Feeding Issues and Interventions in Infants and Children ... · Feeding Issues and Interventions in Infants and Children with Clefts and Craniofacial Syndromes Claire K. Miller, Ph.D.,

FeedingIssuesandInterventionsinInfantsandChildrenwithCleftsandCraniofacialSyndromesClaireK.Miller,Ph.D.,CCC-SLP1

ABSTRACT

Problemswithoralfeedingoccurinvaryingdegreesininfantsbornwithcleftlip/palateand/orcraniofacialsyndromes.Theextentofcleftingisassociatedwiththeseverityoffeedingproblems,andifcleftlip/palate occurs in conjunction with a craniofacial syndrome,additionalstructural,airway,andneuromotorissuesmaybepresent.The infant’s feeding and swallowing skills may be significantlyimpaired,characterizedbyinefficientoralfeedingskillscoupledwithpoorairwayprotectionabilityduringswallowing. Inadequateairwayprotectionduringswallowinghasseriousimplicationsfortheinfant’srespiratory health as sequelae of chronic aspiration during feedingmay include recurrent respiratory illness, pneumonia, and lungdamage. Feeding difficulty in nonsyndromic and syndromic cleftlip/palate infants has been documented as source of considerablestress for parents and can have a potential negative effect on theparent-infant bonding process. Therefore, timely identification offeeding problems by the speech pathologist with subsequentinterventionandmodificationinthefeedingmethodisessential,alongwithprovisionofearlyfeedinginstructiontofamilies.Theobjectiveofthisarticleistoreviewexpertopinionandavailableevidenceregardingfactors that influence feeding success and efficiency in infantswithnonsyndromic and syndromic cleft lip/palate. The types ofcompensatory strategies or interventions that are effective inalleviation of feeding and swallowing difficulties will be described.Descriptive reports, expert opinion, and available evidence fromclinicaltrialstosupporttheuseoffeedinginterventionsintreatmentarereviewed.

1 Speech Pathologist III and Program Director,AerodigestiveandSleepCenter,CincinnatiChildren’sHospitalMedicalCenter,Cincinnati,Ohio.

Addressforcorrespondenceandreprintrequests:ClaireK.Miller,Ph.D.,AerodigestiveandSleepCenter,Cincinnati Children’s Hospital Medical Center, MLC

4011,3333BurnetAvenue,Cincinnati,OH45229-3039(e-mail:[email protected]).

CommunicationDisordersRelatedtoCleftPalate,CraniofacialAnomalies,andVelopharyngealDysfunction;GuestEditor,AnnW.Kummer,Ph.D.,CCC-SLP,ASHA-F.

116 SEMINARSINSPEECHANDLANGUAGE/VOLUME32,NUMBER2 2011

Semin Speech Lang 2011;32:115–126.Copyright # 2011 by Thieme MedicalPublishers, Inc., 333 Seventh Avenue, NewYork,NY10001,USA.Tel:+1(212)5844662.DOI:http://dx.doi.org/10.1055/s-0031-1277714.ISSN0734-0478.

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Cleft lip/cleft palate (CL/CP) andcraniofacial syndromes are oftenassociatedwithspecialchallengesinregardto the feeding and swallowing process ininfancy, as well as beyond in certaincircumstances. Problems vary in degreedependinguponthedegreeofcleftingandif a craniofacial syndrome is present.Severefeedingandswallowingissueshavethepotentialtoresultinnutritionaland/orrespiratory compromise, as well as tocreate significant stress for families andcaretakers. Most reports offeeding/swallowing problems and theefficacy of feeding interventions innonsyndromic and syndromic CL/CP arefrom descriptive independent studies,though there is increasing availability ofempirical evidence to guide interventionsinpractice.

The infant feeding process dependsupon smooth synchronization of sucking,swallowing, and respiration, coordinatedby the neurological system. Rapid,sequential swallowing occurs during theapneicphaseof the inspiratory-expiratoryrespiratorypatterntoensureprotectionofthe airway. Precise coordination of allcomponents is critical; disruption in theprocesscanoccurifanatomic,neurological,orrespiratoryissuesarepresent.Inefficientoral feeding may result in inadequatevolumeofintakeforadequategrowth,andongoing problems with airway protectionduring swallowing present potentially

seriousconsequencestorespiratoryhealth.It is generally reported that CL/CP, in the

absence of other congenital structural,respiratory, or neurological issues, has only aminimal to moderate effect on feeding successinitially.1,2Thedegreeoffeedingdifficultywillvary

withthetypeandseverityofthecleft(unilateral,bilateral,incomplete,complete)andmayrequiresome modification of feeding technique.3,4 Forexample, an isolated cleft lip generallydoesnotinterferewithaninfant’sabilitytoefficientlysuckduringbottle-feeding,whereasanopeninginthepalate has a more profound effect on suckingefficiencyintermsofabilitytogeneratesufficientsucking pressure. This can result in inadequatevolumeoforal intake ifmodifications in feedingmethod are not made. The degree of palatalcleftingisreportedtocorrespondtothedegreeoffeeding difficulty, with more significant feedingproblems reflective of more extensive palatalclefting.5 Usually, an initial assessment by aspeech-language pathologist, with subsequentimplementationoffeedinginterventions,suchasspecializedcleftpalatebottlesandcompensatoryfeeding strategies and techniques, effectivelyalleviatestheinitialfeedingdifficulty.4,6

