supernumerary cusp Dense evaginatus (Leong premolar)


Present on occlusal or lingual surface of premolars Contain a pulp hornperiapical lesion Delay eruption Supernumerary Roots: Most common in mandibular premolars, canines and third molars Significance:

Disturbances in Shape
Gemination Fusion Concrescence Hypercementosis Cervical enamel projection

Fusion
Unusual wide crown Union between adjacent
tooth germs at least with dentin Complete or incomplete according to the stage of development Differentiate from gemination by counting number of teeth Clinical significance: esthetic, crowding, periodontal disease

Gemination
Usually anterior teeth Single rooted tooth
with wide crown Incompletely divided tooth germ Affect deciduous and permanent

Concrescence
Confluence of
cementum after root formation is complete Trauma, crowding Affect maxillary molars Significance: tooth extraction problems

Hypercementosis
Excessive deposits of cementum: Increased or decreased occlusal forces Hyperpituitarism Chronic inflammation Significance: tooth extraction

Cervical enamel extension

Enamel pearl

Alterations in Structure of Teeth

Hereditary or environmental Environmental Bacterial and viral infections, nutritional deficiencies, chemical injuries, trauma Resultant defect: focal or generalized Either deciduous or permanent Affect both types of hard tissues Hereditary Genetically determined Both deciduous and permanent dentitions Either enamel or dentine

Localized Causes
Turner tooth:
local infection related to deciduous predecessor range varies: discoloration (yellow to brown) to grooves (hypoplasia)

Enamel opacities:
hypomineralized, white spot on central incisor very common

Turner tooth

Generalized Causes
Chronological
Hypoplasia
Bands of pits and grooves Distribution depends on enamel developing at time of the disease

Generalized Causes of enamel hypoplasia


Hypocalcemia: pitted type Neonatal line (microscopically) affecting
deciduous teeth and first permanent molars
Systemic insult during birth

Dental Fluorosis: hypomineralized or hypoplastic


Chemically induced: interfere with ameloblast function White flakes or brown discoloration (motteling) smooth enamel surface or pitted Severe cases: soft enamel Resistant to dental caries Susceptible to light exposure

Dental Fluorosis

Table 1.2 in your book


Etiology of developmental abnormalities of enamel REQUIRED

Amelogenesis imperfecta

Developmental Alterations in Structure of Teeth


Amelogenesis imperfecta Hereditary, no associated systemic disorders Affects both deciduous and permanent dentition Witkop classification: 3 major types: 1. Hypoplastic type 2. Hypocalcified: 3. Hypomaturation: e.g snow capped crowns

Deficient matrix production= enamel hypoplasia


Pits and grooves General reduction in thickness

Defective maturation/mineralization:
hypomineralized enamel
White and opaque Brown and pigmented

Amelogenesis imperfecta
Hypoplastic type:
Generalized: Localized:
pits scattered across the surfaces of teeth may be in rows and columns In the middle of the buccal surfaces All the teeth or scattered teeth may be affected affect deciduous and permanent teeth Open contacts White to brown color Like the smooth pattern Denser enamel and more resistant to attrition

AD smooth pattern:

Rough pattern

Amelogenesis imperfecta localized

Amelogenesis imperfecta hypoplastic, rough

Amelogenesis imperfecta rough pattern

Amelogenesis imperfecta hypoplastic, smooth

Amelogenesis imperfecta
Hypocalcification
Teeth erupt in normal shape but are orange to brown in color Enamel is lost easily upon eruption Radiographically: enamel less radio dense than dentin

Hypocalcification

Amelogenesis imperfecta hypocalcified

Amelogenesis imperfecta
Hypomaturation
opaque, white to yellow discoloration softer enamel, chip easily radiographically: density is similar to dentin mottled, fractures easily like a denture dipped in white paint

Pigmented pattern:
Snow capped pattern

Hypomaturation pigmented pattern

Hypomaturation snow capped pattern

Hypomaturation snow capped pattern

Dentinogenesis imperfecta
Developmental disorder results in irrigularly formed dentin, obliteration of root canals Type 1: associated with osteogenesis imperfecta Type2: teeth only affected Type3: Brandywine isolate, rare racial isolates in USA multiple pulpal exposures

