9/12/2013

Team Care of the Child with a Cleft Lip & Palate: Preschool and School-Age Jeff Searl, Ph.D., CCC-SLP, Debby Daniels, Ph.D., CCC-SLP Sandy Keener, AuD, CCC-A Hearing and Speech Department, KUMC

Skylar Bellinger, Ph.D. Center for Child Health and Development, KUMC

The Cleft Palate Team: Professionals Involved Orthodontics

Dentistry

Peggy Tuttle, MMSc, P.A. - C Plastic & Reconstructive Surgery, KUMC John Carter, D.D.S., M.S.D. Plastic & Reconstructive Surgery, KUMC and Private Practice

Social SpeechWork Language PathologyCommunity Based: SLPS, Plastic Teachers, Surgery Psychology Dentists, Peds, Social Workers, etc.

Maxillofacial Surgery

Audiology

Patient & Family

Nursing

ENT

Clinic Coordinator

Genetics

Pediatrics [email protected]

The Cleft Palate Team: Importance of the team approach • coordinated care

• consistent care • proper timing and sequencing of evaluations and treatments • patient’s overall developmental, medical, and psychological needs are considered in the service provision.

Pre-School Age SLP – Evaluation Issues • Don’t forget the toddlers! • Hardin-Jones & Chapman (2008) notes that toddlers should receive direct intervention after palatal repair if….. • They demonstrate restricted consonant inventory • They demonstrate decreased number of oral stop consonants

• Preschool assessment should be broad • Rule out language deficits • Consider phonation • Consider fluency

Overview of tonight’s plan • Focus on… • Preschoolers – primary concerns (Dx/TX per discipline) • School Age (early?) – primary concerns (Dx/TX per discipline) • but . . .

• Isolated clefts of lip/palate (nonsyndromic) • Assume primary lip repair (~2-3months) and palate repair (~10months) have occurred

Pre-School Age SLP – Evaluation Issues • Typical errors in fricatives, affricates & plosives • How to characterize speech deficits? • Restricted phonetic inventory • Articulation errors • Phonological process errors • Compensatory errors • Distortions 2◦ to malocclusions • Reduced oral pressure on stop consonants • Combination? • Specific to speech production, your assessment should include • Measure of overall intelligibility • Conversational sample

• Single word articulation measure • GFTA-2, DEAP, clinic-specific measure

• Sentence imitation task • Stimulability

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Pre-School Age SLP – Evaluation Issues

Pre-School Age SLP – Evaluation Issues • Compensatory Articulation

•Cons onant Production

Initial Position /p/

teapot

/b/ /t/ /d/ /k/ /g/ /th/-vc /th/+vc /f/ /v/ /s/ /z / /sh/ /h/ /ch/-vc /ch/+vc

cowboy bow tie pie dough peacock s eagull too thick try thes e s eafood blue vas e eyes ight free z oo s eas hore keyhole high chair blue jay

Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item.

Cupcake

Final Pos ition Choos e an item.

Bobcat Suitcas e G uide book Blackboard Tug boat Tooth pas te Breathe gas Lifeguard Stove top Bas eball Chees ecake Tras hbag

Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item.

Beach ball Stage coach

Choos e an item. Choos e an item.

/ w/

s eaweed

/ j/ / l/ / l/ / r/ / er/ / m/ / m/ / n/ / n/ / ng/ / kw/ / pl/ / fr/ / s tr/ / nd/ / s t/

three yards s hoelace pillbox hayride birdhous e playmate homerun doughnut rainbow s lings hot bee queen pie plate tree frog s hoes tring s andbox pos tcard

Initial Pos ition Choos e an item.

Final Pos ition

Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item.

• Glottal Stops (build up of pressure below the glottis) often observed in stops, fricatives and affricates • Pharyngeal Fricatives (constriction between tongue and pharyngeal wall)

• Nasal Emission Choos e an item.

Choos e an item. Choos e an item. Choos e an item.

• Stops, fricatives, affricates • Sound specific?

Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item. Choos e an item.

