pediatric omphalocele and gastroschisis follow-up

13/5/2015 PediatricOmphaloceleandGastroschisisFollowup

http://emedicine.medscape.com/article/975583followup 1/4

PediatricOmphaloceleandGastroschisisFollowupAuthor:JamesGGlasser,MD,MA,FACSChiefEditor:TedRosenkrantz,MDmore...

Updated:Apr28,2015

FurtherInpatientCare

Omphalocele

Anintactomphalocelesacprotectstheintestinefromcontactwiththeamnioticfluidhence,thesebabiescanbefedmuchearlierfollowingclosureoftheabdominalwalldefect.Babieswithgiantomphaloceleshaveprolongedhospitalizations,becauseofthedifficultyinobtainingclosureandrespiratoryfailure,whichrequiresprolongedmechanicalventilationandatracheotomy.

Gastroschisis

Primaryclosureoftheabdominalwalldefectispossibleonlyifinflammationoftheintestineisminimalevenso,itisusuallyseveralweeksbeforethebabytoleratesenteralfeedings.

Ifclosureoftheabdominalwalldefectnecessitatesuseofasilotocontaintheevisceratedintestine,thesiloshouldberemovedwithinaweekbecauseoftheriskofwoundinfection(seetheimagebelow).

Siloclosureofababywithgastroschisis.

Parenteralnutritionisuseduntilthebabypasses"starvationstools."Ifthishasnotoccurredwithin34weeks,amechanicalobstruction,ratherthananileus,shouldbesuspectedUpperGI/smallbowelradiographyshouldbeperformedtoassessthetransitofcontrastthroughtheintestinaltract.Ifthisstudydemonstratesanintestinalobstruction,laparotomyisindicated.

FurtherOutpatientCareFollowingdischarge,theseinfantsrequireclosefollowuptoassesstheirgrowth,weightgain,andchronicletheirdevelopmentalmilestones.

Theyfrequentlyhavesymptomsofgastroesophagealreflux,whicharenotoriouslyvariableandoccasionallylifethreatening.Practitionersmustmaintainahighindexofsuspicionforthisdiagnosis.Treatmentfollowsthesameprotocolasforanyotherinfantwiththisproblem.

Hirschsprungdiseasemayalsooccur.Physiciansshouldconsiderthisdiagnosisinachildwithrefractoryconstipationandfailuretothrive.

TransferPlacingawarm,moistlaparotomypadovertheexposedintestineintuitivelymakessense.Theproblemisthatawarm,moistlappadrapidlybecomesacold,wetlappadconsequently,theauthorpreferscoveringtheintestinewithadrylappadandwrappingthebaby'storsoinsuchawayastoavoidpullingorkinkingthebowelmesentery.

Ifintravenous(IV)accesscanbeobtained,IVfluidsandantibioticsshouldbeadministered,andthestomachshouldbedecompressedwithanasogastrictube.

ComplicationsNutritionaldepletionisinevitableinababywithanomphaloceletreatedconservatively,becauseofthelargeopenwound.Positivenitrogenbalanceisrestoredfollowingskinclosure.

Prolongedparenteralnutritioncancausehepatotoxicity,manifestedbycholestasisandhepatomegaly,whichmaycomplicatestagedclosureofagiantomphalocele.Omega3fattyacids(Omegaven)reportedlymayreverse"intestinalfailureassociatedliverdisease."

Infantswithgiantomphaloceleshavepulmonaryinsufficiency(hypoplasiaconsequentuponthesmallthoraciccavity)andmayrequireatracheotomyandprolongedventilatorsupport.Stagedabdominalwallclosuregraduallyincreasestheintraabdominalpressurethiselevatesthediaphragmandmaymakeventilationmoredifficult.

Infantswithgiantomphaloceleshaveanincreasedriskofsepsisbecausetheyaredepletednutritionallybytheopenwoundandbecauseoftheirprolongedneedofventilatorsupportandcentralvascularaccess.



Prognosis

Omphalocele

Infantswithomphalocelesarecomplex,withmultisystemorganinvolvement.Theirprognosisdependsontheseverityoftheassociatedproblems.

Giantomphalocelescanbeclosedhowever,multiplesurgicalproceduresareusuallynecessary.Inaddition,theseinfantsmayhaveadditionalmedicalproblemsthatmakecaringforthemquitechallenging.

