Developmental Defects I

Question 1

Proliferation of ectomesenchyme adjacent to placodes results in

Answer 1

Medial and lateral nasal processes

Question 2

At what week when we see medial and lateral nasal processes

Answer 2

4th

Question 3

At what week do we see the nasal pla code

Answer 3

4th

Question 4

At what week do we see the nasal pit

Answer 4

4th

Question 5

The nasal pit is the depression between

Answer 5

Medial and lateral nasal processes

Question 6

What makes up the upper lip

Answer 6

Medial nasal processes merge with each other and max Larry processes

Question 7

What makes up the alae of nose

Answer 7

Lateral nasal processes

Question 8

At what week do we see the formation of the upper lip

Answer 8

6th-7th

Question 9

At what week do we see the alae of the nose

Answer 9

6th-7th

Question 10

During the 6/7th week of development, the medial nasal processes merge to become

Answer 10

Primary palate

Question 11

During the 6th/7th week of development, medial projection of maxillary processes forms the

Answer 11

Secondary palate

Question 12

At what week does the tongue descend

Answer 12

6th/7th

Question 13

What week do the palatal shelves rotate to horizontal

Answer 13

6th-7th

Question 14

The anterior aspect of the palatal shelves fuse during

Answer 14

8th week

Question 15

During the eighth week, the palatal shelves fuse with

Answer 15

Primary palate and nasal septum

Question 16

When is the fusion of the palate complete

Answer 16

12th week

Question 17

Overall the highly sensitive period for cleft lip occurs in

Answer 17

5th-6th week

Question 18

Teratogens could affect the upper lip during

Answer 18

6th-8th week

Question 19

Defect of a cleft lip

Answer 19

Defective fusion of medial nasal process with maxillary process

Question 20

Defect of cleft palate

Answer 20

Defective fusion of palatal shelves

Question 21

Which combination of cleft lip and cleft palate has the highest incidence

Answer 21

Cleft lip with cleft palate

Question 22

What percentage of oral facial cliffs is a combination of CL + CP

Answer 22

45%

Question 23

Cleft palate only is what percentage of cases

Answer 23

30%

Question 24

Cleft lip only is what percentage of cases

Answer 24

25%

Question 25

True/false: the majority of cleft lips are Unilateral and appear on the right side

Answer 25

False. The majority of cleft lips are unilateral and appear on the left side

Question 26

Percentage of cleft lips will appear on the left side

Answer 26

70%

Question 27

Cleft lip involving maxillary alveolus will likely occur between

Answer 27

Lateral incisor and canine

Question 28

A cleft that extends into nostril is

Answer 28

Complete cleft lip

Question 29

A cleft that has no nostril involvement is

Answer 29

Incomplete cleft lip

Question 30

True false: a cleft lip involving the alveolus could result in missing teeth or supernumerary teeth

Answer 30

True

Question 31

Cleft palates that have no hard palate clefting

Answer 31

Bifid uvula and submucus palatal cleft

Question 32

Minimal manifestation of cleft palate

Answer 32

Bifid uvula

Question 33

Submucous palatal cleft involves

Answer 33

Soft tissue defect, musculature of soft palate, notching of palatal bone. No true clefting

Question 34

Which syndrome has the following clinical features: mandibular micrognathia, cleft palate, glossoptosis

Answer 34

Pierre robin sequence

Question 35

Etiology of Pierre Robin sequence

Answer 35

Constraint of mandibular growth in utero by oligohydroamino (amniotic fluid). Results in failure of tongue to descend and the posterior displacement of tongue.

Question 36

Primary surgeries for oral facial clefts

Answer 36

Primary lip closure, pallet over repair, molding/expansion of Maxilla, bone grafting. Secondary: soft tissue repair.

Question 37

If a first-degree relative was affected by an oral facial cleft, the risk is

Answer 37

10-20%

Question 38

Without a first-degree relative affected, the risk is

Answer 38

3-5%

Question 39

Truffles: patients with a syndromic cleft have a higher chance than non syndromic of their siblings or offsprings also having one

