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Clinical Aspects of Cleft Lip/PalateClinical Aspects of Cleft Lip/Palate
ReconstructionReconstruction
ANJAN K DEBANJAN K DEB
Dept of Plastic surgery BIRDEMDept of Plastic surgery BIRDEM
OverviewOverview
• Relevant Anatomy & PhysiologyRelevant Anatomy & Physiology
• Embryology of Facial CleftingEmbryology of Facial Clefting
• Classification/Epidemiology/Related SyndromesClassification/Epidemiology/Related Syndromes
• Principles of ManagementPrinciples of Management
• Preoperatve Assessment/ OrthodonticsPreoperatve Assessment/ Orthodontics
– Indications/ContraindicationsIndications/Contraindications
• Time table of Procedures/EventsTime table of Procedures/Events
• Surgical TechniquesSurgical Techniques
– Millard / Tennison-RandallMillard / Tennison-Randall
– Wardill-Kilner/ Z-plastyWardill-Kilner/ Z-plasty
– Speech assessment/PharyngioplastySpeech assessment/Pharyngioplasty
– Alveolar bone graftAlveolar bone graft
• Post-op managementPost-op management
– Complications
– Follow up Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Applied AnatomyApplied Anatomy
Normal LipNormal Lip
CENTRAL PHILTRUM
Lateral margins
Philtral columns
Inferior border
Cupids bow and tubercle
VERMILLION-CUTANEOUS
BORDER
White roll
COMMISURE
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Applied AnatomyApplied Anatomy
• PHILTRAL ZONE
– Column/Dimple
• VERMILION
– Cupid’s Bow/Apex
• SUB VEMILION
• PERISTOMAL
– Wet & Dry Line
• COMMISURE
• LATERAL
• CENTRAL
Applied AnatomyApplied Anatomy
• Glabella
• Root Or Nasion
• Dorsum Or Rhinon
• Ala (3)
• Columella (2)
• Tip (8)/ Infratip (1)
• Soft Triangle/Facet (4)
• Nasal Sill (5)
• Columella-philtral Angle (6)
• Alar –Facial Groove (7)
Applied AnatomyApplied Anatomy
MUSCLES
Orbicularis oris (superficial and deep)
Levator labii superioris
Levator superioris alaeque
Levator anguli oris
Zygomaticus major et minor
Buccinator
Depressor labii inferioris
Depressor anguli oris
Mentalis
Disruption of the normal termination of the muscle fibers that cross the embryologicDisruption of the normal termination of the muscle fibers that cross the embryologic
fault line of the maxillary and nasal processes, resulting in abnormal insertion &fault line of the maxillary and nasal processes, resulting in abnormal insertion &
abnormal muscular forces between the normal equilibrium that exists with theabnormal muscular forces between the normal equilibrium that exists with the
nasolabial and oral groups of musclesnasolabial and oral groups of muscles
Applied AnatomyApplied Anatomy
MUSCLES OF LIP INNER VIEWMUSCLES OF LIP INNER VIEW
Applied AnatomyApplied Anatomy
BLOOD SUPPLY LIPSBLOOD SUPPLY LIPSDORSAL NASALDORSAL NASAL
FACIALFACIAL
INF ALVEOLAR terminal brINF ALVEOLAR terminal br
INFERIOR LABIALINFERIOR LABIAL
SUPERIOR LABIALSUPERIOR LABIAL
INFRA ORBITALINFRA ORBITAL
MAXILLARYMAXILLARY
POST SUP ALVEOLARPOST SUP ALVEOLAR
BUCCALBUCCAL
Applied AnatomyApplied Anatomy
BLOOD SUPPLY PALATEBLOOD SUPPLY PALATE
NASAL SEPTUMNASAL SEPTUM
TURBINATESTURBINATESSPHENOPALATINESPHENOPALATINE
GREATER PALATINEGREATER PALATINE
ANASTOMOSIS POSTERIOR SEPTAL & GR PALATINEANASTOMOSIS POSTERIOR SEPTAL & GR PALATINE
LESSER PALATINELESSER PALATINE
Applied AnatomyApplied Anatomy
Normal PalateNormal Palate
Primary palatePrimary palate
Secondary palateSecondary palate
Soft palateSoft palate
Hard palateHard palate
Applied AnatomyApplied Anatomy
Applied AnatomyApplied Anatomy
• SUPERIOR CONSTRICTOR
» primary sphincter
• TENSOR VELI PALATINI
» tenses palate
• LEVATOR VELI PALATINI
» elevates palate
» dilates Eust Tube
• Salpingopharyngeus,
palatopharyngeous,
palatoglossus: minor
contribution
Postero-superior view
PHARYNGEAL VIEWPHARYNGEAL VIEW
Muscle of velum 3D view
Embryology of CleftingEmbryology of Clefting
FACIAL DEVELOPMENT - 4FACIAL DEVELOPMENT - 4THTH
- 10- 10THTH
WEEKWEEK
Formed by the fusion ofFormed by the fusion of 55 prominences/processesprominences/processes
FRONTONASAL x1FRONTONASAL x1
Lateral/medial nasal processesLateral/medial nasal processes
MAXILLARY x2
MANDIBULAR x2
Nose/Philtrum of upper lipNose/Philtrum of upper lip
Cheeks/Upper lip (Cheeks/Upper lip (-philtrum-philtrum))
Lower face (lower lip/chin)
Embryology of CleftingEmbryology of Clefting
FACIAL DEVELOPMENTFACIAL DEVELOPMENT
Medial nasal processesMedial nasal processes
migrate toward
each other and fuse
55thth
-7-7thth
weekweek
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Embryology of CleftingEmbryology of Clefting
• 7th
-8th
week
– Inferior tips of medial nasal
processes expand laterally
form the intermaxillary process
– Tips of maxillary swellings
grow & meet the
intermaxillary process and fuse
Failure of maxillary swellings to fuse with intermaxillary process = cleft lipFailure of maxillary swellings to fuse with intermaxillary process = cleft lip
FORMATION OF THEFORMATION OF THE
PALATEPALATE
66thth
-7-7thth
weekweek
As nasal pits of lateral nasalAs nasal pits of lateral nasal
process invaginate and fuse,process invaginate and fuse,
intermaxillary process extendsintermaxillary process extends
to form primary palateto form primary palate
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Embryology of CleftingEmbryology of Clefting
88thth
- 9- 9thth
weekweek
Medial wallsMedial walls ofof
MAXILLARY PROCESSESMAXILLARY PROCESSES
produceproduce
PALATINE SHELVESPALATINE SHELVES
Shelves grow downwards,Shelves grow downwards,
parallel to lateral sufaceparallel to lateral suface
of tongueof tongue
End ofEnd of week 9week 9,, rotaterotate upwardupward
into ainto a
horizontal position andhorizontal position and
fuse with eachfuse with each
otherother andand
primary palateprimary palate toto
FORMFORM
SECONDARY PALATESECONDARY PALATE Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Embryology of CleftingEmbryology of Clefting
Embryology of CleftingEmbryology of Clefting
• MESODERM H&N derived from
– PARAXIAL MESODERMPARAXIAL MESODERM
• Floor Of Brain CaseFloor Of Brain Case
• Occipital & Parietal BonesOccipital & Parietal Bones
• All Voluntary Muscle of H&NAll Voluntary Muscle of H&N
• Dermis & CT of Dorsal HeadDermis & CT of Dorsal Head
– LATERAL PLATE MESODERMLATERAL PLATE MESODERM
• Laryngeal Cartilages & CTLaryngeal Cartilages & CT
– NEURAL CREST CELLSNEURAL CREST CELLS
(deficient in superoxide dismutase & catalase)(deficient in superoxide dismutase & catalase)
• Mid-facial & Pharyngeal Arch skeletonMid-facial & Pharyngeal Arch skeleton
• Overlying Dermis & Soft TissuesOverlying Dermis & Soft Tissues
• DentinDentin
– ECTODERMAL PLACODES
• Neuron of Sensory Ganglia, V,VII,I X, X
CRANIO-FACIAL CLEFT
• Tessier,s Classification
– Spoke wheel
– Radiating from
orbit
– Magic No- 14
– Incidence:
1:150000
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
Great anatomic variationGreat anatomic variation in types of clefts!in types of clefts!
