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ENT Secrets > Chapter 51 - Cleft Lip and Palate > Flashcards

Chapter 51 - Cleft Lip and Palate Flashcards

1
Q

Embryologic cause of cleft lip/palate

A

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

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2
Q

Cleft lip/palate and race incidence

A

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)

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3
Q

Cleft lip/palate and gender

A

Females: more palate alone
Males: more lip + palate

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4
Q

Incidence of lip/palate separate and together

A

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

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5
Q

How often should cleft palate team meet face to face

A

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

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6
Q

Causes of cleft lip/palate

A

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

Also non-syndromic

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7
Q

Concordance rate of cleft in mono vs dizygotic twins

A

Mono: 40-60%
Di: 5%

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8
Q

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

A

1-16%

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9
Q

6 common syndromes with lip/palate

A

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)

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10
Q

Robin sequence

A

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

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11
Q

Which cleft deformity is the one most commonly syndromic

A

isolated cleft palate

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12
Q

Complete vs incomplete cleft lip

A

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

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13
Q

Simonart’s band

A

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

skin/mucosa/SQ +/- mm

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14
Q

Primary vs Secondary palate

A

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

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15
Q

Primary palate formation

A

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

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16
Q

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

A

fusion of both medial nasal prominences

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17
Q

Fusion of what forms lateral upper lip and maxilla

A

medial nasal prominence and maxillary prominence

18
Q

Fusion of what forms nasal alae

A

lateral nasal prominences with maxillary prominence

19
Q

Formation of secondary palate

A

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

20
Q

Submucous cleft palate: 3 findings, what it is

A

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

21
Q

Cleft nose deformity

A

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)

22
Q

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

A

Prone

most effective if non-syndromic

23
Q

Airway management CF abnl

A

glossopexy
nasal airway
mandibular distraction osteogenesis
trach

24
Q

Feeding cleft infant

A

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

25
Q

Cleft anatomy palatal obturator helps correct

A

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

26
Q

Middle ear disease and cleft

A

> 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

27
Q

Optimal time for cleft lip repair

A

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

28
Q

Presurgical nasoalveolar molding (NAM)

A

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

29
Q

For which types of cleft patients is NAM most beneficial?

A

wide complete bilateral clefts lip + pal

30
Q

Cleft lip adhesion

A

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

31
Q

Describe u/l cleft lip techniques (5)

A

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

32
Q

Describe b/l cleft lip technique

A

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

33
Q

What is used for presurgical molding of nasal tip/columella

A

Help with lengthening columella, tip projection

Acrylic outriggers, orthodontic elastics, tapes attached to palatal appliance

34
Q

Timing of palate repair

A

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)

35
Q

4 common methods of cleft palate repair (name only)

A

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

36
Q

4 postop complications of cleft palate repair

A

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

37
Q

How many have persistent VPI after palate repair

A

10-40%

38
Q

Treatment of VPI

A

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

39
Q

Which cleft concerns are addressed at older ages?

A 
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
40
Q

Early vs late cleft nose repair (controversy)

A

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

41
Q

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

A

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

42
Q

Advantages/Disadv of fetal cleft lip/palate repair

A

ADV: Scar-free wound healing
interrupt, correct facial maldevelopments that occur due to the cleft

DIS: preterm labor, fetal demise, technical limitations

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