In contrast, CL/CP, in conjunction withsyndromes,sequences,andassociations,presentsa more complicated scenario. Oralmotordysfunction and pharyngeal swallowingdysfunctionmayoccursecondarytothestructuralissues, but also because of cranial nerveabnormalities or neuromotor components thatmay be present. The infantmay have problemswith theoralphaseof swallowing, coupledwithanimpairedabilitytoachieveandmaintainairwayprotection during the pharyngeal phase ofswallowing.1,6,7 Therefore, the infant withsyndromic CL/CP and associated neurologicalissues will likely have feeding and swallowingissues that will require ongoing input from thepediatric speech pathologist in regard totreatmentandmanagement.

KEYWORDS:Pediatric,craniofacial,feeding,swallowing,dysphagia

LearningOutcomes:Asaresultofthisactivity,thereaderwillbeableto(1)describecraniofacial issuesthatresult infeedingandswallowingdysfunction,(2)discussvariousassessmentprocedures,and(3)applyappropriateinterventiontechniquesformanagementoffeedingandswallowingdifficulties.

FEEDINGISSUESINCHILDRENWITHCRANIOFACIALSYNDROMES/MILLER 117

CHARACTERISTICSOFFEEDINGPROBLEMSDUETOCL/CPANDCRANIOFACIALCONDITIONS

NonsyndromicCleftsDescriptive reports of infants with CL/CPwithout accompanying medical issuesdocument varying degrees of feedingdifficulty. Usually the feeding difficultiesare attributed to the specific structuralproblem in the oral cavity and areconsidered oral phase in nature.6 Thepharyngeal phase of the swallow, whereswallowing is initiated and coordinatedwithairwayprotection,isintact.

Infantswithunilateralorbilateralcleftsofthelipmayhavesomeinitialdifficultyinlearning how to latch onto the nipple;however, they usually quickly adapt oncethe nipple is placed intraorally with theassistance of the feeder. The infant willinitiatesucking,usingreflexivetongueandjaw movements that are sufficient forefficientcompressionofthenippleagainstthe intactpartof thealveolusandpalate.Theremay be some transient difficulty inachieving negative pressure suction,dependinguponthedegreethatthelipsealon the nipple is compromised, but ingeneral, the infant is able to adapt. Thefeeder can assistwith use of awidebasenipple,aswellasexternalprovisionofliporcheeksupporttohelpmaintainlipclosureonthenipple.4,6

In contrast to infants with cleft lip,infants with cleft palate have difficultyachieving adequate suction due to thecouplingoftheoralandnasalcavities.Asaresult, breast-feeding may be impossible.Therearereportsthatdescribethelackofefficiency in the sucking patterns ofchildren with cleft palate. For example,Masareietalcomparedsuckingpatternsofinfants with nonsyndromic completeunilateralcleftlipandpalateorcleftpalateonlywithanoncleftinfantgroupandfoundsignificantdifferencesinregardtosucking

efficiency.8Shortersuckingbursts, fastersuckingrates, higher suck-swallow ratios, and increasedpositive pressure generation were noted in theinfants with CL/CP or cleft palate only incomparison with the noncleft cohort. The oralphase feeding problems that are typicallyencountered in these patients have beendescribed by multiple investigators and includepoor oral suction, lengthy feeding times, nasalregurgitation, excessive air intake, andinadequatevolumeoforalintake.4–6,9,10

Descriptivereportsofinfantswithaminimalcleftofthepalatesuggestthatthese infantsareoften able to adapt and feed without specialmodifications during bottle as well as breast-feeding.1,11 With a small posterior cleft, infantsmay occlude the cleft with the tongue duringsucking and thereby create the condition fornegative pressure to be generated normally. Incontrast, infantswhopresentwitha cleft in thehard and soft palate region may have moresignificant feeding difficulty initially with bothbottle- and breast-feeding. Use of a modifiedbottle/nippleorcupfeederhasbeenreportedashelpfulinassistingwithfeedingefficiency.9,12,13Ifthe mother wishes to breast-feed, consultationwith a lactation consultant who has experiencewithinfantswithspecialneedssuchcleftlipandpalatecanbeadvantageous.14Interventionsmayalso include nutritional assessment andrecommendationstomaximizenutritionalintake,as well as specific feeding techniques includingfeeder assisted squeezing for milk expression,pacing of oral intake to provide fluid in rhythmwith the infant’s movement and reactions, andoralfacilitationtechniques,suchasjawandcheeksupport to assist with increasing oral controlduringfeeding.6,14