ANATOMIC CLASSIFICATIONANATOMIC CLASSIFICATION basedbased on:on:
1) LOCATION1) LOCATION
2) COMPLETENESS2) COMPLETENESS (Incomplete/Complete)(Incomplete/Complete)
3) EXTENT3) EXTENT
Since lip, alveolus, and hard palate differ in embryologicSince lip, alveolus, and hard palate differ in embryologic
origin,origin,
4) ANY COMBINATION CAN OCCUR4) ANY COMBINATION CAN OCCUR
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
CLEFLIP & PRIMARY PALATECLEFLIP & PRIMARY PALATE
CLEFT OF PRIMARY & SECONDARY PALATECLEFT OF PRIMARY & SECONDARY PALATE
CLEFT OF SECONDARY PALATECLEFT OF SECONDARY PALATE
Veau Classification CLCPVeau Classification CLCP
• 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
Iowa Classification CLCPIowa Classification CLCP
Group IGroup I
Clefts of lip onlyClefts of lip only
Group IIGroup II
Clefts of palate only (Clefts of palate only (22oo
))
Group IIIGroup III
Clefts of lip,Clefts of lip,
alveolus, palatealveolus, palate
Group IVGroup IV
Clefts of lip andClefts of lip and
alveolusalveolus (primary(primary
cleft palate andcleft palate and
lip)lip)
Group VGroup V
MiscellaneousMiscellaneous
Striped Y Modified Kernahan’sStriped Y Modified Kernahan’s
1 & 51 & 5 -- FLOOR OF NOSEFLOOR OF NOSE on right &on right &
left sidesleft sides
2 & 62 & 6 -- LIPLIP
3 & 73 & 7 -- ALVEOLAR RIDGESALVEOLAR RIDGES
4 & 84 & 8 -- PREMAXILLAPREMAXILLA to incisiveto incisive
foramenforamen
9 & 109 & 10 - Each half of the- Each half of the HARDHARD
PALATEPALATE
1111 -- SOFT PALATESOFT PALATE
1212 - Congenital- Congenital VELOPHARYNGEALVELOPHARYNGEAL
INCOMPETENCEINCOMPETENCE without obviouswithout obvious
cleftsclefts
1313 -- PROTRUSIONPROTRUSION of premaxillaof premaxilla
KERNAHANSKERNAHANS
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
Isolated INCOMPLETEIsolated INCOMPLETE
Intact skin/muscle between the lip and noseIntact skin/muscle between the lip and nose
Less distortion brought on by abnormal muscle pullLess distortion brought on by abnormal muscle pull
Bilateral/UnilateralBilateral/Unilateral
CLEFT LIPCLEFT LIP
Expressed in structuresExpressed in structures anterior to incisive foramenanterior to incisive foramen
- prepalatal alveolus, maxilla, lip, nasal structures- prepalatal alveolus, maxilla, lip, nasal structures
GAPING CLEFT of alveolus/lip structures toGAPING CLEFT of alveolus/lip structures to
MERE ‘SCAR’ (MERE ‘SCAR’ (forme frusteforme fruste))
DEFICIENCY INDEFICIENCY IN SKINSKIN,, MUSCLES, MUCOUSMUSCLES, MUCOUS
MEMBRANESMEMBRANES, MAXILLARY/NASAL, MAXILLARY/NASAL BONESBONES, NASAL, NASAL
CARTILAGESCARTILAGES
ISOLATED COMPLETEISOLATED COMPLETE Bilateral/UnilateralBilateral/Unilateral
Cleft runs entire length of lip to floor of noseCleft runs entire length of lip to floor of nose
Abnormal muscle pull distorts nose extensivelyAbnormal muscle pull distorts nose extensively
and creates wide clefts between the lipand creates wide clefts between the lip
segmentssegments
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Ipsilateral Lower Lat CartIpsilateral Lower Lat Cart
flattenedflattened
rotated downwardrotated downward
Bifid tipBifid tip
Short columellaShort columella
Flattened alaFlattened ala
Cleft of alveolusCleft of alveolus
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
ISOLATED CLEFT PALATEISOLATED CLEFT PALATE
COMPLETE/INCOMPLETE/SUBMUCOUSCOMPLETE/INCOMPLETE/SUBMUCOUS
Soft PalateSoft Palate
cleft can extend into the hard palate tocleft can extend into the hard palate to
any extentany extent
Hard PalateHard Palate
Primary PalatePrimary Palate
Secondary PalateSecondary Palate
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
COMBINED CLEFTSCOMBINED CLEFTS
Complete lip &/palate UnilateralComplete lip &/palate Unilateral
Cleft Lip & Palate VariantsCleft Lip & Palate Variants
Complete lip &/palate BilateralComplete lip &/palate Bilateral
PROTRUDED PEMAXILLAPROTRUDED PEMAXILLA
EpidemiologyEpidemiology
• Isolated CLEFT PALATE genetically distinct
from isolated CLEFT LIP OR CLAP
– same among all ethnic groups (1:2000, M:F 1:2)
– More assoc with Syndrome
• Isolated CL or CLAP
– different among ethnic groups
• American Indians: 3.6:1000 (M:F 2:1)
• Asians 3:1000 (M:F 2:1)
• African American 0.3:1000 (M:F 2:1)
• 20% CL (18% unilateral, 2% bilateral)20% CL (18% unilateral, 2% bilateral)
• 50% CL and CP (38% unilateral, 12% bilateral)50% CL and CP (38% unilateral, 12% bilateral)
• 30 % CP alone30 % CP alone
EpidemiologyEpidemiology
• GENETICS (Clustering in families)
FAMILY MAKEUP RISK OF CLEFT
LIP / PALATE
RISK OF CLEFT
PALATE
ONE AFFECTED
SIBLING OR
PARENT
1 IN 25 (4%) 2.5%
TWO AFFECTED
SIBLING
1 IN 11 (9%) 1%
ONE SIBLING AND
ONE PARENT
1 IN 6 (16%) 15%
EpidemiologyEpidemiology
• SYNDROMIC CLAP
– associated with more than 300 malformations
• CHROMOSOMAL
– Trisomy 13, 18, 21 & Deletion 22q11 (Velocardiofacial Syndrome)
• NON MENDELIAN
– PIERRE ROBIN, Goldenhar
• MENDELIAN
– Corlin’s, Dysplasia-Clefting, Treacher-Collins, Van der Woude (AD)
– Smith-Lemli-Opitz (AR)
– Oto-Palato-digital, Oto-Palato-Facial (XL)
• UNKNOWN
– DeLarge , Kabuki
• TERATOGENIC
– Fetal Alcohol, Phenytoin, Valproate
• NONSYNDROMIC CLAP
• diagnosis of exclusion
• OVERALL INCIDENCE OF ASSOCIATED ANOMALIES (e.g.OVERALL INCIDENCE OF ASSOCIATED ANOMALIES (e.g. CARDIACCARDIAC) = 30%) = 30%
EpidemiologyEpidemiology
• RISK FACTORS
– PARENTAL AGE: >30yrs
– VIRAL INFECTIONS: Rubella
– TERATOGENS: Alcohol, Steroids,
Anticonvulsants, Retinoic acid derivatives
Principles of ManagementPrinciples of Management
• Multidisciplinary Approach
• These are not merely surgical problems
– Requires team approach throughout life
• neonatal period
• toddler
• grade school
• adolescence
• young adulthood
Principles of ManagementPrinciples of Management
MULTIDISCIPLINARY APPROACHMULTIDISCIPLINARY APPROACH
Beyond lip repair are other issues:Beyond lip repair are other issues:
