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Which feature is characteristic of the enamel surrounding unerupted teeth in dental anomalies?
Which feature is characteristic of the enamel surrounding unerupted teeth in dental anomalies?
What is a distinguishing histological feature of the dentin in Dentinogenesis Imperfecta?
What is a distinguishing histological feature of the dentin in Dentinogenesis Imperfecta?
Which of the following describes true micrognathia?
Which of the following describes true micrognathia?
What is a common radiographic finding associated with dentin formation disorders?
What is a common radiographic finding associated with dentin formation disorders?
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Which option correctly identifies the two types of true micrognathia?
Which option correctly identifies the two types of true micrognathia?
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What is a defining characteristic of the hypoplastic type of dentinogenesis imperfecta?
What is a defining characteristic of the hypoplastic type of dentinogenesis imperfecta?
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Which of the following describes the histological features of the hypoplastic type of dentinogenesis imperfecta?
Which of the following describes the histological features of the hypoplastic type of dentinogenesis imperfecta?
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In which category is 'dentinogenesis imperfecta I' classified?
In which category is 'dentinogenesis imperfecta I' classified?
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Which statement best describes the clinical implications of enamel defects in dentinogenesis imperfecta?
Which statement best describes the clinical implications of enamel defects in dentinogenesis imperfecta?
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What radiographic characteristic is commonly associated with dentin formation disorders?
What radiographic characteristic is commonly associated with dentin formation disorders?
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What is a key histological feature of dentinogenesis imperfecta II?
What is a key histological feature of dentinogenesis imperfecta II?
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Which clinical implications are associated with dentinogenesis imperfecta type III?
Which clinical implications are associated with dentinogenesis imperfecta type III?
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Which of the following is a characteristic of hypomaturation type of dentinogenesis imperfecta?
Which of the following is a characteristic of hypomaturation type of dentinogenesis imperfecta?
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What is micrognathia also known as?
What is micrognathia also known as?
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What histological feature is typically observed in cases of micrognathia?
What histological feature is typically observed in cases of micrognathia?
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Which radiographic characteristic can indicate dentinogenesis imperfecta?
Which radiographic characteristic can indicate dentinogenesis imperfecta?
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What distinguishes dentinogenesis imperfecta II from type III?
What distinguishes dentinogenesis imperfecta II from type III?
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Which of the following statements regarding dental enamel in dentinogenesis imperfecta is accurate?
Which of the following statements regarding dental enamel in dentinogenesis imperfecta is accurate?
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What is defined as an alteration in shape or structure of a previously normally formed part?
What is defined as an alteration in shape or structure of a previously normally formed part?
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Which term is used to describe defects that are genetically transmitted from parents to offspring?
Which term is used to describe defects that are genetically transmitted from parents to offspring?
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What is an example of a malformation that interferes with function?
What is an example of a malformation that interferes with function?
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Which term best describes any deviation from normal that also indicates lack of functional interference?
Which term best describes any deviation from normal that also indicates lack of functional interference?
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What defines the term 'anomalad' in relation to structural changes?
What defines the term 'anomalad' in relation to structural changes?
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Which of the following correctly describes cleft palate?
Which of the following correctly describes cleft palate?
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What type of developmental anomaly is related to environmental influences during intra-uterine life?
What type of developmental anomaly is related to environmental influences during intra-uterine life?
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Which of the following examples illustrates a congenital defect due to environmental factors?
Which of the following examples illustrates a congenital defect due to environmental factors?
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What is a potential result of hypoplasia of the mandible?
What is a potential result of hypoplasia of the mandible?
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Which of the following describes acquired developmental anomalies?
Which of the following describes acquired developmental anomalies?
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Which anomaly is characterized by uni/bilateral blind tracts at the angle of the lip?
Which anomaly is characterized by uni/bilateral blind tracts at the angle of the lip?
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What is a manifestation of congenital syphilis?
What is a manifestation of congenital syphilis?
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Which age range can developmental anomalies occur?
Which age range can developmental anomalies occur?
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Which statement accurately describes familial developmental anomalies?
Which statement accurately describes familial developmental anomalies?
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What is the prevalence range for commissural lip pits?
What is the prevalence range for commissural lip pits?
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Which developmental anomaly is known to result in notched incisors?
Which developmental anomaly is known to result in notched incisors?
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What is a common histological finding in median rhomboid glossitis?
What is a common histological finding in median rhomboid glossitis?
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Which type of amelogenesis imperfecta is characterized by defective maturation?
Which type of amelogenesis imperfecta is characterized by defective maturation?
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What type of tissue is primarily affected in lingual thyroid nodules?
What type of tissue is primarily affected in lingual thyroid nodules?
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What is a characteristic of the hypoplastic type of amelogenesis imperfecta?
