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Craniofacial Anomalies > Week 3 > Flashcards

Week 3 Flashcards

VPI & VPI Surgery

1
Q

Optiz-G Syndrome

A
  • Autosomal dominant form - X-linked form

- Wide set eyes - may need surgery - laryngeal cleft, etc.

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2
Q

Turner Syndrome

A

Non-inherited chromosomal syndrome - affects females - One x is partially/completely missing - prevents body growth & sexual development -

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3
Q

Anatomy of the anterior 2/3 of the hard palate

A

Premaxilla, palatine processes of maxilla, palatine bones

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4
Q

Anatomy of the posterior 1/3 of the palate (soft palate)

A

Muscle, soft tissue, mucosa

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5
Q

Veolpharyngeal port

A

Space surround the velum, lateral & posterior pharyngeal walls

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6
Q

Muscles of the soft palate: Tensor Veli Palatini Origin

A

Medial pterygoid plate of the sphenoid & lateral Eustachian tube - hook around hamulus of pterygoid plate

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7
Q

Muscles of the soft palate: Tensor Veli Palatini Insertion

A

Ant. to post. - hard palate & opp. aponeurosis in the lateral palate

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8
Q

Muscles of the soft palate: Tensor Veli Palatini Innervation

A

CN V (trigeminal)

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9
Q

Muscles of the soft palate: Tensor Veli Palatini Function

A

Hinge between hard and soft palate - opens Eustachian tube to improve the ventilation & drainage of the auditory tubes

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10
Q

Muscles of the soft palate: Levator Veli Palatini Origin

A

Lower temporal bone and medial Eustachian tube

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11
Q

Muscles of the soft palate: Levator Veli Palatini Insertion

A

Palatal aponeurosis & opposite fibers

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12
Q

Muscles of the soft palate: Levator Veli Palatini Innervation

A

Pharyngeal Plexus (CN IX, X, and XI)

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13
Q

Muscles of the soft palate: Levator Veli Palatini Function

A

Sling action (up & back) - interacts w/faucial pillars

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14
Q

Muscles of the soft palate: Musculous Uvulae Origin

A

Near midline of palatal aponeurosis and travels over levator muscle

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15
Q

Muscles of the soft palate: Musculous Uvulae Insertion

A

Mucosa of uvula

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16
Q

Muscles of the soft palate: Musculous Uvulae Innervation

A

Pharyngeal Plexus (CN IX, X, and XI)

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17
Q

Muscles of the soft palate: Musculous Uvulae Function

A

Adds bulk & length to the velum (extra tissue that helps to close the velopharyngeal port)

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18
Q

Muscles of the soft palate: Palatopharyngeus Origin

A

Lateral pharyngeal wall

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19
Q

Muscles of the soft palate: Palatopharyngeus Insertion

A

Midline of velum

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20
Q

Muscles of the soft palate: Palatopharyngeus Innervation

A

Pharyngeal plexus (CN IX, X, and XI)

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21
Q

Muscles of the soft palate: Palatopharyngeus Function

A

Narrowing of VP port - pulling down on the velum from the lateral pharyngeal wall

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22
Q

Muscles of the soft palate: Palatopharyngeus AKA

A

Posterior faucial pillar

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23
Q

What are the FIVE muscles of the soft palate?

A 
Tensor veli palatini
Levator veli palatini
Musculous Uvulae
Palatopharyngeus (posterior faucial pillar)
Palatoglossus (anterior faucial pillar)
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24
Q

From all of the muscles of the soft palate, which is the ONLY one not innervated by the pharyngeal plexus?