Hearing (Otolaryngologists)Hearing (Otolaryngologists)
Speech (Speech Pathologists / Therapist)Speech (Speech Pathologists / Therapist)
Dental (Periodontologist/Orthodontist / Prosthodontist/)Dental (Periodontologist/Orthodontist / Prosthodontist/)
Maxillofacial Surgeons/Maxillofacial Surgeons/
Psychosocial (Psychologist/ Psychiatrist/ Social Worker)Psychosocial (Psychologist/ Psychiatrist/ Social Worker)
GeneticistGeneticist
Integration with team-based approachIntegration with team-based approach
Each case is assessed independently by those involved and a global treatment planEach case is assessed independently by those involved and a global treatment plan
is instituted based on present need in his/her developmentis instituted based on present need in his/her development
Cleft Lip and PalateCleft Lip and Palate
Nutrition (Nutritionist )Nutrition (Nutritionist )
Principles of ManagementPrinciples of Management
ASSESSMENTASSESSMENT
AIMAIM: RESTORING NORMAL MORPHOLOGIC FORM AND FUNCTION: RESTORING NORMAL MORPHOLOGIC FORM AND FUNCTION
Important forImportant for normal dentition, mastication, speech, hearing,normal dentition, mastication, speech, hearing, andand breathingbreathing
CONTRAINDICATIONSCONTRAINDICATIONS: MALNUTRITION, ANEMIA RESPIRATORY: MALNUTRITION, ANEMIA RESPIRATORY
INFECTION or other conditions that render infantINFECTION or other conditions that render infant UNABLE TO TOLERATEUNABLE TO TOLERATE
GENERAL ANESTHESIA Airway obstruction, Acute otitis mediaGENERAL ANESTHESIA Airway obstruction, Acute otitis media
Work-upWork-up
(1) Thorough(1) Thorough PEPE to uncover anyto uncover any ASSOCIATED ANOMALIESASSOCIATED ANOMALIES
Additional work-up determined by physical findings that suggest involvementAdditional work-up determined by physical findings that suggest involvement
ofof other organother organ systemssystems
(2)(2) WEIGHT, ORAL INTAKE, GROWTH/DEVELOPMENTWEIGHT, ORAL INTAKE, GROWTH/DEVELOPMENT
are of primary concern and must be followed closelyare of primary concern and must be followed closely
(3) Routine lab studies generally not required; Hgb level before surgery(3) Routine lab studies generally not required; Hgb level before surgery
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
• Feeding bottle large hole
The Neonatal Period
• Presurgical
Orthodontics (Baby
Plates)
– Moulds palate into
more anatomically
correct position
– decreases tension
– may improve facial
growth
– Grayson, presurgical
nasal alveolar
moulding (PSNAM)
• c
The Neonatal Period
The Neonatal Period
• Surgical Repair
– Cleft Lip
• In US - “THE RULE OF TENS” - 10 wks, 10 lbs,
Hgb 10gm%
• Lip adhesion vs baby plates
– Cleft Palate
• Varies from 6-18 Months - most around 10 mo
• Early repair may lead to MIDFACE RETRUSION
• Early repair improves SPEECH
Different institutions = different practiceDifferent institutions = different practice
Management Schedule
Palatal obturatorPalatal obturator
Repair cleft lipRepair cleft lip
Repair of PalateRepair of Palate
Repair of Hard palateRepair of Hard palate
Tympanotomy tubeTympanotomy tube
Speech therapy/pharyngoplastySpeech therapy/pharyngoplasty
Bone graftingBone grafting
OrthodonticsOrthodontics
Jaw surgeryJaw surgery
Surgical Principles
• Release the musclesRelease the muscles
from abnormalfrom abnormal
insertionsinsertions
• Repair them inRepair them in
anatomical positionanatomical position
• Lengthen medial sideLengthen medial side
of cleft so that itof cleft so that it
attains normalattains normal
anatomical lengthanatomical length
Surgical ManagementSurgical Management
Unilateral Complete Cleft LipUnilateral Complete Cleft Lip
GOALSGOALS
• SYMMETRY: Nostrils,SYMMETRY: Nostrils,
Nasal Sill, and Alar BasesNasal Sill, and Alar Bases
• WELL DEFINEDWELL DEFINED
PHILTRUM: Dimple andPHILTRUM: Dimple and
ColumnsColumns
• CUPID’S BOW: NaturalCUPID’S BOW: Natural
appearingappearing
• FUNCTION: Good muscleFUNCTION: Good muscle
repairrepair
SURGICAL PRINCIPLESSURGICAL PRINCIPLES
• Lengthen medial side ofLengthen medial side of
cleft so that it equals thecleft so that it equals the
vertical dimensions of non-vertical dimensions of non-
cleft sidecleft side
• Flap designs:Flap designs:
– Triangular (Tennison-Triangular (Tennison-
Randall)Randall)
– QuadrangularQuadrangular
– Rotation-advancementRotation-advancement
(Millard)(Millard)
• MuscleRepair:MuscleRepair:
Surgical ManagementSurgical Management
Unilateral Complete Cleft LipUnilateral Complete Cleft Lip
TENNISON-RANDALLTENNISON-RANDALL
Millard TechniqueMillard Technique
• Cleft Lip Repair
• Unilateral
• Rotation(a &c)-advancement
(b)flap developed by Millard
– Medial flap(a)Medial flap(a) rotatesrotates
downward to achieve necessarydownward to achieve necessary
lengtheninglengthening
– Lateral flap (b)Lateral flap (b) advances intoadvances into
the defect produced by downwardthe defect produced by downward
displacement of medial flapdisplacement of medial flap
– Small pennant-shapedSmall pennant-shaped
medial flap (c)medial flap (c)c an be used toc an be used to
restore nostril sill or lengthen therestore nostril sill or lengthen the
columellacolumella
3 Flaps a,b & c
Millard TechniqueMillard Technique
ADVANTAGES
– ““Cut as you go” techniqueCut as you go” technique
– Preserves’ cupid’s bow andPreserves’ cupid’s bow and
philtral dimplephiltral dimple
– Scar placed in anatomical
position along philtral column
– Tension of closure under theTension of closure under the
alar base; reduces flair andalar base; reduces flair and
promotes better molding of thepromotes better molding of the
underlying alveolar processesunderlying alveolar processes
Millard TechniqueMillard Technique
• COMPLICATIONS
– Tightness at white roll/
cupids bow
– Peaking of vermillion
– Notching of stomal
margin
– Residual nasal
deformity
– Tension?/ dehisence?/
HTS?/infection?
