2/6/2016

Congenitaldiaphragmaticherniaintheneonate

OfficialreprintfromUpToDate
www.uptodate.com2016UpToDate

Congenitaldiaphragmaticherniaintheneonate
Authors
HollyLHedrick,MD
NScottAdzick,MD

SectionEditor
LeonardEWeisman,MD

DeputyEditor
MelanieSKim,MD

Contributordisclosures
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:May2016.|Thistopiclastupdated:Apr05,2016.
INTRODUCTIONCongenitaldiaphragmatichernia(CDH)isadevelopmentaldefectofthediaphragmthat
allowsabdominalvisceratoherniateintothechest.Affectedneonatesusuallypresentinthefirstfewhoursof
lifewithrespiratorydistressthatmaybemildorsosevereastobeincompatiblewithlife.Withtheadventof
antenataldiagnosisandimprovementofneonatalcare,survivalhasimproved,buttherestillremainssignificant
riskofdeathandcomplicationsininfantswithCDH.
Theclinicalmanifestations,diagnosis,andmanagementoftheneonatewithCDHwillbereviewedhere.The
pathogenesis,anatomy,incidence,andprenataldiagnosisandmanagementofCDHarediscussedseparately.
(See"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement".)
EFFECTONPULMONARYDEVELOPMENTBecauseherniationoccursduringacriticalperiodoflung
development,clinicalmanifestationsofCDHresultfromthepathologiceffectsoftheherniatedvisceraonlung
development.Withrisingseverityoflungcompression,therearecorrespondingdecreasesinbronchialand
pulmonaryarterialbranching,resultinginincreasingdegreesofpulmonaryhypoplasia.Pulmonaryhypoplasiais
mostsevereontheipsilateralside.However,pulmonaryhypoplasiamaydeveloponthecontralateralsideif
themediastinumshiftsandcompressesthelung.Arterialbranchingisreduced,resultinginmuscular
hyperplasiaofthepulmonaryarterialtree,whichcontributestotheincreasedriskofpersistentpulmonary
hypertensionofthenewborn(PPHN)[1].
CLINICALMANIFESTATIONS
PrenatalpresentationAlthoughroutineprenatalultrasoundscreeningmayidentifyCDHatamean
gestationalage(GA)of24weeks,thereisawiderangeofreportedsensitivityinitsdetection.Associated
otheranomalies(eg,cardiacabnormalities)areseeninapproximately50percentofCDHcases,andtheir
presenceimprovesthesensitivityofdetectingCDH.Prenatalpresentationanddiagnosisarediscussedin
greaterdetailseparately.(See"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",section
on'Prenataldiagnosis'.)
PostnatalfindingsPostnatally,infantswithCDHmostoftenpresentwithrespiratorydistressinthefirst
fewhoursordaysoflife.Thespectrumcanvaryfromthemorecommonpresentationofacuterespiratory
distressatbirth,tominimalornosymptoms,whichisobservedinamuchsmallergroupofpatientswho
presentlaterinlife.(See'LateCDHpresentation'below.)
Inpatientswhopresentasneonates,thedegreeofrespiratorydistressisdependentontheseverityoflung
hypoplasiaandthedevelopmentofpersistentpulmonaryhypertensionofthenewborn(PPHN).Postdelivery,
hypoxemiaandacidosisincreasetheriskofPPHNbyinducingareactivevasoconstrictiveelementtothe
preexistingfixedarterialmuscularhyperplasiacomponent.Insomecases,pulmonaryhypoplasiaissosevere
astobeincompatiblewithlife.(See'Effectonpulmonarydevelopment'aboveand"Persistentpulmonary
hypertensionofthenewborn".)
InmostcasesofCDH,herniationoccursontheleft.Rightsideddiaphragmaticherniasoccurin11percentof
casesandbilateralherniationin2percent[2].Diseaseseverityappearstobesimilarinpatientswithleftand
rightsidedlesions.
AdrenalinsufficiencyisreportedtobeacommonfindingininfantswithCDH.Inoneretrospectivestudyof58
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patients,adrenalinsufficiency(definedasacortisollevel15mcg/dL[415nmol/L])wasdemonstratedintwo
thirdsofthe34patientswhowereassessedforadrenalfunction[3].Inthisstudy,infantswithadrenal
insufficiencyweremorelikelytohaveherniationoftheliver,andhadmoresevereillnessrequiringepinephrine
forvasopressorsupport,highfrequencyventilation(HFV),andlongerdurationofinhalednitricoxide(iNO)
therapy.Inourpractice,wemayadministerhydrocortisonetherapyinseverelyillpatientswithhypotension
becauseoftheconcernforadrenalinsufficiency.(See"Shorttermcomplicationsofthepreterminfant",section
on'Lowbloodpressure'.)
Associatedanomaliesareseeninapproximately50percentofCDHcasesandincludechromosomal
abnormalities,congenitalheartdisease,andneuraltubedefects.(See"Congenitaldiaphragmatichernia:
Prenataldiagnosisandmanagement",sectionon'Associatedanomalies'.)
PhysicalfindingsPhysicalfindingsincludeabarrelshapedchest,ascaphoidappearingabdomen
(becauseoflossoftheabdominalcontentsintothechest),andabsenceofbreathsoundsontheipsilateral
side.InpatientswithaleftsidedCDH,theheartbeatisdisplacedtotherightbecauseofashiftinthe
mediastinum.
DIAGNOSIS
PrenatalManycasesofCDHarediagnosedprenatallybyroutineantenatalultrasoundscreening.(See
"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",sectionon'Prenataldiagnosis'.)