Answer 39

True

Question 40

Lateral facial cleft defect

Answer 40

Defective fusion of maxillary and mandibular processes

Question 41

Lateral Facial cleft can cause macrostomia when

Answer 41

Left from Commissure to ear

Question 42

Oblique facial cleft etiology 

Answer 42

Defective fusion of lateral nasal process with maxillary process

Question 43

Oblique facial cleft clinical features

Answer 43

Cleft from upper lip to eye

Question 44

Median facial cleft etiology

Answer 44

Defective fusion of medial nasal processes

Question 45

Median facial cleft clinical feature

Answer 45

Cleft of upper lip, midline

Question 46

Commissural lip pits etiology

Answer 46

Unknown

Question 47

Site of commissural lip pits

Answer 47

Corners of mouth. Unilateral or bilateral

Question 48

Commissural lip pits size

Answer 48

Small invagination (blind pit) 1 to 4mm in depth

Question 49

Congenital lip pits is also known as

Answer 49

Paramedian lip pits

Question 50

Which lip pit is congenital only

Answer 50

Paramedian

Question 51

Site of paramedian lip pit

Answer 51

Either side of midline lower lip. Bilateral

Question 52

Depth of paramedian lip pit

Answer 52

1.5mm

Question 53

Paramedian lip pit could be a component of what syndrome

Answer 53

Van der woude syndrome, popliteal Pterygium syndrome, kabuki syndrome

Question 54

van der woude syndrome etiology

Answer 54

Mutation in gene that encodes for interferon regulatory factor 6. Autosomal dominant with variable expressivity

Question 55

Clinical features of Van der Woude syndrome

Answer 55

Paramedian lip pit, cleft lip and/or cleft palette

Question 56

Most common form of syndromic clefting

Answer 56

Van Der woude syndrome

Question 57

True false: double lip etiology is exclusively acquired

Answer 57

False. Congenital or acquired

Question 58

Double lip occurs most frequently on

Answer 58

Upper lip

Question 59

Double lip shows a double lip when

Answer 59

Smiling

Question 60

Double lip is one of the clinical features of

Answer 60

Ascher syndrome

Question 61

Clinical features of Ascher syndrome

Answer 61

Double lip, blepharochalasis, nontoxic thyroid enlargement

Question 62

Blepharochalasis is ____ and is a clinic feature of ____

Answer 62

Adema of upper eyelid and sagging of outer canthus. Ascher syndrome

Question 63

Double lip is caused by the abundance of

Answer 63

Minor salivary glands

Question 64

Blepharochalasis is hyperplasia of

Answer 64

Lacrimal glands

Question 65

Fordyce granules are

Answer 65

Ectopic sebaceous glands

Question 66

Fordyce granules are present in

Answer 66

>80% of population

Question 67

Most common site for Fordyce granules

Answer 67

Buccal or labial mucosa

Question 68

Clinical features of Fordyce granules

Answer 68

Asymptomatic, yellow to yellow white, macules or papules, few to hundreds

Question 69

Which developmental defects only appear in certain facial expressions

Answer 69

Double lip (only when smiling), leukoedema (disappears when stretched)

Question 70

In what way does Fordyce granules have abnormal sebaceous glands

Answer 70

They have normal sebaceous glands

Question 71

Sebaceous cells are

Answer 71

Polygonal cells with foamy cytoplasm central nuclei

Question 72

Leukoedema may be more prominent in

Answer 72

Smokers, African-Americans

Question 73

Site of Leukoedema

Answer 73

Buckle mucosa, label mucosa, bilateral

Question 74

Clinical features of leukoedema

Answer 74

Gray white, milky, folded whitish streaks. Disappears when stretched

Question 75

Histology of leukoedema

Answer 75

Hyperparakeratosis, acanthosis, intercellular edema, intracellular edema

Question 76

Most common causes of macroglossia

Answer 76

Vascular malformation, muscular hypertrophy

Question 77

Syndrome that has macroglossia as a clinical feature

Answer 77

Beckwith Weidman syndrome

Question 78

Clinical features of Beckwith Weidman syndrome

Answer 78

Omphalocele, visceromegaly, gigantism, increased risk for childhood tumors

Question 79

Omphalocele is ____ and is seen in ____

Answer 79

Intestinal protrusion through abdominal wall, Beckwith Weidman syndrome

Question 80

Beckwith Weidman histology of tongue

Answer 80

Muscular hyperplasia

Question 81

Down syndrome can have the clinical feature of ____ with histology showing

Answer 81

Macroglossia, normal structure

Question 82

Lymphangioma and hemangioma both could result in the clinical feature of ____ showing the histological features of _____

Answer 82

Macroglossia, proliferation of lymphovascular vessels

Question 83

Neurofibromatosis has a clinical feature of ____ with a histology showing ____

Answer 83

Macroglossia, neurofibroma

Question 84

Emmaloid doses has a clinical feature of ____ showing histology of ____

Answer 84

Macroglossia, amyloid deposits

Question 85

Ankyloglossia is also known as

Answer 85

Tongue tie

Question 86

Theology of ankyloglossia

Answer 86

Short thick lingual frenum

Question 87

Incidence rate of ankyloglossia

Answer 87

1.7 to 10.7% of neonates

Question 88

Ankyloglossia can cause

Answer 88

Clefting of tongue

Question 89

Surgical corrections for ankyloglossia

Answer 89

Frenotomy, frenuloplasty