What is a characteristic of the hypoplastic type of amelogenesis imperfecta?
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Which feature is not typically observed in median rhomboid glossitis?
Which feature is not typically observed in median rhomboid glossitis?
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Which of the following describes the hypomaturation-hypoplastic type of amelogenesis imperfecta?
Which of the following describes the hypomaturation-hypoplastic type of amelogenesis imperfecta?
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In cases of median rhomboid glossitis, what type of inflammatory response is typically present?
In cases of median rhomboid glossitis, what type of inflammatory response is typically present?
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What is the significance of colloid degeneration in thyroid nodules?
What is the significance of colloid degeneration in thyroid nodules?
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What abnormality is associated with the appearance of teeth in dentinogenesis imperfecta II?
What abnormality is associated with the appearance of teeth in dentinogenesis imperfecta II?
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What histological feature characterizes the dentin in affected individuals?
What histological feature characterizes the dentin in affected individuals?
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Which condition is described as having a very small lower jaw?
Which condition is described as having a very small lower jaw?
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How does dentinogenesis imperfecta III differ clinically from type II?
How does dentinogenesis imperfecta III differ clinically from type II?
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Which abnormality is present in both dentitions in cases of dentinogenesis imperfecta II and III?
Which abnormality is present in both dentitions in cases of dentinogenesis imperfecta II and III?
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What structural alteration occurs in the enamel rod and rod sheath in dentinogenesis imperfecta?
What structural alteration occurs in the enamel rod and rod sheath in dentinogenesis imperfecta?
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Which of the following is NOT a characteristic of micrognathia?
Which of the following is NOT a characteristic of micrognathia?
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What is a common finding in the histology of teeth affected by dentinogenesis imperfecta?
What is a common finding in the histology of teeth affected by dentinogenesis imperfecta?
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Which syndrome is associated with congenital micrognathia?
Which syndrome is associated with congenital micrognathia?
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What encompasses the term 'facial hemiatrophy'?
What encompasses the term 'facial hemiatrophy'?
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What is a common dental anomaly associated with intrauterine acquired conditions?
What is a common dental anomaly associated with intrauterine acquired conditions?
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Which of the following conditions is known to show significant unilateral enlargement of the face?
Which of the following conditions is known to show significant unilateral enlargement of the face?
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What is the primary etiology of congenital micrognathia?
What is the primary etiology of congenital micrognathia?
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Which syndrome is also known as Parry Romberg Syndrome?
Which syndrome is also known as Parry Romberg Syndrome?
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What type of anomalies can be caused by trauma or infection leading to micrognathia?
What type of anomalies can be caused by trauma or infection leading to micrognathia?
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Which condition represents hyperplasia of tissues rather than hypertrophy?
Which condition represents hyperplasia of tissues rather than hypertrophy?
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Study Notes
Dentinogenesis Imperfecta
- Hereditary developmental disturbance of dentin
- Absence of systemic disorder
- Affected teeth are gray to yellowish-brown in color
Hypoplastic Type
- Defective formation of dentin matrix
- Complete absence of enamel or enamel present in focal areas
- Enamel thickness is usually below normal
- Quality of enamel is normal but quantity is affected
- Lack of differentiation of ameloblast cells
- Little or no matrix formation
Hypocalcification Type
- Alteration in enamel rod and rod sheath structures
- Cheesy consistency of teeth
Dentinogenesis Imperfecta Classification
- Dentinogenesis Imperfecta I: Occurs with Osteogenesis Imperfecta. Opalescent teeth. Tooth appears ready for a prosthetic crown.
- Dentinogenesis Imperfecta II: Both dentitions are affected.
- Dentinogenesis Imperfecta III: Similar to type II. Multiple pulp exposures, periapical radiolucencies, and variable appearance. Both dentitions are affected.
Clinical and Radiographic Features
-
Widening of the pre-dentin layer:
- Severe dysplasia of remaining dentin
- Large areas of inter-globular dentin
- Short, disoriented, irregular, and widely spaced dentinal tubules
- Scanty odontoblasts lining the pulp
- Smooth DEJ
- Irregular tubular pattern of dentin
- Reduced enamel epithelium around unerupted teeth showing many irregular calcified bodies
- Abnormal matrix structure and mineral deposition
- Multiple pulpal exposures in deciduous dentition
Micrognathia
- Also known as Mandibular Hypoplasia
- Condition of a very small lower jaw in children
- Two types:
- Congenital: Present at birth
- Acquired: Develops after birth
Dentinal Features
- Normal Enamel and Mantle Dentin
- Irregular tubules in dentin
- Large areas of uncalcified matrix
- Enamel doesn’t form to full thickness on newly erupted developing teeth.
