GIGI GrandGrand RoundsRounds

““AA LifetimeLifetime ofof AbdominalAbdominal PainPain”” 12/9/200412/9/2004 TimTim EdwardsEdwards PMHPMH AugAug 25,25, 19921992

„ 44 yearyear oldold malemale presentspresents toto aa pediatricpediatric gastroenterologistgastroenterologist forfor primaryprimary complaintcomplaint ofof anorexia,anorexia, intermittentintermittent abdominalabdominal painpain whichwhich occasionallyoccasionally awakensawakens himhim atat nightnight „ TheseThese problemsproblems havehave beenbeen presentpresent forfor >1>1 yearyear „ NegativeNegative UGIUGI studystudy „ EGDEGD withwith mildmild gastritisgastritis „ RxRx withwith tagamettagamet 40mg/kg/day40mg/kg/day andand caffeinecaffeine freefree dietdiet MayMay 19,199419,1994

„ SeenSeen forfor c/oc/o abdominalabdominal painpain withwith vomitingvomiting atat bedtimebedtime „ BeenBeen doingdoing wellwell offoff allall medicationsmedications forfor 11 yearyear „ WeightWeight 4444 lbs;lbs; heightheight 4343 inchesinches „ ExamExam withinwithin normalnormal limitslimits „ GESGES T1/2T1/2 prolongedprolonged atat 135135 minutesminutes „ RxRx withwith CisaprideCisapride 5mg5mg 20minutes20minutes QAC+HSQAC+HS forfor gastroparesis.gastroparesis. OctoberOctober 19,19, 19941994

„ SeenSeen inin f/uf/u forfor gastritisgastritis andand GERDGERD „ DoingDoing wellwell onon TagamentTagament andand PropulsidPropulsid „ NoNo abdominalabdominal painpain „ RecentlyRecently startedstarted withwith looseloose stoolsstools „ HeightHeight 4646 in,in, weightweight 47.547.5 lbslbs „ ExamExam withinwithin normalnormal limitslimits „ PropulsidPropulsid stoppedstopped withwith recurrencerecurrence ofof severesevere abdominalabdominal painpain withinwithin severalseveral daysdays SeptemberSeptember 21,21, 19981998

„ F/UF/U ofof GERDGERD andand gastroparesisgastroparesis „ PrilosecPrilosec 20mg/day20mg/day andand PropulsidPropulsid 10mg10mg BIDBID--TIDTID „ C/OC/O crampycrampy abdominalabdominal painpain „ NoNo vomitingvomiting „ 22--33 looseloose stoolsstools perper dayday withoutwithout bloodblood oror mucusmucus „ NoNo weightweight lossloss „ HeightHeight 5555 inches;inches; weightweight 9090 lbslbs FebruaryFebruary 25,25, 20032003

„ SeenSeen forfor recurrentrecurrent abdominalabdominal painpain associatedassociated withwith vomitingvomiting forfor threethree weeksweeks „ OffOff medicationsmedications forfor 22 yearsyears „ DailyDaily epigastric/substernalepigastric/substernal pain.pain. PainPain usuallyusually postprandial.postprandial. „ EmesisEmesis isis nonbiliousnonbilious,, previouslypreviously ingestedingested foodfood „ LostLost 66 lbslbs „ WeightWeight 121121 lbs;lbs; HeightHeight 6565 inin „ ExamExam withinwithin normalnormal limitslimits „ PlacedPlaced onon blandbland dietdiet andand NexiumNexium forfor recurrentrecurrent GERDGERD AugustAugust 18,18, 20032003

„ RecentRecent EGDEGD withinwithin normalnormal limitslimits „ BiopsiesBiopsies normal,normal, nono celiacceliac diseasedisease „ NoNo improvementimprovement withwith ZelnormZelnorm 3mg3mg BIDBID „ OnceOnce perper weekweek withwith severesevere crampycrampy abdominalabdominal painpain relievedrelieved withwith nonbiliousnonbilious vomitingvomiting „ WeightWeight 112112 lb;lb; heightheight 6666 inchesinches „ ExamExam withinwithin normalnormal limitslimits „ 4H4H GESGES withwith T1/2T1/2 161161 minutesminutes JanuaryJanuary 27,27, 20042004

