Term
| Definition of amelogenesis imperfecta |
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Definition
| group of inherited disorders of enamel structure UNassociated w/ systemic disorder |
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Term
| Numerous subtypes of AI exist based upon what? |
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Definition
| Specific hereditary patterns & wide variety of clinical manifestations |
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Term
| How many enamel or enamel-associated genes have been identified as likely candidates behind these disorders? |
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Definition
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Term
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Definition
Hypoplastic
Hypocalcified
Hypomaturation |
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Term
| Definition of hypoplastic AI |
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Definition
| inadequate (insufficient) production of enamel matrix |
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Term
Definition of hypocalcified AI
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Definition
| defect or absence of mineralization of normal enamel matrix |
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Term
| Definition of hypomaturation AI |
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Definition
| defect in maturation of enamel's crystal structure |
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Term
| Five different patterns associated with hypoplastic AI |
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Definition
| Localized, smooth, generalized, rough, enamel agenesis |
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Term
| Localized pattern of hypoplastic AI: |
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Definition
| horizontal rows of pits, linear depression or one large area of hypoplastic enamel surrounded by zone of hypocalcification, usually in middle third of buccal surfaces of teeth |
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Term
| Smooth pattern of hypoplastic AI: |
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Definition
| smooth surface, thin, hard and glassy; teeth shaped like crown perparations; color varies from opaque white to translucent brown, to brown to yellow brown |
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Term
| Generalized pattern of hypoplastic AI: |
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Definition
| pinpoint to pinhead sized pits scattered across surface of teeth, not correlated with pattern of environmental damage; buccal surfaces more severely affected, pits in rows or columns; staining of pits may occur, intervening enamel of normal thickness, hardness & color |
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Term
| Characteristics of hypocalcified AI: |
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Definition
- Teeth appropriately shaped on eruption, enamel is soft and easily lost
- color changes from yellow-brown or orange to brown to black due to staining
- radiographic density similar to that of dentin
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Term
| Characteristics of hypomaturation AI: |
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Definition
- teeth are normal in shape but show mottled, opaque white-brown-yellow discoloration
- Enamel softer than normal and tends to chip away from underlying dentin
- radiographically, enamel density similar to dentin
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Term
| Histologic features of AI |
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Definition
| complex, varied and appreciable only in non-decalcified sections |
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Term
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Definition
- Address esthetics, dental sensitivity and loss of vertical dimension
- Less freq. problems w/ caries, anterior open bite, delayed eruption, impaction, associated gingival inflammation
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Term
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Definition
| Depends on individual type of AI |
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Term
| Dentinogenesis imperfecta definition |
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Definition
| Developmental disturbance of dentin in absence of any systemic disorder |
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Term
| What mutation is DI associated with? |
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Definition
DSPP (dentin sialophosphoprotein) gene
8 mutations known; 7 assoc w/ DI and 1 w/ DDtype2 |
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Term
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Definition
Caucasians of English or French ancestry
1:8,000 caucasians in US |
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Term
| Inheritance pattern of DI |
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Definition
| Autosomal dominant almost 100% penetrance but variable expressivity |
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Term
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Definition
- deciduous most severely affected + permanent teeth
Blue-brown discoloration
Translucent/opalescent appearance
Enamel often separates from underlying defective dentin at CEJ = wears away fast
Occasional kindreds have demonstrated progressive, sensorineural high-freq hearing loss
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Term
| Radiographic features of DI |
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Definition
-Bulbous crowns, cervical constriction, thin roots & early obliteration of root canals and pulp chambers
- Occassionally defective dentin may be eroded from pulpal side resulting in pulp chmabers which are larger than nromal (shell teeth)
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Term
| Histologic features of DI |
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Definition
-Poorly formed dentin - irregularly arranged and malformed dentinal tubules w/ atypical granular dentin matrix and much interglobular calification
-Small # of atypical odontoblasts line pulp surface |
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Term
| Treatment & Prognosis of DI |
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Definition
Challenging restoring problems
Guarded; tendency for teeth to fracture |
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Term
| Dentin dysplasia definition |
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Definition