Dental manifestations: Type II (hereditary opalescent dentin) Both dentitions are affected Distinctive translucent color Enamel separated easily Bulbous crowns, cervical constrictions, early obliteration of pulp spaces Not prone to dental caries

Dentinogenesis Imperfecta

Dentinogenesis imperfecta
Dental manifestations: Shell teeth
Extremely thin dentin and extremely large pulp Especially in deciduous dentition

Dentinogenesis imperfecta
Histopathology: Short misshapen dentinal tubules

Dentin Dysplasia
Hereditary Abnormal dentin and abnormal pulp morphology 2 major types of dentin dysplasia Type I: Radicular dentin dysplasia; rootless teeth Type II: Coronal dentin dysplasia;
Deciduous dentition has many features similar to dentinogenesis imperfecta

Dentin Dysplasia Type I


Far more common than type II All teeth in both dentitions are affected Increased mobility and premature exfoliation in
both dentitions

Loss of organization of root dentin during tooth


development -> variable decrease in root length. Enamel and coronal dentin are normal.

Dentin Dysplasia, Type I


Short blunt roots W shaped roots of
molars Obliterated or chevronshaped pulp champers Periapical radiolucency resembling abscesses, granulomas or cysts.
Deciduous teeth exhibit obliterated pulp champers too

Neville, Color Atlas of Clinical Oral Pathology, 2nd ed, Fig 2.54

Dentin Dysplasia I

Dentin Dysplasia I

Dentin Dysplasia, Type I

Neville, Color Atlas of Clinical Oral Pathology, 2nd ed, Fig 2.53

Dentin Dysplasia Type II


Both dentitions are affected Clinical appearance of deciduous is different that
permanent: Deciduous teeth: features similar to dentinogenesis imperfecta in translucent hue and in pulp obliteration Normal root shape and length

Permanent teeth: normal root length Abnormally large pulp champers with abnormal radicular
extension: flame shaped root pulp Pulp stones and narrowed canals

Dentin Dysplasia Type II


Permanent teeth have normal color but
have apical extension of pulp chamber -> thistle-tube or flame shaped.

Dentin Dysplasia, Type II

Note pulpal calcifications

Dentin Dysplasia, Type II

Regional Odontodysplasia Ghost teeth


Localized developmental
abnormality of several adjacent teeth affecting enamel, dentin, and pulp

Soft tissue is hyperplastic and


contains calcifications

Cause: most are


nonhereditary and idiopathic. Localized ischemic cause

Hypophosphatasia
Autosomal recessive condition Deficiency of alkaline phosphatase in serum and tissues Disorders of bone mineralization: Rickets and osteomalacia
Dental manifestation: Absence (inadequate formation) of cementum Enamel normal, pulp is large Alveolar bone loss + - hypoplastic enamel defects Delayed formation and eruption of dentition Premature loss of primary teeth Spontaneous loss of permanent teeth

Soft Tissue

Congenital lip pits


Commissural lip pit

Invagination: Blind
tracts or dilated ducts Autosomal dominant trait Paramedian pits may be associated with cleft lip/palate (Van der Woude syndrome)
Paramedian lip pits

Double lip

Horizontal fold Inner aspect of upper lip Congenital or acquired Visible when the lip is tensed Ascher Syndrome: drooping of eyelids and non toxic goiter and double lip

Frenal Tag
A redundant piece of
mucosal tissue that projects from the maxillary labial frenum. Familial, AD Histologically: normal oral mucosa

Ankyloglossia
e
Lack of normal
tongue mobility Anteriorliy positioned lingual frenum Adverse effects Treatment

Macroglossia
Congenital: Down syndrome Secondary: tumors
lymphangioma, hemangioma,
neurofibroma, amyloidosis, acromegaly, cretinism.

