Cleft Lip and Palate Katherine Lamb, - PowerPoint Presentation

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Cleft Lip and Palate

Katherine Lamb, Ph.,D. CCC/SLP

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Financial and non-financial disclosure statement

• Relevant Financial Relationship(s): I am a salaried employee of Valdosta State University; University System of Georgia.

• Relevant Nonfinancial Relationship(s): ​I have no relevant non-financial relationship(s) to disclose related to this presentation at GSHA.Lamb_GSHA_2020

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ANATOMY REVIEW

Three cavitiesMuscles of velopharynxLevator veli

palatini Tensor veli palatiniMusculus

uvulae Superior constrictor Palatopharyngeus

Palaoglossus

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Cranial Nerves

Motor GlossopharyngealVagus Accessory

Trigeminal Facial Sensory Vagusglossopharyngeal

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Physiology subsystems

RespirationPhonationBernoulli effectResonance

ArticulationStress and intonationcoordination

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Physiology of velar mechanism

Velar movement SpeechVelar dimpleInferior side or velum

Superior sideElevates and elongates

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Classification of clefts

Primary palateAnterior to incisive foramenLips and alveolusSecondary palate

Posterior to incisive foramenHard and soft palate

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Causes

Disruption or delay:Embryological developmentChromosome/genetic disordersTeratogens or mechanical factors

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Causes

VirusesMaternal nutritional deficiency and obesityLip w & w/o palate 2X malesPalate 2X females

Timing differences in developmentMechanical interferenceMultifactorial inheritance

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Impact

cleft of primary palateNose separation of orbicularis oris

muscles misalignedColumellashortened(appear) attached to noseNasal cavitiesdeformed and airway reduced in size

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Impact

cleft of secondary palateComplete thru velum velar/palatine

Muscle insertions are abnormalLevator veli palatini-midlinepalatophayngeous

insert into posterior borderTensor veli palatini

not functional

cannot regulate eustachian tube

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Submucous cleft palate

OvertBifid uvula, zona pellucida

and notch in posterior HPOccultNo overt signs Nasal regurgitation, chronic ear infection

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Prevalence and treatment of submucous cleft

.02 and 0.08% in general population25-50% will have velopharyngeal dysfunctionSx if leads to velopharyngeal dysfunction

Palatoplastyyoung childpharyngeal flap for older

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Facial clefts

Oblique cleftMouthMidline cleftnotch in midline of vermillion

or slight upper lipor with brain involvementEncephalocele-gap in skullAbsence of corpus callosumHoloprosencephaly-failure of forebrain to divide

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Embryological development of lip and palate

Development of lip and alveolus begin at 6-7 weeks Development of palate begins at 8-9 weeks lingual was highdrop at 7-8 weeks

palatal shelves move from vertical to horizontal and fusepremaxilla at incisive foramenVelum Uvula 12 weeks

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Embryological Development

Variations during:

(1) embryonic period (2) very early fetal period

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Normal Embryological Development

1st

3 weeks: single cell to multicellular Flat disc of 1 layer 7th day

Beginning to 2nd week:

2 layers

3 layers

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Development

3 layers = all tissues and organs

Disc thickens as cells differentiate greater thickening at cranial end

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Genetics evaluation

Dx Hx

Counseling and family supportDysmorphologyMalformationDeformation

TeratogensAmniotic bands

Maternal illness

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Syndromes, sequences, associations

SyndromeSequence

AssociationLamb_GSHA_2020

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Dentition

Normal dentitionAnomaliesIncisor relationship

OverjetOverbiteUnderbite

Missing teethRotated teethSupernumary or ectopic

Crossbite

Protruding

premaxilla

Open bite

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Dental development

Infant stagePrimary stageEarly mixedLate mixed

AdolescentLamb_GSHA_2020

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Psychosocial Issues

Family Shock and adjustmentSupportEmotional

Feeding School EducateMost: average cognitionSocial interaction

Fewer friendsLess socially competentTeasing

Self-perception

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Societal issues

Physical appearanceSpeech qualityHearing impairment

StigmaLamb_GSHA_2020

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Impact

Cleft of primary palateNose separation of orbicularis oris

muscles misalignedcurveColumella: shortened Appears attached to noseNasal cavities deformed

reduced in size

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Impact

Cleft of secondary palateVelumMuscle insertions are abnormal

Levator veli palatini palatophayngeous Tensor veli palatini

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Presurgical management

Lip Alignment neededThree optionsTape lip along with dental elastics for 4-6 weeks