PostnatalForinfantswithCDHnotdiagnosedinutero,thediagnosisshouldbesuspectedinanyterm
infantswithrespiratorydistress,especiallyifthereareabsentbreathsounds.Thediagnosisismadebychest
radiographyshowingherniationofabdominalcontents(usuallyairorfluidcontainingbowel)intothehemithorax
withlittleornovisibleaeratedlungontheaffectedside(image1).Otherradiographicfindingsincludethe
contralateraldisplacementofmediastinalstructures(eg,heart),compressionofthecontralaterallung,and
reducedsizeoftheabdomenwithdecreasedorabsentaircontainingintraabdominalbowel.Thediagnosis
maybefacilitatedbyplacementofafeedingtube,aschestradiographymayshowthefeedingtubewithinthe
thoraciccavityordeviationfromitsexpectedanatomiccourse[4].IftheCDHisrightsided,thelivermaybe
theonlyherniatedorganandwillappearasalargethoracicsofttissuemasswithabsenceofanintra
abdominallivershadowonchestradiograph.
DIFFERENTIALDIAGNOSIS
PrenatalOtherthoraciclesionsincludedinthedifferentialdiagnosisforprenatallydetectedCDHare
diaphragmaticeventration,congenitalcysticadenomatoidmalformation,bronchopulmonarysequestration,
bronchogeniccysts,bronchialatresia,entericcysts,andteratomas.(See"Congenitaldiaphragmatichernia:
Prenataldiagnosisandmanagement",sectionon'Differentialdiagnosis'.)
PostnatalThedifferentialdiagnosisforCDHinatermneonatewithrespiratorydistressincludesother
causesofpulmonaryhypoplasia(eg,oligohydramniosfromchronicamnioticfluidleakorrenal
hypoplasia/dysplasia),andpersistentpulmonaryhypertensionofthenewborn(PPHN)(eg,meconium
aspiration).CDHisdifferentiatedfromtheseconditionsbythecharacteristicchestradiographfindingof
herniatedabdominalcontentsintothethorax.(See'Diagnosis'aboveand"Persistentpulmonaryhypertension
ofthenewborn".)
PRENATALMANAGEMENT(See"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",
sectionon'Obstetricalmanagement'.)
POSTNATALMANAGEMENT
OverviewThetreatmentofCDHintheneonatehaschangedsincethefirstreportsofsurgicalrepairinthe
1940s[5,6].Initially,CDHwastreatedasasurgicalemergencywithearlysurgicalintervention.Inthemid
1980s,itwasrecognizedthatmajordeterminantsofmortalityincludedpulmonaryhypoplasiaandpulmonary
hypertension.Asaresult,theemphasisshiftedfromearlysurgicalinterventiontopreoperativecaredirected
towardsoptimalmanagementofthesetwoassociatedconditions,followedbysurgicalrepair[79].This
approachhasimprovedsurvivaltoitscurrentreportedratesof70to92percent,fromtheprevious50percent
associatedwithearlysurgicalcorrection.(See'Survival'below.)
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ManagementofCDHencompasses:
Preoperativemedicalmanagement,consistingofcorrection(ifneeded)andstabilizationoftheinfant's
oxygenation,bloodpressure,andacidbasestatus.Acidosisandhypoxiaincreasetheriskofpulmonary
hypertension.Hypotensionincreasestheriskofrighttoleftshuntingthatcontributestotissuehypoxia.
Surgicalrepair,consistingofclosureofthediaphragmaticdefectandreductionofthevisceraintothe
abdominalcavity(picture1).
PreoperativemedicalmanagementAlthoughtherearenorandomizedclinicaltrials,severalcaseseries
haveshownthataggressivemedicalmanagementfollowedbysurgicalcorrectionimprovessurvivalin
neonateswithCDH[8,1012].Supportivemedicalmanagementconsistsofreducinglungcompression,
cardiovascularsupportwithfluidsandinotropicagents,andventilatorysupportusinghighfrequencyventilation
(HFV).Extracorporealmembraneoxygenation(ECMO)isusedinseverecasesofpatientswhoarenot
responsivetothesupportivemedicalinterventions.
InitialtreatmentThefollowinginterventionsareinitiatedstartinginthedeliveryroom:
IntubationandventilationOncethediagnosisismadeorsuspected,allpatientsareintubated.This
includesintubationinthedeliveryroomofprenatallydiagnosedpatients.Thisavoidstheuseofblowby
oxygenand/orbagmaskingthatresultingastric/abdominaldistensionandcompressionofthelung.The
infantshouldbeventilatedwithlowpeakinspiratorypressure(PIP,goal<25cmH2O)tominimizelung
injury.Delayinsecuringanadequateairwaymaycontributetoacidosisandhypoxia,whichincreasethe
riskofpulmonaryhypertension.(See"Persistentpulmonaryhypertensionofthenewborn".)
Anasogastrictubeconnectedtocontinuoussuctionisplacedinthestomachofinfantsoncethe
diagnosisofCDHissuspectedormade.Thisdecompressesabdominalcontentsandreduceslung
compression.
LineplacementTheinfantshouldhaveanumbilicalarterylineplacedforfrequentmonitoringofblood
gasesandbloodpressure(BP),andifpossibleanumbilicalvein(UV)catheterforadministrationoffluids
andmedications.Inpatientswiththeliverinthechest,theUVcatheterisoftendifficulttoposition,and,
therefore,oncethepatientisstabilized,othervenousaccessshouldbeobtained.
BPsupportshouldbegiventomaintainarterialmeanBPlevels50mmHgtominimizeanyrighttoleft
shunting.Supportincludestheuseofisotonicfluids,inotropicagentssuchasdopamineand/or
dobutamine,andhydrocortisone.
SurfactantadministrationAlthoughadministrationofsurfactanttherapyhasbeensuggestedintreating
infantswithCDH[13],itdoesnotappearthatsurfactantadministrationimprovesoutcomes[14,15].
