Chapter 51 - Cleft Lip and Palate Flashcards
Embryologic cause of cleft lip/palate
failure of fusion between medial nasal prominence and maxillary prominence, the lateral nasal prominence, or both
Cleft lip/palate and race incidence
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)
Cleft lip/palate and gender
Females: more palate alone
Males: more lip + palate
Incidence of lip/palate separate and together
Usually together (50%), typically left unilateral
Palate alone 35%
Lip alone 15%
How often should cleft palate team meet face to face
6x per yer, evaluate 50 pt/yr, operate on 10+ primary lip/palates per year
surgeon, orthodontist, SLP, etc
Causes of cleft lip/palate
Syndromic: gene transmission, chromosomal aberration, teratogenic, environmental. >400 syndromes
Also non-syndromic
Concordance rate of cleft in mono vs dizygotic twins
Mono: 40-60%
Di: 5%
Recurrence rate for lip/pal or isolated pal in families with children born with nonsyndromic
1-16%
6 common syndromes with lip/palate
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)
Robin sequence
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
Which cleft deformity is the one most commonly syndromic
isolated cleft palate
Complete vs incomplete cleft lip
complete is muscular diastasis orb oris
observe nostril symmetry, appearance with facial movevment
May have simonart’s band
Simonart’s band
thin remnant of tissue, floor nasal vestibule bridging medial and lateral lip elements across cleft
skin/mucosa/SQ +/- mm
Primary vs Secondary palate
separated by incisive foramen
Primary is lip, alveolar arch, palate anterior to incisive foramen (premaxilla)
Secondary is HP posterior to IF, SP
Primary palate formation
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
Fusion of what forms tip of nose, central upper lip, philtrum lower lip
fusion of both medial nasal prominences
Fusion of what forms lateral upper lip and maxilla
medial nasal prominence and maxillary prominence
Fusion of what forms nasal alae
lateral nasal prominences with maxillary prominence
Formation of secondary palate
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
Submucous cleft palate: 3 findings, what it is
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
Cleft nose deformity
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)
Which position can be effective for children with airway obstruction and cleft? (airway obst due to Robin or other CF abnl)
Prone
most effective if non-syndromic
Airway management CF abnl
glossopexy
nasal airway
mandibular distraction osteogenesis
trach
Feeding cleft infant
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
Cleft anatomy palatal obturator helps correct
helps correct protruding premaxilla, lengthens columella, reposition lateral maxillary segments, reshape nostril
Middle ear disease and cleft
> 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
Optimal time for cleft lip repair
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
Presurgical nasoalveolar molding (NAM)
Anterior palatal obturator progressively modified to move lateral maxillary segments
Add attachments to lengthen columella
For which types of cleft patients is NAM most beneficial?
wide complete bilateral clefts lip + pal
Cleft lip adhesion
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
Describe u/l cleft lip techniques (5)
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
Describe b/l cleft lip technique
Millard- elevate prolabium, reconstitute orb, used to do forked prolabium flaps for later columellar lengthening, but now do primary columellar lengthening, rhinoplasty.
What is used for presurgical molding of nasal tip/columella
Help with lengthening columella, tip projection
Acrylic outriggers, orthodontic elastics, tapes attached to palatal appliance
Timing of palate repair
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)
4 common methods of cleft palate repair (name only)
Two-flap palatoplasty
Wardill-Kilner V-Y advancement
Von Langenbeck palatoplasty
Furlow double-opposing Z-plasty
4 postop complications of cleft palate repair
bleed, fistula (5-35%), VPI, Postop upper airway obstruction
How many have persistent VPI after palate repair
10-40%
Treatment of VPI
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
Which cleft concerns are addressed at older ages?
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
Early vs late cleft nose repair (controversy)
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
When did fetal surgery begin? How may OLHNS use fetal surgery?
1981 for life-threatening anomalies (diaph hernia)
secure airway for laryngeal atresia, large tumors
correct cleft lip/palate (very controversial)
Advantages/Disadv of fetal cleft lip/palate repair
ADV: Scar-free wound healing
interrupt, correct facial maldevelopments that occur due to the cleft
DIS: preterm labor, fetal demise, technical limitations
