Cleft Lip & Palate Flashcards

1
Q

Cleft

A

an abnormal opening in an anatomic structure

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2
Q

Cleft Lip/Palate

A

Occurs in utero and is a disruption to embryological development

Clefts are due to delayed migration of neural crest cells

Cleft lip – opening in the lip
Cleft palate – opening in the palate

Individuals with cleft palate without cleft lip are more likely to have other malformations

Cleft lip and/or cleft palate is associated with hundreds of syndromes

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3
Q

Craniofacial anomaly

A

– structural abnormality of the cranium (skull) and/or face

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4
Q

Embryonic Development of the Lip and Alveolus

A

– development begins at 6-7 weeks gestation

Development begins at the incisive foramen and moves forward to the alveolus closing along the incisive suture lines

Next, the base of the nose and then the upper lip fuse

The upper lip consists of two segments which fuse together to form the philtrum and philtrum lines

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5
Q

Embryonic Development of the Hard and Soft Palate

A

Palate development begins at 8-9 weeks gestation

At 7-8 weeks gestation the tongue begins to move down from the nasal cavity
At this time, the palate starts to close

Palate closure begins at the incisive foramen and moves posteriorly (back) along the median palatine suture line

Uvula and velum close at 12 weeks gestation

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6
Q

Oral Anatomy

A

Hard palate – separates the nasal and oral cavities

Formed by – the palatine process of the maxillary bone & horizontal part of the palatine bone

Anatomical landmarks:

  • Incisive foramen
  • Posterior nasal spine
  • Vomer
  • Incisive suture lines
  • Alveolus
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7
Q

Soft Palate Anatomy

A

At rest sits against base of tongue

Has an oral surface and a nasal surface

Uvula has no known function

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8
Q

Pharynx

A

Area between the nasal cavity and esophagus

Pharyngeal wall – have a posterior and lateral portion

  • Posterior pharyngeal wall (PPW)- posterior (back) portion of throat
  • Lateral pharyngeal wall (LPW) – side of throat

Divided into:

  • Nasopharynx– nasal cavity to velum
  • Oropharynx– oral cavity to epiglottis
  • Hypopharynx– epiglottis to esophagus
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9
Q

Eustachian Tube

A

Connects middle ear to pharynx

Closed at rest

Opens during yawning and swallowing

Function:
pressure regulation (air and fluid)
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10
Q

Levator veli palatini

A

pulls the velum up and back

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11
Q

Palatopharyngeus

A

pulls the lateral pharyngeal walls upward and medially

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12
Q

Superior constrictor

A

constricts the pharyngeal walls against the velum

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13
Q

Tensor veli palatini

A

opens the Eustachian tube

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14
Q

Resonance

A

vibration of sound energy throughout cavities and tracts

Can be disrupted with cleft palate

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15
Q

Speech Valves

A

3 valves contribute to the acoustic properties of voice; These valves can change in shape and size

Glottis – space between vocal folds
Vocal tract – glottis through pharynx to oral cavity

Velopharyngeal closure – separates nasal cavity from vocal tract

Constriction of the lips and tongue - articulation

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16
Q

Velopharyngeal valve

A

directs and redirects sound energy

All vowels and oral consonants produced with velopharyngeal closure

4 types of closure patterns – all types include PPW, LPW, and velar movement

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17
Q

Coronal Velopharyngeal Valve Closure Pattern

A

most common pattern

Velum moves up and back (like a bending knee), touching the PPW

PPW may move forward

LPWs move medially to touch the velum (movement is minimal)

