Developmental Disturbances of Oral Structures

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Questions and Answers

What defines a malformation?

A deviation from normal without functional interference

A defect developed due to pathological environmental conditions

An abnormal shape or structure caused by localized error in morphogenesis (correct)

An alteration in shape of a previously formed part

How do acquired developmental anomalies differ from congenital anomalies?

Acquired anomalies are always present at birth

Congenital anomalies develop due to heredity or environmental influences during gestation (correct)

Acquired anomalies occur due to genetic influences only

Acquired anomalies occur after birth due to environmental conditions (correct)

Which of the following best describes an anomaly in the context of developmental disturbances?

Any abnormal shape resulting from environmental factors

A structural change with functional interference

A deviation from normal that may include functional impairment

A defect present at birth with normal function (correct)

Which example illustrates deformation accurately?

Torticollis resulting from an alteration in shape Signup and view all the answers

What term describes malformations that lead to structural changes?

Anomalad Signup and view all the answers

Which of the following is not considered a congenital developmental anomaly?

Acquired torticollis Signup and view all the answers

What is a potential effect of the Robin anomalad?

Hypoplasia of the mandible leading to respiratory difficulties Signup and view all the answers

Which statement regarding congenital anomalies is true?

They may be present due to heredity or environmental influences Signup and view all the answers

What is the primary reason a biopsy is rarely performed on a fissured tongue?

It has a characteristic diagnostic clinical appearance. Signup and view all the answers

Which of the following histological features is NOT associated with a fissured tongue?

Marked accumulation of keratin on filiform papillae. Signup and view all the answers

What histological change is characteristic of hairy tongue?

Loss of papillae with elongated rete ridges. Signup and view all the answers

What type of inflammation is typically noted in hairy tongue?

Mild to moderately intense chronic inflammatory cell infiltrate. Signup and view all the answers

What feature may be present in the subepithelial tissues of median rhomboid glossitis?

Fibrosed stroma with chronic inflammation. Signup and view all the answers

Which statement correctly describes the histological appearance of a fissured tongue?

Loss of filiform papillae with a mixed inflammatory infiltrate. Signup and view all the answers

What type of cellular infiltrate may be identified in a hairy tongue?

Lymphocytic infiltration. Signup and view all the answers

What is a common finding on the dorsal surface of a tongue affected by hairy tongue?

Accumulated debris among the papillae. Signup and view all the answers

What is a common characteristic feature of Down syndrome?

High arched palate Signup and view all the answers

What causes a complete cleft tongue?

Failure of groove obliteration by mesenchymal proliferation Signup and view all the answers

Which of the following statements is correct regarding fissured tongue?

Deep fissures appear in children but increase with age. Signup and view all the answers

Which abnormality commonly presents in individuals with Down syndrome?

Epicanthal folds Signup and view all the answers

What is the main presentation of median rhomboid glossitis?

Erythematous patch of atrophic mucosa Signup and view all the answers

How does a bifid tongue differ from a complete cleft tongue?

Bifid tongue results from incomplete merging of the lingual swellings. Signup and view all the answers

What dental issue can arise due to the presence of a cleft tongue?

Irritation from food debris accumulation Signup and view all the answers

Which feature is associated with Melkersson-Rosenthal Syndrome?

Fissured tongue Signup and view all the answers

What type of enamel defect is characterized by a complete absence of enamel or presence only in focal areas?

Hypoplastic type Signup and view all the answers

Which type of dentinogenesis imperfecta is associated with osteogenesis imperfecta?

Dentinogenesis Imperfecta I Signup and view all the answers

What histological feature is commonly seen in affected teeth of dentinogenesis imperfecta?

Short and irregular dentinal tubules Signup and view all the answers

Which type of enamel defect involves a normal structure but altered mineral deposition?

Hypocalcification type Signup and view all the answers

What is a key characteristic of the remaining dentin in dentinogenesis imperfecta?

Severe dysplasia with interglobular dentin Signup and view all the answers

How is enamel thickness typically affected in the hypoplastic type of enamel defect?

Below normal thickness Signup and view all the answers

What distinct color can affected teeth show in dentinogenesis imperfecta?

Gray to yellowish-brown Signup and view all the answers

What condition is associated with a lack of differentiation of ameloblast cells leading to little or no matrix formation?

Hypoplastic type Signup and view all the answers

Which syndrome is associated with micrognathia and is characterized by cleft palate?

Pierre Robin syndrome Signup and view all the answers

What is a common oral manifestation in cases of facial hemiatrophy?

Deviated jaw alignment Signup and view all the answers

Which of the following conditions may lead to calcification in follicular connective tissue?

Trauma Signup and view all the answers

What is a characteristic feature of Treacher Collins syndrome?

Facial asymmetry Signup and view all the answers

What is the etiology of congenital micrognathia as seen in Pierre Robin syndrome?

Unknown origins Signup and view all the answers

Which chromosomal abnormality is linked to severe dental anomalies and facial asymmetry?

Trisomy 18 Signup and view all the answers

Which syndrome is also referred to as Parry Romberg syndrome?

Progressive facial hemiatrophy Signup and view all the answers

Which condition is NOT associated with congenital micrognathia?

Stickler syndrome Signup and view all the answers

Study Notes

Developmental Disturbances of Oral and Para Oral Structures

Malformation: Defect due to a localized error in morphogenesis resulting in an abnormal shape or structure, with interference in function. Example: cleft palate.
Deformation: An alteration in shape or structure of a previously normally formed part. Example: torticollis.
Anomaly: Any deviation from normal. It is the same as malformation but there is no interference with function. Example: peg-shaped lateral.
Anomalad: Malformation and subsequently derived structural changes. Example: Robin anomalad, where hypoplasia of the mandible results in cleft palate and respiratory difficulty.