A

Tensor Veli Palatini (innervated by CN V)

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25
Q

Muscles of the soft palate: Palatoglossus Origin

A

Oral surface of the velum

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26
Q

Muscles of the soft palate: Palatoglossus Insertion

A

Posterior, lateral tongue

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27
Q

Muscles of the soft palate: Palatoglossus Innervation

A

Pharyngeal plexus (CN IX, X, XI)

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28
Q

Muscles of the soft palate: Palatoglossus Function

A

Pull tongue up / keep palate down

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29
Q

Muscles of the soft palate: Palatoglossus AKA

A

Anterior faucial pillar

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30
Q

Superior pharyngeal constrictors origin

A

Broad - velum, portions of sphenoid, mandible and lateral tongue

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31
Q

Superior pharyngeal constrictors insertion

A

Median pharyngeal raphe

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32
Q

Superior pharyngeal constrictors innervation

A

Pharygneal Plexus (CN IX X XI)

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33
Q

Superior pharyngeal constrictors function

A

Lateral and posterior closure of VP port, velar movement, formation of passavant’s ridge (bulking of muscle on the posterior pharyngeal wall)

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34
Q

Causes of VPI (12)

A
  • Velopharygenal incompetence / insufficiency
  • Abnormal muscle insertion following surgery
  • Poor lateral/posterior pharyngeal wall movement
  • Small oral cavity (s/p surgery)
  • s/p adenoidectomy, adenoid atrophy
  • s/p orthognathic surgery (LeFort 1)
  • Phoneme specific faulty articulation
  • Habituated patterns continuing s/p surgery
  • Hearing loss
  • Dysarthria (UMN, LMN - cerebral, cerebellar, brainstem, muscular)
  • Apraxia of speech (inconsistence, increased severity w/increased length/complexity, HYPO)
  • LMN damage (paralysis/paresis - common w/hemifacial microsomia)
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35
Q

Velopharyngeal incompetence / insufficiency

A

NOT synonyms

  • Incompetence: WEAKNESS - everything is there, it just doesn’t work well
  • Insufficiency: Insufficient muscle / tissue bulk
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36
Q

Flaccid Dysarthria

A

Hypernasality - decreased consonants - nasal emission - velum pulls to non-damaged side

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37
Q

Spastic Dysarthria

A

Hypernasality - decreased pressure consonants - increased gag

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38
Q

Hypokinetic Dysarthria

A

Increased nasal airflow on consonants, slow VP movement - GENERAL SLOWNESS

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39
Q

Submucous cleft

A

Zona pelucida, bifid uvula, notching of posterior border of hard palate (V notch) - V-shaped elevation - reduced movement of the velum

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40
Q

Occult submucous cleft

A

Muscular deficiency on the upper surface of the velum (must visualize from the top - you don’t hear / see it)

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41
Q

VPI effects on speech (6)

A
  • Hypernasality (resonance issue - NOT airflow)
  • Nasal air emission (HEAR air coming out the nose)
  • Weak/omitted consonants
  • Short utterance length
  • Compensatory articulation productions
  • Dysphonia
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42
Q

During nasal articulation…

A

-The velum is DOWN and the VP port is OPEN - the air is coming out of the nose AND the mouth

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43
Q

During oral articulation…

A

When you make an oral sound, the velum is UP and the VP port is CLOSED - the air comes out of the mouth ONLY

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44
Q

VPI effects on speech - Hypernasality

A

Resonance disorder that results form fault coupling of oral and nasal cavities - muffled/nasal - predominant use of nasal sounds (basically no plosives) - moderate to large opening of VP port

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45
Q

VPI effects on speech - Nasal Air Emission

A

Inappropriate release of air pressure through the nasal cavity during speech - audible only on consonants - associated w/air pressure and airflow and affects articulation vs. hyper nasality that is just resonance

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46
Q

Nasal air emission - Nasal rustle/turbulance

A

Bubblying in the back of the nose - small VP opening

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47
Q

Nasal air emission - Nasal snort

A

Forcible emission during consonant production - usually with sibilant sounds

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48
Q

VPI effects on speech - Weak / omitted consonants

A

Leakage through VP port or oronasal fistula, decreased air pressure for oral consents - there greater the nasal air emission, the weaker the consonants - pressure consonants greatly affected (stops. sibilant sounds) - not getting the pressure build up so they turn to nasal sounds

