Cleft 2 Flashcards

1
Q

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A
  • Abnormal opening or fissure in an anatomical structure
  • Usually a congenital malformation due to abnormal fusion of parts during embryological development
  • Can be due to ablative surgery
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2
Q

What Is a Cleft?

Cont’d

A

Clefts:
Vary in type and severity
Follow embryological suture lines (along raphe?)

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3
Q

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A
  • Embryological development is dependent on formation of neural crest cells in embryo.
  • Neural crest cells are found between the neural tube and the epidermis of an embryo.
  • Cells migrate to form skull and face. (should happen 5-6 wks - if not, wont happen ever?)

-(incisive foramen - birthplace - clefting starts away from it?)

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4
Q

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A
  • Genetic or environmental factors delay cell migration and palatal shelf movement.
  • This can cause the embryo to miss the period of epithelial cell fusion.
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5
Q

Causes of Clefts

A

Multifactorial Inheritance

  • Endogenous (internal) factors
  • –Chromosomal disorders
  • –Genetic disorders
  • Exogenous (external) factors (e.g., teratogens)
  • –Drugs: phenytoin (Dilantin), valium, and corticosteroids
  • –Viruses (rubella, influenza)
  • –Teratogens (smoking, lead)
  • –Nutritional deficiencies or maternal obesity
  • –Mechanical interference
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6
Q

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A
  • Lip (primary palate) begins at 7 weeks.
  • Palate (secondary palate) begins at 9 weeks.
  • Development is independent. - can have one OR the other
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7
Q

Embryological Development

A
  • Embryological closure begins at incisive foramen and “zips” …
  • –forward to form the alveolar ridge and then lip
  • –backward to form the hard palate and velum
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8
Q

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A
  • Clefting severity occurs from…
  • –the lip in to the incisive foramen
  • –the uvula in to the incisive foramen
  • Right side of lip may close first.
  • Oral surface of velum may close first.
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9
Q
Classification of Clefts - primary
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A

Primary Palate (Cleft Lip)

  • Fuses around 7 weeks of gestation
  • Anterior to incisive foramen
  • Includes lip and alveolus
  • Clefts include:
  • –Complete or incomplete
  • –Unilateral or bilateral
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10
Q
Classification of Clefts - secondary
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A

Secondary Palate (Cleft Palate)

  • Fuses around 9 weeks of gestation
  • Posterior to incisive foramen
  • Includes hard palate and velum
  • Clefts include:
  • –Complete or incomplete
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11
Q
Clefts of the Primary Palate:Types and Severity
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A

-Unilateral incomplete cleft lip
-Unilateral complete cleft lip
-Bilateral incomplete cleft lip
-Bilateral complete cleft lip
-Simonart’s Band—band of soft tissue that bridges a cleft of the lip
May be due to amniotic bands
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12
Q

Clefts of the Primary Palate: Effects on Structure and Function

A
  • Structure
  • –Cleft lip and orbicularis oris
  • –Wide, flat nose with spreading nasal ala
  • –Short columella
  • –Abnormal dentition
  • Function
  • –Specific articulation errors
  • –Resonance affected
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13
Q
Clefts of the Secondary Palate:Types and Severity
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A
  • cleft palate
  • Cleft palate with Pierre Robin sequence
  • Bilateral complete cleft lip and palate
  • Palatal (oronasal) fistula
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14
Q

Clefts of the Secondary Palate:Effects on Structure and Function

A
  • Structure
  • –Absent velar aponeurosis
  • –Altered insertion of the levator velar muscles (cleft muscles of Veau)
  • –Abnormalities in nasal septum
  • Function
  • –Velopharyngeal insufficiency (speech and resonance affected)
  • –Feeding problems and nasal regurgitation
  • –Eustachian tube malfunction
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15
Q

-

A
  • Overt—visible from the oral surface

- Occult (hidden)—normal on the oral surface, abnormal on the nasal surface

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16
Q
Submucous Cleft Palate:Types and Severity
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A

Classic stigmata of an overt submucous cleft:

  • Bifid or hypoplastic (small and underdeveloped) uvula
  • Zona pellucida—thin, bluish area in middle of the velum
  • Notch in the hard palate where the posterior nasal spine should be, if the submucous cleft extends that far
  • Muscles often affected in the same way as a complete cleft palate
17
Q

Submucous Cleft Palate:Types and Severity

A
  • Affects interdigitation of the levator veli palatini muscle
  • Levator muscle inserts on the posterior border of hard palate rather than in the middle of the velum
18
Q

Submucous Cleft: Effects on Structure and Function

A

Depends on the extent

  • May have virtually no effect
  • May have the same effects as an overt cleft palate as follows:
  • –Structure
  • Altered insertion of the levator velar muscles (cleft muscles of Veau)
  • –Function
  • Velopharyngeal insufficiency (speech and resonance affected)
  • Feeding problems and nasal regurgitation
  • Eustachian tube malfunction
19
Q

Clefts of the Primary Palate:Types and Severity

A

Midline cleft with holoprosencephaly

-Holoprosencephaly—failure of the prosencephalon (the embryonic forebrain) to divide into double lobes of the cerebral hemispheres. The result is a single-lobed brain structure.

20
Q

Facial Clefts: Type and Severity

  • causes
  • two types
A

-Can be caused by:
Genetic defects
Amniotic bands

-Two types of facial clefts:
Oblique
Midline (median)

21
Q

Facial Clefts: Effects on Structure and Function

A

-Structural abnormalities on the outside the head (face or skull) usually suggest abnormalities on the inside of the head (face or skull).

-Abnormalities on the inside the head (face or skull) usually affect function of the following:
cognition
language
speech
resonance
hearing
feeding/swallowing
22
Q

Prevalence of clefts

A

Cleft lip occurs twice as often in males (with/without cleft palate).
Cleft palate occurs twice as often in females.

23
Q

prevalence of clefts

A
  • Submucous cleft
  • -Essentially unknown
  • -May not cause problems with speech
  • -May become symptomatic after adenoidectomy
  • Facial cleft
  • -Very rare
24
Q

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A
  • Cheiloplasty to close the lip
  • Palatoplasty to close the palate
  • Usually pharyngeal flap or sphincter pharyngoplasty if there is velopharyngeal insufficiency affecting speech
25
Q

Clefts are a common birth defect.

Proper intervention is important.

A

Clefts are a common birth defect.

Proper intervention is important.

26
Q

velopharyngeal insufficiency

A

closure not achieved due to anatomy

27
Q

velopharyngeal incompetency

A

anatomy is fine but physiology is messed up - functional problem

28
Q

velopharyngeal mislearning

A

learn incorrect way of producing velar sounds - anatomy and physiology are fine