Developmental anomalies of face and associated structures

26 important questions on Developmental anomalies of face and associated structures

What is oblique facial cleft and what causes it?

Oblique facial cleft extends from the side of the nose to the corner of the mouth. It is caused by failure of fusion between the maxillary process and both the medial and lateral nasal prominences.

What is a cleft that is often accompanied by oblique facial clefts?

Oblique facial cleft is often accompanied by persistent naso-lacrimal cleft (open nasolacrimal duct)

What are the two main types of facial clefts, and what percentage of all facial clefts does each type represent?

1. Oblique facial cleft - 1%
2. Cleft lip and palate - 99%
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What is the difference between the anterior and posterior cleft?

1. Anterior cleft - A cleft anterior to the incisive foramen — involving the lip, alveolar ridge, and primary palate (different combinations of clefts in these stuctures can occur)
2. Posterior cleft - A cleft posterior to the incisive foramen — involving the secondary palate.

What is the cause of anterior cleft?

Failure of fusion between the medial nasal prominence and the maxillary prominence.

What is the cause of posterior cleft?

Failure of fusion between the palatine shelves and the nasal septum

What does image A represent?

Normal development of the lip and palate. (Properly formed primary and secondary palate)

What does image B represent?

Cleft Uvulamild posterior (secondary) cleft palate
- Posterior ends of the palatine shelves failed to fuse completely. 
- Hard palate and most of soft palate fused properly

What does image C represent?

Unilateral cleft of the secondary palate.
- One palatine shelf fails to fuse with the nasal septum and opposite shelf.
- Primary palate and opposite palatine shelf are normal

What does image D represent?

Bilateral cleft of the secondary palate
- Both palatine shelves fail to fuse with each other and with the nasal septum.
- Primary palate is normal

What does image E represent?

Complete unilateral cleft lip and primary palate.
- One medial nasal prominence fails to fuse with the maxillary prominence on the same side.
- Medial nasal prominences fuse with each other (philtrum), opposite side normal

What does image F represent?

Complete bilateral cleft lip and primary palate
- Both medial nasal prominences fail to fuse with their corresponding maxillary prominences.
- Medial nasal prominences fuse with each other (philtrum forms)

What does image G represent?

Complete bilateral cleft lip and primary palate with unilateral cleft secondary palate
- Both medial nasal prominences fail to fuse with their corresponding maxillary prominences + one palatine shelf fails to fuse with the nasal septum and the opposite palatine shelf
- Medial nasal prominences fuse together + One palatine shelf intact

What does image H represent?

Complete bilateral cleft lip and primary palate with bilateral cleft secondary palate.
- Both medial nasal prominences fail to fuse with their corresponding maxillary prominences + both palatine shelves fail to fuse with each other and the nasal septum
- Medial nasal prominences fuse together (philtrum)

Which images are posterior clefts?

B, C, D

Which images are anterior clefts?

E, F

Which images are mixed (both anterior and posterior clefts)?

G, H

What are some conditions where micrognathia is observed?

Treacher-Collins and Pierre-Robin syndromes

What anomaly is shown in image A?

Median cleft of the upper lip caused by failure of fusion between the two medial nasal prominences

What anomaly is shown in image B?

Median cleft of the lower lip caused by failure of fusion between the two mandibular prominences

What anomaly is shown in image C?

Bilateral oblique facial clefts with complete bilateral cleft of upper lip caused by failure of fusion of the maxillary processes with both the lateral and medial nasal prominences

What anomaly is shown in image D?

Macrostomia caused by incomplete fusion between the maxillary and mandibular prominences.

What anomaly is shown in image E?

Microstomia caused by excessive fusion of the maxillary and mandibular prominences.

What anomaly is shown in image F?

Bifid (separated into two) nose and incomplete median cleft lip caused by failure of the two medial nasal prominences to merge and failure to complete formation of the intermaxillary segment.

List the possible five congenital anomalies of the tongue.

1. Ankyloglossia
2. Microglossia
3. Macroglossia
4. Cleft tongue
5. Glossoptosis

What is a cleft tongue (bifid tongue)?

A midline groove or split in the tongue caused by failure of fusion of the lateral lingual swellings.

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