CraniofacialSyndromeswithCleftLipwith/withoutCleftPalateOverall, there is an increased likelihood ofdysphagiaininfantsandchildrenwithcraniofacialmalformations with and without associatedCL/CP.15,16 The oral phase of feeding may beaffected secondary to factors that impact uponthepositionandmobilityof lip, jaw,andtongue


movements necessary for effective andefficient feeding. Craniofacial anomaliesthat involve maxillary or mandibularhypoplasia and/or hypoplasia of themidface create the potential for airwayobstruction.Therefore,theneedforairwaymanagement is the first priority.17,18 Inaddition,multiple cranial nerve palsies orabnormalities may be present, leading toimpairmentoftheessentialoral-motorandsensorynervefunctionsnecessaryforsafeand effective feeding. Poor airwayprotectionduringswallowingwithchronicaspiration may lead to recurrentrespiratory illness, pneumonia, and lungdamage, which presents significantimplications overall in terms of long-termrespiratoryhealth.

The potential for parental stressregarding the feeding issuesandneed forspecific feeding instruction are significantissues that need to be addressed by theprofessionals involved. Overall, it isgenerallyagreedthattheriskforsyndromicCL/CP-associated feeding difficulty andswallowingdysfunctionisgreat,andtherearemultiple issues that arise in regard toadequacy and ease of nutritional intake,respiratory health, and the potential forparental/caretakerstress.15

The presence of oral, oropharyngeal,and/ or pharyngeal dysphagia has beendocumented in conjunction withsyndromesassociatedwithcleftlip,withorwithout cleftpalate, including trisomy13,Van der Woude syndrome, Opitzsyndrome,orofaciodigitalsyndrome,Wolf-Hirschhorn syndrome, hemifacialmicrosomia,CHARGEassociation,andfetalalcoholsyndrome.Perhapsthemostseverefeedingandswallowingdysfunctionoccursin infants with trisomy 13, who are bornwithmultiplebirthdefectsincludingseverebrain abnormalities, congenital heartdefects,spinabifida,andoftenunilateralorbilateral CL/CP. It is known that mostinfantswithtrisomy13donotlivebeyondtheir first birthday, though there arereports of longer-term survivors. These

infants have such severe oral and pharyngealswallowingimpairmentthattotalenteralfeedingsarenecessarytomeettheirnutritionalneeds.15

VanderWoudesyndromeisconsideredtobeamong the most common syndromic causes ofCL/CP. Overall development is normal, and thefeeding issues that occur are similar to thoseexperienced by infants with nonsyndromic CL/CP.15Incontrast,individualswithOpitzsyndrome(also known as hypertelorism-hypospadiassyndrome,Opitz BBB syndrome, andOpitz Friassyndrome) have multiple features includingCL/CP. In addition, a laryngeal cleft may bepresent, thereby creating the potential forsignificant airway protection compromise withfeeding. The laryngeal cleftmay vary in degree,andthereforeobjectiveassessmentofswallowingbyinstrumentalmeans,suchasvideofluoroscopyorendoscopy,isvaluableindeterminingwhetherairway compromise is occurring.19 If aspirationoccurs in association with oral feeding,subsequentsurgicalinterventionofthelaryngealcleftmaybeindicated,aswellasimplementationofthefeedingmod-ificationsforcleftlip/palate.20

Infants born with orofaciodigital syndrometype Ioftenpresentwithamidlinecleft lipwithmultipleoraltissuewebs,cleftpalate,aswellastongue abnormalities, such as notching orlobulations.15 Feeding and swallowing issuesoccur, not only because of the cleft palate, butalsosecondarytotherestrictionsintonguerangeofmotionnecessaryforefficientoraltransferandforswallowinginitiation.

