Bochdalekhernia

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ABochdalekherniaisoneof
twoformsofacongenital
diaphragmatichernia,theother
formbeingMorgagnihernia.A
Bochdalekherniaisacongenital
abnormalityinwhichanopening
existsintheinfantsdiaphragm,
allowingnormallyintra
abdominalorgans(particularlythe
stomachandintestines)to
protrudeintothethoraciccavity.
Inthemajorityofpatients,the
affectedlungwillbedeformed,[1]
andtheresultinglung
compressioncanbelife
threatening.Bochdalekhernias
occurmorecommonlyonthe
posteriorleftside(85%,versus
rightside15%).

Contents
1Causes
2Epidemiology
3Morbidityandmortality
4Symptoms
5Diagnosis
6Treatment
7Mnemonic
8References

Causes

Bochdalekhernia

"Bochdalek",atlumbocostaltriangle,labeledinbottomleft(Captionsin
German.)
Classificationandexternalresources
ICD10

Q79.0
(http://apps.who.int/classifications/icd10/browse/2015/en#/Q79.0)

ICD9

756.6(http://www.icd9data.com/getICD9Code.ashx?icd9=756.6)

DiseasesDB 31492(http://www.diseasesdatabase.com/ddb31492.htm)

Mostlikely,Bochdalekherniasareformedthroughoutthegrowthprocessandorganconstructionduring
fetaldevelopment.Duringfetaldevelopment,thediaphragmisformedbetweentheseventhandtenthweek.
Also,duringthistime,theesophagus,stomach,andintestinesareformed.Therefore,aBochdalekhernia
formseitherfrommalformationofthediaphragm,ortheintestinesbecomelockedintothechestcavity
duringtheconstructionofthediaphragm.AlthoughthesearesomefactorsthatcontributetoaBochdalek
hernia,itdoesnottakeallvariablesintoaccount.Bochdalekhernias,alongwithMorgagnihernias,areboth
multifactorconditions,meaningthattherearemanyreasonsandmultiplevariablesthatcontributetothe
malformations.Forexample,ineachcasetherecouldbegeneticandorenvironmentalcondition(s)thatcan
addtotheprobabilityofthisbirthdefect.[2]

Epidemiology
Bochdalekherniasmakeupabout0.17%to6%ofalldiaphragmaticherniacasesandaboutoneinevery
2200to12,500birthseveryyear.[3]BabieswhoarebornwithaBochdalekherniaaremorethanlikelyto
haveanotherbirthdefectcausedbythehernia.Abouttwentypercentofthosechildrenbornwitha
Bochdalekhernia,alsohaveacongenitalheartdefect.Inaddition,infantsbornwiththisconditionmayalso
haveotherabnormalities.Betweenfiveandsixteen[percentofinfants]haveachromosomal
abnormality.[2]Inmostcases,leftsidedherniasorBochdalekherniashavearatioof3:2ofmalesto
females.Inotherwords,Bochdalekherniasaremorecommoninmen.

Morbidityandmortality
Bochdalekherniacanbealifethreateningcondition.Approximately85.3%ofnewbornsbornwitha
Bochdalekherniaareimmediatelyhighrisk.[4]InfantsbornwithaBochdalekherniahaveahighmortality
rateduetorespiratoryinsufficiency.[5]Between2560%ofinfantsdiefromaBochdalekhernia.[6]The
lungs,diaphragm,anddigestivesystemareallformingatthesametime,sowhenaBochdalekhernia
permitstheabdominalorganstoinvadethechestcavityratherthanremainunderthediaphragminthe
correctposition,itputstheinfantincriticalcondition.These"foreignbodiesinthechestcavitycompress
thelungs,impairingtheirproperdevelopmentandcausingpulmonaryhypoplasia.[2]Sincethelungsof
infantssufferingfromaBochdalekherniahavefeweralveolithannormallungs,Bochdalekherniasarelife
threateningconditionsduetorespiratorydistress.[2]Also,iftheinvasionoftheintestineorstomach
puncturesthelung,thenthelungscannotfillcompletelywithair.Thebabywillnotbehealthyorstable
withthisconditionbecauseheorshecannottakeinenoughairandoxygentokeepthebodyoperating
properly.Likethelungs,theintestinesmayalsohavetroubledevelopingcorrectly.Iftheintestinesare
trappedwithinthelungs,thenthelungsandintestinesmaynotbereceivingtheamountofbloodtheyneed
tostayhealthyandfunctionproperly.