However,wedoadministersurfactantinneonates34weeksgestationwithchestradiographicfindings
ofalveolaratelectasissuggestiveofrespiratorydistresssyndrome(RDS)(see"Pathophysiologyand
clinicalmanifestationsofrespiratorydistresssyndromeinthenewborn",sectionon'Diagnosis').In
addition,weadministersurfactantininfantswhounderwentfetaltrachealocclusionwhenthereleaseof
theocclusionislessthan48hourspriortodelivery.(See"Congenitaldiaphragmatichernia:Prenatal
diagnosisandmanagement",sectionon'Inuterotherapy'.)
Inhalednitricoxide(iNO)AlthoughseveralstudieshaveshownthatiNOdoesnotappeartohavelong
termbenefitsinpatientswithCDH,iNOadministrationiswidespreadintheUnitedStates[1619].Inour
center,priortoplacingthepatientonECMO,weutilizeiNOinselectpatientswithrespiratoryfailuredue
topulmonaryhypertensiondespitehavingreceivingmaximalventilatorysupport.(See"Persistent
pulmonaryhypertensionofthenewborn",sectionon'Congenitaldiaphragmatichernia'.)
VentilationAsnotedabove,oncethediagnosisofCDHismade,allpatientsareintubatedand
mechanicallyventilatedtopreventgastricdistensionandlungcompression.Ventilationstrategyisaimedat
minimizinglungtraumabecausebarotraumatohypoplasticlungsappearstobeacontributingfactorfor
mortalityandmorbidity[19,20].Ourventilationmanagementusestheminimalsettingstomaintainpreductal
oxygensaturationsabove85percentorpreductalpartialpressureofoxygen(PaO2)above30mmHg,and
allowsforpermissivehypercapnia(definedaspartialpressureofcarbondioxide[PaCO2]<65mmHgandan
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arterialpH>7.25)[15].Initiationofconventionalventilatormanagementincludespressurelimitedventilationat
ratesof30to70breathsperminuteatPIPof20to25cmH2O[7,11].PIPexceeding28cmH2Oisused
transientlyasabridgetoECMO.Positiveendexpiratorypressure(PEEP)shouldbemaintainedatphysiologic
levels(3to5cmH2O)wheneverpossible.Hyperventilation,hypocarbia,andalkalosismaydecreaseductal
shuntingandcontrolpulmonaryhypertensioninCDH[21,22],butattheexpenseofincreasedbarotrauma.
PermissivehypercapniahasbeenusedinneonateswithCDH,withreportsofincreasedsurvivalcompared
withhyperventilationandalkalization[79,11,23].(See"Mechanicalventilationinneonates",sectionon
'Conventionalventilation'.)
HFVisgenerallyreservedforneonateswhocontinuetohavehypoxiaandhypercarbia(PaCO2>65mmHg)
refractorytoconventionalventilation.AlthoughtheindicationsforHFVarenotclearlydefined,thereare
retrospectivereportsofeffectivePaCO2reductionandincreasedsurvivalinneonateswithCDH[2426].
However,atrialofconventionalmechanicalventilation(CMV)versusHFVforinfantswithprenatallydiagnosed
CDHfoundnostatisticallysignificantdifferenceinthecombinedoutcomeofmortalityorbronchopulmonary
dysplasia(BPD)betweenthetwomodesofventilation[27].PatientswhowererandomlyassignedtoCMVhad
bettersecondaryoutcomes,withfewerventilatordays,decreaseduseofECMOandpulmonaryvasodilators,
andshorterdurationofvasoactivedrugs.FurtherdataareneededtodeterminewhetherHFVhasarolein
managingneonateswithCDHbeyondtheinitialmode,andifso,todeterminetheindicationsforHFV[19].
(See"Mechanicalventilationinneonates",sectionon'Highfrequencyventilation'.)
Frequentbloodgasesareimportantparameterstofollowintheadjustmentofventilatorsettings.Oxygenis
startedatfractionalinspiredconcentration(FiO2)of0.5andadjustedbasedonmaintainingpreductaloxygen
saturationabove85percent.WeconsideriNOadministrationifpreductaloxygensaturationis<85percentor
pre/postductaldifferentialis>10percentdespitemaximalventilatorysupport.
Althoughparalysisandsedationreduceairswallowingandmayenhancecomplianceandreducesympathetic
vasoconstriction,potentiallyleadingtolowerventilatorsettings,someexpertsinthefieldbelievethattheloss
oftheinfant'sspontaneouscontributiontominuteventilationandincreasedthirdspaceedemanegatethe
benefitsofparalysis[7].Inourpractice,weavoidtheuseofparalyticagents,andusepancuroniumonlywhen
necessary(eg,failureofpatientventilatorsynchrony).
EchocardiographyEchocardiographyisperformedearlytodetectanyassociatedcardiacanomalies,
andtoestablishthepresenceandseverityofpulmonaryhypertensionandshunting,astheseconditionsmay
impactmanagementdecisions(image2AB).
CongenitalheartdiseaseThepresenceofassociatedseverecardiacanomaliesmayhaveanimpacton
howaggressivesubsequenttreatmentshouldbe,asthesurvivalrateofpatientsislowerinpatientswith
CDHandmajorcardiacanomalies(suchashypoplasticleftheartsyndrome)[2831].Thiswasillustrated
inthelargestcaseseriesof2636patientsfromtheCongenitalDiaphragmaticHerniaStudyGroup
(CDHSG)thatreportedsurvivalof41percentinpatientswithhemodynamicallysignificantcardiac
defects(n=280,11percentofcohort)comparedwitha70percentsurvivalforpatientswithoutcardiac
defects[29].
Itremainsunclearwhetherthereisadifferenceinsurvivalbetweenpatientswithsingleventriclecardiac
defectscomparedwiththosewithtwoventriclecardiaclesions.IntheCDHSGreport,survivalwas
poorerinthosewithsingleversustwoventricleanatomy(5versus47percent)[29],whereasintwoother
studies,therewasnodifferenceinsurvival[30,31].