Pre-School Age SLP – Evaluation Issues

Pre-School Age SLP – Evaluation Issues • Info we seek

• VP Related • Ideal situation for early VP Dx post 1 0 repair • Info we seek • Why it can be hard to get at this age –

• Oral Mech – • Structural issues • Movement – soft palate, lateral walls Not Obs.

No Mvmnt

Min/ Slight

•What is the speech sample to use? •How many times should you do it? •Any other special instructions or considerations?

Mod/ Marked

stinkers not thinkers • What the team asks for from community SLPs and families

Pre-School Age SLP – Evaluation Issues • Info we seek • Ratings of hypernasality

Pre-School Age SLP – Evaluation Issues • Info we seek • Ratings of Nasal Emission

• Speech sample • The task: nasal flutter

• Speech sample • The task: no nose pinch

• The rating scale:

• The rating scale:

(From KUMC CLEFT CLINIC FORM 2005, M.A. Carpenter) (From KUMC CLEFT CLINIC FORM 2005, M.A. Carpenter)

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Pre-School Age SLP – Evaluation Issues • Info we seek • Ratings of hyponasality

Pre-School Age SLP – Evaluation Issues • Info we seek • Stimulable

• Speech sample (another reminder!) • The task: no pinching of the nose

• Can they alter hyper-, hypo-, nasal emission - direct instruction, modeling, biofeedback (aerodynamic, acoustic)?

• The rating scale:

• Degree to which they can do it

• Consistency with which they can do it

• Instrumental measures/information – more in school age section but we can get this for quite a few of our kids

(From KUMC CLEFT CLINIC FORM 2005, M.A. Carpenter)

Pre-School Age SLP – Evaluation Issues • What we often need from community professionals • Confirmation/comparison of what we are seeing and hearing vs. what you are seeing/hearing • Stinkers: • Your impressions of their speech – reports, phone calls, etc. • Your guidance as to how we can get the best set of observations from them • Your presence, if possible • Coaching/training of the kids to participate in our team eval

• Support to families/kids for whom we have made recommendations – communication between you and us

Pre-School Age SLP – Treatment Issues • VP Related – issues that influence VPI management plan at this age • Confounders to VP Sx observations? • Fistula – deal with via physical fix • Nasal patency – ID cause and deal with it • Artic related – significant ‘backing’/glottal stopping; more global oral artic movement issues

• Confidence in our observations/data – often an issue with 2-4yr olds • Degree of impact of VP symptoms on speech – various perspectives to consider • Expectation that improving VP situation will improve communication?

• Aerodynamic assessment – age 3 and up almost guaranteed • Aero-acoustic assessment – age 3 and up almost guaranteed • Flexible fiberoptic nasopharyngoscopy – a little riskier but can get valid/useable views and info for quite a few with good scopist

Pre-School Age SLP – Treatment Issues • Goal is age appropriate communication • Increase phonetic diversity • Increase number of oral stop consonants (focus on placement) • Facilitate emergence of oral airflow for oral fricatives • Therapy issues • Frequency • Individual vs. group? • Traditional treatment vs. phonological approach? • Sounds stimulable • Begin with anterior consonants (visible!) • Consider starting with voiceless rather than voiced consonants

• Don’t reinforce glottal stop substitutions for absent pressure consonants

Pre-School Age SLP – Treatment Issues • VP Related – • No confounders, solid confidence, notable impact, expect improved VP [or masked VP sx] to improve communication – intervene • Surgeries • Behavioral – involves SLPs in home community often times • Maximize VP function • Compensatory Tx – minimize VP sx

• Reduce/eliminate maladaptive behaviors

• Confounders – deal with and re-eval (use community SLPs in some instances) • Poor data set • Behavioral work in home community • Familiarize with protocol, instrumental approaches • Convince to work for the team

• Work on other communication needs while they mature

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Pre-School Age Audiology – Evaluation issues • With Universal Newborn Hearing screening, any permanent hearing loss would already be identified by preschool and school age. • The majority of hearing loss in this population is conductive and typically medically treatable. Care should be taken as middle ear dysfunction may arise at any time and conductive loss often fluctuates. Any concern that a child may not be hearing well warrants referral. • Annual testing should be provided to those children who are not already followed due to middle ear pathology and/or identified hearing loss.