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49
Q

VPI effects on speech - Short Utterance Length

A

*Real indicator that air is being lost!
Nasal leakage shortens the supply of oral air pressure for speech - increased respiratory effort needed - speech becomes physically difficult - fatigue - short, choppy utterances

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50
Q

VPI effects on speech - Compensatory Productions

A

Airflow and utterance length changes are considered passive speech characteristics or “obligatory errors” - articulation changes are the reaction to the VP dysfunction - compensatory articulation productions - Maintain MANNER - BACK place of artic to get behind the cleft (glottal stops, pharyngeal stops, pharyngeal fricatives) - nasalization of oral phonemes

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51
Q

Compensatory Productions - Glottal stop

A

Rapid voice onset - pressure built up below the level of the glottis - plosive substitution

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52
Q

Compensatory Productions - Pharyngeal plosive

A

Back of the tongue against posterior pharyngeal wall - difficult to produce - used for k/g

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53
Q

Compensatory Productions - Pharyngeal fricative / Affricate

A

Back of the tongue against the posterior pharyngeal wall - with small opening left - substituted for sibilants

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54
Q

Compensatory Productions - Velar fricative

A

Tongue in position for /j/ sound w/frication - used for sibilant sounds

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55
Q

Compensatory Productions - Posterior nasal fricative

A

Incomplete closure of VP port- using nasal air emission / friction - typically for /s/

56
Q

Compensatory Productions - What is substituted for voiceless plosives?

A

/h/

57
Q

Compensatory Productions - Nasal sniff

A

Phoneme is produced through forced nasal inhalation - for /s/ in final position

58
Q

Compensatory Productions - General backing of phonemes

A

Using back of the tongue and velum - assists w/ VP closure - prevents later loss through fistulae

59
Q

VPI effects on speech - Dysphonia

A

Alteration of normal vocal quality - characterized by breathiness, hoarseness, low intensity, and glottal fry - typically causes vocal nodules - breathiness as a compensatory strategy

60
Q

CP population has an increased risk for…

A
  • Congenital abnormality of larynx
  • Hyperfunction w/compensatory strategies (glottal stops)
  • Careful monitoring of therapy so as not to increase tension/explore surgical option
61
Q

Other resonance disorders - Hyponasality / Denasality

A
  • Blockage in nasopharynx or nasal cavity (swelling of nasal passages, adenoid/tonsil hypertrophy, aprxia)
  • In CP population: deviated septum, chaonal atresia, stenotic naris, maxillary retrusion (Crouzon / Aperts)
62
Q

Other resonance disorders - Cul de Sac Resonance

A

-Only an entrance to but no outlet from the nasal cavity - “potato in the mouth” speech - combination of VPI and anterior blockage in nasal cavity

63
Q

Instrumentation for assessment of VPI (6)

A
  • Human ear
  • Oral mech examination (nasal emission mirror)
  • Radiography (VFSS)
  • Nasendoscopy (GOLD STANDARD)
  • Acoustic Analysis
  • Aerodynamics
64
Q

Radiography options

A

VFSS: lateral plane, AP view, oblique views, base view - contrast - not perfect b/c you won’t be able to see if there is a GAP in closure, you’ll just see that it’s closed.
CT Scan

65
Q

Videonasendoscopy

A

Optical instrument: viewing lens, fiberoptic tubing, eyepiece attached to camera - assess the contribution of all parts of VP sphincter, images *w/in our scope of practice

66
Q

Nasendoscopy is used to…

A
  • Determine presence / extent of VPI (size of gap, consistency of VPC, timing, movement of individual structures)
  • Visualize the mechanism during speech (before, during, after development of prosthesis, determine location/size of additions)
67
Q

Endoscopic Examination

A

Speech sample (Iowa Pressure articulation test) - Templin-Darley articulation test - oral / nasal consonants, oral only sentence, sustained fricatives, mixed contexts, words - phrases - sentences - swallow

68
Q

VP closure patterns

A

Coronal, sagittal, circular, circular w/passavant’s ridge

69
Q

VP closure patterns: Coronal

A

Most common (55% of people) - most of closure is from soft palate; little medial motion is required - velum goes up and back