PulmonaryhypertensionTheechocardiographicsignsofpulmonaryhypertensionincludepoor
contractilityoftherightventricle,enlargedrightheartchambers,pulmonicandtricuspidvalve
regurgitation,andpresenceofductalshunting.Leftventricularhypoplasiamayalsobeidentified.
Pulmonaryhypertensionwithrighttoleftshunting,leftventriculardysfunction,orsystemichypotension
areindicationsforpromptadministrationofinotropicagentsaswellasselectivepulmonaryvasodilators
(eg,iNO).(See"Persistentpulmonaryhypertensionofthenewborn",sectionon'Management'.)
ExtracorporealmembraneoxygenationThefirstCDHsurvivortreatedwithECMOwasreportedin
1977[32].Subsequently,severalsinglecentercaseserieshaveshownimprovedsurvivalrateswiththeuseof
ECMOininfantswithCDH[3336].However,theresultsofECMOareheavilydependentonselectioncriteria,
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whichcanvarybyinstitution.
AlthoughtherearenorandomizedcontrolledstudiesthathavedemonstratedtheefficacyofECMOininfants
withCDH,ECMO,ifavailable,isconsideredforalmostallinfantswhocannotbemanagedwithconventional
medicaltherapy,asthesepatientswouldnotsurvivewithoutECMO.ECMOisadvocatedasameansto
supportthepatientuntilthereactivecomponentofpulmonaryhypertensionresolves,whichmaytakeweeks.
DatafromtheExtracorporealLifeSupportOrganization(ELSO)Registryfrom1989throughJuly2004
demonstratedthatCDHwasthesecondmostcommondiagnosisforneonatalpatientsundergoingECMO
(totalof4491cases)[37].TheproportionofcasesofCDHhasalsoincreasedsincethestartoftheregistry
withapproximatelyonethirdofreportedneonatalpatientsin2003and2004diagnosedwithCDH[38].The
survivalrateofpatientswithCDHintheELSOregistryis53percent.
CriteriaTheprimaryindicationforECMOisfailureofconventionaltherapy[8,20,39].ECMOis
initiatedifanyoneofthefollowingcriteriaismet:

InabilitytomaintainpreductalPaO2saturations>85percentorpostductalPaO2>30mmHg
PIP>28cmH2Oormeanairwaypressure(MAP)>15cmH2O
Hypotensionthatisresistanttofluidandinotropicsupport
Inadequateoxygendeliverywithpersistentmetabolicacidosis

ExclusionarycriteriaforECMOvarybetweeninstitutions.MostECMOcentersexcludepatientswithlethal
chromosomalabnormalitiesorsevereintracranialhemorrhage.
Asaresult,traditionalinclusioncriteriaforECMO,includinginourcenter,alsoincludeallofthefollowing[40]:

Birthweight(BW)>2kg
Gestationalage(GA)>34weeks
AbsenceofintracranialhemorrhagegreaterthangradeI
Absenceofchromosomalanomalies

Therehavebeenreportsofpatientswithcardiacdisease,lowgradeintracranialhemorrhage,andprematurity
whohavebeenplacedonECMOandsurvived[28,33,41,42].Inourpractice,ECMOisofferedtoallpatientsif
theinfanthasinitiallyrespondedtotherapeuticinterventions,irrespectiveofpresenceofaheartdefect,and
meetstheaboveinclusioncriteria.
WithdrawalCriterionforwithdrawalofECMOsupportatourcenterincludesanyextensionof
intracranialhemorrhage.Infantsareatriskforintracranialbleedingduetotheneedforcontinuous
anticoagulationwithheparin.
Inourpractice,headultrasoundsusedtomonitorforintracranialbleedingareperformedbeforeinitiationof
ECMO,dailyforthefirstfivedaysandtheneveryotherdaywhileonECMOsupporttodetectandmonitorany
extensionofintracranialhemorrhage.Inaddition,headultrasoundsareperformedemergentlyforonsetof
seizures,changeinneurologicstatus,orfollowinganysignificantclinicalevent(eg,surgicalrepairofCDH,
andepisodesofhypotensionorhypertension).
Anotherconsiderationforwithdrawalisworseningclinicalstatusdespiteoptimaltherapy.Inourpractice,we
utilizeastepwiseapproachofinterventionstoimprovepulmonaryfunctionanddonotsetanarbitrarytimefor
pulmonaryhypertensionresolution[43].Weinitiallyoptimizethepatient'sfluidstatusandlungaerationwhile
he/sheisonECMO.Ifthereremainsevidenceofpulmonaryhypertensiondespitethesemeasureswhile
attemptingtowithdrawECMOsupport,wewillconsidersurgicalrepairwhilethepatientisonECMO.
Followingrepairandoptimizingfluidandcardiorespiratorysupport,wewillagaintrytoweanfromECMO
support.Ifthisisnotfeasible,dexamethasone(decreaselunginflammation),prostaglandintherapy(maintain
anopenductusarteriosus),andprostacyclin(pulmonaryvasodilator)therapyareprovidedasadditionalmedical
measures.
EfficacyItisdifficulttoaccuratelyassessthebenefitofECMOgiventheabsenceofclinicaltrials.
However,efficacyisdemonstratedbycomparingobservationaldatashowing50percentsurvivalforthese
patientswhohavefailedconventionaltherapy,withhistoricaldatasuggestingamuchlowersurvivalrate
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withoutECMOintervention[44].
OncetheinfantisweanedfromECMO,thereremainsariskofrecurrenthypoxiasecondarytopulmonary
hypertensionandrighttoleftshunting[45].InareportofpatientstreatedwithasecondcourseofECMO,16of
34(47percent)CDHpatientssurvived[46].