Pre-School Age Audiology Issues-Treatment • Referral to ENT as indicated for any new issues and to ensure follow-up for existing patients is in place. • For newly identified hearing losses, close communication is needed with the education • If amplification is in place, hearing aid follow-up services are ongoing • Work with the school team to ensure optimal hearing aid settings and any needed assistance in the classroom as it relates to hearing are provided

• Continue to monitor hearing status for children with known hearing loss.

Pre-School Age Psychology • Sklylar Bellinger

Preschool Age 

Assessment / Screening  Family

History

 Developmental

Milestones

 Social-Emotional  Anxiety/

coping with surgeries

 Behavior  Physical symptoms-

Preschool Age 

Treatment  Provide

resources/ guidelines to talk to children about condition and how to prepare for surgeries an(books, websites, coloring books).  Teach importance of monitoring/ early intervention.  Brief

Intervention

 Coach  Work  Referral  Infant

Case Example 

 Prevention

behavior management strategies on coping skills/ calming plan

to appropriate services

Toddler/Early Childhood  Local Mental Health Provider

eating/ sleeping

Gary 4

y/o boy Syndrome- many medical issues  Difficulties with hearing and vision  Behavior problems/ temper tantrums  Attends Head Start  IEP for speech- mom reports trouble getting adequate services from school  Mom would like evaluation for Bipolar and Autism Spectrum Disorder  Stickler

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Pre-School Age Dentistry/Orthodontics

Gary cont… 

Psychology treatment/ Recommendations  Continue  Families

current services Together Strategy Consultation

 B ehavioral  Catch

him being good warnings  Timers on ipad  Transition

 Outside

• Consider these as part of management plan • Might serve as a viable replacement for missing teeth later on so not always removed • Movement of the tooth later on via ortho work can increase alveolar bone growth

service referrals

 Behavioral Therapy  KU

CCHD for Autism Evaluations

Pre-School Age Dentistry/Orthodontics

ectopic

• Maximize dental care of primary teeth – primary out of alignment, partially erupted, difficult to clean • Deal with ectopic teeth as needed – often happens with primary and permanent incisors • Dental X-rays – looking for permanent teeth yet to erupt to better plan orthodontics that will start in a few years • Identification of supernumerary teeth – about 20-23% of patients

Pre-School Age Plastic Surgery

supernumerary

Velopharyngeal Insufficiency

Surgical Management of Velopharyngeal Insufficiency

Work-up

• Clinical speech assesment • Closed nose speech • Mirror test • Aerodynamic testing

Imaging

• Videonasal endoscopy • (ie flexible NP scope) • Nasopharyngeal fluoroscopy

Peggy Tuttle, MMSc, PA-C Cleft and Craniofacial Clinic KU Medical Center Kansas City, KS

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Functional Assessment of VP Closure

Initial Management • Speech Therapy with SLP • - help correct articulation errors • •

- help correct compensatory speech - work on timing and tempo of speech

• Pharyngeal appliance or obturator

Surgical Management of VPI 1) Palate lengthening and pushback 1) VWK palatal pushback 2) Furlow Double Opposing Z-plasty

2) Pharyngeal flap 3) Lateral sphincter pharyngoplasty 4) Augmentation of the posterior pharynx (Passavant’s ridge)

Lateral Sphincter Pharyngoplasty • Use the palatopharyngeus muscles bilateral and create a smaller sphincter at VP opening • Originally thought to be dynamic but not much motion present • Best when poor sidewall movement present and some AP movement present

•

- poorly tolerated

• •

- best for non-surgical candidates - rarely good long-term plan

Palate Lengthening • Two techniques • 1) VWK palatal pushback • • • •

Use if muscles are already reapproximated

2) Furlow double opposing Z-plasty Use if muscles are dehiscent at midline Great for submucosal clefts

Pharyngeal Flap • Superiorly based flap of the posterior pharyngeal wall is sewn into the midline of the palate • Two side “ports” are lateral to the midline flap • Lateral sidewall movement required to close VP ports (ie touch the midline flap)