„ F/UF/U GERDGERD andand gastroparesisgastroparesis „ RecurrentRecurrent abdominalabdominal painpain hashas returnedreturned „ ProminentProminent regurgitationregurgitation „ FrequentFrequent nauseanausea „ NoNo diarrheadiarrhea „ DecreasedDecreased appetite;appetite; lostlost 44 lbslbs sincesince OctoberOctober „ IncreasedIncreased NexiumNexium 40mg40mg BID;BID; IncreasedIncreased ReglanReglan toto 55 mgmg TIDTID FebruaryFebruary 8,8, 20042004

„ StillStill withwith epigastric/substernalepigastric/substernal painpain 22-- 3X/wk3X/wk „ WillWill vomitvomit whenwhen painpain isis severesevere „ ReglanReglan increasedincreased toto 7.5mg7.5mg TIDTID „ ReferredReferred toto DrDr WoWo forfor evaluationevaluation andand potentialpotential useuse ofof DomperidoneDomperidone AprilApril 20,20, 20042004

„ DoingDoing wellwell onon NexiumNexium QAMQAM andand reglanreglan BIDBID „ NoNo N/V/abdominalN/V/abdominal painpain „ RepeatedRepeated UGIUGI withwith SBFTSBFT andand CTCT abd/pelvisabd/pelvis werewere withoutwithout evidenceevidence ofof obstruction,obstruction, stricture,stricture, oror IBDIBD „ BloodBlood teststests werewere withoutwithout suggestionsuggestion ofof secondarysecondary causescauses ofof gastroparesisgastroparesis „ TrialTrial ofof DomperidoneDomperidone 10mg10mg TIDTID JulyJuly 30,30, 20042004

„ AdmittedAdmitted toto UU ofof LL HospitalHospital fromfrom DrDr WoWo’’ss clinicclinic forfor 22 daysdays ofof sharp,sharp, constant,constant, nonradiatingnonradiating,, epigastricepigastric painpain whichwhich waswas relievedrelieved withwith vomitingvomiting „ NoNo POPO intakeintake forfor 22 daysdays „ IncreaseIncrease inin typicaltypical GERDGERD painpain „ NoNo diarrheadiarrhea „ PMH:PMH: AsAs outlinedoutlined previously.previously. O/WO/W negative.negative. „ PSHxPSHx:: NoneNone „ FmHxFmHx:: NoncontributoryNoncontributory „ SocialSocial Hx:Hx: DoesDoes wellwell inin school,school, nono ETOH,ETOH, drugsdrugs „ All:All: NKDANKDA „ Meds:Meds: NexiumNexium 40mg40mg POPO BID,BID, DomperidoDomperidonene 1010 mgmg POPO BIDBID „ ROS:ROS: 1111 lblb weightweight lossloss previousprevious 33 weeksweeks PhysicalPhysical ExamExam

„ VS: 112/79 12 96.8 68 „ Gen: NAD „ HEENT: NC/AT, EOMI, anicteric, o/p without lesion „ Neck: No TM, no LAN „ CV: RRR „ Lungs: CTA B „ Abd: S/ND/minimal TTP mid epigastrium, no HSM, no masses „ Ext: No c/c/e „ Neuro: AAOX3, nonfocal. LaboratoryLaboratory

„ Hgb/HctHgb/Hct 16/4616/46 WBCWBC 77 PltPlt 284284 „ NaNa 137137 ClCl 9494 BUNBUN 1818 KK 3.63.6 CO2CO2 3030 CrCr 1.11.1 CaCa 99 TpTp 8.18.1 AlbAlb 4.74.7 „ AmylaseAmylase 5353 LipaseLipase 110110 ASTAST 2525 ALTALT 2424 TbiliTbili 0.080.08 ImagingImaging

„ 7/30/20047/30/2004 CTCT AbdomenAbdomen „ MarkedMarked dilatationdilatation ofof thethe stomachstomach andand proximalproximal duodenumduodenum withwith aa transitiontransition pointpoint nearnear thethe thirdthird portionportion ofof thethe duodenum.duodenum. „ MayMay bebe secondarysecondary toto focalfocal dysmotilitydysmotility versusversus obstructionobstruction secondarysecondary toto thethe mesentarymesentary andand itsits vascularvascular structuresstructures simulatingsimulating aa SMASMA syndrome.syndrome.