Wolf-Hirschhorn syndrome is a rarechromosome disorder caused by a deletion ormissingportionoftheshortarmofchromosome4.Featuresofthissyndromeincludecleftlipandpalate, hypotonia, microcephaly, and acharacteristic facial appearance (described as a‘‘Greekwarriorhelmet’’)withhypertelorismanda prominent nasal bridge.15 Feeding/suckingdifficultieshavebeenreportedsecondarytotheCL/CP and poorly coordinated swallowing withaspiration. Poor growth is frequent, along withheartdefectsandseizures.21

Hemifacial microsomia (also referred to asGoldenhar syndrome, oculoauriculovertebral


dysplasia, or facioauriculovertebralspectrum) is characterized by orofacialclefts, facial nerve weakness, limited jawrange, unilateral velar paresis, as well ascleft palate. The facial nerve weaknesslimits active cheek and lip movementsduring feeding. The restriction in jawexcursionlimitssuckingefficiency,andthevelarparesisand/orcleftpalatecontributeto difficulty with generation of negativepressureduringsuckingandwitheffectiveposteriortransferofliquidforswallowing.6

Infants with CHARGE syndrome(acronym for coloboma, heart defect,choanal atresia, retarded growth and/ordevelopment, genitourinary anomalies,and ear anomalies and/or deafness) havebeendocumented tohave significant oralfeeding dysfunction. The choanal atresiaandresultingrespiratory issues,aswellassignificant cranial nerve abnormalitiesimpacting sensory and motor function inthepharynxandlarynx,createsevereoral-motor and swallowing difficulty in someindividu-als.22,23

Lastly, infants born with fetal alcoholsyndrome,causedbyintrauterineexposuretosignificantamountsofalcohol,mayalsopresent with significant feeding andswallowing issues, secondary to cleft lipand palate. Structural and neurologicalissues are reflected in varying degrees oforal-motorandswallowingdysfunction. Inaddition, fetal alcohol syndrome isoneofthemorecommoncausesofPierre Robin sequence (PRS) and cleftpalate.15

CraniofacialSyndromesandSequenceswithCleftPalateVarying degrees of oral and pharyngealdysphagiahavebeendescribedinpatientswith sequences and syndromes known tobe associated with cleft palate.1,7,15,16,24

Such sequences and syndromes include:

PRS, Stickler syndrome, velocardiofacialsyndrome,andMoebiussyndrome.

PRS is characterized by retrognathia,posterior placement of the tongue, and often acleftpalate.Theposteriorpositionofthetongue(glossoptosis) causes a blockage in the pharynxandairway.Theresultantairwayobstructioncancause difficulty with coordination of respirationduring feeding and swallowing and seriouslycompromised airway protection, resulting inaspiration.1,16,24 In a prospective longitudinalstudyoffeedingskills inacohortof infantswithclefts and syndromes, infants with PRS werefoundtohaveasignificantlyhigherriskoffeedingproblems.7 Lidsky et al reported the benefit ofearlyairwayinterventionforinfantswithPRS.25Instudyofinfantsdividedintogroupsaccordingtoisolated PRS and PRS with additional disordersandsyndromes, the investigators foundthattheinfants with isolated PRS who received earlyairway intervention to relieve upper airwayobstructionweremoresuccessfulwithoralfeeds.AgreaternumberofinfantswithisolatedPRSwhoreceived later airway intervention requiredgastrostomytubes,andtheinfantswithPRSwithadditional disorders and syndromes tended torequire gastrostomy feedings regardless of thetimingofairwayintervention.

Reports indicate that airway managementinterventions for PRS vary across centers, andtreatment decisions are individualized. Theoptions include the use of prone or side-lyingpositioning,placementofanasopharyngealtube,tracheostomytobypasstheareaofupperairwayobstruction,ormandibulardistractiontoimproveposteriorairwayspace.

Feedingtrialsarepossiblewhiletheinfantisplaced inpronepositioning,andtherearesomereports that indicate this strategy is effective inimprovingfeedingability.4,6Sidelyingpositioning,withtheinfant’strunkstraightandwellsupportedbythefeeder,hasalsobeenreportedaseffectiveinhelpingtheretractedtonguefallforward,andtherefore facilitating feeding.4 Mandibulardistraction is a recent advancement in thetreatmentofairwayobstructionthatinvolvesanosteotomy (mandibular incision) and placementofadevicethatallowsseparationofthetwoendsof bone. New bone is created in the gap, and


thereby elongates the mandible. Thiscauses forward movement of the tonguebase and surrounding soft tissues awayfrom the pharyngealwall, thus alleviatingtheupperairwayobstruction.Infantswhoundergomandibulardistractionareunabletofeedduringthedistractionastheirjawsare immobilized and they are unable tosuck. However, once the distractionprocedure is completed, feeding can beresumed. Several reports indicatesignificant improvement of oral feedingfollowingmandibulardistraction.26,27

Stickler syndrome can be variable interms of the clinical presentation, but ischaracterized by the presence of PRSincluding cleft palate, myopia, andsensorineuralhearingloss.TheissueswithfeedingaresimilartothoseininfantswithPRS.15