– scar stretching
Bilateral Cleft Lip Repair
• MILLARD’S
ADAPTATION:
– Philtral from central
prolabium
– Prolab paring banked for
collumelar lengthening
– Prolabial white roll &
vermillion discarded
– Cupids bow tubercle from
lateral lip segments
Bilateral Cleft Lip Repair
• MODIFIED
MANCHESTER
– Preserves white roll
vermillion of prolabium
– Philtral flap cut to desired
width
– Prolabial paring used for
nasal floor
– Lateral muscle are sutured
to prolabial sub cut tissues
Bilateral Cleft Lip Repair
• BLACK’S METHOD
Post-op ManagementPost-op Management
1) FEEDINGS administered with catheter tip syringe1) FEEDINGS administered with catheter tip syringe
fitted with small red rubber catheter for the first 10 daysfitted with small red rubber catheter for the first 10 days
post-oppost-op
2) AVOID SUCKING Nipples are avoided to minimize2) AVOID SUCKING Nipples are avoided to minimize
strain on the muscle/skin suturesstrain on the muscle/skin sutures
3) ARM RESTRAINT Velcro arm restraints to3) ARM RESTRAINT Velcro arm restraints to
protect repair from flailing hands/fingersprotect repair from flailing hands/fingers
4) SUTURE LINE CARE: PRN cleansing with half4) SUTURE LINE CARE: PRN cleansing with half
strength peroxide followed with polymixin B-bacitracinstrength peroxide followed with polymixin B-bacitracin
ointmentointment
Cleft LipCleft Lip
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Post-op ManagementPost-op Management
Scar contractureScar contracture
ErythemaErythema
FirmnessFirmness
Inform the parents of:Inform the parents of:
Avoid placing in direct sunlight until the scar fully maturesAvoid placing in direct sunlight until the scar fully matures
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
ComplicationsComplications
Post-op ManagementPost-op Management
• AestheticAesthetic
– vermilion-cutaneousvermilion-cutaneous
mismatchmismatch
– vermilion notchingvermilion notching
– Whistle deformityWhistle deformity
– tight appearing lateraltight appearing lateral
lip segementlip segement
– lateral muscle buldgelateral muscle buldge
– laterally displaced alalaterally displaced ala
– constricted appearingconstricted appearing
nostrilnostril
• OtherOther
– dehiscencedehiscence
– excessive scarexcessive scar
formationformation
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Surgical ManagementSurgical Management
Cleft PalateCleft Palate
Goal: Production of a competent velopharyngeal sphincterGoal: Production of a competent velopharyngeal sphincter
Two most common repairs:Two most common repairs:
1) V-Y (Veau-Wardill-Kilner)*1) V-Y (Veau-Wardill-Kilner)*
2) von Langenbeck2) von Langenbeck
Main difference: V-Y repair involvesMain difference: V-Y repair involves elongation of the palateelongation of the palate, while, while
von Langenbeck does notvon Langenbeck does not
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Wardill-KilnerWardill-Kilner
1) Incisions made along free margins of cleft and extend1) Incisions made along free margins of cleft and extend
anteriorly to apexanteriorly to apex
2) Dissection continued posteriorly along oral side of2) Dissection continued posteriorly along oral side of
alveolar ridge to retromolar trigonealveolar ridge to retromolar trigone
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Wardill-KilnerWardill-Kilner
3) Mucoperiosteal flaps are elevated from3) Mucoperiosteal flaps are elevated from
nasal/oral surfaces of bony palatenasal/oral surfaces of bony palate
4) Dissection of the greater palatine vessels from4) Dissection of the greater palatine vessels from
the foramen lengthens the pediclethe foramen lengthens the pedicle
5) Tensor veli palatini muscle is elevated off the5) Tensor veli palatini muscle is elevated off the
hamulus to aid in relaxing the midline closurehamulus to aid in relaxing the midline closure
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Wardill-KilnerWardill-Kilner
6) Nasal mucosa freed from bony palate6) Nasal mucosa freed from bony palate
and closed to either side, or if necessaryand closed to either side, or if necessary
closed by using vomer flapsclosed by using vomer flaps
7) Muscle and oral mucosa closed in a7) Muscle and oral mucosa closed in a
second single layer in a horizontal fashionsecond single layer in a horizontal fashion
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Wardill-KilnerWardill-Kilner
8) Anteriorly, the oral mucoperiosteal flaps are8) Anteriorly, the oral mucoperiosteal flaps are
attached to the third flap (mucosa overlying theattached to the third flap (mucosa overlying the
primary palateprimary palate
9) Posteriorly, the palate is closed in 3 layers9) Posteriorly, the palate is closed in 3 layers
Nasal mucosaNasal mucosa
Levator muscleLevator muscle
Oral mucosaOral mucosa
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Post-op ManagementPost-op Management
• Cleft PalateCleft Palate
– Immediate concerns:Immediate concerns:
• AIRWAY MANAGEMENT: Change in nasal/oral airwayAIRWAY MANAGEMENT: Change in nasal/oral airway
dynamicsdynamics
• ANALGESIA: Risk of over-sedation and subsequentANALGESIA: Risk of over-sedation and subsequent
airway compromiseairway compromise
• ARM RESTRAINTS to prevent placing fingers in mouthARM RESTRAINTS to prevent placing fingers in mouth
• DIET restricted to liquids, soft foods (x3wks): bottlesDIET restricted to liquids, soft foods (x3wks): bottles
avoidedavoided
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Post-op ManagementPost-op Management
• Airway obstructionAirway obstruction
• Intraoperative bleeding/ AspirationIntraoperative bleeding/ Aspiration
• Palatal fistula/ DehisencePalatal fistula/ Dehisence
• Midface abnormalities (early interventions)Midface abnormalities (early interventions)
COMPLICATIONSCOMPLICATIONS
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
Cleft Palate ClinicsCleft Palate Clinics
Through a protocol of sequential, regular evaluations by aThrough a protocol of sequential, regular evaluations by a
team composed of plastic surgeon, speech pathologist,team composed of plastic surgeon, speech pathologist,
orthodontist, and audiologist, great strides have been made inorthodontist, and audiologist, great strides have been made in
improving all aspects of care of the child with cleft palateimproving all aspects of care of the child with cleft palate
Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
The Toddler Years
• Priority: Speech
• VELOPHARYNGEAL DYSFUNCTION
– A. VELOPHARYNGEAL MISLEARNING
“i.e. Phoneme Specific Nasal Air Emission”
– B. VELOPHARYNGEAL INCOMPTENCY
i.e.“apraxia”, neurological deficit
– C. VELOPHARYNGEAL INSUFFICENCY
i.e. Anatomical deficit
The Toddler Years
• Priority: Speech
– “CLEFT ERRORS OF SPEECH” in 30%