70
Q

VP closure patterns: Sagittal

A

Majority of closure is from medial motion of lateral pharyngeal walls (10-15% of people) - some lateral wall movement is typical, but not when most of the closure is due to lateral walls

71
Q

VP closure patterns: Circular

A

When both the lateral walls and soft palate provide closure (10-20% of people)

72
Q

VP closure patterns: Circular w/Passavant’s Ridge

A

Has posterior wall motion as well as lateral walls and soft palate - uses all 4 walls to close port (15-20%)

73
Q

Acoustic Analysis: Nasometer

A

-Computer based equipment - computes ratio of acoustic energy between the microphones at nose & mouth (called nasalance) - dividing the intensity of nasal acoustic energy by the total of nasal + oral acoustic energy (score of 0-100)

74
Q

Nasometery typical scores

A

Nasal sounds should be >50

Oral sounds you want a score to be <30 is fine)

75
Q

Aerodynamic Study

A

Area of opening can be measured by fluid flow amount through opening & accompanying pressure change - production of stop consonants, pressure sensing tubes placed in mouth and one nasal passage to determine difference - nasal airflow measured through other nasal passage

76
Q

What is a mean oral air pressure and nasal flow for aerodynamic study?

A

A mean oral air pressure 300mL/sec is ABNORMAL

77
Q

VPI management - Large gap or Flaccid/paretic palate

A

Palatal lift prosthesis - tight sphincter pharyngoplasty - superior based pharyngeal flap

78
Q

VPI management - Moderate gap (2-10 mm) / good palatal lift

A

Sphincter pharyngoplasty

79
Q

VPI management - Small (1-2 mm) central gap / irregular posterior wall

A

Loose sphincter pharyngoplasty - folded flap paryngoplasty - revision adenoidectomy

80
Q

VPI management - Submucous cleft palate

A

Palatoplasty - double opposing Z-plasty

81
Q

VPI management - Tonsil something causing VPI

A

Tonsillectomy

82
Q

Prosthodontics for VPI

A
  • Provide optimal VPC for speech production
  • Training approach to increase VP closure capability (when they’ve mislearned sounds)
  • Increase probability of successful surgical outcome
  • Assist swallowing/articulation gestures (limited tongue mobility / coordination)
83
Q

What are two prostheses that ASSIST in VP closure

A

Speech bulb

Palatal lift

84
Q

Speech bulb

A

When the soft palate is too short to contact the posterior pharyngeal wall - the bulb contacts the posterior pharyngeal wall - maintains some opening on the sides of the bulb for nasal breathing - need lateral wall movement to close
*used in cases of INSUFFICIENCY - there is just not enough tissue

85
Q

Palatal lift

A

Soft palate of sufficient length, but lacks sufficient mobility - soft palate contacts posterior pharyngeal wall - maintains some opening on the sides of the elevated palate for nasal breathing - again, need lateral wall movement to completely close
*used in cases of INCOMPETENCE - there is enough tissue, it just doesn’t function well

86
Q

Criteria for patient selection

A

Based on diagnostic information, including:

  • speech characteristics, oral-pharyngeal examination, nasendoscopy, & VFSS assessment during speech
  • VP closure is NOT accomplished & further function efforts are contraindicated
87
Q

Patient selection criteria: Issue 1: Surgical approach may be contraindicated due to: (3)

A
  • Significant surgical risk
  • Patient and/or family’s choice
  • Cultural / religious concerns/issues
88
Q

Patient selection criteria: Issue 2: Surgical prognosis may be guarded or poor because: (3)

A
  • Concern that lack of palatal or pharyngeal wall movement will prevent successful surgical outcome
  • Extent of movement
  • Timing & coordination
89
Q

Patient selection criteria: Issue 3: Will an improved VP mechanism help?

A

Further diagnostic information may be needed to determine the effect of improved velopharyngeal closure on speech production

90
Q

Patient selection criteria: Issue 4: What is the status of oral hygiene?