| hereditary disorder of dentin (autosomal dominant) unaccompanied by systemic disaese and unrelated to dentinogenesis imperfecta, one type is caused by mutation in DSPP gene |
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Term
| How many types of DD are there? |
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Definition
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Term
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Definition
| characterized by highly disorganized root dentin, often resulting in short stubby roots, extremely mobile teeth and early exfoliation & weakness of radicular dentin predisposing to fracture under trauma |
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Term
| Radiographic features of DD type 1 |
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Definition
Little to no detectable pulp, narrow ribbon of pulp about where the base of pulp chamber should be
Short, stubby roots w/ frequent periapical radiolucencies, periapical inflammatory disease from tiny ribbons of residual pulp present w/in defective dentin being exposed by caries |
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Term
| Which type of DD is associated with short, stubby roots? |
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Definition
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Term
| Histologic features of DD type I |
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Definition
- Coronal enamel and dentin are normal
- Apical to pulp chamber the dentain disorganization in central portion of root forms whorls of tubular dentin and atypical osteodentin (stream flowing around boulders)
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Term
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Definition
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Term
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Definition
| characterized by deciduous teeth closely resembling those of DI but permanent teeth which appear essentially normal clinically except that they exhibit enlarged thistle tube shaped pulp chambers w/ prominent pulp stones |
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Term
| Which type of DD can histologically be described as "stream flowing around boulders" |
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Definition
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Term
| Which type of DD contains thistle-tube shaped pulp chambers w/ prominent pulp stones? |
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Definition
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Term
| Another name for DD Type 2 |
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Definition
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Term
| Clinical features of DD type II |
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Definition
-Deciduous teeth closely resemble those of DI: blue-amber-brown translucency
- Perm teeth show normal clinical color and shape
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Term
| deciduous teeth w/ DD type 2 clinically represent what other diseased teeth? |
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Definition
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Term
| Radiographic features of DD type 2 |
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Definition
- Deciduous teeth show bulbous bell-shaped crowns and pulpal obliteration (like DI)
- Permanent teeth show thisle-shaped pulp chambers w/ prominent pulp stones
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Term
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Definition
preventive care to minimize caries and PerioD very important
Endo very difficult |
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Term
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Definition
preventive care minimze caries and PD
Endo treatment more successful |
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Term
| regional odontodysplasia "ghost teeth" definition |
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Definition
| localized NONhereditary developmental abnormality of teeth which results in one or a group of teeth w/ extremely enlarged pulps and thin enamel and dentin, most often either only partially erupted or not erupted at all |
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Term
| Pathogenesis of Ghost teeth |
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Definition
| Unknown but thought to be due to localized restricted vascular flow during development |
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Term
| Clinicoradiographic features of regional odontodysplasia: |
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Definition
- Occurs in both dentitions
- F>M
- 2-4 yrs; 7-11 yrs
- Typically affects specific region including several adj teeth
- max > mand |
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Term
| True or false: Ghost teeth are always unilateral |
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Definition
| False, can be bilateral but usually unilat |
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Term
| Characteristics of erupted Ghost teeth: |
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Definition
| - Small irregular crowns which are yellow to brown, often rough surface, caries & periapical inflammatory disease common (maybe due to dentinal clefts and long pulpal horns leading to pulpal necrosis) |
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Term
| How are ghost teeth characterized radiographically? |
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Definition
- Very thin enamel and dentin surrounding enlarged lucent pulp; lack of contrast b/t dentin and enamel
Short roots w/ open apices; pulp stones |
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Term
| Histologic features of Regional Odontodysplasia |
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Definition
- Ground variations show in enamel thickness & quality of enamel prism structure
- Decalcified standard sections show clefts & interglobular areas in dentin
- Pulp stones in pulp
- Dental follicle thickened and shows many "enameloid conglomeates," thought to be result of abnormal enamel deposition/metabolism
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Term
| Treatment & prognosis for Ghost teeth |
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Definition
Aimed at retention of altered teeth when & where possible to allow for appropriate dev and preservation of surrounding alveolar ridge -
Generally good depends on success of treatment
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