Chapter 51 - Cleft Lip and Palate

Question 1

Embryologic cause of cleft lip/palate

Answer 1

failure of fusion between medial nasal prominence and maxillary prominence, the lateral nasal prominence, or both

Question 2

Cleft lip/palate and race incidence

Answer 2

Overall frequency with lip and/or palate 1:700
Cleft lip + palate: higher in native americans, asian, latin american (1:400)
Least often in african american (1:1500-2000)
Cleft palate alone consistent between ethnicities (1:2000)

Question 3

Cleft lip/palate and gender

Answer 3

Females: more palate alone
Males: more lip + palate

Question 4

Incidence of lip/palate separate and together

Answer 4

Usually together (50%), typically left unilateral
Palate alone 35%
Lip alone 15%

Question 5

How often should cleft palate team meet face to face

Answer 5

6x per yer, evaluate 50 pt/yr, operate on 10+ primary lip/palates per year
surgeon, orthodontist, SLP, etc

Question 6

Causes of cleft lip/palate

Answer 6

Syndromic: gene transmission, chromosomal aberration, teratogenic, environmental. >400 syndromes

Also non-syndromic

Question 7

Concordance rate of cleft in mono vs dizygotic twins

Answer 7

Mono: 40-60%
Di: 5%

Question 8

Recurrence rate for lip/pal or isolated pal in families with children born with nonsyndromic

Answer 8

1-16%

Question 9

6 common syndromes with lip/palate

Answer 9

Apert’s (craniosynostosis, syndactyly)
Sitckler’s (face flat, eye, hearing, joint)
Treacher Collins (small jaw/chin, downslant eye, coloboma lower eyelid)
22q11 deletion (DiGeorge, velocardiofacial, Sphrintzen)
Van der Woude…heart, immune, low Ca, retardation)
Goldenhar (hemifacial microsomia, ear/nose/SP/mandible)

Question 10

Robin sequence

Answer 10

French stomatologist
Micrognathia, relative glossoptosis (tongue relatively large comp to mandible), airway obstruction

6.5-10wk gestation relative macroglossia –> tongue high and posterior in OP –> upper airway obstruction at birth, U-shaped cleft palate in most
Rarely isolated, typically occurs in syndrome

Question 11

Which cleft deformity is the one most commonly syndromic

Answer 11

isolated cleft palate

Question 12

Complete vs incomplete cleft lip

Answer 12

complete is muscular diastasis orb oris
observe nostril symmetry, appearance with facial movevment
May have simonart’s band

Question 13

Simonart’s band

Answer 13

thin remnant of tissue, floor nasal vestibule bridging medial and lateral lip elements across cleft

skin/mucosa/SQ +/- mm

Question 14

Primary vs Secondary palate

Answer 14

separated by incisive foramen
Primary is lip, alveolar arch, palate anterior to incisive foramen (premaxilla)
Secondary is HP posterior to IF, SP

Question 15

Primary palate formation

Answer 15

weeks 4-7
4: frontonasal prominence forms (incl nasal placodes)
5: frontonasal prominence elevates, forms medial/lateral nasal prominences around placode
Placode invaginates to form pits
6-7: maxillary prominences enlarge, grow medially, forces medial nasal prominence toward midline

Question 16

Fusion of what forms tip of nose, central upper lip, philtrum lower lip

Answer 16

fusion of both medial nasal prominences

Question 17

Fusion of what forms lateral upper lip and maxilla

Answer 17

medial nasal prominence and maxillary prominence

Question 18

Fusion of what forms nasal alae

Answer 18

lateral nasal prominences with maxillary prominence

Question 19

Formation of secondary palate

Answer 19

Weeks 6.5-10
Grow, shelf elevation, fusion
Outgrowths of maxillary prominences extend vertically downward along tongue
Shelves assume horizontal position above tongue
Palatal shelves then fuse

Question 20

Submucous cleft palate: 3 findings, what it is

Answer 20

diastasis of palate w/ NL mucosa
bifid uvula, midline bluish mucosa/furrow due to abnormal muscle insertion, notch posterior HP

Endoscopy: midline furrow nasal surface of posterior palate

Question 21

Cleft nose deformity

Answer 21

short columella, nasal spine, caudal septum deflected toward non-cleft side
compensatory hypertrophy of cleft side inferior turbinate
LLC cleft side rotated laterally, medial crura collapsed inferiorly, lateral crura collapsed and bucled, leading to deflection of nasal tip toward cleft side
Stenosis/nasal valve collapse on cleft side
hypoplastic maxilla on cleft side, leading to lateralized alar base, wide nares
broad nasal dorsum
horizontal not vertical nostril orientation (basal view)

Question 22

Which position can be effective for children with airway obstruction and cleft? (airway obst due to Robin or other CF abnl)