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Posterior Pharyngeal Wall Augmentation • Controversial • Various techniques used • Main principle is to augment Passavants ridge in the posterior pharynx to aid in closure • - Fat injection • •

Complications 1) 2) 3) 4)

Hyponasal speech Snoring SLEEP APNEA Sinus drainage problems

- Cosmetic fillers - Rolled pharyngeal flap

School Age SLP – Evaluation Issues

School Age SLP – Evaluation Issues • VP Related

• We consider the same issues as noted in the preschool assessment • Increased focus on the presence of compensatory articulation errors • Increased focus on presence of distortions related to malocclusion • Presence of continued developmental errors

School Age SLP – Evaluation Issues

• If not done already we now need a definitive VP diagnosis – GOAL is to know by age 4 but…; earlier the better • Info we seek • oral mech, perceptual ratings as earlier • NOW we really look for solid data from instrumental exams to help understand VP status and plan appropriate intervention

Aerodynamics – Pressure-Flow testing

• Instrumental Exam Options – more common ones • Flexible fiberoptic video nasopharyngoscopy (FFVN) http://www.youtube.com/watch?v=Bp3GVgAo2-k

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Combo Acoustic and Airflow

Combo Acoustic and Airflow

• Nasalance and Nasal Emission System (Glottal Enterprises)

Combo Acoustic and Airflow

School Age SLP – Treatment Issues • Suggestions for remediating compensatory errors • Glottal stops • Sustained /h/ sound followed by sustained vowel /a/→ hhhhaaa • Sustained /a/ followed by sustained /h/ followed by sustained /a/ →aaahhhaaa • Insert light /p/ contact → hhhhaaaapaaaa • Pharyngeal fricatives • Produce sibilant sounds with nares occluded and then open (i.e., alternating oral and nasal airflow) • Work on production of /s/ by making hard /t/ with teeth closed, increase duration until it becomes /ts/ • For /ʃ/, have child do big sigh with teeth closed. Increase oral air pressure and shape lip position. • For /tʃ/, have child make /t/ with teeth closed or trying big sneeze with teeth closed. Once mastered, add voiced component for /dʒ/

School Age SLP – Treatment Issues • VP related • Firm decision on need for physical management or not in early early school age at the latest • Possible behavioral VP intervention – but usually only for • Limited resonance symptoms that can be masked, reduced, controlled • Poor surgical or physical fix candidates • Options? • • • • • •

School Age SLP – Treatment Issues • Dental/occlusal related – save for after a couple of slides from our dentist/ortho doc

No non-oral exercises for VP – lack of empirical support Oral pressure focus Mouth/jaw opening Rate manipulation Articulatory precision Normalize all other aspects of artic (place, manner, voice; eliminate compensatory articulation if present)

• Impact of fistula on speech (resonance, nasal emission, artic) – in a minute

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School Age Audiology Issues

• Children with a history of normal findings in the past may be monitored through public school and/or health screenings with referrals for further testing pending those results. If coming annually for a team evaluation, hearing would be assessed if not completed elsewhere. • Children with hearing loss are followed on a regular basis with frequency of visits determined by each child’s needs and hearing loss. • Many issues related to middle ear disease have resolved by this time making fluctuating hearing loss less common.

School Age Psychology

School Age Audiology Issues-Treatment • Referral to ENT as indicated for any new issues and to ensure children with tubes, chronic ear issues are being followed. • For newly identified hearing losses, close communication is needed with the education • If amplification is in place, hearing aid follow-up services are ongoing • Work with the school team to ensure optimal hearing aid settings and any needed assistance in the classroom as it relates to hearing are provided

Elementary Age 

Assessment/ Screening  Interview      

Family History Development Behavior School Functioning Social interactions/ experiences Mental Health  Anxiety  Depression

 Pediatric 

Elementary Age 

Treatment 

Prevention



Brief Intervention



  



Provide resources to prepare for surgery

Create coping plans to manage anxiety Coach on behavioral strategies Collaboration with current service providers

