Cleft Palate Flashcards by Kathryn isaac

what is the epidemiolgy of CP?

CP is a separate epidemiological entity from CL and CLP (which are on a spectrum)

Overall CP is 1:2000, no race distinction
most common cleft is bifid uvula 2/100
this is in contrast to CL/P 1:1000 with race distinctions (higher in asian, less in africain)
CP more common in GIRLS 2:1
Opposite is true for CL/P

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What is the timing + prominences involved in embryologic developement of CP

and pathogenesis of CP

for primary palate
for secondary palate

Palatogenesis week 4-12

Wk 4, MNP and LNP form from the FNP
Wk 4- 6; MxP and MNP and LNP FUSE to form the primary palate and lip - fusion from posterior to anterior
- failure of fusion at wk 6 leads to cleft of 1’ palate+cleft lip/alveolus
Wk 6: MxP forms vertical shelves which lie adjacent to tongue = Lateral palatine Processes (LPP)
wk 7: as neck extends, tongue descends, mandible moves forward and LPP ELEVATE horizontally to be dorsal to tongue . R shelf before L shelf = ADHESION and CONTACT between the shelves
FUSION between shelves with apoptosis of epidermis and mesenchymal penetration - fusion from anterior to posterior

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What is the arterial supply and nerve supply to the hard palate and soft palate

Hard Palate

Artery: Greater Palatine Artery

Nerve: Sensory - GPn via V2

Soft Palate

Artery: AScending Palatine (most important from facial), Lesser Palatine, Ascending pharyngeal, recurrent pharyngeal

Nerve: Motor: 9,10 (all velar muscles PP, PG, Mus.Uvalae, Sup. Constrictor, LVP) EXCEPT TVP by CV 5

Nerve Sensory: V2

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What muscles are more important VeloPharyngeal competance

LVP

Superior constrictor

Musculus uvulae

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Name the muscles of the soft palate and their function

LVP (9,10) - elevate velum superior and posterior, E tube dilatation
- O: petrous bone, Etube
- I: midline velum, at midpoint
TVP (5) - dilate Etube
- O: Etube, Sphenoid, pterygoid process
- I: paaltine aponeurosis, in anterior 1/3 of velum)
Musculus Uvulae (9,10) - elevates uvula
- O: palatine aponeurosis
- I: posteriro Connective tissue
Superior Pharyngeal constrictor (9,10) - move lateral pharyngeal walls medially
- O: midline raphe
- I: pteryogomandibular raphe
Palatoglossus (9,10) - elevate tongue
- O: palatine aponeurosis
- I: anterior tonsillar pillar and tongue
Palatopharyngeus (9,10)- depress velum, pull inferior pharynx upward
- O: palatine aponeurosis
- I: posterior tonisillar piallar, lateal/posterior pharyngeal wall

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List the aberrant anatomic features of the cleft Palate (Bone, muscle, fascia)

Fascia
- no palatine fascia on cleft side
Bone
- Vomer: failed fusion with palatine shelves
  - Unilat CLP - Vomer atatched to non cleft palate shelf fro entire length
  - Bilat CLP - Vomer only attached aneriorly at premaxilla
  - Isolated CP - vomen atatched as far atnerio as is start of cleft
Muscle
- LVP - orgin same, insertion - runs parallel and along medial margin of cleft (with PG, PP). Can be separated from tensor white fibers
- TVP - origin same - runs along hamulus but no palatine aponeurosis - instead has tendon insertion lateral to LVP/PP/PG at posterior palatine shelf
- PP - inserts with PG and LVP at posterior edge of hard palate

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How do you classify CP?

By Anatomy
- Veau
- Kernahan and stark Y
- LAHSHAL
By Etiology
- non-syndromic
- syndromic

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Describe the veau classificaiton of CLP

SP = 1

SP/HP = 2

Unilat CLP = 3

Bilat CLP = 4

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Describe the Kernahan stark y classification

Classified according to embryology
- Y point is incisive foramen to separate 1’ and 2 palate
1/4 = lip
2/5 = alveolus
3/6 = HP anterior to incisve foramen
7/8 - ant/post half of HP
9 = SP
Millard added triangel for nasal deformity

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What are causes/etiologies of CP?

Environmental
- Meds (isotretinoin, diazepa, valproate/phynitoin, steroids
- Smoking
- Maternal infection (toxo rub CMV)
- Nutrition (vit b6,a)
- Alcohol
Non-syndromic (geenticcs+environmental)
Chromosomal
- trisomy 21, 13
- microdeletion 22q11 (digeorge, VCF, Conotruncal anomaly face syndrome)
Syndromic (VolkSWagon GT catching Pierre Klip Ed)
- Van der woude
- Stickler
- Waardenberg
- Goldenhar
- Treacher collins
- VCF CATCH - most common submucous cleft
- EED - ectrodactyly, ectrodermal dysplasia clefting
- Klippel Feil
- PRS
  *

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What is Velocardio facial syndrome

due to microdletion of 22q11
Clinical features
- C- cardiac anomalie
- A- abnormal facies
- T- thymic aplasia
- C-Cleft palate
- H- hypocalcemia
Most common clefting syndrome
has medialized carotids

CARDIAC= VSD, TOF

Abn FACIES= long face w VME. flat zygoma, small alae, long lip and philtrum, retrognathia

Thymic aplasia = 10% of VCF have digeroge (3/4 BA devlopmental issue w thymic aplasia, hypoPTH, conotruncal anomalie)

CLEFT PALATE - most common submucous cleft (triad of bifid uvula, rdige notch in poseriro palate, decussation/diastasis of velar musculature

Hypocalcemia = low PTH

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What percentage of CLP vs CP are syndromic and no syndromic?