UpperUpper GIGI limitedlimited

„ AugustAugust 3,3, 20042004 „ ThereThere isis aa fillingfilling defectdefect seenseen inin thethe secondsecond oror thirdthird portionportion ofof thethe duodenumduodenum whichwhich maymay bebe c/wc/w intrinsicintrinsic vsvs extrinsicextrinsic defect,defect, butbut intrinsicintrinsic defectdefect isis favored.favored. TheThe etiologyetiology maymay bebe ectopicectopic pancreaticpancreatic tissue,tissue, largelarge adenomatousadenomatous polyp,polyp, smallsmall bowelbowel tumortumor oror otherother multiplemultiple extrinsicextrinsic causescauses suchsuch asas SMASMA syndrome.syndrome. „ NonNon--obstructiveobstructive bowelbowel patternpattern EGDEGD DefinitiveDefinitive TherapyTherapy

„ GivenGiven endoscopicendoscopic andand radiologicradiologic evidenceevidence ofof extrinsicextrinsic lesionlesion resultingresulting inin obstructionobstruction aa surgicalsurgical consultationconsultation waswas obtained.obtained. „ OnOn 8/4/20048/4/2004 thethe patientpatient waswas takentaken toto thethe OROR forfor exploratoryexploratory laparotomylaparotomy forfor diagnosisdiagnosis ofof sourcesource ofof duodenalduodenal obstructionobstruction OperativeOperative FindingsFindings

„ Liver,Liver, gallbladder,gallbladder, spleen,spleen, andand stomachstomach appearedappeared normalnormal „ UnableUnable toto locatelocate thethe ligamentligament ofof TrietzTrietz „ TheThe duodenumduodenum waswas notnot fixedfixed inin usualusual retroperitonealretroperitoneal positionposition th „ ThickThick fibrousfibrous bandband ofof tissuetissue crossedcrossed thethe 44th portionportion ofof thethe duodenumduodenum asas anan obstructionobstruction pointpoint „ CecumCecum andand ascendingascending coloncolon werewere mobilemobile andand notnot attachedattached toto thethe laterallateral abdominalabdominal wallwall „ ThisThis waswas c/wc/w intestinalintestinal malrotationmalrotation

IntestinalIntestinal MalrotationMalrotation inin thethe AdolescentAdolescent

„ MidgutMidgut malrotationmalrotation isis estimatedestimated toto occuroccur inin approximatelyapproximately 1/5001/500 livelive birthsbirths „ TheThe truetrue incidenceincidence isis unknownunknown owingowing toto thosethose whowho remainremain asymptomaticasymptomatic andand gogo undiagnosedundiagnosed „ SurgicalSurgical seriesseries estimateestimate 5050--80%80% presentpresent inin thethe firstfirst monthmonth ofof lifelife „ 20%20% presentpresent withinwithin firstfirst yearyear „ 1010--20%20% presentpresent olderolder thanthan 11 yearyear PathophysiologyPathophysiology

„ IntestinalIntestinal malrotationmalrotation cancan bebe simplysimply defineddefined asas anyany deviationdeviation fromfrom thethe normalnormal 270270 degreedegree counterclockwisecounterclockwise rotationrotation ofof thethe midgutmidgut duringduring embryonicembryonic developmentdevelopment NormalNormal RotationRotation

„ InIn thethe firstfirst twotwo monthsmonths ofof developmentdevelopment thethe growthgrowth ofof thethe intestinesintestines exceedsexceeds thethe capacitycapacity ofof thethe abdomenabdomen toto containcontain themthem „ TheThe bowelbowel developsdevelops outsideoutside thethe abdomenabdomen inin thethe yolkyolk sacsac „ NormalNormal counterclockwisecounterclockwise rotationrotation ofof thethe bowelbowel isis drivendriven byby thethe greatergreater raterate ofof proximalproximal bowelbowel growthgrowth asas comparedcompared toto distaldistal bowelbowel andand thethe rapidrapid growthgrowth ofof thethe fetalfetal liverliver ReturnReturn toto thethe AbdomenAbdomen

„ FirstFirst thethe duodenojejunalduodenojejunal junctionjunction passespasses behindbehind thethe SMASMA andand becomesbecomes fixedfixed toto thethe upperupper leftleft retroperitoneum.retroperitoneum. ThisThis formsforms thethe ligamentligament ofof TrietzTrietz „ Second,Second, thethe cecocoliccecocolic junctionjunction passespasses fromfrom thethe leftleft sideside ofof thethe abdomen,abdomen, anterioranterior toto thethe SMA,SMA, assumingassuming itsits positionposition rightright ofof midlinemidline „ Overall,Overall, thethe bowelbowel rotatesrotates 270270 degreesdegrees counterclockwisecounterclockwise fromfrom thethe originaloriginal primaryprimary looploop DuodenojejunalDuodenojejunal RotationRotation CecocolicCecocolic LimbLimb NormalNormal FixationFixation