Referral to appropriate services   



Special Education Evaluations Connect to school supports (i.e. check ins with counselor etc…) Families Together Local Mental Health Providers for Behavior therapy  Family counseling  Evaluations 

Symptoms Checklist

Broad screener for concerns

Case example 

Dana 7

y/o girl is in therapy due to traumatic history  IEP for speech and academics  Good social skills  History of Developmental Delay (late walker)  Pediatric Symptom Checklist- within normal limits  Family history positive for mental health problems  Family



  

Depression Intellectual Disability Conduct Disorder Asperger’s Disorder

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Dana cont… 

Reported anxiety about upcoming surgery  B rother



told her they were going to “cut off her tongue”

Treatment/ Intervention  Family

resources

 Surgery

prep coloring book  Consult with Dr. Andrews  Visit

in hospital if needed and encourage current services

 Support

School Age Dentistry/Orthodontics

School Age Dentistry/Orthodontics • “Soon and Often” – palatal expansion • Do NOT need permanent first molars to cement appliance to; can use well developed primary second molars with full exposed clinical crown • Lots of benefits to doing it early – beyond normalization of palatal width • Suture cleft area is more labile for movement – need fewer teeth anchors for skeletal expansion device • Increased arch length (less crowding) • Earlier est. of skeletal/dental muscular function – less lower jaw muscular shifting as compensation (can cause asymmetric mandible growth) • Speech? Function follows form • Improved nasal airway (wider floor to maxillary sinus) • Etc.

Expansion devices

Modified Haas Butterfly Standard Hyrax Standard Modified Haas

Butterfly Hyrax

Phase I Orthodontic Treatment

• Limited braces to align the upper incisors - always be a part of early treatment for Cleft Cases.

Combined Palatal Expansion & Phase 1 Orthodontic Treatment

Initial Left Cleft Lip & Jaw with Left posterior Crossbite

• Often used in combo with palatal expansion

Post Butterfly Modified Haas RPE

Phase I Orthodontic Treatment-note alveolar molding in the premaxilla with the central incisor alignment

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Combined Palatal Expansion & Phase 1 Orthodontic Treatment

Combined Palatal Expansion & Phase 1 Orthodontic Treatment Skeletal Expansion of Maxilla to Coordinate with Normal Mandible

Initial Bilateral Cleft Lip, Jaw & Palate with Almost No Premaxilla-Missing upper incisors & Severe Constriction & Retrognathia of the Maxilla

Stage 1; Standard Hyrax for width on a very shallow flat palate

2 stages of expansion were indicated to increase the width of the maxilla as much as possible before orthognathic surgery

Advancement of the upper incisors to regain positive overjet and overbite to eliminate a functional Class III development Stage 2; Butterfly Hyrax to widen the maxilla interiorly

Pre-surgery Orthodontics Completed with Decompensated Lower Dentition

Palatal Bone Graft (following expansion)

Plastic Surgeon

• Bone grafting is done as soon as expansion is complete in the mixed dentition before the cuspids erupt so that its eruption may fill in the cleft defect with new alveolar bone.

• harvest the patent’s own bone from the iliac area. There is a 90% success rate with vascularization using iliac donor bone; vascularizes in 2-3weeks (cancellous bone).

School Age SLP – Treatment Issues • Dental/occlusal related – • Class III maloclusion – possible impact on speech • Interdental production of alveolars • Mid palatal production of alveolars using tongue dorsum • Labiodentals – inverted production, exaggerated lower lip movement

• Missing or misplaced teeth – disruptions to tongue placement; filling voids (“function follows form”); lateralizations • Cross bites - Anterior –interdental production of alveolars; tongue dorsum to palate • Open bite – interdental production of alveolars • Dental hardware - Distortions -palatal expanders

School Age SLP – Treatment Issues • Fistula – • Caused by … • Causing… • Hypernasality • Nasal emission • Week oral pressure • Adaptions in tongue placement that might = distortion/substitutions

Q&A

• Separate out fistula vs. VP as cause for symptoms • Covering fistula, surgery

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