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CLEFT LIP AND PALATEIt is congenital abnormal space in upper lip, alveolus and palate.INCIDENCECommon in south asians 1 in 500Less frequent in africans 1 in 200Prevalance in pakistan 1 in 523Boys are more affected than girls 3:275% are unilateralLeft side is more affected than right sideEMBRYOLOGY

DEVELOPMENT OF PALATE

CLASSIFICATIONGenerally classified asIsolated cleft of lip/palateCombined CL+CPUnilateral or bilateral complete (if it crosses nasal philtrum) or incomplete (if it does not cross philtrum)Veau classificationKernehans stripped YMillard modification of kernehan stripped YKriens LAHSHALVEAU CLASSIFICATIONGROUP 1 Cleft of the soft palateGROUP 2Cleft of hard and soft palate up to incisive foramenGROUP 3Complete unilateral cleft lip and palateGROUP 4Complete bilateral cleft lip and palate

LAHSHAL CLASSIFICATIONL=lip (right)A=alveolus (right)H=hard palate (right)S=soft palate (median)H=hard palate (left)A=alveolus (left)L=lip (left)Capital letter = complete cleftLower case letter = incomplete cleft. or , = normal ETIOLOGYGenetics, inherited from one or both parentsEnvironmental factorsDrugs, phenytoin, retinoidsInfection, rubellaSmokingAlcoholHypoxiaVitamin A defeciencyFolic acid defeciencyAge DIGANOSISCleft can be diagnosed prenatallyCan be diagnosed by using 3-D imaging in ultrasonography and genetic tests for screening of birth defects.In second trimesterPremaxillary protrusion is an important clue to the presence of cleft lip and palate.ADVANTAGEPrenatal educationPsychological preperation of parentsHelps to asses and investigate other associated anomaliesNON SYNDROMIC CLEFTPIERRE ROBIN SEQUENCECleft palateMicrognathiaGlossoptosis (retrarction of tongue)

SYNDROMIC CLEFTSTICKLERS SYNDROMEFlttened facePRSEar infectionsOccular HTNRetinal detachmentVELOCARDIOFACIAL SYNDROMECardiac abnormalityAbnormal faciesThymic aplasiaCleft palateHypocalcemia/hypoparathyroidism

VAN-DER-WOUDES SYNDROMEGenetic disorderCombination of lower lip pits+CL with or without cleft palate and CP alone.HypodontiaCongenital heart diseaseCerebral abnormalitiesSyndactyly

PROBLEMSFeedingBreathing Speech problemsHearing Esthetic problemsNasal deformityDental problemsFEEDINGInfant cannot suckMay cause milk to be accidently taken in nasal cavityUnable to create negative pressureFrequent regurgitationUpper respiratory tract infections

Mead-Johnson cleft palate nurser

Haberman feeder

Pigeon nipple

EAR, NOSE AND THROAT EVALUATIONAudiology assessment is recommended soon after birthHigh frequency of otitis mediaMyringotomy is performed to relese otitis media fffusionDENTAL PROBLEMSMissing teethHypodontiaHyperdontiaNatal teethMicrodontia/macrodontiaEnamel hypoplasiaPoor PDL supportEarly loss of teethClass III tendencySpacingCrowdingXbiteNASAL DEFORMITYPoor nasal shapeScar mark due to surgeryESTHETIC PROBLEMSPoor dental alignmentPoor smilePoor lip function during speechEAR PROBLEMMiddle ear infectionHearing lossSPEECH PROBLEMSVelopharyngeal insufficiency (VPI)Poor pronounciation of bilabial, labiodental, linguoalveolar soundsMANAGEMENTTEAM APPROACHGenetic scientistPeditricianENT surgeonPaedodontistOral surgeonProsthodontistPlastic surgeonSpeech therapistPshyciatristSocial worker TIMING OF TREATMENT IN CLEFT PATIENTPRENATALDiagnosis and parental counseling0-6 MONTHSGeneral assessment for associated anomaliesENT evaluation- breathing, feeding, swallowing and hearingPre surgical orthopedics (0-3 months)Primary lip repair (3-4 months)6 MONTHS-2 YEARSSpeech and oral sensory motor assessmentGrommets/ear tubes (as needed)Primary palate repair (9-12 months)PRESCHOOL: 3-5 YEARSDental careSpeech assessment and therapy asses need for lip revisionCHILDHOOD: 6-12 YEARSCorrection of velo-pharyngeal dysfunction (as needed)Orthodontic treatment-phase IAlveolar cleft repair (8-11 years)ADOLESCENCE: 13-18 YEARSOrthodontic treatment-phase IIOrthognathic surgery (if needed)-14-16 years (female), 16-18 years (male)Revision chielo-rhinoplastyReplacement of missing teeth (as needed)

