Developmental Defects Flashcards

1
Q

When does central face development begin?

A

At week 4 of development

Upper lip formation at week 6-7

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2
Q

The merger of the medial nasal process forms what?

A

Primary Palate

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3
Q

The merger of the maxillary processes forms what?

A

Secondary palate

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4
Q

A defective fusion of the medial nasal process with the maxillary process results in what?

A

Cleft lip

*80% are unilateral. Treat with the rule of 10 (10 lbs, 10 weeks, 10 gm %HM)

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5
Q

Failure of the palatal shelves to fuse results in what?

A

Cleft palate

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6
Q

What is the minimal manifestation of cleft palate?

A

Bifid uvula

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7
Q

______% of Cleft palate are CL and CP, _____% are only CP and ____% are CL only

A

45
30
25

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8
Q

What three things make up Pierre Robin sequence?

A

1-Cleft Palate
2-Mandibular Micrognathia
3-Glossoptosis (downward displacement)

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9
Q

Lack of fusion of the maxillary and mandibular processes results in what?

A

Lateral facial cleft

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10
Q

Failure of fusion of the lateral nasal process with the maxillary process results in what?

A

Oblique facial cleft

*upper lip to the eye, almost always with CP

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11
Q

Failure of fusion of the medial nasal process results in?

A

Median cleft of upper lip

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12
Q

What is the prevalence of orofacial clefts across races?

A

Native americans 1:250
Asians 1:300
Caucasian 1:700
African Americans 1:1500

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13
Q

When the surface is intact but there is a defect in the underlying musculature of the soft palate, what is it called?

A

Submucous palatal cleft

*appears as a bluish midline discoloration

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14
Q

Mucosal invaginations that occur at the corners of the mouth on the vermillion border that are not associated with clefts are called what?

A

Commissural Lip pits

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15
Q

Congenital invaginations of the lower lip, usually bilaterally located are called what?

A

Paramedian lip pits

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16
Q

What are 4 things to know about Van der Woude syndrome?

A

1-Greatest significance of paramedic lip pits
2-Autosomal dominant
3-CL + CP
4-Most common form of syndromic clefting

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17
Q

A redundant fold of tissue on mucosal side of lip is typical of ascher syndrome and called what?

A

Double Lip

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18
Q

What are the 3 characteristics of Ascher syndrome?

A

1-Double lip
2-Blepharochalasis (eyelid edema)
3-Nontoxic Thyroid enlargement

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19
Q

Ectopic sebaceous glands found in 80 % of the population appearing as yellow papular lesions on the buccal mucosa are called what?

A

Fordyce Granules

*More common in adults

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20
Q

Diffuse, gray-white, milky opalescent lesions found bilaterally on buccal mucosa that do not rub off and disappear when the cheek is stretched are is called what?

A

Leukoedema

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21
Q

Abnormally small tongue is usually syndromic and called what?

A

Microglossia

*often associated with limb features such as hypodactylia and hypomelia

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22
Q

Abnormally large tongue is called what and is most frequently cause by what 3 things?

A

Macroglossia
1-Vascular malformations
2-Muscular hypertrophy
3-Other (down syndrome, tumors etc.)

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23
Q

What feature is characteristic of Beckwith-Wiedmann syndrome?

A

Macroglossia

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24
Q

A short, thick lingual frenum resulting in limited tongue movement is called what?

A

Ankyloglossia

*more common in men

25
Q

Abnormal descending of the primitive thyroid gland can result in a mass found on the foramen cecum and is called what?

A

Lingual Thyroid

*Diagnose via thyroid scan with iodine isotopes of technetium-99m, CT and or MRI

26
Q

Also known as scrotal tongue, what is it called when the tongue exhibits multiple grooves/furrows and may contribute to mild burning sensation?

A

Fissured Tongue

*Strong association with geographic tongue

27
Q

AKA benign migratory glossitis, what condition presents as inflammation on the dorsal and/or lateral tongue?

A

Geographic tongue

*Erythema migrans when in a place other than the tongue. May burn or hurt

28
Q

Accumulation of keratin on the filiform papilla of dorsal tongue is called what?

A

Hairy Tongue

*may cause halitosis and stain depending on diet

29
Q

Superficial dilated veins, most commonly on the sublingual varix presenting as blue-purple papular nodules are called what?

A

Varicosities

*if calcified=phlebolith

30
Q

An arterial branch extending into the superficial submucosa without reduction in diameter in which you can feel at pulse is called what?