Latham appliance uses 2-piece acrylic device gradually brings palate together and lip as well; used with nasal alveolar molding deviceLip adhesion at 6 weeks of age with full repair 3-4 mos. laterLamb_GSHA_2020

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Techniques for unilateral lip repair

Millard

rotation advancement flap technique; aka cut as you goRandall-Tennisontriangular flap technique; fixed technique that is precise and measured

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Cleft lip repair

Cheilopasty is usually completed @10 lbs

10 weeks oldHemoglobin of 10gmMost repair lip between 4 and 12 weeks

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Techniques for bilateral lip repair

Millard

Broadbent-ManchesterBoth done between 9 months and 5 yearsOnly difference is how white roll of philtrum is created

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Palatoplasty

Two groups for repair

Early = 6-15 mos. Late = 15-24 mos.LOTS of debate on when and how but overall, but generally accepted that delayed closure of HP has negative effect on speech.

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Techniques for Palatoplasty

Von

Langenbeck – oldest and most successfulWardill-Kilner V to Y – higher incidence of anterior fistulae

Intravelar veloplasty (IVVP) – to repair

levator

sling

Two-flap palatoplasty

Furlow

palatoplasty

Early repair is better

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Fistula repair techniques

Most cases, fistula is deliberately left in alveolus

Usually closed during time of mixed dentition with bone graft from rib or iliac crestUnintentional fistulas can be fixed but carry >37% recurrence risk

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Surgery for VPI

PharyngoplastyCannot dx until able to produce speechEarliest: 3.5 to 4 y/oVPI is a surgical disorder

Needs of patientLamb_GSHA_2020

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Techniques

Redo palatoplastyLengthens the palatePharyngeal wall augmentationVP opening is small

Implant deep in superior pharyngeal constrictorsRolled flap –pharyngeal wall to produce bulgeLamb_GSHA_2020

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Techniques

PharyngoplastyPharyngeal flapBest for midline gaps

Long and high as possibleSphincter pharyngoplastyMyomucosal flaps

Rotate posteriorlyInset into nasopharynx

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Complications

EdemaApneaLess common with pharyngeal flap

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Facial structures

MaxillaRetrusion: small maxilla relative to mandibleFacial nerveImpacts what phonemes?

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Oral cavity

LipsShorten upper lipPhonemes affected?

MouthMacrostomiaMicrostomia

lingualMacroglossiaMicroglossia

Lobulated: multiple lobes with fissures

Ankyloglossia

Tonsils and adenoids

Faucial

Lingual

Pharyngeal

Palate

High arched vs. low flatPalatal or oronasal fistula40

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Upper airway obstruction

Adenotonsillar hypertrophyTonsillar hypertrophy

Lingual tonsil hypertrophyAdenoid hypertrophyImpact nasal breathingObstruct Eustachian tube functioningSpecific facial presentation

Mouth breathingAnterior lingual carriageLowered mandible

Puffy eyes

Pinched nostrils

Stertorous

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Treatment of UAO

TonsillectomyAdenoidectomyTracheostomyUvulopalatopharyngoplasty (UPPP)

CPAPLamb_GSHA_2020

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Nasometry

Nasometer: measures nasal acoustic energymeasuring acoustic energy in nasal cavity and oral cavity

calculate ratio Score higher = hypernasality; lower = hyponasalityClinical useMeasure Equipment

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Nasometric procedures

Sensitivityabnormal resonance

Specificitynormal resonanceInterpreting numbers

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Sensitivity

ID individuals with abnormal resonance

SpecificityCorrectly excludes those with normal resonanceVariability of scores and lack of agreement PERCEPTION not just numbers

Interpreting numbers

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Interpreting

nasogram

Normal oral resonance is 15-20% pointsExpected difference between /a/ (ah) and /i/ (

ee) is about 10 points with oral consonants and 20% with nasal consonants

Higher contour on screen, more hypernasality to expect

If most data points are normal, but have occasional peaks, suggests normal resonance with nasal emission

Gradual rise indicates muscle fatigue

No data points on /s/ or /

sh

/, but if do get data points on these without others then consider phoneme specific nasal emission

