GI DISTRESS — MALROTATION, VOLVULUS, BOWEL … · symptoms and treatments of malrotation,...

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GIDISTRESS—MALROTATION,VOLVULUS,BOWELOBSTRUCTION,PANCREATITISByCarolPotterAsPartIIofatwo-partreviewofGIissuesinpersonswithCdLS,Dr.CarolPotterdescribessymptomsandtreatmentsofmalrotation,volvulus,bowelobstruction,andpancreatitis.MalrotationandVolvulusSymptomsofmalrotationandvolvulusmayincludevomiting,pain,aswollenabdomen,bloodyorloosestools,rapidheartrateandbreathing,littleornourine,andfever.Malrotationoccurswhentheintestineorboweldoesnotfoldorrotateproperlyinearlyfetaldevelopment.Malrotatedintestinesmay“flop”aroundsincetheyarenotproperlyattachedtotheabdomenwall.Thiscanresultintheintestinestwistingaroundoneanother.Thisiscalledvolvulus.Asthishappens,theduodenummaybetwistedshutwhichcaninducevomitingwithorwithoutpain.Iftheintestinestwistfurther,theymaycompressthebloodsupply.Thiscancauseseverepainasthebloodsupplytothesmallintestineiscutoff.Sometimes,thetwistrelaxesandthepainresolves.However,witheachepisodethereisthechancethatthebloodvesselcompressionwilllasttoolongandtheintestinewilldie.Unfortunately,theentiresmallintestineisusuallyinvolved,withtheexceptionofthetopfewinchesandthelowerhalfthecolon.Whenthismuchbowelislost,mostchildrenrequireintravenousnutritionfortherestoftheirlives.Malrotationisdiagnosedbyhavingthechildswallowbarium.Itisimportanttotrackthebariumasittravelsthroughthefirstpartofthesmallintestinetomakesuretheintestinepassescorrectlyfromrighttoleft.Sometimesbariumenemasareusedtoseethelocationofthebeginningofthecolon.Becausemalrotationcanbealife-threateningemergencythatmostcommonlyoccursinchildhood,pediatricradiologistsareoftenmorefamiliarwithitssubtleformsandmoreaggressiveaboutwatchingbariumlongenoughtomakesuremalrotationisnotpresent.Forthisreason,werecommendthatstudiesdonetoevaluatevomitinginchildrenbedonebypediatricradiologistswheneverpossible.Malrotationisamedicalemergency.Ifvolvulusisnotpresent,i.e.thebowelisnotblockedfromtwistingandthebloodsupplyisnotbeingcompressed,surgicalcorrectionisusuallydonewithinafewdays.Ifvomitingispresentandx-raystudiesshowthatvolvulusispresent,surgicaltherapyshouldoccurimmediately.Thesurgeonwilluntwistthebowelandtrytodetermineifanybowelhasdied.Oncedeadbowelisremoved,theremainingintestineisattachedtothebackoftheabdomentopreventfurthervolvulus.

BowelObstructionSignsofobstructionorblockageincludepain,vomiting(greenoryellowbile),anddistentionoftheabdomen.Unfortunatelynoneofthesecantellyoudefinitivelyifthechilddoesordoesnothaveobstruction.Theyare,however,worrisomesignsandshouldbeinvestigated.Bowelobstructionsimplymeansblockageofthegastrointestinaltract.Thiscanoccuranywherefromthemouthtotherectum.Congenitalmalformations(malformationspresentatbirth)cancausebowelobstruction.Atresia,anabsenceofopeningsinareasoftheintestine,isacommoncauseseenatbirth.Othercongenitalproblemsmayshowuplaterinlife,includingwebs(thinmembraneswhichstretchacrosspartoftheintestine).Malrotationandvolvulusarealsoexamplesofcongenitalmalformationsthatcanshowupatanyage.SometimespartoftheGItracthasaduplication,orasecondGItractrunningbesideit.Thiscanbeshortorlongandcansometimescauseblockageofthemainintestinaltract.Somebowelobstructionsdonotoccurbecauseofproblemswearebornwithbutproblemsweacquireduringlife.Adhesions,orbandsofscartissuecanresultafteranysurgeryintheabdomenandcanblocktheintestines.Fundoplications(surgerydoneforgastroesophagealreflux)thataretootightcancauseobstructionorblockage.Inflammationintheintestineorabdomencanalsocauseobstruction.Finally,severeconstipationcancauseareversibleobstructionoftheGItract.PancreatitisPancreatitismeansinflammationofthepancreas.Therearemanycausesbuttheyleadtoasimilarpicture.Thechildmaydevelopasevereabdominalpain,oftenaccompaniedbyvomiting,overarelativelyshortperiodoftime.Thechild’sabdomenisoftenverytenderandthechildappearssick.Thereareseveralcausesofpancreatitis.Virusescancausepancreatitisinchildren.Ablowtotheupperabdomencanalsocausepancreatitisasthefragileductsofthepancreasaredamagedagainstthespine.Manymedications,includingsomeseizuremedicationsandsteroids,canalsocausepancreatitis.Gallstonesandcongenitalmalformationsofthebileductsandpancreascanalsopredisposechildrentoinflammationofthepancreas.Thediagnosisofpancreatitisismadebybloodtests.X-raystudiessuchasultrasound,CATscanandMRImayhelpdeterminethecauseandseverityofthepancreatitis.Thepancreasmakestheenzymesweneedtobreakdownfood.Whenthepancreasisininflamed,thesesameenzymesstarttodigestthepancreas.Iftheprocessismild,thechildmaygetbetterwithminimalornointervention.MoreseverecasesofpancreatitisoftenrequireadmissiontothehospitalforIVfluidsandpaincontrol.Achildmaynotbeabletoeathis/herregulardietuntiltheconditionimproves.Inaveryseverecaseofpancreatitis,thechildcanbecomeextremelyillanddeveloplongtermcomplications.

Theprognosisforchildrenwithpancreatitisisdependentonthecauseoftheinflammationandtheseverityoftheillness.ChildrenwithCdLSareatriskfordevelopingGIissues.Earlyrecognitionofthesignsandsymptomsoftheseconditions,quickmedicalaction,andperseverancewithmedicalprofessionalswillequipparentsandcaregiverswiththebestdefenseagainstthedevelopmentofmoreseriouscomplicationsintheirchildren.

GI DISTRESS — MALROTATION, VOLVULUS, BOWEL … · symptoms and treatments of malrotation,...

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