A

Caliber persistent artery

31
Q

Mandibular deviation toward the affected side can be due to what hyperplasia?

A

Coronoid Hyperplasia

32
Q

Mandibular deviation toward the opposite side is likely due to what hyperplasia?

A

Condylar Hyperplasia

33
Q

Loclaized bony protuberances are called what?

A

Exostoses

*Torus palatinus, Torus mandibularis or Buccal, Palatal, Solitary exostoses

34
Q

Where does a torus palatinus occur?

A

Midline of the hard palate

35
Q

AKA lingual mandibular salivary gland depression, what well circumscribed radiolucency is found below the mandibular canal?

A

Stafne defect

*Histologically normal salivary gland tissue

36
Q

What is the definition of a cyst?

A

Pathologic cavity lined by epithelium

*typically fluid filled

37
Q

Keratin pearls found along the median palatal raphe are called what?

A

Epstein’s Pearls or Palatal Cysts of the newborn

38
Q

When Keratin pearls are found scattered all over the hard palate, usually near junction or soft palate, what are they called?

A

Bohn’s nodules

39
Q

A swelling of the upper lip, lateral to the midline, resulting in elevation of the ala of the nose is called what?

A

Nasolabial Cyst

*always in soft tissue, often lined with respiratory epithelium

40
Q

A globulomaxillary radiolucency near the maxillary canine/lateral incisor is most likely what?

A

Periapical cyst

*second most common is periapical granuloma

41
Q

What is the most common non-odontogenic cyst of the oral cavity?

A

Nasopalatine duct cyst (incisive canal cyst)

*Lined with respiratory epithelium

42
Q

What is the upper limit of the normal size for the incisive foramen?

A

6 mm (less than 6 is normal, 6 or larger is likely nasopalatine duct cyst)

43
Q

What is it called when the nasopalatine duct cyst is in the soft tissue only, without bony involvement?

A

Cyst of the incisive papilla

44
Q

What is the most common cyst of the skin?

A

Epidermoid cyst

45
Q

What are 4 characteristics of an epidermoid cyst?

A

1-Nodular
2-Fluctuant (rebounds)
3-Subcutaneous
4-White or yellow

46
Q

An epidermoid cyst found on the scalp, derived from a hair follicle, is called what?

A

Pilar cyst

47
Q

A cyst lined by epidermis like epithelium and contains adnexal structures (hair follicle, sebaceous gland, pilar muscle) are called what?

A

Dermoid cyst

*can occur at midline as a submucosal fluctuant swelling on the floor of mouth

48
Q

Commonly found on the midline, what cyst develops from epithelial remnants of the thyroglossal tract?

A

Thyroglossal duct cyst

*usually inferior to the hyoid bone

49
Q

What is one of the most common yellow things, will have lymphoid tissue in the biopsy, and is found in waldeyers ring (tonsils) and FOM?

A

Lymphoepithelial cyst

*most commonly found in FOM

50
Q

What forms in the upper lateral neck along anterior border of the SCM as a soft, fluctuant mass?

A

Branchial Cleft cyst/Cervical lymphoepithelial cyst

*If bilateral, HIV testing is required

51
Q

Asymmetric overgrowth of one of more body parts is called?

A

Hemihyperplasia

52
Q

Having many features similar to scleroderma, what may be caused by boreal spp infection (lyme disease)?

A

Progressive hemifacial atrophy

53
Q

Painless unilateral enlargement of the maxillary bone, along with overgrowth of overlying gingiva is called what?

A

Segmental odontomaxillary dysplasia

*one or both maxillary premolars are frequently missing

54
Q

What syndrome is characterized by craniosynostosis, cloverleaf skull?

A

Crouzon syndrome (craniofacial dysostosis)

55
Q

What does a skull x-ray of a crouzon syndrome patient look like?

A

Beaten-metal

56
Q

What mutation causes Crouzon syndrome and apert syndrome?

A

fibroblast growth factor receptor 2 (FGFR-2) on chromosome 10q26

57
Q

What are 4 characteristics of Apert syndrome?

A

1-Ocular proptosis
2-Hypertelorism (wide spaced eyes)
3-Downward slant lateral palpebral fissures
4-Syndactyly of 2, 3, 4 digits of hands and feet

58
Q

A notch on the outer portion of the lower eyelid called coloboma is characteristic of what syndrome?

A

Treacher-collins syndrome or mandibulofacial dysostosis

*also have hypo plastic zygoma, and underdeveloped mandible