Surgery
PrimaryversuspatchrepairSurgicalrepairconsistsofreductionoftheabdominalvisceraandprimary
closureofthediaphragmaticdefect(picture1).Thediaphragmaticdefectmayberepairedwithsuturesalone
(primaryrepair).However,aGoreTexpatchrepairisoftenrequiredinpatientswithlargeCDHsinwhom
increasedtensionusingaprimaryrepaircompromisestotalthoraciccompliance[47].Reportedpatchrelated
complicationsincludeanincreasedriskofinfection,chestwalldeformitiesbytetheringoftheribs,anda
potentialincreaseinCDHrecurrence[48].However,inourcenter,theriskofrecurrenceofCDHwassimilarin
patientswhounderwentpatchrepaircomparedwiththosewithprimaryrepair[47].(See'Patchrelated'below.)
Iftheabdominalwallisdifficulttoclosefollowingreductionofthehernia,theuseofatemporaryabdominal
wallsiloorpatchmaybehelpful[49].InaretrospectivereviewofCDHrepairsatasingleinstitution,delayed
abdominalwallclosurewasrequiredin9percentofoverallcases,2percentofcasesoffECMO,and40
percentofrepairsonECMO[50].Delayedabdominalwallclosurewasassociatedwithanincreasedneedfor
bloodtransfusionsbutnosignificantdifferenceinmortality[50].Alesscommonlyusedtechniqueisthesplit
abdominalwallmuscleflapusedtorepairlargediaphragmaticherniaswhenprimaryclosureisnotfeasible[51].
Themuscleflapisagoodalternativewhenthepatienthasalreadyhadinfectiousissuestoavoidusingan
implantofGoreTex.
TimingWithabetterunderstandingofthepathophysiologyandvariationinthedegreeofpulmonary
impairment,thetimingofsurgeryhasshiftedfromearlysurgicalinterventiontodelayingsurgicalcorrectionuntil
thepatienthasbeenstabilizedmedically[15].Inparticular,surgeryisusuallydelayedinneonateswithmore
severeformsofpulmonaryhypoplasiaandpulmonaryhypertensionwhorequireadditionalmedicalcare,which
mayincludeECMO.Inourcenter,weusethisapproachinthetimingofsurgeryasdiscussedinthenext
section.
ReportedsurvivalratesinnewbornswithCDHusingthismanagementapproachofpreoperativestabilization
andselectiveuseofECMOfollowedbydelayedsurgicalcorrectionrangefrom79to92percent[8,1012,15].In
areviewofdatafromtheCDHSG,ananalysisadjustedforseverityofillnessfoundnodifferencesinmortality
basedontimingofthesurgerystratifiedasdayoflife0to3,dayoflife4to7,andafterdayoflife8[52].
OurapproachOurmanagementapproachisbasedontheseverityofpulmonaryimpairmentanddictates
thetimingofsurgery.Initialmedicaltreatmentasdiscussedabovefocusesonstabilizingtheneonate,
especiallythosewithpulmonaryhypertensionandhypoplasia.
Oncethediagnosisismadeorsuspected,allpatientsareintubatedandanasogastrictubeconnectedto
continuoussuctionisplacedinthestomachtoreducelungcompression.Inaddition,echocardiographyis
performedtodetectthepresenceofpulmonaryhypertensionand/orcardiacabnormality.(See'Initialtreatment'
above.)
Managementincludingtimingofsurgeryisdependentontherespiratorystatusofthepatientasfollows:
Inpatientswithonlymildsymptomsonminimalsupport,inwhomthereisnoevidenceofpulmonary
hypertensionorpulmonaryhypoplasia,repairistypicallyundertakenat48to72hoursofage.
Inpatientswithnoormildpulmonaryhypoplasiaandreversiblepulmonaryhypertension,thetimingof
repairisdelayeduntilpulmonaryhypertensionisresolvedandpulmonarycomplianceimproves[53].The
timecourseisvariableandisdependentontheresponsetomedicalmanagement(stabilizationofblood
pressure,oxygenation,andcorrectionofacidosis).Themostcommongroupofpatientswilldemonstrate
initiallability,butthenstabilize,allowingrepairafter5to10days.(See'Initialtreatment'aboveand
'Ventilation'above.)
Inasmallgroupofpatientswithseverepulmonaryhypoplasiaand/orpulmonaryhypertension,therewill
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benoresponsetotherapyofanykind,includingtoECMO.Inthisgroup,supportisoftenwithdrawn.
InpatientswhorequireECMOtherapybecauseoffailuretorespondtomedicalmanagement(see
'Criteria'above),thereissomecontroversyabouttimingoftheoperativerepair.Inourcenter,surgical
repairisperformeddependingupontheindividualclinicalsettingasfollows:
ForpatientswithseverepulmonaryhypertensionwhocontinuetorequireECMOsupport,one
approachistorepairthedefectwhiletheinfantisonECMO[54].Earlierstudiesreportedahighrate
ofhemorrhagiccomplicationsandhighmortalityoncebleedingdeveloped[55,56].Thisproblemhas
beenpartiallyresolvedbyadministeringperioperativeaminocaproicacid[54,57],puttingfibringlue
onthesutureline[58],andavoidingextensivedissectionofthediaphragmaticleaves.(See
'Withdrawal'above.)
RepairisdelayeduntilpatientsarereadytobeweanedoffofECMOafterresolutionofpulmonary
hypertension[59].InaretrospectivereviewofpatientswithCDHandECMO,survivalwas
improvedwithlowerratesofsurgicalbleedingandtotaldurationofECMOtherapyifpatientscould
besuccessfullyweanedfromECMOpriortorepair[60].
Ifthecircuitclotssecondarytotheuseofaminocaproicacidorthereisexcessivebleeding,the
infantmaybedecannulatedwithoutsignificantclinicalcompromise.
Finally,somepatientsmayreturntoconventionalventilationanddecannulationbeforerepairofCDH
[40].ThisstrategyisindicatedforneonateswhocanbeweanedfromECMOandwhenthereare
coagulation,infectious,ormechanicalcomplicationsfromECMO.