„ AtAt thethe completioncompletion ofof thethe rotationrotation thethe intestinesintestines becomebecome fixedfixed toto thethe retroperitoneumretroperitoneum byby aa broadbroad basedbased mesenterymesentery „ TheThe mesenterymesentery extendsextends fromfrom thethe ligamentligament ofof TrietzTrietz toto thethe ileocecalileocecal junctionjunction NormalNormal MesentericMesenteric FixationFixation NonrotationNonrotation

„ NonrotationNonrotation occursoccurs whenwhen thethe duodenojejunalduodenojejunal andand cecocoliccecocolic limbslimbs returnreturn thethe abdomenabdomen withoutwithout anyany rotationrotation „ TheThe smallsmall bowelbowel isis locatedlocated inin thethe rightright abdomenabdomen „ TheThe coloncolon isis locatedlocated inin thethe leftleft abdomenabdomen

MalrotationMalrotation

„ MalrotationMalrotation occursoccurs withwith thethe duodenojejunalduodenojejunal limblimb havinghaving nono rotation.rotation. TheThe cecocoliccecocolic limblimb hashas partialpartial rotationrotation „ TheThe cecumcecum willwill bebe fixedfixed toto thethe rightright centralcentral abdominalabdominal wallwall byby thickthick peritonealperitoneal bands.bands. TheseThese bandsbands maymay causecause extrinsicextrinsic compressioncompression ofof thethe duodenumduodenum „ ThisThis configurationconfiguration resultsresults inin aa veryvery narrownarrow mesentericmesenteric attachmentattachment „ TheThe narrownarrow vascularvascular pediclepedicle predisposespredisposes toto volvulusvolvulus withwith subsequentsubsequent ischemiaischemia andand necrosisnecrosis

ClinicalClinical PresentationPresentation

„ Majority of symptomatic malrotation is diagnosed within the first week of life „ The presentation of malrotation in adolescents and adults is highly variable „ Most will have intermittent abdominal pain „ The pain has an unusual nature in the it will be transient, vague, and not necessarily associated with any physical findings „ Often the pain is postprandial and may or may not be associated with vomiting „ Less common presentations include failure to thrive , malabsorption, diarrhea, motility disorders, and biliary obstruction ClinicalClinical PresentationPresentation

„ CaseCase studiesstudies reportreport thethe timetime toto diagnosisdiagnosis rangingranging fromfrom monthsmonths toto 1717 yearsyears „ CommonCommon misdiagnosesmisdiagnoses includeinclude cycliccyclic vomiting,vomiting, foodfood allergy,allergy, IBS,IBS, andand motilitymotility disordersdisorders „ OftenOften malrotationmalrotation isis firstfirst suspectedsuspected inin adolescentsadolescents duedue toto abnormalabnormal imagingimaging studiesstudies oror atat laparotomylaparotomy PlainPlain FilmsFilms

„ ConventionalConventional radiographyradiography isis neitherneither sensitivesensitive nornor specificspecific forfor malrotationmalrotation „ RightRight sidedsided jejunaljejunal markingsmarkings andand absenceabsence ofof stoolstool filledfilled coloncolon inin thethe rightright lowerlower quadrantquadrant cancan bebe suggestivesuggestive ofof malrotationmalrotation „ PlainPlain radiographsradiographs maymay bebe completelycompletely normalnormal UpperUpper GIGI SeriesSeries

„ AA limitedlimited UpperUpper gastrointestinalgastrointestinal bariumbarium seriesseries remainsremains thethe mostmost accurateaccurate tooltool forfor detectiondetection ofof malrotationmalrotation „ FindingsFindings includeinclude failurefailure ofof thethe duodenojejunalduodenojejunal junctionjunction toto crosscross thethe midlinemidline andand lyinglying belowbelow thethe levellevel ofof thethe duodenalduodenal bulbbulb andand aa clearlyclearly misplacedmisplaced duodenumduodenum thatthat hashas aa corkscrewcorkscrew appearanceappearance „ 75%75% ofof casescases havehave obviousobvious signssigns ofof malrotationmalrotation