Symptoms
InnormalBochdalekherniacases,thesymptomsareoftenobservablesimultaneouslywiththebabysbirth.
AfewofthesymptomsofaBochdalekHerniaincludedifficultybreathing,fastrespirationandincreased
heartrate.Also,ifthebabyappearstohavecyanosis(bluetintedskin)thiscanalsobeasign.[3]Another

waytodifferentiateahealthybabyfromababywithBochdalekHernia,istolookatthechestimmediately
afterbirth.IfthebabyhasaBochdalekHerniaitmayappearthatonesideofthechestcavityislargerthan
theotherandortheabdomenseemstobeconcave(cavedin).[2]

Diagnosis
OnewaytodetermineifababydoesinfacthaveaBochdalekhernia,wouldbetohaveapediatrician
performaphysicalontheinfant.Achestxraycanalsobedonetoexaminetheabnormalitiesofnotonly
thelungsbutalsothediaphragmandtheintestine.Inadditiontothese,adoctorcanalsotakeabloodtest,
drawingarterialbloodtocheckanddeterminehowwellthebabyisbreathingandhisorherabilityto
breathe.Achromosomaltest(donebytestingtheblood)canalsobeperformedtodeterminewhetherornot
theproblemwasgenetic.Thedoctorscanalsotakeanultrasoundoftheheart(echocardiogram)toevaluate
thehealthoftheheart.

Treatment
InordertotreataBochdalekhernia,thebabysphysicianmusttake
intoaccountmultiplefactors.First,thediagnosiswillvary
dependingonwhethertheBochdalekherniawasfoundduringfetal
developmentorafterbirth.Thekeytosurvivalliesinprompt
diagnosisandtreatment.[6]Second,thebabysoverallhealthand
medicalhistorywillbeevaluated.Third,thedoctorwilllookatthe
seriousnessofthecondition.Fourth,thebabywillneedtobe
evaluatedatthelevelofmedication,procedureandtherapyheor
shecanhandle,andfinally,thedoctorwilltakeintoconsideration
theopinionandpreferenceoftheparents.Afterthesethingsareall
Bochdalekherniaduringsurgery
takenintoconsiderationandevaluated,thedoctorwilldetermine
howtotreatthebaby.Therearethreedifferenttreatmentsavailable.
ThefirsttreatmentincludesthebabysadmissionintotheNICU(NeonatalIntensiveCareUnit).[2]Inmost
BochdalekHerniacases,babieswhoareadmittedintheNICU,areplacedonamechanicalventilatorto
helpbreathing.Anothertreatmentinvolvesputtingtheinfantsonatemporaryheart/lungbypassmachine,
calledanECMO.[2]Thisnormallypertainstochildrenwhohavesevereproblems.ECMOperformsthe
taskstheregularlyfunctioningheartsandlungsdo.ECMOallowsoxygentoberegulatedintotheblood
andthenpumpsthebloodthroughouttheentirebody.Normally,thismachineisusedtostabilizethebabys
condition.Thethirdoptionintreatmentissurgery.[7]
Afterthebabyisstableandhisorherstatehasimproved,thediaphragmcanbefixedandthemisplaced
organscanberelocatedtotheircorrectposition.[2]AlthoughthesearevarioustreatmentsforBochdalek
Hernias,itdoesnotguaranteethebabywillsurvive.[2]Sincethebabymustgothroughsomeorallofthe
previoustreatments,thebabyshospitalstayisusuallylongerthanthatofanormalnewborn.Theaverage
infantsbornwithaBochdalekHerniastayinthehospitalbetween23.1and26.8days.[6]

Mnemonic

Ausefulwaytorememberthelocalizationofthisherniavs.Morgagniis"Bochdalekisbackandtotheleft"
(retheposterolaterallocalization).

References
1. ^DiaphragmaticHernia.2007.Children'sHospitalofWisconsin,Wisconsin.3Feb.2007
<http://www.chw.org/display/PPF/DocID/22791/router.asp>
2. ^abcdefghi"DiaphragmaticHernia."HealthSystems.24Nov.2006.UVAHealth.3Feb.2007.
<http://www.healthsystem.virginia.edu/UVAHealth/peds_digest/diaphrag.cfm>.
3. ^abJeffrey,MarkE.,andWilburA.Gorodetsky."AdultBochdalekHernia."Medind.10,Sept.2004,accessed
3Feb.2007<http://medind.nic.in/maa/t05/i3/maat05i3p284.pdf>.
4. ^Klein,JaquierM."HospitalStays,HospitalCharges,andinHospitalDeathsAmongInfantswithSelected
BirthDefectsUnitedStates,2003."CDC.19Jan.2007.3Feb.2007
<http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5602a1.htm>.
5. ^"Hernia."Encarta.2006.MicrosoftCorporation.8Feb.2007
<http://encarta.msn.com/encyclopedia_761574157/Hernia.html>.Archived
(http://www.webcitation.org/5kwQByUDr)20091031.
6. ^abcHekmatnia,Ali,andKieranMcHugh."CongenitalDiaphragmaticHernia."EMedicine(2003).8Feb.2007
<http://www.emedicine.com/RADIO/topic187.htm>.
7. ^Larrazbal,Natasha.DiaphragmaticHernia,Left.2003.CaracasVenezuela.Diaphragmatichernia.6Feb.2007
<http://www.thefetus.net/page.php?id=1218>.

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Categories: Diaphragmatichernias Congenitaldisordersofmusculoskeletalsystem
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