PRESURGICAL ORTHOPEDICSGentle application of pre surgical orthopedic force allow the molding of alveolar segments and the nostrils within 0-3 months of birth and provide benefit in correction of the nasal deformity in childern with bilateral CLP and wide unilateral clefts.Nasoalveolar molding increases the surface area of nasal mucosal liningHelps to elongate columellaMake columella uprightAllows suturing of alar cartilages without tensionDecreases wideningof the noseTwo categories of appliances are usedPassiveactive.

ACTIVE APPLIANCEActive appliancesThey are fixed intra orally apply traction through mechanical means such as elastic chains, screws, and plates.

Passive appliances maintain the distance between the 2 maxillary segments while external force is applied to the primarily to reposition it posteriorly. The naso alveolar molding device commonly used passive deviceit consists of an intraoral acrylic plate held in place with extraoral elastics and tape. In later phases of treatment, wire are added to apply force to the vestibule of the nares, lengthening the columella

OTHER METHODSExternal force can be applied byexternal taping of the lip a head cap with elastic straps or a surgical lip adhesion.

CLEFT LIP REPAIRGoal is to reconstruct a functional lip, with normal appearnceTimining is between 3-6 monthsA RULE OF TEN is followed to ensure the infant is fit for surgery.Infant should be at least 10 weeks of ageWeigh 10lbsHaemoglobin level 10g/100mlUNILATERAL CLEFT LIPANATOMYAsymmetric deformity of lip and noseNasal deformity severity of cleftDue to abnormal position of orbicularis oris muscle because they terminate medially beneath the base of clumella and laterally beneath alar base and periosreum of piriform rimDistortion of noseDeflection of ANSDeflect nasal septum

TECHNIQUEBlair, Le Mesurier, Tennison and Randall contributed to evolution of cleft lip repair.Popular is Millard Technique who described ROTATION-ADVANCEMENT concept.Medial flap is rotated downward to achieve lengthLateral is advanced.Can be modified at the time of operationADVANTAGE:Suture lies on the recreated philtrumIncision allows easy access for primary rhinoplasty to reposition nasal septum, lateral cartilage and alar base.

PRINCIPLESROTATION OR LENGTHENING OF SHORTENED VERTICAL HEIGHT OF LIPADVANCEMENT OF FLAP FROM LABTERAL TO MEDIALRETAINING CUPIDS BOW AND CREATE PHILTRAL COLUMNMUSCLE RECONSTRUCTIONRESTORATION OF ALVEOLAR CONTINUITYPRIMARY REPAIR OF DISTORTED NASAL ANATOMYBILATERAL CLEFT LIPANATOMYDifficult to repairAbsence of muscle in prolabial segmentLack of philtrum dimpleLack of Philtral columnsAbsent Roll marginsPremaxillary segment protrudes making tension free margins of muscle repair difficult.Columella shortWide nasal tipFlare alar base