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18
Q

Circular Velopharyngeal Valve Closure Pattern

A

second most common pattern

All velopharyngeal structures move

Velum moves posterior, lateral walls move medially, and PPW moves anterior

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19
Q

Circular with Passavant’s Ridge Velopharyngeal Valve Closure Pattern

A

Passavant’s ridge occurs in typical and atypical speakers

Superior constrictor muscle constricts creating a ridge on the PPW

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20
Q

Sagittal Velopharyngeal Valve Closure Pattern

A

least common pattern

Lateral walls move medially to meet midline behind the velum

Minimal velar movement

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21
Q

Velar Movement Summary

A

Velum moves up and back to close velopharyngeal valve during speech
-Also when you sing, vomit, whistle, gag, and suck

Velum bends like a knee but also stretches to reach PPW

Lateral walls move medially to touch velum

PPW moves anterior to meet velum

All movements vary by person

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22
Q

Velopharyngeal insufficiency

A

velum is too short to close against the PPW

Results in hypernasal resonance because of a STRUCTURAL deficit

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23
Q

Velopharyngeal incompetence

A

poor movement of the velopharyngeal structures due to physiological deficits

Results in hypernasal resonance because structures cannot MOVE well

24
Q

Incomplete Cleft Lip

A

cleft of the upper lip that doesn’t extend to the floor of the nose

25
Q

Complete Cleft Lip

A

cleft extends to floor of the nose

May include a cleft of the alveolar ridge

26
Q

Unilateral Cleft LIp

A

cleft on one-side

27
Q

Bilateral Cleft LIp

A

cleft on both sides of the upper lip

28
Q

Submucous Cleft lip

A

outer layers of skin are intact, but the orbicularis oris muscle is incomplete underneath

Looks like a repaired cleft lip

29
Q

Complete Cleft Palate

A

cleft is through uvula, all of the soft and hard palate and alveolus

30
Q

Unilateral Cleft Palate

A

one-side of alveolus

31
Q

Bilateral Cleft Palate

A

both sides of the alveolus

32
Q

Incomplete Cleft Palate

A

cleft is through the uvula, soft and hard palate

33
Q

Unilateral/Bilateral Cleft Palate

A

Unilateral – one-side of palate

Bilateral – both sides of palate

Soft palate only – called “central” cleft

34
Q

Submucous Cleft Palate

A

palate is covered in tissue/mucosa, but muscles underneath are incomplete

Has 3 features:

  • Bifid uvula
  • Zone pellucida – located in the central portion of the velum
  • A notch at posterior end of hard palate
35
Q

Occult Submucous Cleft Palate

A

only visible from the nasal cavity

36
Q

Cleft Lip Repair

A

usually completed by 3 months of age

Repair schedule follows a rule of 10:

  • Infant must weigh 10 lbs.
  • Be at least 10 weeks old
  • Have a hemoglobin of at least 10 grams
  • Have a white cell count no higher than 10,000
37
Q

Cleft Palate Repair

A

usually performed between 12-18 months of age

Repair can be done in 1-2 steps (hard & soft palate)

Early repair (12-18 months) associated with better speech development

38
Q

Pharyngeal flap

A

surgical procedure to improve speech production by reducing hypernasal resonance

does not repair a cleft

allows for velopharyngeal closure

39
Q

Syndrome

A

A pattern of 2 or more abnormities that are related or have a known or suspected cause

40
Q

Sequence

A

2 or more anomalies that occur together, but the second anomaly is caused by the first

41
Q

Etiologies of Clefts, Sequences, & Syndromes

A
  • Chromosomal abnormalities
  • Genetic abnormalities
  • Teratogenic agents
  • Mechanical influences – in utero crowding

Some clefts are caused by a single factor; others result from a combination of factors

42
Q

Pierre Robin Sequence

A

Presents in isolation or with a syndrome

A U-shaped cleft palate may be present

Micrognathia

Glossoptosis:

  • This can cause significant breathing problems.
  • The infant may need to be closely monitored and/or require tracheostomy
43
Q