Velocardiofacial syndrome (alsoreferred to as DiGeorge syndrome or22q11.2 deletion syndrome) is variable inpresentation. It typically includes palatalanomalies, such as cleft palate andvelopharyngeal insufficiency, congenitalheart defects, and dysmorphic facialfeatures.28 Feeding issues have beendescribed inpatientswithvelocardiofacialsyndrome, which range from oral-motordysfunction secondary to low orofacialmuscle tone and difficulty with motorsequencing of oral motor movementsnecessary for efficient feeding. Moreserious swallowing issues have beenreported in patients with velocardiofacialsyndrome with accompanyinglaryngotracheal abnormalities andpharyngealhypotonia.15

Moebiussyndromeischaracterizedbybilateral facial and oral musculatureweakness secondary to absence orunderdevelopment of the abducent andfacial nerves. Therefore, the infant mayhavelimitedabilitytoachieveandmaintainan adequate lip seal on the nipple forefficient sucking.Ahighpalatal vaultmay

be present, resulting in potential difficulty inmaintaining an adequate tongue-palate seal forefficient sucking. The possible presence of cleftpalate contributes to the overall feedingdifficulties experienced by the infant withMoebiussyndrome.29,30

CraniosynostosisSyndromesCraniosynostosisistheprematurefusionofoneormoreofthecranialsutures,resultinginabnormalgrowth of the skull. Children with isolatedcraniosynostosis generally do not have othermalformationsoraccompanying issuesandtendto have an excellent prognosis.15 In contrast,craniosynostosis syndromes, such as Crouzonsyndrome, Apert syndrome, Pfeiffer syndrome,and Saethre-Chotzen syndrome, have varyingclinical features that can include feedingproblems.17 Midface hypoplasia is a commonpresentingfeatureinSaethre-Chotzensyndrome,and a small number of infantswill also have anaccompanyingcleftpalate,creatingthepotentialfor difficulty with the oral-motor mechanicsnecessary for efficient feeding. Infants withCrouzon syndrome have cranial and facialinvolvement that can also interfere with oral-motor movements necessary for feeding. Cleftpalate may also occur with Crouzon syndrome,though this is reported to be uncommon.15,17

Infants with Apert syndrome have midfacehypoplasia, a narrow palate, and an increasedfrequency of cleft palate in comparison toCrouzonsyndrome.15Inaddition,choanalstenosisandnarrowinginthenasalandpharyngealairwaycreate the potential for respiratory obstruction,whichmustbe resolvedprior topresentationoforalfeeding.Lastly,infantswithPfeiffersyndromemaypresentwith significantmidfacehypoplasiaand airway compromise resulting from trachealanomalies and upper airway stenosis. Thechildren have been reported to have significantdifficultywithfeedingandswallowingininfancy,withpoorgrowth.31

OtherCraniofacialAnomalies


Treacher-Collins syndrome andBeckwithWiedemann syndrome areadditionalgeneticdisordersthatareoftenlinkedwithfeedingandswallowingissues.Treacher-Collins syndrome has a variablepresentation,butusuallyincludesPRSwiththeaccompanyingfeedingdifficulties.17OfnoteisthatmostindividualswithTreacher-CollinsandPRSdonothaveclefts. InfantswithBeckwith-Wiedemannsyndromehavemacroglossia(largetongue),whichhasthepotential tocauserespiratoryandfeedingproblems.The tonguemaybeobstructiveand interfere with normal breathing.Limitationsinthemovementofthetonguemay cause difficulty with the degree ofmotionneeded foranefficientoralphaseoffeeding.6

COMMONLYUSEDFEEDINGINTERVENTIONSBestpracticedictatesthatinterventionsareevidence-based; however, the availabilityofevidencesupportingspecificstrategiesinthe management of feeding andswallowing issues in patients with CL/CPand craniofacial syndromes is limited.Interventions described in the literaturevary between centers and betweenindividual practitioners. Often,combinations of interventions areimplemented,whichmakesdeterminationofspecificeffectonoutcomesproblematic.Variation in the severity of clefting, thepresence and features of accompanyingsyndromes, as well as other complicatingmedical issues contribute to the difficultyof determining the effects of specificinterventiononpatientoutcome.

The following is a summary of data-driven reports, as well as reports basedupon expert opinion. There is also be adescriptionoffeeding interventionsfoundtobeeffectiveintheclinicalenvironment.

Oral Feeding Facilitation TechniquesCommonly used techniques to facilitate

oral feeding in infants with nonsyndromic andsyndromic CL/CP and craniofacial syndromesinclude positioning, oral facilitation techniques,assistedfluiddelivery,pacingoffluiddelivery,andchangesintheviscosityoffluid.ThesetechniquesaresummarizedinTable1.