PRINCIPLESESTABLISH SYMMETRYDESIGNIGNING A PROLABIAL FLAP OF APPROPRIATE WIDTHFORMING THE CUPIDS BOW AND MEDIAN TUBERCLE FROM LATERAL LIP ELEMENTSESTABLISH MUSCLE CONTINUITYRECONSTRUCTION OF NASAL TIP AND COLUMELLAREPOSITIONING THE ALAR BASEMANAGEMENT OF PREMAXILLA

REPAIR OF CLEFT PALATEGOALS:To normalize speechAlign the aberrant attachment of palatal musclesSeal communication between the oral and nasal cavitiesTIMING OF REPAIR:Historically cleft palate repair of the hard palate was delayed to minimize impairment of maxillofacial growthNowadays, literature shows that speech outcomes are better when soft and hard palate repair is completed before speech developmentIt is around 9-12 monthsMost prefer to repair one stage before 12 months of ageSome prefer two-stage repair soft palate repair at 3-6 months and hard palate at 12-15 monthsANATOMY:Minor submucous cleft: affect soft palate onlyBifid uvulaNotching of posterior nasal spineTranslucency in mid palatine region of soft palate due to lack of muscleAbnormal orientation and attachment of the muscles, primarilyLevator palatiniTensor palatineMusculus uvulaMuscle bundle insert longitudinally into the posterior edge of palatine bone instead of in midline

PRINCIPLEMain principle is to detach and retropose the abnormal insertion of levator palatini and join the in midlineIn hard palate reflect mucoperiosteal flap based on greater palatine arteriesTWO-FLAP PALATOPLASTYCommonly usedFor complete unilateral and bilateral cleft of palateEdges of cleft are incised from alveolus to base of uvulaBilateral full thickness muco periosteal flap is raisedLevator palatini muscles are released and dissectedReposition horizontally and suturedBilateral releasing incision are made to decrease tensionVON LANGENBECK REPAIRFor secondary palate cleftBilateral releasing incisions are made muco periosteum is elevatedComplete stripping of nasal mucosaThen close nasal layer, muscle and oral mucosa in layers

FURLOW Z-PLASTY REPAIRUses two reversed Z-plasties of oral and nasal mucosa to repair the cleftADVANTAGE:Restore the normal anatomic position of levator palatini in midlineIncreases soft palate length

VEAU-WARDWILL-KILNER (V-Y PUSHBACK) REPAIRwas the commonest technique V-Y procedure is performed mucoperiosteal flap and the soft palate are retroposed and the palate is lengthened. it leaves an extensive raw area anteriorly and laterally along the alveolar margin with exposed bare membranous boneheals with secondary intention.This causes shortening of the palate and velopharyngeal incompetence. alveolar arch deformity anddental malalignment.

SPEECH DYSFUNCTIONResonanceArticulationPhonationLanguage delayCAUSEVelo pharyngael insufficiencyOro nasal fistulaWeak lip pressureAbnormal tongue pressureMalposed teethAbnormal jaw relationshipNeuromuscular dysfunctionHearing lossVELOPHARYNGEAL INSUFFICIENCY(VPI)It is as a failure of the separation between nose and mouth, because of an anatomical dysfunction of thesoft palate, the lateral or posterior wall of the pharynx.leads to functional problems with speech (hypernasality), eating and breathingDIAGNOSIS OF VPISPEECH ANALYSIS:The main symptom is hypernasality of the voice. The patient is unable to create normal resonance because of nasal air emission.NASOMETRY:It is a test which calculates a ratio between the nasal and oral sound emissions. Ratios are compared with a normal ratio. Preoperative ratios are compared with postoperative ratios.NASOENDOSCOPY:provides an overview of the anatomy of the velopharynx during phonationCannot be used in kidsWith nasal obstructionVIDEOFLUROSCOPY:a radiographic technique, mostly to demonstrate the lateral and posterior wall of the pharynx.MRITREATMENT (SURGICAL)FLAP PHARYNGOPLASTY:When the pharyngeal flap is used, a flap of the posterior wall is attached to the posterior border of thesoft palate.The flap consists of mucosa and the superior pharyngeal constrictor muscle. The muscle stays attached to the pharyngeal wall at the upper side (superior flap) or at the lower side (inferior flap).The function of the muscle is to obstruct the pharyngeal port at the moment that the pharyngeal lateral walls move towards each other