Congenital Developmental Anomalies

These defects are present at birth or before birth during the intrauterine life because of either heredity or environmental influences.
Example: Cleft lip & palate.

Acquired Developmental Anomalies

These defects develop during intrauterine life due to some pathological environment conditions.
Can be prenatal, neonatal, or postnatal.
Example: Down syndrome (Trisomy 21 syndrome).

Down Syndrome (Trisomy 21 Syndrome)

Flat face.
Large anterior fontanel.
Open sutures.
Small slanting eyes with epicanthal folds.
Open mouth.
Cardiac abnormalities.
Macroglossia.
High arched palate.

Cleft Tongue/Bifid Tongue

Complete cleft tongue: occurs due to lack of merging of lateral lingual swellings.
Partial cleft tongue: occurs due to incomplete merging and failure of groove obliteration by underlying mesenchymal proliferation.
Partially cleft tongue is more common and is manifested as a deep groove in the midline of the dorsal surface.
Food debris and microorganisms collect in the base of the cleft and cause irritation.

Fissured Tongue/Scrotal Tongue

Deep fissures may be seen in children or adults but increase with age.
Down syndrome and Melkersson-Rosenthal Syndrome.
Histologic features:
- Atrophic stratified squamous epithelium.
- Occasionally pseudoepitheliomatous hyperplasia.
- Loss of papillae, elongated rete ridges, and lymphocytic infiltration.

Median Rhomboid Glossitis

Central papillary atrophy of the tongue or posterior lingual papillary atrophy.
It is an asymptomatic elongated erythematous patch of atrophic mucosa on the mid-dorsal surface of the tongue.
A biopsy is rarely performed on a fissured tongue because of its characteristic diagnostic clinical appearance.
Histologic examination has shown an increase in the thickness of the lamina propria, loss of filiform papillae of the surface mucosa, hyperplasia of the rete pegs, neutrophilic micro abscesses within the epithelium, and a mixed inflammatory infiltrate in the lamina propria.

Hairy Tongue

Characterized by marked accumulation of keratin on filiform papillae of the dorsal surface resulting in a hair-like appearance.
Histologic Features:
- Presence of fungal hyphae.
- Consist of elongated filiform papillae, with mild hyperkeratosis and occasional inflammatory cells.
- Debris accumulation among the papillae and candidal pseudohyphae is not unusual finding.
- A mild to moderately intense chronic inflammatory cell infiltrate may be seen within subepithelial and deeper fibrovascular tissues.

Black Hairy Tongue, Lingua Nigra, Lingua Villosa

Elongated filiform papillae, with mild hyperkeratosis and occasional inflammatory cells.
Debris accumulation among the papillae and candidal pseudohyphae is not unusual finding.

Enamel Hypoplasia

Teeth exhibit complete absence of enamel or there may be presence of enamel on some focal areas.
Hypoplastic type: Lack of differentiation of ameloblast cells with little or no matrix formation.
Hypocalcification type: Abnormal matrix structure & mineral deposition.
Hypomaturation type: Alteration in the enamel rod & rod sheath structures.

Dentinogenesis Imperfecta

It is the hereditary developmental disturbance of the dentin in the absence of any systemic disorder.
Affected teeth are gray to yellowish-brown.
Histologic Features: Enamel & mantle dentin are normal. The dentin, on the other hand, is composed of irregular tubules, with large areas of uncalcified matrix. Also known as “Hereditary opalescent dentin” & “Capdepont’s teeth”.
Remaining dentin is severely dysplastic & exhibits vast areas of interglobular dentin.
Dentinal tubules are short, disoriented, irregular & widely spaced.
Classification:
- Dentinogenesis imperfecta I: It occurs with Osteogenesis Imperfecta with opalescent teeth. Deciduous teeth more severely affected. Large pulp chamber with pulp stones.
- Dentinogenesis imperfecta II: It is an autosomal dominant condition without associated bone disorder. Both deciduous and permanent teeth are equally affected.
- Dentinogenesis imperfecta III: It is a rare autosomal recessive condition where the dentin is abnormal and radiolucent. It does not occur with Osteogenesis Imperfecta or any other systemic disorder.

Etiology of Micrognathia

Congenital:
- Pierre Robin syndrome.
- Catel Manzke syndrome.
- Cerebrocostomandibular syndrome.
Intrauterine acquired conditions:
- Congenital syphilis.
- Turner syndrome.
Chromosomal abnormality:
- Trisomy 18.
Autosomal dominant conditions:
- Treacher Collins syndrome.
- Pallister Hall Syndrome.

Facial Hemiatrophy

Also known as Parry Romberg Syndrome, Progressive Facial Hemiatrophy, Progressive hemifacial atrophy.
Parry and Romberg in 1846.

Oral Manifestations of Facial Hemiatrophy

Dental anomalies: incomplete root formation, delayed eruption, and severe facial asymmetry.
Eruption of teeth on the affected side may be retarded.
Atrophy of half of the upper lip and tongue.
Deviation of jaws while opening the mouth.
A characteristic loss in the soft tissues of essentially half the face.

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Description

Explore the various types of developmental disturbances affecting oral and para oral structures. From malformations to acquired anomalies, learn how these conditions arise and impact function. This quiz will test your knowledge of congenital and acquired developmental anomalies and their implications.