• PRIMARY DEFECTS - due to VPD (hypernasality)
– consonants are most difficult sounds (plosives)
• SECONDARY DEFECTS - due to attempted
correction
– Glottic Stops, Nasal Grimace
– VELOPHARYNGEAL DYSFUNCTION
• diagnosed by fiberoptic laryngoscopy or BaSw
• surgical repair after failed speech therapy - usually
around age 4
VELOPHARYNGEAL DYSFUNCTION
• SIGNS AND SYMPTOMS
– History of NASAL REGURGITATION post
cleft palate repair
– History of need for multiple placement of PE
tubes
– Nasal GRIMACE
– HOARSE Vocal Quality
– Decreased INTELLIGIBILITY

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Clinical aspects of cleft lip and palate reconstruction

  • 1. Clinical Aspects of Cleft Lip/PalateClinical Aspects of Cleft Lip/Palate ReconstructionReconstruction ANJAN K DEBANJAN K DEB Dept of Plastic surgery BIRDEMDept of Plastic surgery BIRDEM
  • 2. OverviewOverview • Relevant Anatomy & PhysiologyRelevant Anatomy & Physiology • Embryology of Facial CleftingEmbryology of Facial Clefting • Classification/Epidemiology/Related SyndromesClassification/Epidemiology/Related Syndromes • Principles of ManagementPrinciples of Management • Preoperatve Assessment/ OrthodonticsPreoperatve Assessment/ Orthodontics – Indications/ContraindicationsIndications/Contraindications • Time table of Procedures/EventsTime table of Procedures/Events • Surgical TechniquesSurgical Techniques – Millard / Tennison-RandallMillard / Tennison-Randall – Wardill-Kilner/ Z-plastyWardill-Kilner/ Z-plasty – Speech assessment/PharyngioplastySpeech assessment/Pharyngioplasty – Alveolar bone graftAlveolar bone graft • Post-op managementPost-op management – Complications – Follow up Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 3. Applied AnatomyApplied Anatomy Normal LipNormal Lip CENTRAL PHILTRUM Lateral margins Philtral columns Inferior border Cupids bow and tubercle VERMILLION-CUTANEOUS BORDER White roll COMMISURE Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 4. Applied AnatomyApplied Anatomy • PHILTRAL ZONE – Column/Dimple • VERMILION – Cupid’s Bow/Apex • SUB VEMILION • PERISTOMAL – Wet & Dry Line • COMMISURE • LATERAL • CENTRAL
  • 5. Applied AnatomyApplied Anatomy • Glabella • Root Or Nasion • Dorsum Or Rhinon • Ala (3) • Columella (2) • Tip (8)/ Infratip (1) • Soft Triangle/Facet (4) • Nasal Sill (5) • Columella-philtral Angle (6) • Alar –Facial Groove (7)
  • 6. Applied AnatomyApplied Anatomy MUSCLES Orbicularis oris (superficial and deep) Levator labii superioris Levator superioris alaeque Levator anguli oris Zygomaticus major et minor Buccinator Depressor labii inferioris Depressor anguli oris Mentalis Disruption of the normal termination of the muscle fibers that cross the embryologicDisruption of the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in abnormal insertion &fault line of the maxillary and nasal processes, resulting in abnormal insertion & abnormal muscular forces between the normal equilibrium that exists with theabnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of musclesnasolabial and oral groups of muscles
  • 7. Applied AnatomyApplied Anatomy MUSCLES OF LIP INNER VIEWMUSCLES OF LIP INNER VIEW
  • 8. Applied AnatomyApplied Anatomy BLOOD SUPPLY LIPSBLOOD SUPPLY LIPSDORSAL NASALDORSAL NASAL FACIALFACIAL INF ALVEOLAR terminal brINF ALVEOLAR terminal br INFERIOR LABIALINFERIOR LABIAL SUPERIOR LABIALSUPERIOR LABIAL INFRA ORBITALINFRA ORBITAL MAXILLARYMAXILLARY POST SUP ALVEOLARPOST SUP ALVEOLAR BUCCALBUCCAL
  • 9. Applied AnatomyApplied Anatomy BLOOD SUPPLY PALATEBLOOD SUPPLY PALATE NASAL SEPTUMNASAL SEPTUM TURBINATESTURBINATESSPHENOPALATINESPHENOPALATINE GREATER PALATINEGREATER PALATINE ANASTOMOSIS POSTERIOR SEPTAL & GR PALATINEANASTOMOSIS POSTERIOR SEPTAL & GR PALATINE LESSER PALATINELESSER PALATINE
  • 10. Applied AnatomyApplied Anatomy Normal PalateNormal Palate Primary palatePrimary palate Secondary palateSecondary palate Soft palateSoft palate Hard palateHard palate
  • 12. Applied AnatomyApplied Anatomy • SUPERIOR CONSTRICTOR » primary sphincter • TENSOR VELI PALATINI » tenses palate • LEVATOR VELI PALATINI » elevates palate » dilates Eust Tube • Salpingopharyngeus, palatopharyngeous, palatoglossus: minor contribution Postero-superior view PHARYNGEAL VIEWPHARYNGEAL VIEW
  • 13. Muscle of velum 3D view
  • 14. Embryology of CleftingEmbryology of Clefting FACIAL DEVELOPMENT - 4FACIAL DEVELOPMENT - 4THTH - 10- 10THTH WEEKWEEK Formed by the fusion ofFormed by the fusion of 55 prominences/processesprominences/processes FRONTONASAL x1FRONTONASAL x1 Lateral/medial nasal processesLateral/medial nasal processes MAXILLARY x2 MANDIBULAR x2 Nose/Philtrum of upper lipNose/Philtrum of upper lip Cheeks/Upper lip (Cheeks/Upper lip (-philtrum-philtrum)) Lower face (lower lip/chin)
  • 15. Embryology of CleftingEmbryology of Clefting FACIAL DEVELOPMENTFACIAL DEVELOPMENT Medial nasal processesMedial nasal processes migrate toward each other and fuse 55thth -7-7thth weekweek Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 16. Embryology of CleftingEmbryology of Clefting • 7th -8th week – Inferior tips of medial nasal processes expand laterally form the intermaxillary process – Tips of maxillary swellings grow & meet the intermaxillary process and fuse Failure of maxillary swellings to fuse with intermaxillary process = cleft lipFailure of maxillary swellings to fuse with intermaxillary process = cleft lip
  • 17. FORMATION OF THEFORMATION OF THE PALATEPALATE 66thth -7-7thth weekweek As nasal pits of lateral nasalAs nasal pits of lateral nasal process invaginate and fuse,process invaginate and fuse, intermaxillary process extendsintermaxillary process extends to form primary palateto form primary palate Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction Embryology of CleftingEmbryology of Clefting
  • 18. 88thth - 9- 9thth weekweek Medial wallsMedial walls ofof MAXILLARY PROCESSESMAXILLARY PROCESSES produceproduce PALATINE SHELVESPALATINE SHELVES Shelves grow downwards,Shelves grow downwards, parallel to lateral sufaceparallel to lateral suface of tongueof tongue End ofEnd of week 9week 9,, rotaterotate upwardupward into ainto a horizontal position andhorizontal position and fuse with eachfuse with each otherother andand primary palateprimary palate toto FORMFORM SECONDARY PALATESECONDARY PALATE Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction Embryology of CleftingEmbryology of Clefting