NON SURGICALused for nonsurgical closure.There are two types of prostheses.speech bulb: The speech bulb is an acrylic body that can be placed in the velopharyngeal port and can achieve obstructionpalatal lift prosthesis: is comparable with the speech bulb, but with a metal skeleton attached to the acrylic body to obstruct the velopharyngeal port.

ALVEOLAR CLEFT REPAIRGoal is to maintain bony continuity of alveolar ridgeProvide bone support for the teeth adjacent ti cleftSeal nasal and oral cavityFacilitate eruption and orthodontic movement of teeth mostly canineMaintain healthy periodontiumProvide alar base supportImprove nasal symmetryTIMING OF REPAIRSecondary bone grafting at the age of 7-11 years is doneThis coincides with development of one half to two thirds of root of canine or lateral incisor if present.Graft material is corticocancellous bone from iliac crest, rib, symphysis, calvarium and tibia.Before grafting of bone maxillary expansion should be completed to provide acess to the defect.

PRINCIPLESClosure of nasal floorRemoval of any supernumerary teeth in the cleftFill with boneApproximate oral mucosaOrthodontic movement can be initiated in 3-4 months if requiredPalatal retainer should be placed after surgery to prevent loss of maxillary width ORTHODONTIC MANAGEMENT2-10 weeks:Pre surgical orthopedics6-10 years:Phase I orthodontics:Maxillary expansion for alveolar bone graftMaxillary protraction with face mask if required10-14 years:Maintain maxillary expansion and alignment of teethMonitor facial growth and eruption of teeth14-18 years:Phase II orthodontics:Orthodontic treatment with full fixed appliancePrepare orthognathic surgery if requiredExtract teeth if mandibular arch is crowdedDecide to replace absent teethREPLACEMENT OF TEETHMost common teeth is maxillary lateral incisor Managed by closing the space and substitute the adjacent canine in its positionOr by opening the space and replace it with a fixed or removal prosthesisEnd osseous implant can be placed when adequate bone space and bone quantity is presentAlveolar ridge augmentation with cortical onlay can be performed 4 months before placement of implantRemovable prosthesis can be given if there isLoss of pre maxillaSevere deficiency of bone heightDeficient bone heightScarring of overlying soft tissueLack of lip support

CORRECTION OF MAXILLARY HYPOPLASIAMaxillary hypoplasia is due to restriction of midfacial growthIt can be attributed partly due to congenital malformationScar contracture following primary palate surgeryCan be done by:Orthodontic management with dental compensationSurgical maxillary advancementSECONDARY LIP AND NOSE DEFORMITYFinal phase for the cleft patientInvolve correction of residual lip and nasal deformityLip and nose revision is done during late adolescence after correction of maxillary hypoplasiaDone to achieve balanced facial estheticsSECONDARY LIP DEFORMITYResidual deformity in unilateral cleft includeMismatch of cutaneous vermillion border realignment and small triangular flap or Z-plasty procedureVertical shortening of lip full thickness revision of skin, muscle and mucosaHypertrophic scarResidual deformity in bilateral cleft includeTight upper lip Abbe flapPoor philtral columnPoor cupids bowPoor vermillion show in midlineScarShallow labial sulcus perform Z-plastyCLEFT NASAL DEFORMITYCorrection is difficult becauseSoft tissue distortionScarringNasal stenosisUneven bony foundationDone by open rhinoplasty under direct visionAn esthetic smile and normal speech are two important requirements to make an identity and self esteemAbility to restore lip and palate function sre key to management of cleft patientTHANK YOU