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
Complete Cleft Lip
cleft extends to floor of the nose | May include a cleft of the alveolar ridge
26
Unilateral Cleft LIp
cleft on one-side
27
Bilateral Cleft LIp
cleft on both sides of the upper lip
28
Submucous Cleft lip
outer layers of skin are intact, but the orbicularis oris muscle is incomplete underneath Looks like a repaired cleft lip
29
Complete Cleft Palate
cleft is through uvula, all of the soft and hard palate and alveolus
30
Unilateral Cleft Palate
one-side of alveolus
31
Bilateral Cleft Palate
both sides of the alveolus
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Incomplete Cleft Palate
cleft is through the uvula, soft and hard palate
33
Unilateral/Bilateral Cleft Palate
Unilateral – one-side of palate Bilateral – both sides of palate Soft palate only – called “central” cleft
34
Submucous Cleft Palate
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
Occult Submucous Cleft Palate
only visible from the nasal cavity
36
Cleft Lip Repair
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
Cleft Palate Repair
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
Pharyngeal flap
surgical procedure to improve speech production by reducing hypernasal resonance does not repair a cleft allows for velopharyngeal closure
39
Syndrome
A pattern of 2 or more abnormities that are related or have a known or suspected cause
40
Sequence
2 or more anomalies that occur together, but the second anomaly is caused by the first
41
Etiologies of Clefts, Sequences, & Syndromes
- 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
Pierre Robin Sequence
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
Glossoptosis
Back of tongue rests in airway
44
Down Syndrome
Chromosomal abnormality Extra chromosome 21 Cleft lip and/or palate can occur in some cases
45
Velo-Cardio-Facial syndrome
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
Velo-Cardio-Facial Syndrome
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
Van der Woude Syndrome:
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
Treacher Collins Syndrome:
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
Stickler Syndrome
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
Fetal Alcohol Syndrome
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
Non-Syndromic Clefts
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
Syndromic Clefts
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
Speech Concerns
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
Obligatory Speech Errors
The result of structural deficits Nasal air emission, consonants, and short utterances Can only be changed by repairing the underlying structural deficit
55
Compensatory Speech Errors
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