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PediatricOmphaloceleandGastroschisisFollowup

PediatricOmphaloceleandGastroschisisFollowup
Author:JamesGGlasser,MD,MA,FACSChiefEditor:TedRosenkrantz,MDmore...
Updated:Apr28,2015

FurtherInpatientCare
Omphalocele
Anintactomphalocelesacprotectstheintestinefromcontactwiththeamnioticfluidhence,thesebabiescanbe
fedmuchearlierfollowingclosureoftheabdominalwalldefect.Babieswithgiantomphaloceleshaveprolonged
hospitalizations,becauseofthedifficultyinobtainingclosureandrespiratoryfailure,whichrequiresprolonged
mechanicalventilationandatracheotomy.

Gastroschisis
Primaryclosureoftheabdominalwalldefectispossibleonlyifinflammationoftheintestineisminimalevenso,it
isusuallyseveralweeksbeforethebabytoleratesenteralfeedings.
Ifclosureoftheabdominalwalldefectnecessitatesuseofasilotocontaintheevisceratedintestine,thesiloshould
beremovedwithinaweekbecauseoftheriskofwoundinfection(seetheimagebelow).

Siloclosureofababywithgastroschisis.

Parenteralnutritionisuseduntilthebabypasses"starvationstools."Ifthishasnotoccurredwithin34weeks,a
mechanicalobstruction,ratherthananileus,shouldbesuspectedUpperGI/smallbowelradiographyshouldbe
performedtoassessthetransitofcontrastthroughtheintestinaltract.Ifthisstudydemonstratesanintestinal
obstruction,laparotomyisindicated.

FurtherOutpatientCare
Followingdischarge,theseinfantsrequireclosefollowuptoassesstheirgrowth,weightgain,andchronicletheir
developmentalmilestones.
Theyfrequentlyhavesymptomsofgastroesophagealreflux,whicharenotoriouslyvariableandoccasionallylife
threatening.Practitionersmustmaintainahighindexofsuspicionforthisdiagnosis.Treatmentfollowsthesame
protocolasforanyotherinfantwiththisproblem.
Hirschsprungdiseasemayalsooccur.Physiciansshouldconsiderthisdiagnosisinachildwithrefractory
constipationandfailuretothrive.

Transfer
Placingawarm,moistlaparotomypadovertheexposedintestineintuitivelymakessense.Theproblemisthata
warm,moistlappadrapidlybecomesacold,wetlappadconsequently,theauthorpreferscoveringtheintestine
withadrylappadandwrappingthebaby'storsoinsuchawayastoavoidpullingorkinkingthebowelmesentery.
Ifintravenous(IV)accesscanbeobtained,IVfluidsandantibioticsshouldbeadministered,andthestomachshould
bedecompressedwithanasogastrictube.

Complications
Nutritionaldepletionisinevitableinababywithanomphaloceletreatedconservatively,becauseofthelargeopen
wound.Positivenitrogenbalanceisrestoredfollowingskinclosure.
Prolongedparenteralnutritioncancausehepatotoxicity,manifestedbycholestasisandhepatomegaly,whichmay
complicatestagedclosureofagiantomphalocele.Omega3fattyacids(Omegaven)reportedlymayreverse
"intestinalfailureassociatedliverdisease."
Infantswithgiantomphaloceleshavepulmonaryinsufficiency(hypoplasiaconsequentuponthesmallthoraciccavity)
andmayrequireatracheotomyandprolongedventilatorsupport.Stagedabdominalwallclosuregraduallyincreases
theintraabdominalpressurethiselevatesthediaphragmandmaymakeventilationmoredifficult.
Infantswithgiantomphaloceleshaveanincreasedriskofsepsisbecausetheyaredepletednutritionallybytheopen
woundandbecauseoftheirprolongedneedofventilatorsupportandcentralvascularaccess.

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Prognosis
Omphalocele
Infantswithomphalocelesarecomplex,withmultisystemorganinvolvement.Theirprognosisdependsonthe
severityoftheassociatedproblems.
Giantomphalocelescanbeclosedhowever,multiplesurgicalproceduresareusuallynecessary.Inaddition,these
infantsmayhaveadditionalmedicalproblemsthatmakecaringforthemquitechallenging.
Thecriticalfactoraffectingthesurvivalofababywithagiantomphaloceleisthesizeofthethoraciccavitywith
associatedpulmonaryhypoplasiaandchronicrespiratoryfailure.Evenifthebabyhasasmallthorax,thepotential
forlunggrowthanddevelopmentencouragesoptimismregardingtheultimateprognosis.