- Teeth exhibit complete absence of enamel or there may be presence of enamel on some focal areas
- Enamel thickness is usually below normal
- Quantity is affected, but quality of formed enamel is normal
Dentinogenesis Imperfecta Histological Features
- Enamel and mantle dentin are normal
- Dentin composed of irregular tubules with large areas of uncalcified matrix
Developmental Anomalies
-
Malformation: A defect caused by a localized error in morphogenesis leading to abnormal shape or structure, interfering with function. Example: Cleft palate
-
Deformation: An alteration in the shape or structure of a previously normally formed part. Example: Torticollis
-
Anomaly: Any deviation from normal. Similar to malformation but without interference in function. Example: Peg-shaped lateral incisors
-
Anomalad: Malformation resulting in subsequent structural changes. Example: Robin anomalad, where hypoplasia of the mandible leads to cleft palate and respiratory difficulties.
Congenital Developmental Anomalies
- Present at birth or before birth during intrauterine life
- Result from either heredity or environmental influences
- Examples: Cleft lip and palate, Downs syndrome
Hereditary Developmental Anomalies
- Genetically transmitted from parents to offspring.
- Definite genetic location identified: Example: Downs Syndrome (Trisomy 21)
- Definite genetic location NOT identified: Example: Diabetes
Acquired Developmental Anomalies
- Defects develop during intrauterine life due to pathological environmental conditions.
- Can be prenatal, neonatal, or postnatal.
- Example: Congenital Syphilis
- Notched incisors
- Mulberry molars
Developmental Disturbances of Oral and Para-Oral Structures
-
Lip Pits: Congenital lip pits.
- Paramedian lip pits: Common (1-20%), can be as deep as 2 cm. Can be autosomal dominant in some cases.
- Commissural: Bilateral blind tracts at the angle of the lip, up to 4 mm.
Histopathology
- Median rhomboid glossitis: Smooth or nodular surface covered by atrophic stratified squamous epithelium, overlying a moderately fibrosed stroma. Debris accumulation and candidal pseudohyphae are not uncommon.
- Lingual thyroid nodule: Consist of normal mature thyroid tissue. May exhibit colloid degeneration.
- Fungiform and filiform papillae: Not seen, although surface nodules may mimic or represent anlage of these structures.
- Inflammation: Mild to moderately intense chronic inflammatory cell infiltrate may be seen within subepithelial and deeper fibrovascular tissues.
Amelogenesis Imperfecta
- Also known as Hereditary Enamel Dysplasia/Hereditary Brown Enamel/Hereditary Brown Opalescent Tooth.
- A group of conditions caused by defects in the genes encoding enamel matrix proteins.
- Affects both dentition (deciduous and permanent).
- Types:
- Hypoplastic type: Defective matrix deposition.
- Hypocalcification type: Defective calcification.
- Hypomaturation type: Defective maturation.
- Hypomaturation-hypoplastic with taurodontism: Abnormal matrix structure and mineral deposition.
### Dentinogenesis Imperfecta
- Type II: Appearance of cheesy consistency. Both dentition affected.
- Type III: Similar to type II but with clinical and radiological variations, including multiple pulp exposures, periapical radiolucencies, and variable radiographic appearance. Both dentition affected.
- Multiple pulpal exposures in deciduous dentition
Micrognathia
- Also known as Mandibular hypoplasia.
- Condition where a child has a very small lower jaw.
- Etiology of congenital: Unknown, but associated with Congenital Heart Disease or Pierre Robin Syndrome.
- Acquired postnatal: Due to ankylosis of the joint, as a result of trauma or infection.
Causes of Micrognathia
-
Congenital:
- Pierre Robin syndrome
- Catel Manzke syndrome
- Cerebrocostomandibular syndrome
-
Intrauterine acquired conditions: Congenital syphilis.
Facial Hemiatrophy
- Also known as Parry Romberg Syndrome, Progressive Facial Hemiatrophy, Progressive Hemifacial Atrophy.
- Description: Unilateral atrophy of the face.
-
Oral manifestations:
- Dental anomalies: Incomplete root formation, delayed eruption, severe facial asymmetry.
- Eruption of teeth on the affected side may be retarded.
Facial Hemihypertrophy
- Also called Friedreich’s Disease.
- Significant unilateral enlargement of the face.
- Represents hyperplasia of tissues rather than hypertrophy.
- Associated Syndromes:
- Neurofibromatosis
- Beckwith Wiedemann syndrome
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Description
This quiz explores the hereditary condition of Dentinogenesis Imperfecta, detailing its types and their characteristics. Learn about the differences between hypoplastic and hypocalcification types, as well as the classification of Dentinogenesis Imperfecta. Perfect for dental students and professionals!