CP is more likley syndromic
- 40% of CP are syndromic
- 15% of CLP are syndromic
CLP is more likely non-syndromic
- 85% of CLP are non-syndromic
- 60% of CP are non-syndormic

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What is Klippel Fell syndrome

cogenital fusion of Cspine & CP

scoliosis, spina bifida

heart, renal, deformities

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What are issues to address with parents once child with CP is born

Feeding
- Haberman
Airway
- mainly for PRS
Middle Ear disease
- myringotomy+silastic/grommet tubes
Etiology (syndromic vs non-syndromic)
- syndromic in 40% CP and 15% CLP
- full Hx/PE, investigation heart/skeletal, renal
Sequelae of unrepaired CP
- poor speech, facial growth disturbance
Treatment plan
- prenatal - counseling, geentic/prenatal
- birth-4wks - diagnosis, w/u for other abN, feednig, airway, orthodontics
- 3-6month - 1’ lip
- 9mth - prepalate ENT consult
- 9-14 mths - 1’ palate
- 2.5yo - Speech
- 5-6yo - VPI/secondary lip/nose
- 9-11 alveolar BG
- Orthognathic/rhino - F 16 M 18

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What is the predicted recurrence of another child with CLP/CP

Cleft Palate

1child 2%
1 parent 7%
1 child and parent 15%

CLP

1child 4%
1 parent 4%
1 child and parent 15%

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What are 2 factors to consider in CP surgery

Speech
Facial growth
- note; CP/CLP differ on affect of maxillary growth
  - alveolus repair restricts max growth but isolated palate repair doesnt

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What are the goals of CP surgery

close cleft
recreate VP sphincter (for speech and feeding)
Improve Etube fux
minimize effect on facial growth and dentoalveolar growth

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What are the adv/disad of performing the palate repair early (3-9mths) versus late

EARLY 3-9MTHS

Adv: less speech problem, less pharyngoplasty
DisAdv: technically harder b/c smaller, high rate A/W problems

MIDRANGE 9-14MTHS * AT HSC

adv/disadv of both

LATE (SP only ay 6-12mth + obturator) then HP at 6-14yr

Adv: less facial growth impedance
DisAdv: more problems with speech, hearing, chronic ear infection

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What are surgical options for Repair of the Cleft Hard Palate

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Von Lagenbeck
Veau-Wardill-Kilner “puch back”
Hybrid (Clarke)
Two-flap (Bardach)
No palatal insicions (Sommerlad)

Describe briefly the technique and use of each HP cleft repair

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Von lagenbeck
- bipedicled mucoperiosteal flaps
- Use: Complete CP
- No lengthening, requires relaxing incisions ???higher VPI rate/poor speech
Veau WardillKIlner push back
- axila MC flaps based on greater palatine artery
- Use: Incomplete CP only
- Lengthening achieved, high Max growth restriction b/c scarring of denuded bone ???hgihger fistula
Hybrid (Clarke)
- bipedicled MC flap on noncleft isde and axial unipedicled VWK flap on cleft side
- Use: Complete unilat
- Lengthens cleft side
Two flap Bardach
- similar to VWK unilat flaps except no puchback
Sommerlad
- Superioyly based vomerine flap - single layer closure of hard palate
- Use: complete unilat CP

Describe technique of both options for repair of a cleft SP

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Furlow Double opposing Zplasty
- used for :
  - submous cleft
  - to lengthen palate
  - to correct VPI
- Adv: restores normal anatomy
- Disadv: difficul in wide clefts
Intravelar velopalsty
- Used for any SP cleft
- Used in conjunciton with HP repair
  - divide TVP and LVP muscle insertion at HP and recreate sling

What are your treatemtn otions for a incomplete cleft of the SP

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furlow
IVV

What are your treatment options for an incomplete cleft of hard and soft palate

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Von lagenbeck with med/lat relaxing incisions
Veau Wardill Kilner
Two flap Bardach

+ IVV or furlow

What are your treatment options for a complete cleft palate that is unilateral and bialteral

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Unilateral

Hybrid = Veau+VLb + Vomer + IVV
2 flap bardach
Sommerlad
All with IVV

Bilateral

Veau= bilateral Veau, bilateral vomerine flaps, anterior V flap of 1’ HP AND IVV or furlow

What are complications following cleft palate repair

EARLY * Hemorrhage (especially VWK) * Airway obstruction (tongue stitch, NP tube) * dehiscence * INfection * mortality LATE * Palatal fistula * Poor speech outcome (1/3 have good speech, 1/3 need SLP, 1/3 need SLP + VPI OR) * Sleep apnea * disrupted facial growth