COMPLICATIONS
AcutecomplicationsThemostseriouscomplicationpostrepairofCDHispersistentpulmonary
hypertensionofthenewborn(PPHN)[6163].Somepatientsmayrequireextracorporealmembrane
oxygenation(ECMO)[6163].Othercomplicationsearlyinthepostoperativecourseincludehemorrhage,
chylothorax,andpatchinfection.
LatecomplicationsLatecomplicationsincludechronicrespiratorydisease,recurrenthernia/patch
problems,spinal/chestwallabnormalities,gastrointestinaldifficulties,andneurologicalsequelae[64].
PulmonarySurvivors,especiallythosetreatedwithECMO,areatriskforrespiratoryinfectionsand
chroniclungdisease[6568].Inafollowupstudyfromourcenterof98patients,pulmonaryfunctiontestswere
abnormalthroughoutthefirstthreeyearsoflife[68].Theseverityofimpairmentincreasedwithincreasing
degreesofinitialpulmonaryhypoplasiaanddurationofmechanicalventilation.However,pulmonaryfunction
andstatusimprovesinmostpatientswithlunggrowth[69].Outcomestudieshavereportedthatadolescent
survivorshavemildtomoderateairwayobstructiondetectedbypulmonaryfunctionstudiesandamild
decreaseinexercisecapacity[70,71].Inmostcases,thereislittletonoimpactondailylifeactivities.
RecurrentherniaRecurrentdiaphragmaticherniaoccursin2to22percentofallCDHsurvivors[72].
Recurrentdiaphragmaticherniationisusuallydiagnosedbychestorcontraststudiespromptedbyrespiratoryor
gastrointestinalsymptoms.Earliercaseseriesreportedrecurrencewashighest(27to57percent)inpatients
requiringpatchrepairsandECMOsupport,astheygenerallyhadlargerdefects[66,7376].However,
subsequentreportshavenotedasubstantiallylowerrateofrecurrentherniationinpatientscorrectedwitha
patchrepair.Thesiteofrecurrencewithapatchistypicallymedial.
PatchrelatedPatchesmaybecomechronicallyinfected,requiringremovalofthepatchand
diaphragmaticreconstruction,preferablywithnativetissue[73].Althoughrepairusingapatchwasgenerally
associatedwithahighrateofrecurrenceofhernia(upto40percent)inearliercaseseries,subsequentlylower
recurrenceratesof4to5percenthavebeenreportedusingGoreTexpatches[77,78].
Theneedforpatchrepairhasbeenassociatedwithahigherrateofchestwalldeformitiessuchaspectus
excavatum,pectuscarinatum,andthoracicscoliosis.Chestwalldeformitieshavebeenreportedinupto50
percentofpatientswhowereinitiallyrepairedwithapatch[48,70,76,79].Itremainsuncertainwhetherthe
deformityisdirectlyrelatedtotheuseofapatch,oraconsequenceoftheseverityoftheCDHandsubsequent
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incongruentlunggrowth.
GastrointestinalGastroesophagealrefluxdisease(GERD)andforegutdysmotilityareprominentchronic
abnormalitiesinCDHpatients.ThereportedincidenceofGERDinpatientswithCDHrangesfrom40to50
percent[8082].Inseveralcaseseries,antirefluxsurgeryisperformedin15to20percentofallpatientswith
CDH[8083].
Thereareseveralanatomicfactorsthatmaycontributetothedevelopmentofreflux[8486]:
Disturbanceofnormalesophagealandgastroesophagealjunctionduetomediastinalshiftand
compression
Shortenedintraabdominalesophagus
ObtuseangleofHis(angleatwhichtheesophagusintersectsthestomachatthecardioesophageal
juncture)
Deformationofthediaphragmaticcrusbyclosure
Pressurechangesrelatedtoincreasedworkofbreathing
Potentialneurologicdefects
Intestinalobstructionsecondarytoadhesionsoccursin10percentofpatientswithCDH[79,87,88].All
patientswithCDHhavemalrotationormalfixationoftheintestines,andthus,apredispositiontodevelopment
ofvolvulus.Ratesofthispotentiallydevastatingcomplicationvaryfrom3to9percent[79,89].
FailuretothriveSubsequentfailuretothrivehasbeenreportedin30to86percentofinfantswithCDH.
Riskfactorsincludeprematurity,prolongedventilation,andoxygenrequirementatdischarge[67,9093].
Increasedworkofbreathingmayalsocontributetofailuretothrivebymakingoralfeedingandswallowing
challenging.Affectedpatientsmayrequiresupplementalfeedingvianasogastricorgastrostomytubesfor
adequatecaloricintake.
Manypatientswillrequiregastrostomytubefeedingsbecauseoforalaversiontofeedingduetorefluxorneed
forsupplementation.Theincidenceofgastrostomytubefeedingsmaybeincreasingwiththeincreasein
survivalrate,especiallyinpatientswithsevereCDH[23,93].Inalargecaseseries,15percentofpatients
continuedgastrostomytubefeedingsthroughchildhood(agesevenyears)becauseofpoorgrowthandthe
needfornutritionalsupplementation[94].
NeurodevelopmentimpairmentAbnormalitiesdetectedbycranialimagingincludeintraventricular
hemorrhage(IVH),infarction,periventricularleukomalacia(PVL),andextraaxialfluidcollections.Magnetic
resonanceimaging(MRI)ofsurvivorsofsevereCDHdemonstratesdelayedmaturationandstructuralbrain
abnormalitiesincludingPVLandvaryingdegreesofintracranialhemorrhage[95].
Theseabnormalitieslikelyleadtolongtermneurologiccomplications.Neurodevelopmentalimpairmenthas
beenreportedin30to80percentofpatients,andhasincludedbothmotorandcognitivefunction[96104].
Neurocognitiveimpairmentanddelayhasbeenreportedtopersistintoschoolage[97,98,100,105].In
particular,hearinglossiscommon,withreportedprevalenceof30to50percent[67,106,107].