Lingua-

alveolars and bilabials higher, consider fistulaHigh on vowels but prolonged /s/ is zero, consider thin velum, high tongue placement or vowel-specific nasalityLow data points overall, hyponasality and maybe upper airway obstructionLamb_GSHA_2020

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Pressure –Flow technique

Small bore cathetersoral cavitynostril

Other nostrildetermining presence and extent of VPIEquipment and calibrationLamb_GSHA_2020

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Assessment of nasal airway

Obstruction via nasal resistancePosterior & anterior rhinomanometryClinical procedures

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Low or No technology assessment

Counseling and support1st yearAnnual screenings & assessments

Language screeningSpeechArticStimulabilityNasal air emission

Weak consonantsUtterance lengthOral-motor dysfunction

Resonance

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Basic Assessment

Visual detectionMirror test figureAir paddle-figure Tactile detection

Auditory detection Nose pinch Stethoscope Listening tube

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Differential dx of cause

VPIOrofacial fistulaArtic disorder

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Follow up

RecommendationsFamily counselingEvaluation report

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Videofluoroscopy

Multiple viewsLateralFrontalBase

Oblique Barium:orally noseSpeech sample

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Advantages and limitations

AdvantagesMovementVelumlateral/posterior

Length of velumDisadvantagesRadiation Not as good as direct viewNot 3D

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Nasopharyngoscopy

Flexible fiberoptic nasopharyngoscopyCameraHigh res monitorMicrophone

RecordingLamb_GSHA_2020

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Nasopharyngoscopy

Advantages/disadvantagesNo radiationDetailBiofeedback

Minimal risk CooperationLamb_GSHA_2020

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Resonance disorders and VP dysfunction

VPD Not = VP insufficiencyNot = VP incompetence

Not = VP mislearningLamb_GSHA_2020

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Nasality

HypernasalityMore noticeable on vowelsNasal twang Hyponasality and denasality

Hypo Denasal Cul-de-sac resonanceMixed resonance

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More effects of VPD

Nasal emission: build up oral pressure Leak

Pressure-sensitive phonemes Sibilant soundsNasal grimaceeffort to close VPWeak or omitted consonants

Short utterance lengthAltered rate and speech segment durations

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Compensatory and obligatory articulation

Passive speech characteristics structural abnormalityActive speech characteristics

response to VPDMiddorsum palatal stop: sub: /t,d,n,l,k,g/; sometimes: /s,z,,zh,

sh,ch,j/Generalized backingVelar fricative

Nasalization of v/c

Nasal

snort

sniff

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Compensatory and obligatory articulation

Pharyngeal fricativeGlottal stop/h/ for voiceless plosives

BreathinessDysphoniabreathy, hoarse, low intensity, glottal fryIncreased muscular effort and respirationNodules

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Factors impacting speech and severity

Size of VP openingInconsistency of VP closureAbnormal artic and phonation

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Causes of VPI dysfunction

VPI (insuff)Hx of cleft palate

Submucous cleft palateShort velum/deep pharynxAdenoid atrophyIrregular adenoidsHypertrophic tonsils

VPI post treatmentAdenoidectomyTonsillectomyMaxillary advancement

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Causes of VPI dysfunction

VPI (incomp)Abnormal muscle insertion

Hypotonia/poor pharyngeal wall movementDysarthriaApraxiaCranial nerve defectVelar fatigue or stress incompetence

VP mislearningFaulty articHabituated speech patterns

Lack of auditory feedback

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Anatomy of the ear

External Middle Inner

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Malformation of ear

ExternalMicrotia: malformed pinnaAural atresiaMiddle

Eustachian tube dysfunction and middle ear diseaseInnerRare (syndromes)Sensorineural HL

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Infant anatomy for feeding

Smaller / closer Oral cavity is ideal for sucklingRestricted ability to open oral cavity Extension/retraction of lingual

Larynx Epiglottis protrudes into nasopharynx Lamb_GSHA_2020

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Oral phase of swallowing

Rhythmic suckling create pressurePalate must be intact; compression of nippleOral cavity must be closed posteriorly

Any opening Lamb_GSHA_2020

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Pharyngeal phase of swallow

Bolus channeledCoordination of breathing and swallow and sucklelingual, velum and pharyngeal wall Velum elevates and closes VP

lingual base movesBolus diverts around epiglottisBreathing thru noseAbduction of VFArytenoids

