TOPIC: DEVELOPMENTAL DEFECTS  OF CRANIOFACIAL REGION

🌟 IMPORTANT POINTS TO REMEMBER

🔸Facial Hemihypertrophy: It is also known as Friedreich disease. Enlargement of half face occurs. On radiograph there is enlargement of bone to the affected side of the skull.

🔸Facial Hemiatrophy: It is also known as Parry–Romberg syndrome. There is appearance of white furrow on one side of face. Radiologically there is reduction of size of bone on the affected side.

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🔸CCD: It is also known as cleidocranial dysostosis. Patient is brachycephalic and bridge of the nose is depressed. Hypertelorism and micrognathia may be present. Radiologically there is presence of wormian bone (small irregular bone in the skull) in skull.

🔸Craniofacial Dysostosis: It is also known as Crouzon disease or syndrome. Premature closure of fibrous joint, exophthalmos, strabismus and hypertelorism may be present. Radiologically, absence of suture and beaten-metal appearance are important findings.

🔸Mandibulofacial Dysostosis: It is also known as Treacher Collins syndrome and Franceschetti syndrome. Development of zygomatic bone is retarded. Mandible is also underdeveloped with acute mandibular angle. Radiologically, most striking features are reduced size of zygomatic bone.

🔸Ectodermal Dysplasia: It is also known as hereditary hypohidrotic (anhidrotic) ectodermal dysplasia. Typical features include hypotrichosis, hypohidrosis and anhidrosis with ‘saddle nose’ appearance. Radiologically there is absence of teeth seen in the jaws.

🔸Developmental Salivary Gland Defect: It is also known as static bone cavity or cyst, Stafne cyst or defect, lingual mandibular bone cavity and latent bone cyst. It is radiolucent in appearance. Shape of radiolucency is circular or ovoid. Size rarely exceeds 3 cm.

🔸Cleft Lip And Cleft Palate: When there is bilateral involvement of cleft of lip, it is called ‘harelip’. In case of cleft palate, premaxillary portion is missing. This results in midline defect. Teeth are malformed and position abruptly in the jaw.

🔸Phlebectasia: It is also known as venous congenital cyst, venous aneurysm, venous ectasia or essential venous dilatation. Swelling is not visible before the compression, but as soon as patient clinches the jaws, swelling is visible. Dilated blood vessels are usually seen on ultrasonography.

📌 MULTIPLE CHOICE QUESTIONS (MCQs)

💡 Facial hemihypertrophy is also known as

1. Progressive facial hemiatrophy
2. Parry–Romberg syndrome
3. Friedreich disease
4. None of the above

Answer : 3

💡 Which one of the following is not the synonym of facial hemiatrophy?

1. Parry–Romberg syndrome
2. Romberg hemifacial atrophy
3. Progressive facial hemiatrophy
4. Marie and Sainton disease

Answer : 4

💡 Which one of the following is not the synonym of phlebectasia?

1. Essential venous dilatation
2. Venous ectasia
3. Franceschetti syndrome
4. Venous aneurysm

Answer: 3

💡Disorder which is caused by the inability of differentiation of maxillary mesoderm is known as

1. Treacher Collins syndrome
2. Cleidocranial dysplasia
3. Mandibulofacial dystosis
4. None of the above

Answer: 1

💡Another name for cleidocranial dysplasia is

1. Treacher Collins syndrome
2. Marie and Sainton disease
3. Crouzon disease
4. Hemifacial microstomia

Answer: 2

💡Absence of suture is an important radiological finding of

1. Cleidocranial dysplasia
2. Mandibulofacial dystosis
3. Hemifacial microstomia
4. Craniofacial dysostosis

Answer: 4

💡Absence of clavicle is seen in

1. Cleidocranial dysplasia
2. Franceschetti syndrome
3. Hemifacial microstomia
4. Ectodermal dysplasia

Answer: 1

💡Craniofacial dysostosis is

1. Autosomal dominant trait
2. X-linked recessive Mendelian disorder
3. X-linked autosomal
4. None of the above

Answer: 1

💡Facial hemiatrophy is also known as

1. Parry–Romberg syndrome
2. Romberg hemifacial atrophy
3. Progressive facial hemiatrophy
4. All of the above

Answer: 4

💡Crouzon disease or syndrome is the synonym of

1. Facial hemihypertrophy
2. Ectodermal dysplasia
3. Mandibulofacial dysostosis
4. Craniofacial dysostosis

Answer : 4

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