  • 19. Embryology of CleftingEmbryology of Clefting • MESODERM H&N derived from – PARAXIAL MESODERMPARAXIAL MESODERM • Floor Of Brain CaseFloor Of Brain Case • Occipital & Parietal BonesOccipital & Parietal Bones • All Voluntary Muscle of H&NAll Voluntary Muscle of H&N • Dermis & CT of Dorsal HeadDermis & CT of Dorsal Head – LATERAL PLATE MESODERMLATERAL PLATE MESODERM • Laryngeal Cartilages & CTLaryngeal Cartilages & CT – NEURAL CREST CELLSNEURAL CREST CELLS (deficient in superoxide dismutase & catalase)(deficient in superoxide dismutase & catalase) • Mid-facial & Pharyngeal Arch skeletonMid-facial & Pharyngeal Arch skeleton • Overlying Dermis & Soft TissuesOverlying Dermis & Soft Tissues • DentinDentin – ECTODERMAL PLACODES • Neuron of Sensory Ganglia, V,VII,I X, X
  • 20. CRANIO-FACIAL CLEFT • Tessier,s Classification – Spoke wheel – Radiating from orbit – Magic No- 14 – Incidence: 1:150000
  • 21. Cleft Lip & Palate VariantsCleft Lip & Palate Variants Great anatomic variationGreat anatomic variation in types of clefts!in types of clefts! ANATOMIC CLASSIFICATIONANATOMIC CLASSIFICATION basedbased on:on: 1) LOCATION1) LOCATION 2) COMPLETENESS2) COMPLETENESS (Incomplete/Complete)(Incomplete/Complete) 3) EXTENT3) EXTENT Since lip, alveolus, and hard palate differ in embryologicSince lip, alveolus, and hard palate differ in embryologic origin,origin, 4) ANY COMBINATION CAN OCCUR4) ANY COMBINATION CAN OCCUR Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 22. Cleft Lip & Palate VariantsCleft Lip & Palate Variants CLEFLIP & PRIMARY PALATECLEFLIP & PRIMARY PALATE CLEFT OF PRIMARY & SECONDARY PALATECLEFT OF PRIMARY & SECONDARY PALATE CLEFT OF SECONDARY PALATECLEFT OF SECONDARY PALATE
  • 23. Veau Classification CLCPVeau Classification CLCP • 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
  • 24. Iowa Classification CLCPIowa Classification CLCP Group IGroup I Clefts of lip onlyClefts of lip only Group IIGroup II Clefts of palate only (Clefts of palate only (22oo )) Group IIIGroup III Clefts of lip,Clefts of lip, alveolus, palatealveolus, palate Group IVGroup IV Clefts of lip andClefts of lip and alveolusalveolus (primary(primary cleft palate andcleft palate and lip)lip) Group VGroup V MiscellaneousMiscellaneous
  • 25. Striped Y Modified Kernahan’sStriped Y Modified Kernahan’s 1 & 51 & 5 -- FLOOR OF NOSEFLOOR OF NOSE on right &on right & left sidesleft sides 2 & 62 & 6 -- LIPLIP 3 & 73 & 7 -- ALVEOLAR RIDGESALVEOLAR RIDGES 4 & 84 & 8 -- PREMAXILLAPREMAXILLA to incisiveto incisive foramenforamen 9 & 109 & 10 - Each half of the- Each half of the HARDHARD PALATEPALATE 1111 -- SOFT PALATESOFT PALATE 1212 - Congenital- Congenital VELOPHARYNGEALVELOPHARYNGEAL INCOMPETENCEINCOMPETENCE without obviouswithout obvious cleftsclefts 1313 -- PROTRUSIONPROTRUSION of premaxillaof premaxilla KERNAHANSKERNAHANS
  • 26. Cleft Lip & Palate VariantsCleft Lip & Palate Variants Isolated INCOMPLETEIsolated INCOMPLETE Intact skin/muscle between the lip and noseIntact skin/muscle between the lip and nose Less distortion brought on by abnormal muscle pullLess distortion brought on by abnormal muscle pull Bilateral/UnilateralBilateral/Unilateral CLEFT LIPCLEFT LIP Expressed in structuresExpressed in structures anterior to incisive foramenanterior to incisive foramen - prepalatal alveolus, maxilla, lip, nasal structures- prepalatal alveolus, maxilla, lip, nasal structures GAPING CLEFT of alveolus/lip structures toGAPING CLEFT of alveolus/lip structures to MERE ‘SCAR’ (MERE ‘SCAR’ (forme frusteforme fruste)) DEFICIENCY INDEFICIENCY IN SKINSKIN,, MUSCLES, MUCOUSMUSCLES, MUCOUS MEMBRANESMEMBRANES, MAXILLARY/NASAL, MAXILLARY/NASAL BONESBONES, NASAL, NASAL CARTILAGESCARTILAGES
  • 27. ISOLATED COMPLETEISOLATED COMPLETE Bilateral/UnilateralBilateral/Unilateral Cleft runs entire length of lip to floor of noseCleft runs entire length of lip to floor of nose Abnormal muscle pull distorts nose extensivelyAbnormal muscle pull distorts nose extensively and creates wide clefts between the lipand creates wide clefts between the lip segmentssegments Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction Ipsilateral Lower Lat CartIpsilateral Lower Lat Cart flattenedflattened rotated downwardrotated downward Bifid tipBifid tip Short columellaShort columella Flattened alaFlattened ala Cleft of alveolusCleft of alveolus Cleft Lip & Palate VariantsCleft Lip & Palate Variants
  • 28. Cleft Lip & Palate VariantsCleft Lip & Palate Variants ISOLATED CLEFT PALATEISOLATED CLEFT PALATE COMPLETE/INCOMPLETE/SUBMUCOUSCOMPLETE/INCOMPLETE/SUBMUCOUS Soft PalateSoft Palate cleft can extend into the hard palate tocleft can extend into the hard palate to any extentany extent Hard PalateHard Palate Primary PalatePrimary Palate Secondary PalateSecondary Palate Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 29. Cleft Lip & Palate VariantsCleft Lip & Palate Variants COMBINED CLEFTSCOMBINED CLEFTS Complete lip &/palate UnilateralComplete lip &/palate Unilateral
  • 30. Cleft Lip & Palate VariantsCleft Lip & Palate Variants Complete lip &/palate BilateralComplete lip &/palate Bilateral PROTRUDED PEMAXILLAPROTRUDED PEMAXILLA
  • 31. EpidemiologyEpidemiology • Isolated CLEFT PALATE genetically distinct from isolated CLEFT LIP OR CLAP – same among all ethnic groups (1:2000, M:F 1:2) – More assoc with Syndrome • Isolated CL or CLAP – different among ethnic groups • American Indians: 3.6:1000 (M:F 2:1) • Asians 3:1000 (M:F 2:1) • African American 0.3:1000 (M:F 2:1) • 20% CL (18% unilateral, 2% bilateral)20% CL (18% unilateral, 2% bilateral) • 50% CL and CP (38% unilateral, 12% bilateral)50% CL and CP (38% unilateral, 12% bilateral) • 30 % CP alone30 % CP alone
  • 32. EpidemiologyEpidemiology • GENETICS (Clustering in families) FAMILY MAKEUP RISK OF CLEFT LIP / PALATE RISK OF CLEFT PALATE ONE AFFECTED SIBLING OR PARENT 1 IN 25 (4%) 2.5% TWO AFFECTED SIBLING 1 IN 11 (9%) 1% ONE SIBLING AND ONE PARENT 1 IN 6 (16%) 15%
  • 33. EpidemiologyEpidemiology • SYNDROMIC CLAP – associated with more than 300 malformations • CHROMOSOMAL – Trisomy 13, 18, 21 & Deletion 22q11 (Velocardiofacial Syndrome) • NON MENDELIAN – PIERRE ROBIN, Goldenhar • MENDELIAN – Corlin’s, Dysplasia-Clefting, Treacher-Collins, Van der Woude (AD) – Smith-Lemli-Opitz (AR) – Oto-Palato-digital, Oto-Palato-Facial (XL) • UNKNOWN – DeLarge , Kabuki • TERATOGENIC – Fetal Alcohol, Phenytoin, Valproate • NONSYNDROMIC CLAP • diagnosis of exclusion • OVERALL INCIDENCE OF ASSOCIATED ANOMALIES (e.g.OVERALL INCIDENCE OF ASSOCIATED ANOMALIES (e.g. CARDIACCARDIAC) = 30%) = 30%
  • 34. EpidemiologyEpidemiology • RISK FACTORS – PARENTAL AGE: >30yrs – VIRAL INFECTIONS: Rubella – TERATOGENS: Alcohol, Steroids, Anticonvulsants, Retinoic acid derivatives
  • 35. Principles of ManagementPrinciples of Management • Multidisciplinary Approach • These are not merely surgical problems – Requires team approach throughout life • neonatal period • toddler • grade school • adolescence • young adulthood