Fordyce granules

Fordyce granules
Sebaceous glands Not associated with
hair Bilaterally on buccal mucosa, upper vermilion border, gingiva

Leukoedema
accumulation of fluid within the epithelial cells-edema Bilateral More in black Variation of normal White milky appearance that disappears upon stretching

White Sponge Nevus


AD Mutation in keratin pair K4 and K13 White thickened and folded oral mucosa Other mucosal surfaces Generalized involvement of the oral mucosa and other mucosal surfaces Histopath: hyperkeratosis, acanthosis, edema of spinous layer.

Lingual Thyroid nodule


Accessory thyroid tissue Midposterior tongue Symptoms: dysphagia,
dyspnea, dysphonia May be the only thyroid tissue More in females during puberty and adolescence

Oral Tonsil
Lingual tonsil: at base of the tongue Part of Waldeyer ring Reactive hyperplasa: Foliate papillitis Oral tonsil Sides of lingual frenum Oral lymphoepithelial cysts may develop

Retrocuspid papilla
A slightly raised area lingual to mandibular
canine Uni or bilateral More in children Histopathology: resemble incisive papilla
May be covering a fibrovascular canal

Developmental Disturbances of Bone



Hemifacial hypertrophy Hemifacial atrophy: Romberg syndrome Cleft lip and palate Lingual mandibular salivary gland depression Focal osteoporotic bone marrow defect Cleidocranial dysplasia Crouzon syndrome Treacher Collins syndrome Down syndrome Papillon-Lefevre syndrome

Hemifacial hypertrophy

Increased neurovascular supply to the affected side Increased incidence of visceral tumors Involves all tissues in the affected side even brain hemispheres Mental retardation and seizures in 15-20%

Progressive hemifacial atrophy (Parry-Romberg Syndrome)



Degenerative condition 1st or 2nd decade Skin, muscle, bone. Left side more than right Sympathatic nervous system malfunction??? Hereditary?, trauma? Infection? Contra lateral epilepsy Teeth may exhibit delayed development Retarded tooth eruption Atrophic lip and tongue

Cleft lip and palate

Stafne bone defect (lingual mandibular salivary gland depression)


Focal concavity in the
cortical bone on the lingual aspect of the mandible May contain salivary gland tissue May contain muscles, nerves or be devoid of contents 80-90% seen in men

Stafne bone defect (lingual mandibular salivary gland depression)


Radiographically:

Focal Osteoporotic Bone Marrow Defect


Ill defined Edentulous area
After extraction, the defect is filled with hematopoetic bone marrow No pain or expansion Biopsy may be needed To rule out malignancy

Cleidocranial Dysplasia
Genetic disease, abnormal growth of bones. Skull, clavicle, failure of tooth eruption
Fontanels are open or have late closure Sutures remain open Prominent frontal, parietal and occipital bones

Shoulders
Aplasia, hypoplasia or thinning of clavicles Hypermobility of shoulders

Oral
High narrow palate. May be cleft Hypoplastic maxilla and sinuses Supernumerary teeth

Cleidocranial dysplasia

Cleidocranial Dysplasia
Teeth
Prolonged retention of primary teeth Succedaneous teeth may fail to erupt or have delayed eruption due to lack of cellular cementum Multiple unerupted supernumerary teeth Severe malocclusion

Craniofacial Dysostosis (Crouzon Syndrome)


Genetic disease. Manifestations are due to
synostosis = early closure of sutures Mutation of FGF2

Clinical Features: frog-like face due to midface


hypoplasia and exophthalmos Craniofacial
Hypoplastic maxilla Short upper lip Parrot-beak nose

Crouzon Syndrome

Craniofacial Dysostosis (Crouzon Disease)


Eyes
Exophthalmos Increased interpupillary distance Optic nerve damage and possible blindness

Oral
High-arched palate, sometimes cleft Hypoplastic maxilla --> malocclusion

Mandibulofacial dysostosis (Treacher Collins syndrome)

Mandibulofacial dysostosis (Treacher Collins syndrome)


Genetic disease Defects in first and second branchial
arches Associated with increased paternal age, Characteristic faces: midface hypoplasia, underdeveloped mandible, hypoplasia of condyle and coronoid Lateral facial clefting Trt: cosmetic surgery