EpiglottisLamb_GSHA_2020

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Esophageal phase of swallowing

Pharynx to esophagusUESLES After swallow

suckling and breathing resumeUES and LES closeSuck-swallow-breathe ratio is 1:1:1 to 2:1:1Lamb_GSHA_2020

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Characteristics of feeding problems with clefts

Poor oral suctionInadequate volume Lengthy feeding Nasal regurgitation

Excessive air intakeCoughingChokingVaries

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Characteristics of feeding problems with clefts

Pharyngeal phase normalProblems maintaining airwaypharyngeal, esophageal or CNS abnormalities airway protection

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Characteristics of feeding problems with clefts

Cleft of lip usually not as problematicMinimal cleft of velum may be able to feedMore anterior cleft Nasal regurgitation (common)

StressfulLamb_GSHA_2020

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Breast or Bottle feeding

Breast feedingNot challenge (lip)Modified nippleBottle feeding

PliabilityShapeSizeHole type and size

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Oral-motor facilitation techniques

Positioningupright at 60 degreeschin-tuckarms forward

trunk in midlinehips flexedNipple under shelf of hard palatePacing intakeOral facilitation

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Oral-motor facilitation techniques

Manage nasal regurgitationConsistency of method Feeding obturatorOral hygiene

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Feeding older infant

Soft cup feeder to aid transitionSolid foods-thickened

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Prosthetic devices

Dental appliancesFixed bridgeDenturesOverlay dentures

Facial prosthesesFeeding obturatorsCovers unrepaired cleftlingual out of cleftProvides surface

Does not help with velum

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Speech Appliances

Palatal liftRemovable deviceelevates velumVP incompetence

Takes timeInterferes with nasals and resonancePalatal ObturatorCover palatal fistulaSx

not in near future

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Speech Appliances

Speech bulb obturatorRemovableVP insufficiency

Stops nasal regurgitationSwallowingRarely used with childrenFabricationFittingEasier to fit palatal obturator for fistula

Lift or bulb function

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Indicators and contraindicators

ProsPersistent VPI Sx

not workNeurological compromisedGood teeth ConsRemoveNight & eating

ExpensiveEasy to lose and damageUncomfortable

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Speech therapy

Eliminate compensatory artic productions can help eliminate nasal rustleReduction therapyLength gradually shortened or…

Time wearing it reducedLamb_GSHA_2020

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Timeline for Intervention

Infants and toddlersFeeding is number one Language development next

Quantity of speechSLP & language stimulation activitiesEnroll if feeding or language issuesFacilitate appropriate placement

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Intervention

Preschool childrenAssess language and speech development and VP function (3y/o)Sx best between ages 3-5

Include parents and siblingsEasier to fix errors earlyMay alleviate teasing later

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Intervention

School age childrenVPI should be corrected Hypernasality or nasal emission Focus is on any artic errors

Only time we target obligatory errorsTarget compensatory errors < developmental Rarely provide treatment for teen or adult

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Interventions

Basic PrinciplesEarly involvement ParentsPlan aimed at problems identified

Simple explanations for the childStart with an area of likely successFocus on motivation and increase insight

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Interventions

Attention to volume, rate, and prosody Insufficient or incompetent SP: exercises will not helpBlowing and sucking exercises are not effective

Follow normal phonetic and phonological developmentAddress both language and speech developmentIncrease sensory awareness and movement

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Interventions: Lips

Gentle stroking from top in midline encourages lip pursingStimulation with soft brush

Rub lips with Vaseline: sensation of contact Soft toysMassage with a facial spongeFunny facesPull small object to extract object; lip pressure

Paint face; pay attention to lipsLip movement--encourage vocalization/babblingMouth opening

Wide-open

Yawning

Encourage vocalization with wide-open mouth

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Interventions:

lingual tip/Alveolar ridge

Sensory awareness with toothbrushPressure on alveolar ridgePlace lingual-tip on pressed spot

Identify spots with cotton swabs or toothettes“Sticky” to identify points

Food around the mouth and lick

Oral air-stream

Feel the air-stream on back of their hand

Blowing activities to demonstrate result of air-stream

cotton balls

bubbles

feathers

Blow into cupped hands or down a tube89

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Interventions:

AirstreamBlow up cheeks Introduce consonants /f/ or /s/

Feeling the vocalization (parent/clinician)Drumming fingers on lipsAssist in producing bilabials, labio-dentals