  • 36. Principles of ManagementPrinciples of Management MULTIDISCIPLINARY APPROACHMULTIDISCIPLINARY APPROACH Beyond lip repair are other issues:Beyond lip repair are other issues: Hearing (Otolaryngologists)Hearing (Otolaryngologists) Speech (Speech Pathologists / Therapist)Speech (Speech Pathologists / Therapist) Dental (Periodontologist/Orthodontist / Prosthodontist/)Dental (Periodontologist/Orthodontist / Prosthodontist/) Maxillofacial Surgeons/Maxillofacial Surgeons/ Psychosocial (Psychologist/ Psychiatrist/ Social Worker)Psychosocial (Psychologist/ Psychiatrist/ Social Worker) GeneticistGeneticist Integration with team-based approachIntegration with team-based approach Each case is assessed independently by those involved and a global treatment planEach case is assessed independently by those involved and a global treatment plan is instituted based on present need in his/her developmentis instituted based on present need in his/her development Cleft Lip and PalateCleft Lip and Palate Nutrition (Nutritionist )Nutrition (Nutritionist )
  • 37. Principles of ManagementPrinciples of Management ASSESSMENTASSESSMENT AIMAIM: RESTORING NORMAL MORPHOLOGIC FORM AND FUNCTION: RESTORING NORMAL MORPHOLOGIC FORM AND FUNCTION Important forImportant for normal dentition, mastication, speech, hearing,normal dentition, mastication, speech, hearing, andand breathingbreathing CONTRAINDICATIONSCONTRAINDICATIONS: MALNUTRITION, ANEMIA RESPIRATORY: MALNUTRITION, ANEMIA RESPIRATORY INFECTION or other conditions that render infantINFECTION or other conditions that render infant UNABLE TO TOLERATEUNABLE TO TOLERATE GENERAL ANESTHESIA Airway obstruction, Acute otitis mediaGENERAL ANESTHESIA Airway obstruction, Acute otitis media Work-upWork-up (1) Thorough(1) Thorough PEPE to uncover anyto uncover any ASSOCIATED ANOMALIESASSOCIATED ANOMALIES Additional work-up determined by physical findings that suggest involvementAdditional work-up determined by physical findings that suggest involvement ofof other organother organ systemssystems (2)(2) WEIGHT, ORAL INTAKE, GROWTH/DEVELOPMENTWEIGHT, ORAL INTAKE, GROWTH/DEVELOPMENT are of primary concern and must be followed closelyare of primary concern and must be followed closely (3) Routine lab studies generally not required; Hgb level before surgery(3) Routine lab studies generally not required; Hgb level before surgery
  • 38. 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 • Feeding bottle large hole
  • 39. The Neonatal Period • Presurgical Orthodontics (Baby Plates) – Moulds palate into more anatomically correct position – decreases tension – may improve facial growth – Grayson, presurgical nasal alveolar moulding (PSNAM) • c
  • 41. The Neonatal Period • Surgical Repair – Cleft Lip • In US - “THE RULE OF TENS” - 10 wks, 10 lbs, Hgb 10gm% • Lip adhesion vs baby plates – Cleft Palate • Varies from 6-18 Months - most around 10 mo • Early repair may lead to MIDFACE RETRUSION • Early repair improves SPEECH Different institutions = different practiceDifferent institutions = different practice
  • 42. Management Schedule Palatal obturatorPalatal obturator Repair cleft lipRepair cleft lip Repair of PalateRepair of Palate Repair of Hard palateRepair of Hard palate Tympanotomy tubeTympanotomy tube Speech therapy/pharyngoplastySpeech therapy/pharyngoplasty Bone graftingBone grafting OrthodonticsOrthodontics Jaw surgeryJaw surgery
  • 43. Surgical Principles • Release the musclesRelease the muscles from abnormalfrom abnormal insertionsinsertions • Repair them inRepair them in anatomical positionanatomical position • Lengthen medial sideLengthen medial side of cleft so that itof cleft so that it attains normalattains normal anatomical lengthanatomical length
  • 44. Surgical ManagementSurgical Management Unilateral Complete Cleft LipUnilateral Complete Cleft Lip GOALSGOALS • SYMMETRY: Nostrils,SYMMETRY: Nostrils, Nasal Sill, and Alar BasesNasal Sill, and Alar Bases • WELL DEFINEDWELL DEFINED PHILTRUM: Dimple andPHILTRUM: Dimple and ColumnsColumns • CUPID’S BOW: NaturalCUPID’S BOW: Natural appearingappearing • FUNCTION: Good muscleFUNCTION: Good muscle repairrepair SURGICAL PRINCIPLESSURGICAL PRINCIPLES • Lengthen medial side ofLengthen medial side of cleft so that it equals thecleft so that it equals the vertical dimensions of non-vertical dimensions of non- cleft sidecleft side • Flap designs:Flap designs: – Triangular (Tennison-Triangular (Tennison- Randall)Randall) – QuadrangularQuadrangular – Rotation-advancementRotation-advancement (Millard)(Millard) • MuscleRepair:MuscleRepair:
  • 45. Surgical ManagementSurgical Management Unilateral Complete Cleft LipUnilateral Complete Cleft Lip TENNISON-RANDALLTENNISON-RANDALL
  • 46. Millard TechniqueMillard Technique • Cleft Lip Repair • Unilateral • Rotation(a &c)-advancement (b)flap developed by Millard – Medial flap(a)Medial flap(a) rotatesrotates downward to achieve necessarydownward to achieve necessary lengtheninglengthening – Lateral flap (b)Lateral flap (b) advances intoadvances into the defect produced by downwardthe defect produced by downward displacement of medial flapdisplacement of medial flap – Small pennant-shapedSmall pennant-shaped medial flap (c)medial flap (c)c an be used toc an be used to restore nostril sill or lengthen therestore nostril sill or lengthen the columellacolumella 3 Flaps a,b & c
  • 47. Millard TechniqueMillard Technique ADVANTAGES – ““Cut as you go” techniqueCut as you go” technique – Preserves’ cupid’s bow andPreserves’ cupid’s bow and philtral dimplephiltral dimple – Scar placed in anatomical position along philtral column – Tension of closure under theTension of closure under the alar base; reduces flair andalar base; reduces flair and promotes better molding of thepromotes better molding of the underlying alveolar processesunderlying alveolar processes
  • 48. Millard TechniqueMillard Technique • COMPLICATIONS – Tightness at white roll/ cupids bow – Peaking of vermillion – Notching of stomal margin – Residual nasal deformity – Tension?/ dehisence?/ HTS?/infection? – scar stretching
  • 49. Bilateral Cleft Lip Repair • MILLARD’S ADAPTATION: – Philtral from central prolabium – Prolab paring banked for collumelar lengthening – Prolabial white roll & vermillion discarded – Cupids bow tubercle from lateral lip segments
  • 50. Bilateral Cleft Lip Repair • MODIFIED MANCHESTER – Preserves white roll vermillion of prolabium – Philtral flap cut to desired width – Prolabial paring used for nasal floor – Lateral muscle are sutured to prolabial sub cut tissues
  • 51. Bilateral Cleft Lip Repair • BLACK’S METHOD