Optimal positioning as key to successfulfeedingforfacilitationofcoordinatedjaw,cheek,lip, and tongue movements forsucking/swallowing is frequently described inreports of oral-motor intervention.4,10 Generalpositioningrecommendationsforfeedingincludeprovision of head support, with arms forward,trunkinmidline,andhipsflexed.10Positioningtheinfantwithacleftpalateinanuprightpositionofatleast60degreeswillallowgravitytoassistwithposteriortransferoffluidandwithswallowing.Italso helps to prevent nasal regurgitation,whichmayoccursecondarytothecleftpalate.4,6,32

Direct oral-motor techniques to facilitatenutritive intakemay include lip, cheek, and jawsupportprovidedbythefeeder.33Stabilizationofthe jaw by placing the middle finger under thechin and the index finger between the chin andthelowerlipassistsinprovidingastableplatformformovementsof the tongue, lips,andcheeks.4

Provisionofproprioceptiveinputtothetonguebyuse of slight downward pressure to themidlinemay help to initiate an appropriate suckingpattern.


Table1 OralFeedingFacilitationTechniques

Assistedfeedingrequiresthefeedertosqueeze the nipple in synchronywith theinfant’s sucking efforts. Turner et aldemonstratedthatassistedfeedingusingaspecialized nipple/bottle, such as theSpecial Needs feeder,was effective in asmall prospective study of lactationeducation and use of an obturator onfeeding efficiency.34 Though there are nodata-driven studies that provide a strongevidencebasisforthistechnique,therearenumerous descriptive reports of thebenefit in regard to feeding success andincreasedvolumeoforalintake.4,6,35

Pacingtherateofintakeisdonesothatorganization in sucking, swallowing, andrespiration will be maintained duringfeeding.36 The feeder monitors infantreaction(color,oxygensaturation,rhythmof sucking, feeding efficiency, respiratoryrate)duringfeedingandidentifiescuesthatsignifytheneedforpausingorchangingthepaceofintaketohelptheinfantmaintainastable and organized swallowing pattern.Whether the implementation of pacinginterfereswiththeusualrhythmoffeedingand createsmore difficulty remains to beseen. Current research in identificationofrespiratoryairflowandapneicpauseduring

swallowing will likely provide a means forobjective evidence in the future to support theuseof pacing as an evidence-based feedingstrategy.37

Increasingtheviscosityoffluidstoassistwithmaintenance of airway protection duringswallowing is another frequently describedinterventional strategy with little objectiveevidence to support. The rationale supportingchanges in liquid viscosity or thickened liquid isthat the slower flowof the liquidwill assist theinfant in maintaining airway protection duringswallowing. At this time, the reports of theefficacy of using thickened fluid are primarilyanecdotal in nature. Medical and nutritionalconsultationisnecessarypriortoimplementationof thickened fluids to confirm that the infant’scaloric, fluid, and nutritional requirements aremet.

Technique Rationale

Positioning Maintainingflexion,midlineorientation,andneutralalignmentoftheheadandneckfacilitatesoral-motorpatterns,airwayprotection,andefficientfeeding.

Positioningat60degreesassistswithposteriortransferoffluidanddecreasestendencyfornasopharyngealreflux.

Assistedfeeding Assistivesqueezing,insynchronywithinfant’ssuckingefforts,helptocompensateforinfant’sinabilitytocreatesuctionforfluidextraction.

Thisrequiresspecializedfeedingequipment.Externalpacing Imposingpausesduringinfantfeedinghelpstomaintainappropriaterespiratoryphase

patternforairwayprotection.Lip,cheek,andchinsupport Supportinglips,cheek,andchinbyexternalsupportfacilitatessucking

movements.Liquidviscosity Thickeningliquidsslightlycreatesamorecohesivebolusthatmovesmoreslowly

throughthehypopharynx,allowingmoretimeforairwayclosuretooccur.Medicalandnutritionalconsultationisessentialsothatcaloric,fluid,andnutritionalneedsaremet.


Table2 SpecializedFeedingEquipment

SpecializedFeedingEquipmentThere is a wide variety of specializednipples,bottles,andcupsavailableforusewith infants who present with CL/CP orother feeding difficulties (Table 2).Likewise, there is a wide variety ofindependent descriptive studies regardingthesespecializeditemsthatreportvaryingdegrees of effectiveness with feeding. Ofnoteare two randomizedcontrolled trialsspecific to infants with CL/CP thatcomparedfeedingequipmentandprovidemoderatetostrongevidenceregardingtheuseofspecifictypeofequipment.12,13,38TheMead Johnson cleft palate feeder,combined with a nutrition interventionprotocol, was found to be effective in

support of normal growth in infants with cleftpalateorcombinedCL/CP.38Acomparisonof

TypeofEquipment Rationale

Nippletype Considerationsincludenippleshape,nipplelength,andtypeandsizeofnipplehole.