Imitation of oral vs. nasalImitated vegetative noises & reinforce

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Interventions: Babbling and Sounds

CV combinations: includevoiced/voiceless variations of loudness and intonation

Repeat /wa/wa/wa

/Encourage blowingInterrupt: stop air-stream by opening/closing lips or moving lingual up and down

Feel larynx of others

Sing /la/la/la

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Interventions: Young Children

Perceptual skills

Discriminatory work (phonological contrast) Auditory input tied to attempts at production Explain correctedCorrect articulation

presence of persisting nasal emission place is prime

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Interventions

Introduce new sounds:developmental sequence

difficultydemonstrationUse any procedures=results

air-stream on handfeeling larynx

movement of air-stream

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Interventions Hypernasality

Adequate breath support

Ear training to recognize desired target Encourage:low lingual carriagemouth openingAdequate loudness

Optimum pitchDo not precipitate laryngeal elevation to reduce hypernasality Practice CV sequences/words for desired resonance (easy)

Practice sequences low in nasals

Eliciting Plosives

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Principles

Auditory firstAnd on production

Imitation firstMirrorPlace and manner

Diagrams

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Plosives /p/b

Achieving manner and voicingshort sounds, bursts, puffs

voiceless, puffy; voiced, buzzy Begin with voicelessInterrupt gentle air-streamShow visible resultStrengthen lips for

plosionmaintaining seal against resistance

Puff out cheeks before release

Feel larynx for /b/

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Achieving place

Awareness of articulators by increasing sensory awarenessGood lip movement /w/

Vaseline Imitate silent open/closing of lipsgradually introduce breathHum with pressed lips, followed by a /p/ or /b/

Test stimulability in all phonotactic positions

Imitate silently, words which include bilabial plosives

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Alveolar plosives t/d

Frequently replaced by glottalLack precise lingual-tip contactNasal emission

Achieving manner and voicingNature of soundsSound concepts

Voiceless first

Symbols for voiced

Diagrams

Feel larynx for /d/

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Achieving place

Children: stories Adolescents/adults: diagrams

Head forward Clinician press on alveolar ridgeChild presses Lift lingual to contact

Dental elastic between lingual-tip and contactHold

Out of mouth on lingual-tip

Return, holding elastic

/n/ for start

Increase sensory awareness in lingual-tip

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Velar plosives /k/g/

May be replaced by glottal plosive or fricativeachieving place is difficult

Achieving manner and voicingConcepts: tapping, knockingBe careful introducing /g/ may trigger glottal

Diagrams of vocal tract and functionslingual and velum tap for plosives

Vocal cords buzz

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Achieving place

Mirror and diagramsLingual at posterior

clinician touch with toolchild with fingerBig open mouth

Tap under chin at base of lingualId placeEncourage movement

Introduce /k/ and /g/ next to high vowel

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Fricatives

TermsPlace lacks frication

nasal emissionabsentglottal or pharyngeal fricativeLabio-dental easiest fricative

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Achieving manner and voicing

Gentle air-stream without strictureFeel air-stream from clinician

Feel voice/no voiceProduction of VC first Use mirror

Manually assist to achieve labio-dental contact

Increase awareness of contact point on lower lip:

Prolong frication initially to increase sensory and auditory awareness

If incisors are missing

upper gum ridge to lower lip

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Alveolar fricatives /s/z/

Usually most difficult to achieveIncreased intra-oral pressure and precision

Achieving manner and voiceFeel air-stream from clinician to handConcepts of fine air-stream Press spot-to identify

Lift lingual-tip up to spot stimulated by pressure on alveolar ridge

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Exercises to strengthen lingual tip

Is it easier to produce with lowered or raised lingual-tip

Lingual tip with elastic exerciseEstablish lip spread with teeth close together, Facilitate with /s/z/ adjacent to /t/d/

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Palato-alveolar

Achieving manner and voiceThick stream vs. thin stream for /s/z/

Achieving placeBroad palatal contactlingual (lateral) pressing teeth

Forward square lips106

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Affricates

Last Until /t/d/ can be produced

Achieving manner and voiceConceptsCompare fricatives/affricates

Contrastive pairAvoid words that trigger old patterns

Use nonsense words for older children and adults

 

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Achieving place

Elicit from place of plosive elementPlace for/t/d/ with slow release

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