Gastroschisis
Thepatient'sprognosisdependsontheseverityoftheassociatedproblemssuchasprematurityandintestinal
inflammatorydysfunction,intestinalatresia,andshortgut.Apopulationbasedcohortstudy(from28pediatric
surgicalcentersintheUnitedKingdomandIreland)analyzedthe1yearoutcomesofinfantswithgastroschisis.
Babieswithcomplexgastroschisisrequiredlongerhospitalstaysandhadmorecomplicationsthanbabieswith
simplegastroschisis.Classifyinginfantswithgastroschisisinto"simple"versus"complex"(macroscopicintestinal
abnormalities)maybeareliablepredictorofoutcome. [39]
Manypediatricsurgeonsbelievethattheprognosishasimprovedbecauseofmaternalsonographicdiagnosisand
monitoring,whichallowsexpeditiousdeliveryofthesebabiesattertiarycenters.
Obtainingprimaryabdominalwallclosureinababywithgastroschisisrarelyoccurredinthepastitwasusually
necessarytouseasilo.Primaryclosureiscommonplacetoday.Thisprogressisattributedtoimprovementsin
prenatalandobstetricalcare. [6,7,9]

PatientEducation
Parentsshouldbeinstructedregardingthesignificance(ominous)ofbiliousemesis,becausethismayindicatethat
adhesivesmallbowelobstructionormidgutvolvulushasoccurred.
Theyshouldbeinformedthattheirchild'sappendixislocatedinanunusuallocationandthatCTscanningisthe
mostreliablewaytodiagnoseacuteappendicitis.

ContributorInformationandDisclosures
Author
JamesGGlasser,MD,MA,FACSAssociateProfessorofSurgeryandPediatrics,UniversityofSouthAlabama
CollegeofMedicineAttendingStaff,USAChildren'sandWomen'sHospital
JamesGGlasser,MD,MA,FACSisamemberofthefollowingmedicalsocieties:AmericanPediatricSurgical
AssociationandChristianMedical&DentalSociety
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
DavidNSheftelMD,AssistantProfessorofPediatrics,ChicagoMedicalSchoolatRosalindFranklinUniversity
ofMedicineandScience
DavidNSheftelisamemberofthefollowingmedicalsocieties:AmericanAcademyforCerebralPalsyand
DevelopmentalMedicineandAmericanAcademyofPediatrics
Disclosure:Nothingtodisclose.
MaryLWindle,PharmDAdjunctAssociateProfessor,UniversityofNebraskaMedicalCenterCollegeof
PharmacyEditorinChief,MedscapeDrugReference
Disclosure:Nothingtodisclose.
BrianSCarter,MD,FAAPProfessorofPediatrics,UniversityofMissouriKansasCitySchoolofMedicine
AttendingPhysician,DivisionofNeonatology,Children'sMercyHospitalandClinicsFaculty,Children'sMercy
BioethicsCenter
BrianSCarter,MD,FAAPisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,American
AcademyofHospiceandPalliativeMedicine,AmericanAcademyofPediatrics,AmericanPediatricSociety,
AmericanSocietyforBioethicsandHumanities,AmericanSocietyofLaw,Medicine&Ethics,NationalHospice
andPalliativeCareOrganization,andSocietyforPediatricResearch
Disclosure:Nothingtodisclose.
CarolLWagner,MDProfessorofPediatrics,MedicalUniversityofSouthCarolina
CarolLWagner,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,American
ChemicalSociety,AmericanMedicalWomen'sAssociation,AmericanPublicHealthAssociation,American
SocietyforBoneandMineralResearch,AmericanSocietyforClinicalNutrition,MassachusettsMedicalSociety,
NationalPerinatalAssociation,andSocietyforPediatricResearch
Disclosure:Nothingtodisclose.
ChiefEditor
TedRosenkrantz,MDProfessor,DepartmentsofPediatricsandObstetrics/Gynecology,DivisionofNeonatal
PerinatalMedicine,UniversityofConnecticutSchoolofMedicine

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TedRosenkrantz,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
AmericanMedicalAssociation,AmericanPediatricSociety,ConnecticutStateMedicalSociety,EasternSociety
forPediatricResearch,andSocietyforPediatricResearch
Disclosure:Nothingtodisclose.

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