Selectiondependsuponindividualinfantanatomyandoralmotorskills.

Mustsupportappropriateoral-motorpatternsduringsucking.

SpecialNeedsFeeder(formerlyHabermanandMini-HabermanFeeder)

Thenippleisdesignedtoreleasemilkfrominfantcompressionefforts,sosuctionisnotrequired.

Thenippleissoftandpliablewithone-wayvalvethatpreventsrapidfluidflowandopensonlywheninfantsucks.

Thenippleisdesignedforassistivesqueezing.Flowcontrolcanberegulatedbyfeeder.Mini–specialneedsfeederisdesignedforsmallerorpremature

infantswithcleftlip/palate.SoftCupAdvancedCupFeeder(Medela;McHenry,IL)

Thecupismadeofsoftpliablesiliconeandiscontoured.Acontrolvalve,withself-fillingcuplikereservoirwithflowrate,iscontrolledbythefeeder.

Activesuckingisnotrequired.RossSyringeNipplewithcompressiblebottle(AbbottNutrition;AbbottPark,IL)

Thisverythin,longnipplerequiresassistivesqueezeforformuladeliverywithclosemonitoring.

PigeonNipple/Bottle(PigeonCorp.;Chuo-ku,Tokyo,Japan)

Thenipplehasonesidewiththickwallforplacementagainstroofofmouthandonesidewithathinwallfortheinfanttosuck.

Thebottleissoftandcompressible,allowingassistedfeeding.Thenipplecanbeusedwithotherbottles.

MeadJohnsonCleftPalateNurser(MeadJohnsonNutrition;Glenview,IL)

Soft,compressiblebottleiseasilysqueezed.Itisfittedwithacross-cutnipple.Astandardnipplecanbeusedwithamodifiedholeasnecessary.


a compressible bottle used with anorthodontic nipple was shown to lead togreater weight gain and headcircumferenceinagroupofnonsyndromicinfantswithCL/CP.13

There are varying reports fromexperimental evidence regarding the typeof nipples that are most effective,considering the following four nippleparameters:pliability,shape,size,andholetype (Table 3).6,39 There is generalagreement that the nipple needs to bepliableenoughtoallowreleaseofformulaor breast milk with limited compression,but that the nipple must also be firmenough to provide an appropriate degreeof proprioceptive input to stimulatesucking. Reports regarding appropriateflow rate are varied. Overall, there iscurrentlynodefinitiveevidence,otherthanexpert opinion and clinical experience, toguide decision making regarding nippleselection. Generally, the type of nipplechosen should be based on each infant’soral-motorskillabilityandonthetypeanddegree of clefting.4 Therefore, clinicaldecisionmakingatthistimeisbasedontheinfant’ssuccessandeaseoffeedingwithaparticular nipple, as well as theinstrumental assessment of airwayprotectionandswallowingwhilefeeding.

Parental/CaretakerEducationonFeedingTechnique

Feedingisoneofthemostimmediatechallengesfaced by parents and caretakers following thebirth of a baby with a CL/CP or craniofacialanomaly.Therefore,theclinician’sroleinassistingwithappropriatefeedingadaptationsis

Table3 NippleCharacteristics

essential.Prenataldiagnosisoforofacialcleftsandearly provision of information regarding feedingtechniques has been found to be effective inimprovingpatientsatisfaction.43Thisisimportanttoconsiderastheearlymaternalbondingprocesshas been reported to bemoredisruptedby theinitial feeding problems than by the facialdefect.44 Studies have shown that instructionregardingfeedingmethodsinthenewbornperiodis a parent/caretaker priority immediatelyfollowing birth.8,40–42 Of concern are parentalreports detailing the poor quality of adviceregarding appropriate feeding techniques andinterventionsandthefactthattheyoftenreceiveconflicting information regarding therecommendedfeedingtechniquewithinthesameinstitution.44

PalatalObturatorsAn additional feeding intervention is a feedingobturator, which consists of an acrylic plateinserted into the mouth over the hard palate,essentially closing thepalatal defect. Separationbetweenthenasalcavityandtheoralcavitycanthus be obtained. There are differing reports inthe literature regarding the effectiveness of a

NippleType Pliability FlowRate Shape HoleTypeandSize

Nukorthodonticnipple(GerberCo.;Fremont,MI)