A

Oral hygiene may need to improve so that the prosthesis will not worsen dental health

91
Q

Patient selection criteria: Issue 5: What is dental & occlusal status?

A
  • Tooth eruption / health / shape
  • Occlusal structure / function (mandibular / maxillary alignment, limited mouth opening, other orthodontic appliances)
  • All factors are important to allow satisfactory retention
92
Q

Patient selection criteria: Issue 6: Patient & family cooperation, tolerance, and support are essential

A
  • Multiple visits to construct prosthesis
  • Multiple visits to modify / adjust / repair
  • Prosthesis must be worn consistently
  • Prosthesis must be cleaned consistently
  • Easy to lose & expensive to replace
93
Q

Patient selection criteria: Issue 7: Patient education

A

Patient, family, and support systems MUST understand what the prosthesis CAN and CANNOT accomplish

94
Q

Patient selection criteria: Issue 8: Train out

A

The patient may be able to “train out” of the prosthesis, following systematic reductions of size, resulting in acceptable speech production

95
Q

What percent of kids suffer from VPI following cleft palate repair?

A

10-36%

96
Q

It’s important to keep in mind the causes of the VP dysfunction

A
  • Anatomical (mechanical)
  • Neurogenic
  • Behavioral
  • Combination
97
Q

It’s important to keep in mind the characteristics of the VP dysfunction

A

VP gap size - VP closure pattern

98
Q

What are 3 signs of a sub mucous cleft palate?

A
  • Bifid uvula
  • Zona pellucidum (velar muscle diastasis)
  • Palatal notch at junction of hard/soft palate
99
Q

Management of velopharygenal dysfunction

A
  • Speech therapy
  • Surgical options
  • Prosthetic Devices
100
Q

What are the surgical options for velopharyngeal dysfunction? (3)

A
  • Pharyngeal flap*
  • Sphincter pharygnoplasty*
  • Palatal lengthening and/or re-repair
  • = two main surgical options
101
Q

Goals of VPI Surgery

A

-Partial obstruction of the VP port
1- reduction of opening between the nasal & oral pharynges
2- Lengthening the palate by retro-positioning the velum

102
Q

What surgeries are used for reduction of opening between the nasal & oral pharynges?

A
  • Pharyngeal flap

- Sphincter pharyngoplasty

103
Q

What surgeries are used for lengthening the palate by retro-positioning the velum

A
  • V-Y Pushback
  • Intravelar veloplasty
  • Double-opposing z-plasty
104
Q

When is diagnosis of VPI usually established?

A

Diagnosis of VPI usually not established until around 4 years old - need to let them develop their articulation systems & functional speech before you can evaluate

105
Q

When is VPI surgery usually performed?

A

VPI surgery is usually performed ~5 years

106
Q

Pharyngeal Flap Surgery

A
  • Creates a central static obstruction & leaves 2 lateral ports
  • MOST COMMON for VPI
  • Most efficient in patients w/satisfactory lateral pharyngeal wall movement
  • Sagittal or circular VP closure patterns
107
Q

Where is the donor site for a pharyngeal flap?

A

-Posterior pharyngeal flap as donor site -unscarred, well vascularized, expendable tissue -minimal long-term donor site morbidity
Boundaries: adenoid pads superiorly, 3rd cervical body inferiorly, lateral pharyngeal walls

108
Q

What do musculomucosal flaps incorporate?

A

Musculomucosal flaps incorporate segments of the superior pharyngeal constrictor muscles and palatopharyngeus

109
Q

Posterior pharyngeal flaps can be based…

A
  • superiorly (preferred)

- inferiorly (length is limited by adenoid pad)

110
Q

Inferiorly based posterior pharyngeal flap

A
  • May had advantages as far as vascularity
  • Limitations w/length, ability to inset the flap secondary to incorporated adenoid tissue at tip
  • Low position of base in pharynx - tethers velum inferiorly
111
Q

Superiorly based posterior pharyngeal flap

A
  • May be lengthened and inset at the level of the soft palate
  • May be lacking as a robust vasculature
112
Q

Average pharyngeal flap width:

A

2-3 cm (depends on lateral wall movement)

113
Q

Length of pharyngeal flap is dependent on what?

