ppt
DESCRIPTION
TRANSCRIPT
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Clefts of the Lip, Alveolus and Palate
Michael E. Prater, MDNorman R. Friedman, MD
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Overview
Introduction Basic Science Timetable of Events
• neonatal• toddler• gradeschool• teenage
Surgical Procedures Conclusion/Future Directions
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Introduction
A TEAM APPROACH IS REQUIRED• pediatrician• surgeon• OMFS• dentist• ENT• psychiatrist• speech• nurse coordinator
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Introduction
Most common congenital malformation of H and N (1:1000 in US; 1:600 in UK)
Second most common overall (behind club foot)
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Epidemiology
Syndromic CLAP associated with more than 300
malformationsPierre Robin Sequence; Treacher-Collins,
Trisomies 13,18,21, Apert’s, Stickler’s, Waardenburg’s
Nonsyndromic CLAP diagnosis of exclusion
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Syndromic CLAP
Single Gene Transmission trisomies 21, 13, 18
Teratogenesis fetal alcohol syndrome Thalidomide
Environmental factors materal diabetes amniotic band syndrome
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Epidemiology: continued
Isolated cleft palate genetically distinct from isolated cleft lip or CLAP same among all ethnic groups (1:2000, M:F
1:2)Isolated CL or CLAP
different among ethnic groupsAmerican Indians: 3.6:1000 (m:f 2:1)Asians 3:1000 (m:f 2:1)African American 0.3:1000 (m:f 2:1)
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Embryology
Primary versus secondary palate divided by incisive foramen
primary palate develops 4-5 wkssecondary palate develops 8-9 wks
Primary palate mesodermal proliferation of frontonasal
and maxillary processes never a cleft in normal development
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Embryology: continued
Secondary palate medial ingrowth of lateral maxillae with
midline fusion always a cleft in normal development
macroglossia, micrognathia may provide anatomical barriers to fusion
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Classification
Veau Classification - 1931 Veau Class I: isolated soft palate cleft Veau Class II: isolated hard and soft
palate Veau Class III: unilateral CLAP Veau Class IV: bilateral CLAP
Iowa Classification - a variation of Veau Classification
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Classification; continued
Complete Clefts absence of any connection with
extension into nose vomer exposed
Incomplete Clefts midline attachment (may be only
mucosal)ex: submucous cleft (midline diasthasis, hard
palatal notch, bifid uvula)
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Anatomy - Normal
Lip: “Cupid’s Bow”Maxilla
primary/secondary palates
soft palate alveolus maxillary tuberosity hamulus
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Anatomy: palatal muscles
Superior constrictor– primary
sphincterTensor veli palatini
– tenses palateLevator Veli palatini
– elevates palate– dilates ET
Salpingopharyngeus, palatopharyngeous, palatoglossus: minor contribution
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Cleft Anatomy
Unilateral Cleft Lip and alveolus
lack of mesodermal proliferation
• cleft of orbicularis– medial portion
to columella– lateral portion
to nasal ala• cleft of alveolus
– alveolar bone graft
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Cleft Anatomy - The Nose
Ipsilateral LLC flattened rotated downward
Short columellaBifid tip
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Cleft Antatomy: continued
Bilateral Cleft Lip/Alveolus/nose duplication of
unilateral defectpremaxillaorbicularis to alar
cartilages bilaterallybifid tipextremely short
columella
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Cleft Anatomy: continued
Clefts of the primary hard palate/alveolus cleft alveolus always associated with
cleft lip cleft lip not necessarily associated with
cleft alveolus by definition there is opening into nose
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Cleft Anatomy: continued
Clefts of secondary palate
Failure of medial growth maxillae
• fusion at incisive foramen
• macroglossia Submucous vs.