Soft High Broadbase Holeonsurfacetip

StandardNipples Moderatelyfirm

Lowtovariable Round,crosssection;straight,tapertoflaredbase

Single,cross-cutormultipleholes

RossCleftPalateSyringeNipple

Soft Feederregulated Long,thin Largehole

MeadJohnsonCleftPalateFeederNipple

Soft Feederregulated Customized Cross-cut

SpecialNeedsFeederNipple

Soft Feederregulated Customized Slit


palatalobturator for improvementoforalfeedingability.45,46Theobturatorprovidesasurface by which the infant can achievenipple compression; however, there arediffering opinions regarding the ability togeneratenegativepressure.Thecombineduse of a palatal obturator, Habermanfeeder, and education by a lactationconsultant has been reported to reducefeeding time and significantly increasevolume of oral intake.34 Disadvantages ofobturators include the expense and thedifficultyofuse.Intraoralplacementoftheobturator can be challenging, irritation tothe oral tissues may occur, and ongoingadjustment and replacement toaccommodategrowthisnecessary.

PalatalObturatorsAn additional feeding intervention is afeeding obturator, which consists of anacrylicplate inserted into themouthoverthe hard palate, essentially closing thepalatal defect. Separation between thenasalcavityandtheoralcavitycanthusbeobtained.Therearedifferingreportsintheliterature regarding the effectiveness of apalatalobturator for improvementoforalfeedingability.45,46Theobturatorprovidesasurface by which the infant can achievenipple compression; however, there arediffering opinions regarding the ability togeneratenegativepressure.Thecombineduse of a palatal obturator, Habermanfeeder, and education by a lactationconsultant has been reported to reducefeeding time and significantly increasevolume of oral intake.34 Disadvantages ofobturators include the expense and thedifficultyofuse.Intraoralplacementoftheobturator can be challenging, irritation tothe oral tissues may occur, and ongoingadjustment and replacement toaccommodategrowthisnecessary.

InterventionstoAssistwithBreast-Feeding

Whetheror not breast-feeding is feasible in theinfantwithCL/CP(syndromicornonsyndromic)isoften an immediate question, and historicallyopinions have varied. There are descriptivestudiesthatindicateinfantswithCL/CPareabletosuccessfullybreast-feedatleasttosomedegree,though supplemental bottle-feeding may benecessary to meet nutritional needs.47 Otherreports cite less successwith breast-feeding forinfantswithcleftpalatethanforinfantswithcleftlipornocleft.5Ingeneral,thesuccessofbreast-feeding(asinbottlefeeding)willdependuponthedegree of clefting and the status of the airway.Infantswithasmallcleftinthesoftpalatemaybeable to breast-feed with some success; incontrast, infants with more significant palatalcleftingwilllikelyhaveconsiderabledifficultyandrequireanalternativefeedingmethod.

Mostpediatriciansandhealthcareprovidersagree that breast milk is best for infants, andevidencedoesshowthatbreast-feedingappearsto be protective against otitismedia in childrenwith CL/CP.48,49 Fortunately, clinical protocolshave been developed by the Academy ofBreastfeeding Medicine to manage commonmedical problems that may impact uponbreastfeeding success, including CL/CP.14 Theprotocols serve as guidelines and acknowledgethat individual variations in treatment areappropriateaccordingtotheneedsofeachinfant.Ininfantswithacleftlip,theguidelinesadvisethatthe infantshouldbepositionedso that thecleftlipisorientedatthetopofthebreast.Themothermay occlude the cleft with her thumb or herfinger,orprovidecheeksupporttodecreasethewidthofthecleftandincreaseclosurearoundthenipple.6,14 Semiupright positioning is advised tominimizenasalregurgitation.

For infantswith cleftpalate,positioning thebreasttowardthesideofthepalatethathasthemost bone promotes better compression andprevents the nipple frombeing pushed into thearea of the cleft. The guidelines state thatassessmentofbreast-feedingpotentialininfantswithsyndromicCL/CPshouldbedoneonacase-by-case basis, taking into consideration theadditional features of the syndrome that mighthaveanimpactonbreast-feedingsuccess.


CONCLUSIONFeedingandswallowingdifficultiesvary inseverity in the nonsyndromic andsyndromic CL/CP population. There are amultitude of medical and neurologicalfactors that impact feeding success andefficiency that the pediatric speech-language pathologist must consider.Compensatorystrategiesandinterventionsto assistwith the feeding and swallowingdifficultieshavepreviouslybeendescribed,primarily in descriptive studies and casereports rather than extensive systematicresearch. However, the availability ofevidence to support the use of specifictechniques,suchasspecificequipmentandassisted feeding methods, is emerging.Continued research to develop a strongevidence base to support the efficacy ofspecificfeedinginterventionsisneededforbestclinicalpractice.

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Feeding Issues and Interventions in Infants and Children ... · Feeding Issues and Interventions in Infants and Children with Clefts and Craniofacial Syndromes Claire K. Miller, Ph.D.,