Answer 22

Prone

most effective if non-syndromic

Question 23

Airway management CF abnl

Answer 23

glossopexy
nasal airway
mandibular distraction osteogenesis
trach

Question 24

Feeding cleft infant

Answer 24

Many can breastfeed/bottle
Palatal insufficiency –> more difficulty generating negative pressure in OC –> expend more energy feeding, long feeding time –> poor WG/dehydrate

Medela special needs feeder (Haberman), Mead Johnson squeeze bottle, Pigeon feeder, Dr. Brown’s nipple/bottle
Palatal obturator/prosthetic

Question 25

Cleft anatomy palatal obturator helps correct

Answer 25

helps correct protruding premaxilla, lengthens columella, reposition lateral maxillary segments, reshape nostril

Question 26

Middle ear disease and cleft

Answer 26

>90% of children <2yo with unrepaired cleft have MEE Variable hearing loss Tube placement in first year of life or sooner if infected effusion or hearing markedly impaired

Question 27

Optimal time for cleft lip repair

Answer 27

2-6 mo Decrease in respiratory complications after anesthesia once 10wk old (must correct for premie) Rule of 10's: At least 10wk, 10lb, Hb 10mg/dL More important: efficient feeding, proven weight gain, good general health predict successful repair

Question 28

Presurgical nasoalveolar molding (NAM)

Answer 28

Anterior palatal obturator progressively modified to move lateral maxillary segments Add attachments to lengthen columella

Question 29

For which types of cleft patients is NAM most beneficial?

Answer 29

wide complete bilateral clefts lip + pal

Question 30

Cleft lip adhesion

Answer 30

Sometimes performed for wide b/l or u/l cleft lip Stage 1 (adhesion)- reapproximate m/l lip elements, orb Converts complete cleft into more easily repaired incomplete Stage 2- formal repair

Question 31

Describe u/l cleft lip techniques (5)

Answer 31

Millard rotation advancement- medial element rotated inferiorly, lateral advanced into resulting upper lip defect, columellar flap used to lengthen columella or create nasal sill Tennison-Randell- medial part lengthened using triangular flap from inf portion lateral part Hagedorn-LeMesurier- quadrilateral flap from lat part lengthens medial part Rose-Thompson- curved/angled pairing of margins lengthens lip to straight line closure Skoog- medial part lengthened with two traingular flaps from lateral part

Question 32

Describe b/l cleft lip technique

Answer 32

Millard- elevate prolabium, reconstitute orb, used to do forked prolabium flaps for later columellar lengthening, but now do primary columellar lengthening, rhinoplasty.

Question 33

What is used for presurgical molding of nasal tip/columella

Answer 33

Help with lengthening columella, tip projection | Acrylic outriggers, orthodontic elastics, tapes attached to palatal appliance

Question 34

Timing of palate repair

Answer 34

Goal is speech, facial growth No repair --> less abnormal growth, but poor speech Repair prior to 12mo has better speech outcomes than around 24 mo and does not have significant negative impact on growth Some centers advocate for repair as early as 7 mo (7-12)

Question 35

4 common methods of cleft palate repair (name only)

Answer 35

Two-flap palatoplasty Wardill-Kilner V-Y advancement Von Langenbeck palatoplasty Furlow double-opposing Z-plasty

Question 36

4 postop complications of cleft palate repair

Answer 36

bleed, fistula (5-35%), VPI, Postop upper airway obstruction

Question 37

How many have persistent VPI after palate repair

Answer 37

10-40%

Question 38

Treatment of VPI

Answer 38

SLP, may need fluoroscopy or nasopharyngoscopy to eval speech therapy, oral appliance Surgery: separate OP/NP during speech...pharyngeal flap, sphincter pharyngoplasty, Furlow palatoplasty, posterior pharyngeal wall augmentation

Question 39

Which cleft concerns are addressed at older ages?

Answer 39

``` Elementary school- dental/speech Late elementary school- orthodontic/alveolar cleft repair High school- orthodontic, secondary cosmetic surgery Skeletal maturity (16-18F, 18-21M) orthodontics, orthognatic surgical procedures ```

Question 40

Early vs late cleft nose repair (controversy)

Answer 40

Early: with cleft lip repair, better symmetry, less psychological stress, may have disrupted growth. Late teens: avoid potential growth disturbance, less scarring, avoid multiple surgeries (unexpected changes due to growth) Recent increased support for limited primary rhinoplasty at time of lip repair

Question 41

When did fetal surgery begin? How may OLHNS use fetal surgery?

Answer 41

1981 for life-threatening anomalies (diaph hernia) secure airway for laryngeal atresia, large tumors correct cleft lip/palate (very controversial)

Question 42

Advantages/Disadv of fetal cleft lip/palate repair

Answer 42

ADV: Scar-free wound healing interrupt, correct facial maldevelopments that occur due to the cleft DIS: preterm labor, fetal demise, technical limitations