Thecriticalfactoraffectingthesurvivalofababywithagiantomphaloceleisthesizeofthethoraciccavitywithassociatedpulmonaryhypoplasiaandchronicrespiratoryfailure.Evenifthebabyhasasmallthorax,thepotentialforlunggrowthanddevelopmentencouragesoptimismregardingtheultimateprognosis.

Gastroschisis

Thepatient'sprognosisdependsontheseverityoftheassociatedproblemssuchasprematurityandintestinalinflammatorydysfunction,intestinalatresia,andshortgut.Apopulationbasedcohortstudy(from28pediatricsurgicalcentersintheUnitedKingdomandIreland)analyzedthe1yearoutcomesofinfantswithgastroschisis.Babieswithcomplexgastroschisisrequiredlongerhospitalstaysandhadmorecomplicationsthanbabieswithsimplegastroschisis.Classifyinginfantswithgastroschisisinto"simple"versus"complex"(macroscopicintestinalabnormalities)maybeareliablepredictorofoutcome.[39]

Manypediatricsurgeonsbelievethattheprognosishasimprovedbecauseofmaternalsonographicdiagnosisandmonitoring,whichallowsexpeditiousdeliveryofthesebabiesattertiarycenters.

Obtainingprimaryabdominalwallclosureinababywithgastroschisisrarelyoccurredinthepastitwasusuallynecessarytouseasilo.Primaryclosureiscommonplacetoday.Thisprogressisattributedtoimprovementsinprenatalandobstetricalcare.[6,7,9]

PatientEducationParentsshouldbeinstructedregardingthesignificance(ominous)ofbiliousemesis,becausethismayindicatethatadhesivesmallbowelobstructionormidgutvolvulushasoccurred.

Theyshouldbeinformedthattheirchild'sappendixislocatedinanunusuallocationandthatCTscanningisthemostreliablewaytodiagnoseacuteappendicitis.

ContributorInformationandDisclosuresAuthorJamesGGlasser,MD,MA,FACSAssociateProfessorofSurgeryandPediatrics,UniversityofSouthAlabamaCollegeofMedicineAttendingStaff,USAChildren'sandWomen'sHospital

JamesGGlasser,MD,MA,FACSisamemberofthefollowingmedicalsocieties:AmericanPediatricSurgicalAssociationandChristianMedical&DentalSociety

Disclosure:Nothingtodisclose.

SpecialtyEditorBoardDavidNSheftelMD,AssistantProfessorofPediatrics,ChicagoMedicalSchoolatRosalindFranklinUniversityofMedicineandScience

DavidNSheftelisamemberofthefollowingmedicalsocieties:AmericanAcademyforCerebralPalsyandDevelopmentalMedicineandAmericanAcademyofPediatrics


MaryLWindle,PharmDAdjunctAssociateProfessor,UniversityofNebraskaMedicalCenterCollegeofPharmacyEditorinChief,MedscapeDrugReference


BrianSCarter,MD,FAAPProfessorofPediatrics,UniversityofMissouriKansasCitySchoolofMedicineAttendingPhysician,DivisionofNeonatology,Children'sMercyHospitalandClinicsFaculty,Children'sMercyBioethicsCenter

BrianSCarter,MD,FAAPisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanAcademyofHospiceandPalliativeMedicine,AmericanAcademyofPediatrics,AmericanPediatricSociety,AmericanSocietyforBioethicsandHumanities,AmericanSocietyofLaw,Medicine&Ethics,NationalHospiceandPalliativeCareOrganization,andSocietyforPediatricResearch


CarolLWagner,MDProfessorofPediatrics,MedicalUniversityofSouthCarolina

CarolLWagner,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,AmericanChemicalSociety,AmericanMedicalWomen'sAssociation,AmericanPublicHealthAssociation,AmericanSocietyforBoneandMineralResearch,AmericanSocietyforClinicalNutrition,MassachusettsMedicalSociety,NationalPerinatalAssociation,andSocietyforPediatricResearch


ChiefEditorTedRosenkrantz,MDProfessor,DepartmentsofPediatricsandObstetrics/Gynecology,DivisionofNeonatalPerinatalMedicine,UniversityofConnecticutSchoolofMedicine



TedRosenkrantz,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,AmericanMedicalAssociation,AmericanPediatricSociety,ConnecticutStateMedicalSociety,EasternSocietyforPediatricResearch,andSocietyforPediatricResearch


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pediatric omphalocele and gastroschisis follow-up

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