Glossoptosis

A

Back of tongue rests in airway

44
Q

Down Syndrome

A

Chromosomal abnormality

Extra chromosome 21

Cleft lip and/or palate can occur in some cases

45
Q

Velo-Cardio-Facial syndrome

A

Genetic Abnormality

Most common syndrome associated with cleft palate

Velar deficits- incomplete cleft palate or submucous cleft palate
Cardio deficits – congenital heart defects
Facial- dysmorphic facial features including microcephaly, narrow palpebral fissures, wide nasal root, bulbous nose, micrognathia, & minor auricular anomalies

46
Q

Velo-Cardio-Facial Syndrome

A

Genetic Abnormality

Most common syndrome associated with cleft palate

  • Velar deficits- incomplete cleft palate or submucous cleft palate
  • Cardio deficits – congenital heart defects
  • Facial- dysmorphic facial features including microcephaly, narrow palpebral fissures, wide nasal root, bulbous nose, micrognathia, & minor auricular anomalies

Can present with Pierre Robin Sequence

Speech & language characteristics:

  • Compensatory artic errors
  • Hypernasal resonance
  • Hoarse voice & high pitch
  • Language delay
  • Learning disabilities
  • Psychiatric disorder
47
Q

Van der Woude Syndrome:

A

Genetic Abnormality

Facial features:

  • Variable cleft lip and/or palate
  • Congenitally absent premolars
  • Lower lip pits

Speech and language characteristics:

  • Hypernasal resonance
  • Compensatory artic errors
48
Q

Treacher Collins Syndrome:

A

Genetic Abnormality

Facial – hypoplastic zygomatic arches, macrostonia, and narrow vocal tract

Eyes - downward slanting palpebral fissures and lower eyelid fissures

Ears – microtia

Pierre Robin Sequence may co-occur

Speech, Hearing, and Language characteristics:

  • Conductive hearing loss
  • Speech disorders
  • Resonance issues
49
Q

Stickler Syndrome

A

Facial- incomplete cleft palate or submucous cleft palate

Eyes – nearsightedness

Joints- early onset arthritis
Scoliosis

Pierre Robin Sequence- commonly co-occurs

Speech, hearing, and language characteristics:

  • Sensorineural hearing loss
  • Hypernasal resonance
  • Compensatory artic errors
50
Q

Fetal Alcohol Syndrome

A

Result of Teratogen Exposure in utero

Facial- microcephaly, short palpebral fissures, short nose, flat philtrum, and thin upper lip

Can present with cleft lip and/or palate

Cardio- congenital heart defects

Pierre Robin Sequence- commonly co-occurs

Speech, hearing, and language characteristics:

  • Chronic ear infections
  • Compensatory artic errors
  • Hypernasal resonance
  • Learning disabilities
  • Other deficits: prenatal and postnatal growth deficits, intellectual disability, and behavior problems
51
Q

Non-Syndromic Clefts

A

Usually exhibit typical cognitive and language abilities

May demonstrate language delay early in life, but these children typically catch-up to peers over time

Hearing deficits must be addressed

52
Q

Syndromic Clefts

A

Children with syndromes most at risk for cognitive and language development issues

Isolated cleft palate is more commonly associated with cognitive and/or language disorders

53
Q

Speech Concerns

A

Children with isolated cleft lip and/or palate typically develop normal articulation and resonance after repair

Children that do NOT receive team-based care at infancy are more likely to demonstrate long-term speech disorders

Speech sound/articulation errors may be related to source:

  • Developmental delay
  • Hearing loss
  • Malocclusion
  • Palatal fistula
  • Velopharyngeal insufficiency or incompetence
54
Q

Obligatory Speech Errors

A

The result of structural deficits

Nasal air emission, consonants, and short utterances

Can only be changed by repairing the underlying structural deficit

55
Q

Compensatory Speech Errors

A

Learned errors in response to reduced intraoral air pressure for speech

Maintain manner, but place of articulation is altered

Examples: glottal stops/stop consonants, pharyngeal fricative, and nasal snort for /s, z/

These errors can be modified

Speech evaluation should include production of nasal and nasal-free words and sentences