Where are palatal fistulas located, how do they present and what is the etiology

* Most commonly immediate post-alveolar or in HP * Most commonly assocaited w severity of cleft BCLP\>UCLP\>CP * Present w nasal crusting, nasal emissions, nasal regurg * Etiology * post- op - trauma, infection, hematoma, techqnieu * not yet repaired

what options for treatment of Palatal alveolar fistula

* Non-surgical - obturator * Surgical * two layer closure w * palatal ucoperiosteal flap * tongue flap * buccal flap * free flap RFF

What is required for proper speech

* airstream * functional VP sphincter * lip/tongue movement * normal occlusal/skeletal relations * normal hearing/intelligence

What are features of a Cleft palate speech

* Hypernasality * Difficulty with pressure consonants (fricative/plosive) and compensating for this * error in tongue and lip placement * Nasal emissions dugin consonants

What are fricative and plosive consonents

Fricative Th, V, F, S, Z, Sh Plosive P B K D T G

How would you manage a patient presenting with VPI

History * FHx clefting/VPI * Nasal regurg/emission * ObSA (daytime sleepiness, snoring) * exercise intolerance, mouth breathing * Difficulty with hearnig, speaking * ear infections * Hx of SLP * PMHx, surgery, adenoidectomy/tonsillectomy Exam * fistula, SMC, bifid uvula, tonsil/adenoid, denttiion Nasoendoscoy Radiography

What is VPI?

Dysfunction of the velopharyngeal sphinter to close during speech/eating and open for nasal emissions

How is VPI diagnosed?

Dx is made based on history (hypernasality, nasal regurg, compensatory articulations Invstigations (abnormal closure pattern on nasoendoscopy or videofluoroscopy

What is the etiology or DDX of VPI

STRUCTURAL * Palatopharyngeal mismatch (repaired cleft, tonil/adenoid removed, submucous clef) * abN Levator (submucous cleft) * oronasal fistula * adenoid/tonisl hypertrophy * tumor FUNCTIONAL * developmental delay, hearing D * CNS paresis (infection mningitis/encephalitis), CP

What investigations are done for VPI

* nasometry * Nasoendoscopy * Video flurometry * Oral nasal acoustis radio

What closure patterns are seen on nasoendosopy

* coronal - velum closing * sagittal - lateral pharyngeal walls closig * circular - velum and lat pharyngeal wall * circular _ passavant ridge = all velum, lat wall and posteriro pharyngal wall

What are options for management of VPI?

ALL OPTIONS REQUIRE SLP Non-operative * obturator * paaltal lift Operative * Lengthen/reconstruct soft palate * furlow * IVV * V-Y pusch back * VP narrowing procedure * pharyngeal flap * sphincter pharyngoplasty * posterior pharyngela wall augmentation

How will you manage the VPI patient and based on what

Cloure rating and patterm dictate management\*\* Timing age 5-6 * 2’ Furlow Palatoplasty - for closure rate \>0.9 and small central defects * double opposing z plasty - to lengthen velum * Pharyngeal flap - for noncoronal closure patterns * superior based pharyngeal wall w superior pharyngeal constrictors are raised and secured to velum - * Sphicter pharyngoplasty - for coronal closure pattesm

What are options for management of an alveolar bone cleft?

* Presurgical orthotics (NAM) and gingivoperiosteoplasty * Alveolar bone grafting * primary age 0-2 * secondary age 7-12 * LeFort osteotomy 2piece * Distraction osteogenesis of palate (interdental) * Dental rehab (bridge/Osseointegrated implants)

What are the pros/cons of NAM+GPP, ABG early vs late

* NAM GPP- arches aligned until muscosa abutting. GPP stuulates bone growth * pro - reduced rate of needing 2’ ABG * con - may constrict arch w molding, GPP-stimulated bone of lesser quality, potential risk of tooth buds * primary ABG early ag 0-2 - after CL and before CP * pro - early closure of fistula, less time needed w orthotics * secondary ABG late 6-12 for mixed dentition and lat incisor eruption or 11-22 for canine eruption. Use ABG. Good for tooth movement, tooth eruption

What is a submuous cleft

due to failure of fusion of velar musculature post palatal shelf fusion

Classic triad of SMCP

* bific uvula * notched hard palate * diastasis of velar musculature (zona pellucida

What is the presentation and treatment of SMCP

50% have VPI, Treatment - investigate for VPI and treatment as VPI * SLP and * non-op vs * op: furlow, push back V-Y, pharyngeal flap, sphincter pharyngoplasty

What secondary deforities are CLP pateints at risk for

* maxillary hypoplasia (Vertical hegith restriction and max arch constriction) * midface retrusion * class 2,3 malocclusion * lower face increased height * residual Oronasal fistula * nasal defomrities

What are orthognathic options for correction of secondary CLP deformities

* LeFort 1 (1 or 2,3 pieces) to correct malocclusion, midface/max hypoplasia/retrusion, close ORF, stbailize arches * Lefort 2/3 * BSSO, genioplasty

Cleft Palate Flashcards

(46 cards)