A

Length of flap dependent on access and requirement for tension-free insetting - average 2.5-3cm

114
Q

Pharyngeal flap success rate:

A

63-98% success rate

115
Q

Results if the pharyngeal flap is too wide:

A

HYPOnasality, mouth breathing, obstructive sleep apnea - sometimes transient and resolves ~6 months

116
Q

Results if the pharyngeal flap is too narrow:

A

Continued VPI symptoms

117
Q

Sphincter Pharyngoplasty

A
  • Provides a static & possibly dynamic lateral & posterior obstruction, creating smaller central orifice
  • Patients who demonstrate good velar elevation bout poor lateral wall motion may be good candidates
118
Q

Where is the mucosal incision for a sphincter pharyngoplasty?

A

Over the posterior tonsillar pillar - pillar flaps rotated 90 degrees and inset into posterior pharyngeal wall mucosa

119
Q

Hynes: Sphincter Pharyngoplasty

A

Elevated lateral pharyngeal flaps 3-4 cm long with salpingopharyngeus muscle, sutured anterior to Passavant’s ridge

120
Q

Orticochea modification: Sphincter Pharyngoplasty

A

Incorporated posterior tonsillar pillar with palatopharyngeus muscle

121
Q

Jackson modification: Sphincter Pharyngoplasty

A

Added small superior pharyngeal flap

122
Q

Post-Op Course

A
  • Admitted to PICU to monitor airway (liquid/soft diet after 24 hours
  • Continued multidisciplinary team approach
  • Go home when pain is controlled, no signs of bleeding, no longer nauseated, breathing easily
123
Q

Early complications of VPI surgery

A

-Airway compromise (usually w/in 1st 24hours, <1% require reintubation), hemorrhage, infection, aspiration/pneumonia, flap dehiscence, cervical subluxation

124
Q

When do you usually see a flap dehiscence in a VPI surgery?

A

Usually when the flap sutured to the friable tonsillar tissue

125
Q

Late complications of VPI surgery

A
  • Sleep apnea
  • Hyponasality
  • Residual VPI
126
Q

Contraindications to surgery

A

-Patient declines surgical management by choice, has known/suspected risk for airway obstruction, has intermittent/inconsistent closure that responds well to speech therapy, has incomplete diagnostic results, visible pulsations on the posterior pharyngeal wall

127
Q

Velocardiofacial Syndrome (VCFS) characteristics

A
  • long face w/prominent upper jaw
  • Flattening of cheeks
  • Underdeveloped lower jaw
  • Bluish color below eyes
  • Prominent nose w/narrow nasal passages
  • Long thin upper lip
  • Down-slanting mouth
  • Cleft palate
  • Cardiac defects
128
Q

Furlow Palatoplasty

A

-Double-opposing Z-plasty is a method of primary repair of cleft palate - has been used secondary for VPI - reposition the palatal muscles transversely, creating a functional muscle sling by overlapping them - adds velar length

129
Q

Who is the Furlow Palatoplasty reserved for?

A

Patients w/submucous cleft palates with VPI and patients with small VP gaps (<8-10mm)

130
Q

Which VPI surgery is more likely to cause nasal airway obstruction and obstructive sleep apnea?

A

The furrow palataoplasty

131
Q

What are two types of VPI prosthetics?

A
  • Obturator prosthesis

- Palatal lift prosthesis

132
Q

What is an obturator prosthesis?

A

Fills residual gaps when tissue is deficient

133
Q

What is a palatal lift prosthesis?

A

Used when there is adequate tissue but poor coordination and movement

134
Q

When would you use a prosthetic for VPI?

A

May be used when surgery is contraindicated or not desired or as a temporary trial to test the expected effectiveness of surgery

135
Q

Poor candidates for prosthetics for VPI

A

Patients w/reduced mental capacity, uncooperative patients/parents, poor oral hygiene/uncontrolled dental caries

Craniofacial Anomalies (4 decks)

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