Onelongitudinalstudyof47CDHsurvivorsoverthefirstthreeyearsoflifedemonstratedthatmostchildren
whohadearlydelaysshowedimprovementintheirneurodevelopmentaloutcome,butchildrenwithdelaysinall
domainsweretheleastlikelytoshowimprovement[104].
MusculoskeletaldeformitiesChestdeformitiesincludingpectusexcavatum,pectuscarinatum,and
scoliosisarecommon,particularlyinpatientswithrepairedlargeCDH[70,108].
SURVIVALThepostnatalsurvivalrateattertiarycentershasimproved,withreportedratesof70to92
percent[8,1012,109,110].Thisincreasedsurvivalrateappearstobearesultoftheshiftfromearlysurgical
interventiontointensivepreoperativesupportivecareaimedatavoidinglunginjury,followedbysurgical
correction.However,thesedatarepresentthesurvivalrateofcasesofCDHthatwerefullterminfantsbornor
transferredtotertiarycarecenterswithavailableskilledpersonnelandaccesstoadvancedtechnology(eg,
extracorporealmembraneoxygenation[ECMO]).ThesesurvivalratesdonotaccountforthecasesofCDH
thatarestillbornordiedoutsideatertiarycenter,orfetallossduetospontaneousortherapeuticabortion[110
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114].
Factorsassociatedwithdecreasedsurvivalinclude:
PrematurityAreviewfromtheCongenitalDiaphragmaticHerniaStudyGroup(CDHSG,formerlycalled
theCDHRegistry)reportedlowersurvivalforpreterminfantscomparedwithterminfants(54versus73
percent)[115].Preterminfantswerelesslikelytoundergosurgicalrepair(69versus86percent)andbe
treatedwithECMO(26versus33percent).Survivalwashigherinpreterminfantswhounderwentsurgical
repair(77percent),butthiswasstilllowerthaninterminfantswithsurgicalrepair(85percent).Preterm
infantsweremorelikelytohavechromosomalabnormalities(8versus4percent)andmajorcardiac
defects(12versus6percent).
CardiacabnormalitiesDatafromtheCDHSGshowedthatpatientswithmajorcomplexcardiacdefects
(eg,singleventriclephysiology,leftheartobstructivelesions,andtranspositionofthegreatarteries)have
asignificantlylowersurvivalrate(36percent)comparedwiththosewitheitherminorheartdefects(67
percent)ornoheartdefect(73percent)[116].
Persistentandseverepulmonaryhypertension[6163,117]
NeedfortransportNeonataltransportofinfantswithCDHisassociatedwithpoorersurvivalcompared
withinfantswhoareinbornatatertiarycenterwithexpertiseinthemanagementofCDH[118,119].
LowpreductaloxygenandhighcarbondioxidesaturationSurvivalispoorerininfantswhosehighest
recordedpreductaloxygensaturationisbelow85percentinthefirst24hoursoflifecomparedwiththose
withhigherlevels[120,121].Inaddition,elevatedarterialbloodgasPaCO2(partialpressureofcarbon
dioxide)greaterthan70mmHgisassociatedwithdecreasedsurvival[121,122].
DefectsizeInfantswithverylargedefectshaveapooreroutcome[123126].InanotherCDHSGreport
thatincluded3062liveborninfantsbetween1995and2004,logisticregressionanalysisofinfantswho
underwentrepair(n=2524)demonstratedthatdefectsizealonepredictedsurvival[123].
RightversusleftsidedlesionItisunclearwhetherthesideofthelesionaffectssurvival.Inonecase
seriesof267patients,diseaseseverityappearedtobegreaterinpatientswithrightsidedlesions,
resultinginalowersurvivalrate(50versus75percent)[127].Agreaterproportionofinfantswithright
sidedlesionscomparedwiththosewithleftsidedlesionsrequiredECMO(40versus15percent)anda
diaphragmaticpatch(76versus41percent).However,intwootherlargecaseseriesof220patients,
therewasnodifferenceinsurvivalbetweenpatientswithrightversusleftsidedlesions,although
patientswithrightsidedlesionsweremorelikelytoundergoapatchrepairandhavearecurrenthernia
[117,128].
BecauseofthecontinuedpostnatalmortalityofCDH,researchattemptsareongoingtodevelopinutero
therapytopreventorreversepulmonaryhypoplasiainfetuseswithCDH,therebyrestoringadequatelung
growthforneonatalsurvival.(See"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",
sectionon'Inuterotherapy'.)
POSTDISCHARGEMANAGEMENTBecauseoftheassociatedsignificantmorbidities(ie,pulmonary
complications,neurodevelopmentaldelay,gastroesophagealreflux,hearingloss,andpoorgrowth),careof
survivorswithCDHfollowingdischargefromthehospitalischallenging.Structuredfollowup,ofteninvolvinga
multidisciplinaryteam,facilitatesrecognitionandtreatmentofthesecomplications.
In2008,theAmericanAcademyofPediatrics(AAP)sectiononSurgeryandtheCommitteeonFetusand
Newbornpublishedacomprehensiveplanforthedetectionandmanagementoftheassociatedmorbiditiesfor
clinicianswhoprovidecareforthesepatients(table1)[129].Thisplanprovidesarecommendedschedulethat
includesthefollowing:
Measurementofgrowthparametersateachvisit
Chestradiographyifapatchwasusedinrepairofthedefectoriftherearerespiratoryorgastrointestinal
symptoms
Pulmonaryfunctiontestingbaseduponclinicalstatus
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Respiratorysyncytialvirus(RSV)prophylaxis
Echocardiographyifpreviouslyabnormalorsupplementaloxygenused
Brainimagingifpreviousabnormalheadultrasound,abnormalneurologicstatus,patchrepair,or
extracorporealmembraneoxygenation(ECMO)used
Hearingevaluation
Developmentalscreening
Oralfeedingassessment
Uppergastrointestinalstudybaseduponclinicalstatus
Scoliosisandchestwalldeformityscreening
LATECDHPRESENTATIONInfrequently,CDHwillpresentaftertheneonatalperiod.Inonecaseseriesof
15children,patientspresentedwithrespiratorysymptoms(n=6),gastrointestinalsymptoms(n=6),orboth(n
=3)atameanageof1.5years(range3.8daysto9.9years)[130].Fivepatientshadfailuretothrive.The
diagnosiswasmadebychestradiographyinsixpatients,andtheotherpatientswerediagnosedby
gastrointestinalcontrastseriesorcomputedtomography.Primaryrepairwithoutapatchwassuccessfulinall
patientswitha100percentsurvivalrateatameanfollowupoftwoyears.
INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"
and"BeyondtheBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6th
gradereadinglevel,andtheyanswerthefourorfivekeyquestionsapatientmighthaveaboutagiven
condition.Thesearticlesarebestforpatientswhowantageneraloverviewandwhoprefershort,easytoread
materials.BeyondtheBasicspatienteducationpiecesarelonger,moresophisticated,andmoredetailed.
Thesearticlesarewrittenatthe10thto12thgradereadinglevelandarebestforpatientswhowantindepth
informationandarecomfortablewithsomemedicaljargon.
Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthese
topicstoyourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingon
"patientinfo"andthekeyword(s)ofinterest.)
Basicstopics(see"Patientinformation:Congenitalherniaofthediaphragm(TheBasics)")
SUMMARYANDRECOMMENDATIONS
Congenitaldiaphragmatichernia(CDH)isadevelopmentaldefectinthediaphragmthatallowsabdominal
visceratoherniateintothechest,therebyinterferingwithnormallungdevelopment.Compressionofthe
developinglungbytheherniatedabdominalcontentsdecreasesbronchialandpulmonaryarterial
branching,resultinginlunghypoplasiaandpulmonaryarterialmusclehyperplasia(pulmonary
hypertension).(See'Effectonpulmonarydevelopment'above.)
PatientswithCDHmostoftendeveloprespiratorydistressinthefirstfewhoursordaysoflife.The
spectrumofpresentationcanvaryfromacute,severerespiratorydistressatbirth,whichiscommon,to
minimaltonosymptoms,whichisobservedinamuchsmallergroupofpatients.Physicalexamination
willrevealabarrelshapedchest,ascaphoidappearingabdomenbecauseoflossoftheabdominal
contentsintothechest,andtheabsenceofbreathsoundsontheipsilateralside.(See'Clinical
manifestations'above.)
Diagnosiscanbemadeprenatallywithultrasoundexamination.AmonginfantsinwhomCDHisnot
diagnosedinutero,thediagnosisismadebychestradiographyshowingherniationofabdominalcontents.
(See"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",sectionon'Prenatal
diagnosis'and'Diagnosis'above.)
Inourcenter,managementofCDHconsistsofpreoperativemanagementwithstabilizationofpulmonary
andcardiovascularfunction,anddelayingsurgeryuntilthereisresolutionofearlypulmonaryinsufficiency
andacutepulmonaryhypertension.(See'Postnatalmanagement'above.)
IntheinitialmanagementofallinfantswithCDH,werecommendthefollowingmeasures(Grade1B)
(see'Initialtreatment'above):
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Immediateendotrachealintubationtopreventfurtherdilationofabdominalcontents.Blowbyoxygen
and/orbagmaskingshouldbeavoidedastheyleadtogastric/abdominaldistensionand
compressionofthelung.
Placementofanasogastrictubeconnectedtocontinuoussuctionfordecompressionofthestomach
andintestine.
Administrationoffluidsandinotropicagentstomaintainmeanarterialbloodpressure(BP)50
mmHg.
Administrationofsurfactanttherapytoonlypreterminfants(gestationalage[GA]34weeks)who
alsohavefindingssuggestiveofrespiratorydistresssyndrome.
Wesuggestventilatorysupportwithminimalairwaypressuretomaintainpreductaloxygensaturations
above85percent(Grade2C).Conventionalmechanicalventilation(CMV)isfirstusedandhigh
frequencyventilation(HFV)isreservedforpatientswhofailCMV.(See'Ventilation'above.)
Werecommendextracorporealmembraneoxygenation(ECMO)forinfantswhofailtorespondto
supportivemedicalmanagement(Grade1A).(See'Extracorporealmembraneoxygenation'above.)
Werecommendthatthetimingofsurgicalrepairbebasedonthepatient'spulmonarystatus,whichis
dependentontheseverityofpulmonaryhypoplasiaandpulmonaryhypertension,andhis/herresponseto
preoperativemedicalcare(Grade1B).(See'Ourapproach'above.)
ThemortalityandmorbidityofCDHarerelatedtotheseverityoflunghypoplasiaandpulmonary
hypertension.Otherfactorsthatincreasemortalityincludethepresenceofassociatedanomalies(eg,
cardiacdefectsandchromosomalabnormalities)andprematurity.(See'Survival'aboveand'Late
complications'aboveand"Congenitaldiaphragmatichernia:Prenataldiagnosisandmanagement",
sectionon'Prognosticfactors'and"Congenitaldiaphragmatichernia:Prenataldiagnosisand
management",sectionon'Associatedanomalies'.)
LongtermcomplicationsinsurvivorsofCDHincludechronicrespiratorydisease,gastroesophageal
reflux,failuretothrive,recurrence,neurodevelopmentaldelay,andmusculoskeletaldeformities.(See
'Latecomplications'above.)
StructuredfollowupfacilitatesrecognitionandtreatmentofthemorbiditiesassociatedwithCDHas
outlinedbytheAmericanAcademyofPediatrics(AAP)'srecommendedscheduleforfollowupofthese
patients(table1).(See'Postdischargemanagement'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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