  • 52. Post-op ManagementPost-op Management 1) FEEDINGS administered with catheter tip syringe1) FEEDINGS administered with catheter tip syringe fitted with small red rubber catheter for the first 10 daysfitted with small red rubber catheter for the first 10 days post-oppost-op 2) AVOID SUCKING Nipples are avoided to minimize2) AVOID SUCKING Nipples are avoided to minimize strain on the muscle/skin suturesstrain on the muscle/skin sutures 3) ARM RESTRAINT Velcro arm restraints to3) ARM RESTRAINT Velcro arm restraints to protect repair from flailing hands/fingersprotect repair from flailing hands/fingers 4) SUTURE LINE CARE: PRN cleansing with half4) SUTURE LINE CARE: PRN cleansing with half strength peroxide followed with polymixin B-bacitracinstrength peroxide followed with polymixin B-bacitracin ointmentointment Cleft LipCleft Lip Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 53. Post-op ManagementPost-op Management Scar contractureScar contracture ErythemaErythema FirmnessFirmness Inform the parents of:Inform the parents of: Avoid placing in direct sunlight until the scar fully maturesAvoid placing in direct sunlight until the scar fully matures Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 54. ComplicationsComplications Post-op ManagementPost-op Management • AestheticAesthetic – vermilion-cutaneousvermilion-cutaneous mismatchmismatch – vermilion notchingvermilion notching – Whistle deformityWhistle deformity – tight appearing lateraltight appearing lateral lip segementlip segement – lateral muscle buldgelateral muscle buldge – laterally displaced alalaterally displaced ala – constricted appearingconstricted appearing nostrilnostril • OtherOther – dehiscencedehiscence – excessive scarexcessive scar formationformation Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 55. Surgical ManagementSurgical Management Cleft PalateCleft Palate Goal: Production of a competent velopharyngeal sphincterGoal: Production of a competent velopharyngeal sphincter Two most common repairs:Two most common repairs: 1) V-Y (Veau-Wardill-Kilner)*1) V-Y (Veau-Wardill-Kilner)* 2) von Langenbeck2) von Langenbeck Main difference: V-Y repair involvesMain difference: V-Y repair involves elongation of the palateelongation of the palate, while, while von Langenbeck does notvon Langenbeck does not Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 56. Wardill-KilnerWardill-Kilner 1) Incisions made along free margins of cleft and extend1) Incisions made along free margins of cleft and extend anteriorly to apexanteriorly to apex 2) Dissection continued posteriorly along oral side of2) Dissection continued posteriorly along oral side of alveolar ridge to retromolar trigonealveolar ridge to retromolar trigone Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 57. Wardill-KilnerWardill-Kilner 3) Mucoperiosteal flaps are elevated from3) Mucoperiosteal flaps are elevated from nasal/oral surfaces of bony palatenasal/oral surfaces of bony palate 4) Dissection of the greater palatine vessels from4) Dissection of the greater palatine vessels from the foramen lengthens the pediclethe foramen lengthens the pedicle 5) Tensor veli palatini muscle is elevated off the5) Tensor veli palatini muscle is elevated off the hamulus to aid in relaxing the midline closurehamulus to aid in relaxing the midline closure Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 58. Wardill-KilnerWardill-Kilner 6) Nasal mucosa freed from bony palate6) Nasal mucosa freed from bony palate and closed to either side, or if necessaryand closed to either side, or if necessary closed by using vomer flapsclosed by using vomer flaps 7) Muscle and oral mucosa closed in a7) Muscle and oral mucosa closed in a second single layer in a horizontal fashionsecond single layer in a horizontal fashion Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 59. Wardill-KilnerWardill-Kilner 8) Anteriorly, the oral mucoperiosteal flaps are8) Anteriorly, the oral mucoperiosteal flaps are attached to the third flap (mucosa overlying theattached to the third flap (mucosa overlying the primary palateprimary palate 9) Posteriorly, the palate is closed in 3 layers9) Posteriorly, the palate is closed in 3 layers Nasal mucosaNasal mucosa Levator muscleLevator muscle Oral mucosaOral mucosa Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 60. Post-op ManagementPost-op Management • Cleft PalateCleft Palate – Immediate concerns:Immediate concerns: • AIRWAY MANAGEMENT: Change in nasal/oral airwayAIRWAY MANAGEMENT: Change in nasal/oral airway dynamicsdynamics • ANALGESIA: Risk of over-sedation and subsequentANALGESIA: Risk of over-sedation and subsequent airway compromiseairway compromise • ARM RESTRAINTS to prevent placing fingers in mouthARM RESTRAINTS to prevent placing fingers in mouth • DIET restricted to liquids, soft foods (x3wks): bottlesDIET restricted to liquids, soft foods (x3wks): bottles avoidedavoided Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 61. Post-op ManagementPost-op Management • Airway obstructionAirway obstruction • Intraoperative bleeding/ AspirationIntraoperative bleeding/ Aspiration • Palatal fistula/ DehisencePalatal fistula/ Dehisence • Midface abnormalities (early interventions)Midface abnormalities (early interventions) COMPLICATIONSCOMPLICATIONS Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 62. Cleft Palate ClinicsCleft Palate Clinics Through a protocol of sequential, regular evaluations by aThrough a protocol of sequential, regular evaluations by a team composed of plastic surgeon, speech pathologist,team composed of plastic surgeon, speech pathologist, orthodontist, and audiologist, great strides have been made inorthodontist, and audiologist, great strides have been made in improving all aspects of care of the child with cleft palateimproving all aspects of care of the child with cleft palate Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction
  • 63. The Toddler Years • Priority: Speech • VELOPHARYNGEAL DYSFUNCTION – A. VELOPHARYNGEAL MISLEARNING “i.e. Phoneme Specific Nasal Air Emission” – B. VELOPHARYNGEAL INCOMPTENCY i.e.“apraxia”, neurological deficit – C. VELOPHARYNGEAL INSUFFICENCY i.e. Anatomical deficit
  • 64. The Toddler Years • Priority: Speech – “CLEFT ERRORS OF SPEECH” in 30% • PRIMARY DEFECTS - due to VPD (hypernasality) – consonants are most difficult sounds (plosives) • SECONDARY DEFECTS - due to attempted correction – Glottic Stops, Nasal Grimace – VELOPHARYNGEAL DYSFUNCTION • diagnosed by fiberoptic laryngoscopy or BaSw • surgical repair after failed speech therapy - usually around age 4
  • 65. VELOPHARYNGEAL DYSFUNCTION • SIGNS AND SYMPTOMS – History of NASAL REGURGITATION post cleft palate repair – History of need for multiple placement of PE tubes – Nasal GRIMACE – HOARSE Vocal Quality – Decreased INTELLIGIBILITY