completeVomer
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Multidisciplinary Approach
These are not merely surgical problems Requires team approach throughout life
neonatal periodtoddler grade schooladolescenceyoung adulthood
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The Neonatal Period
Pediatrician: directs care establishes feeding
complete clefts preclude feeding
• breast feeding not possible
• a soft, large bottle with large hole is required
• a palatal prosthesis may be required
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The Neonatal Period
Presurgical Orthodontics (Baby Plates)
• Molds palate into more anatomically correct position
• decreases tension• may improve facial
growth• Grayson,
presurgical nasal alveolar molding (PSNAM)
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The Neonatal Period
Surgical Repair Cleft Lip
In US - “the rule of tens” - 10 wks, 10 lbs, Hgb 10
Lip adhesion vs baby plates
Cleft PalateVaries from 6-18 months - most around 10 moEarly repair may lead to midface retrusionEarly repair improves speech
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The Toddler Years
Priority: Speech “Cleft errors of speech” in 30%
primary defects - due to VPI (hypernasality)• consonants are most difficult sounds (plosives)
secondary defects - due to attempted correction• glottic stops, nasal grimace
Velopharyngeal insufficiencydiagnosed by fiberoptic laryngoscopy or BaSwsurgical repair after failed speech therapy -
usually around age 4
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The Toddler Years
Growth hormone deficiency 40 times more common in CLAP suspects when below 5% on growth
chart
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The Grade School Years
Three primary issues Orthodontics
poor occlusioncongenitally absent teeth
alveolar bone graftingfills alveolar defect - around age 12
psychological growthconsidered standard of care
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The Teenage Years
Midface retrusionetiology - ?early palatal repairsurgical correction around age 18
Psychological developmentcounseling standard of care
Rhinoplastyusually last procedure performed, around
age 20
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Surgical Techniques
Cleft Lip Repair unilateral
rotation-advancement flap developed by Millard
complications• dehiscence
– infection• thin white roll
– excess tension
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Surgical Techniques
Cleft Lip Repair bilateral
bilateral rotation advancement with attachment to premaxilla mucosa
complications• dehiscence • thin white roll
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Surgical Techniques
Velopharyngeal Incompetnece superior based
pharyngeal flap sphincter
pharyngoplasty • palatopharyngeus
complications• continued VPI• stenotic side ports
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Surgical Techniques
Alveolar Bone Grafting iliac crest bone
graft complications
infected donor site• hematoma
failed graft• dehiscence• palatal prosthesis
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Surgical Techniques
Midfacial Advancement LeForte osteotomies
leave vascular pedicle attached in back of maxilla - prevents necrosis
complications• malocclusion• infection• necrosis
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Surgical Techniques
Rhinoplasty standard
techniquestip projectionalar rotationcolumellar length
complicationsalar stenosis
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Controversies: Otologic Disease
>90% have COMERobinson, et al
• prospective, 150 patients - 92%
Muntz, et al.• retrospective, 96%
Pathology: ETD (controversial)abnormal muscular attachmentHuang, et al. - Cadaveric study
• palatal repair restores ET function. ?Midface growth?
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Controversies:Timing of Repair
Early repairAdvantage: improved speech
• Rohrich, et. al; retrospective study. The earlier the repair, the better speech.
Disadvantage: worsening midface retrusion• Rohrich, et. al; people with unrepaired palates
have less midface retrusion
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Controversies: VPI
Surgical Repair Reserved for failure of speech pathology Pharyngeal Flap - superiorly based
Advantage: time tested, severe casesDisadvantage: passive obturator
Sphincter Pharyngoplasty (palatopharyngeus rotation flap)Advantage: active sphincterDisadvantage: new technique
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Controversies
Presurgical Nasal Alveolar Molding molds palate,
alveolus and noseAdvantage:
excellent early results
Disadvantage: no long term results
Grayson, et al.
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Conclusion andFuture Directions
Multidisciplinary approachNot merely a “surgical problem”Alveolar bone graftingPSNAMPharyngoplasty vs. pharyngeal flap