BariumBarium EnemaEnema

„ ContrastContrast enemaenema examinationexamination usuallyusually showsshows malpositionmalposition ofof thethe rightright coloncolon „ ContrastContrast enemaenema findingsfindings areare nonspecificnonspecific becausebecause thethe cecalcecal locationlocation cancan bebe variablevariable withoutwithout malrotationmalrotation „ 20%20% ofof patientspatients withwith malrotationmalrotation willwill havehave aa cecumcecum whichwhich assumesassumes aa normalnormal positionposition givinggiving aa falsefalse negativenegative studystudy

UltrasoundUltrasound

„ TheThe rolerole ofof ultrasoundultrasound forfor diagnosingdiagnosing malrotationmalrotation isis notnot establishedestablished „ AA normalnormal U/SU/S doesdoes notnot rulerule outout malrotationmalrotation „ FindingsFindings whichwhich suggestsuggest malrotationmalrotation areare anan abnormalabnormal relationshiprelationship ofof thethe SMVSMV andand SMA;SMA; eithereither anterioranterior oror toto thethe leftleft ofof thethe SMASMA „ TheThe ““whirlpoolwhirlpool”” signsign ofof volvulusvolvulus causedcaused byby thethe twistingtwisting ofof thethe vesselsvessels aroundaround thethe narrownarrow mesentericmesenteric pediclepedicle CTCT ImagingImaging

„ ManyMany casescases ofof quiescentquiescent malrotationmalrotation inin adolescentsadolescents andand adultsadults areare detecteddetected byby CTCT’’ss obtainedobtained forfor otherother reasonsreasons „ CTCT cancan depictdepict extraextra--intestinalintestinal findingsfindings notnot seenseen onon conventionalconventional imagingimaging „ DeviationDeviation ofof thethe normalnormal SMVSMV toto SMASMA relationshiprelationship (vertical(vertical oror leftleft--rightright inversioninversion isis suspicioussuspicious forfor malrotation)malrotation) „ TheThe pancreaspancreas maymay showshow underdevelopmentunderdevelopment oror absenceabsence ofof thethe uncinateuncinate processprocess

TreatmentTreatment

„ TheThe treatmenttreatment forfor malrotation,malrotation, whetherwhether asymptomatic,asymptomatic, relatedrelated toto acuteacute duodenalduodenal obstruction,obstruction, oror incidentallyincidentally foundfound isis surgicalsurgical „ TheThe ““LaddLadd”” procedureprocedure isis usedused forfor treatmenttreatment ofof duodenalduodenal obstructionobstruction secondarysecondary toto malrotationmalrotation „ HeHe wrotewrote hishis paperpaper onon thisthis procedureprocedure inin 19321932

OutcomeOutcome

„ OverallOverall mortalitymortality raterate isis relatedrelated toto thethe presencepresence oror absenceabsence ofof volvulusvolvulus andand intestinalintestinal necrosisnecrosis atat thethe timetime ofof surgerysurgery „ MortalityMortality approachesapproaches 0%0% inin healthyhealthy patientspatients withoutwithout intestinalintestinal ischemiaischemia „ RecurrentRecurrent volvulusvolvulus cancan notnot bebe eliminatedeliminated duedue toto thethe inabilityinability toto correctcorrect thethe underlyingunderlying defectdefect ofof malrotation.malrotation. EstimatedEstimated recurrencerecurrence isis 22--5%5% SummarySummary

„ TheThe clinicalclinical diagnosisdiagnosis ofof malrotationmalrotation inin adolescentsadolescents andand adultsadults isis rarelyrarely consideredconsidered „ Adolescents/adultsAdolescents/adults mostmost oftenoften presentpresent withwith chronicchronic abdominalabdominal painpain withwith oror withoutwithout vomitingvomiting oror chronicchronic diarrheadiarrhea „ MalrotationMalrotation shouldshould bebe consideredconsidered inin anyany adolescentadolescent withwith intermittentintermittent abdominalabdominal pain,pain, vomiting,vomiting, diarrhea,diarrhea, oror malabsorptionmalabsorption „ SurgerySurgery isis indicatedindicated inin allall casescases ofof malrotationmalrotation regardlessregardless ofof thethe discoverydiscovery toto reducereduce riskrisk ofof volvulusvolvulus andand associatedassociated complicationscomplications