Median Arcuate Ligament Syndrome in a patient with Crohn's disease.

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InternationalJournalofSurgeryCaseReports4 (2013) 399–402

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International

Journal

of

Surgery

Case

Reports

jo u r n al h om ep a g e :w w w . e l s e v i e r . c o m / l o c a t e / i j s c r

Median

Arcuate

Ligament

Syndrome

in

a

patient

with

Crohn’s

disease

Alessandro

Sturiale

, Giovanni

Alemanno, Francesco

Giudici, Rami

Addasi,

Francesco

Bellucci,

Francesco

Tonelli

DigestiveSurgeryUnit,DepartmentofClinicalPathophysiology,UniversityofFlorenceMedicalSchool,CareggiUniversityHospital,Florence,Italy

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t

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l

e

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Articlehistory:

Received19December2012 Accepted15January2013 Available online 29 January 2013 Keywords:

MedianArcuateLigamentSyndrome Dunbar’sSyndrome

Crohn’sdisease

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INTRODUCTION:TheMedianArcuateLigamentSyndromeisarareconditioncharacterizedbypostprandial abdominalpain,bowelfunctiondisorderandweightloss.Wereportthefirstcasetoourknowledgeof Crohn’sdiseaseandMedianArcuateLigamentSyndrome.

PRESENTATIONOFCASE:Thepatientwasa33year-oldfemalewithapreviousdiagnosisofCrohn’s dis-ease.Acutepostprandialabdominalpainaffectedthepatienteveryday;shewas,therefore,referredto US-Dopplerandmagneticresonanceangiographyoftheabdominalvesselsandreceivedadiagnosisof MedianArcuateLigamentSyndrome.Consequently,thepatientwassurgicallytreated,releasingthe vas-cularcompression.Aftertheoperation,shereportedacompleterelieffrompostprandialpainwhichwas oneofhermajorconcerns.Subocclusivesymptomsoccurredaftersixmonthsduetotheinflammatory reactivationoftheterminalileitis.

DISCUSSION:ThediagnosisofMedianArcuateLigamentSyndromeismainlybasedontheexclusionof otherintestinaldisordersbutitshouldbealwaysconfirmedusingnoninvasivetestssuchasUS-Doppler, angio-CTormagneticresonanceangiography.

CONCLUSION:ThiscasedemonstratesthattheMedianArcuateLigamentSyndromecouldbethemajor causeofsymptoms,eveninpresenceofotherabdominaldisorders.

© 2013 Surgical Associates Ltd. Published by Elsevier Ltd.

1. Introduction

Clinical radiological studies based on the relation between abdominal symptoms and celiac trunk compression by right diaphragmatic crux, ledto theinclusion of a new conditionin the field of vascular pathology – the Celiac Trunk Compres-sionSyndrome.1 _{Thisdisordermainlyaffectsyoungectomorphic} females betweentheageof 20and 50 (F:Mratio of4:1).2 _The symptomsaretypicallythree:postprandialabdominalpain,bowel functiondisorderandweightloss.Thistriadresemblesthe symp-toms of intestinal angina caused by atherosclerotic lesions of themajorsplanchnicvessels;postprandialpain,however,isless severe.1,3,4_{Thegrade ofstenosis,thesystolicanddiastolic flow} velocitiesintheceliactrunkcanbeevaluatedbymeansof non-invasivetestssuchasUS-Doppler,angio-CTormagneticresonance angiography(MRA).5–8 _{Whatfollowsis ourfirstcase ofMedian} ArcuateLigamentSyndrome(MALS)inapatientwithCrohn’s dis-ease(CD).

Abbreviations:MALS,MedianArcuateLigamentSyndrome;MAL,Median Arcu-ateLigament;CD,Crohn’sdisease;CT,computerizedtomography;MRA,magnetic resonanceangiography;US,ultra-sonography;WBC,whitebloodcells;ESR, eryth-rocytesedimentationrate.

∗ Correspondingauthorat:LargoBrambilla3,50134Florence,Italy. Tel.:+390557947449;fax:+390557947449.

E-mailaddresses:alexstur@yahoo.it,a.sturialemd@gmail.com(A.Sturiale).

2. Presentationofcase

A33year-oldfemalepatientarrivedwithahistoryof abdomi-nalpainassociatedwithalternatingbowelfunctionandnauseashe experiencedsincetheageof20.Intheprevious3yearsshelost5kg andexperiencedadegenerationofabdominalpain,especiallyafter meals,weakness,fatigue,nauseaand diarrhea.Thepatient was admittedtotheGastroenterologyDepartment.Laboratory evalu-ationrevealedhigherWBC(13.7mg/dl)andinflammatorymarkers (ESR33mm/h,CR-Protein48mg/L).Apancolonoscopywith retro-gradeileoscopyshowedhyperemicmucosawithvascularpattern alterationattheilealsite. Thehistopathologicalexaminationof biopsiesshowedinflammatorychanges,typicalofCD.Thickening oftheterminalilealloopanditsmesenterywasseenonCTscan.The patientstartedatreatmentofmesalazine1600mg×2/die.Aperiod ofremissionwasfollowedbyarelapse,astreatmenthadtobe sus-pendedbecauseofapregnancy.Thepatient,however,continued suffering frompostprandial pain;a US-Doppler of the abdomi-nalvesselswasperformed(Fig.1)whichshowedstenosisofthe celiactrunkwithanincreaseinflowvelocity.TheMRAconfirmed thestenosis ofceliac trunkwithextrinsiccompression (Fig.2). AlthoughCDwasconfirmedbythehistopathologicalexamination, theclinicalhistoryofpostprandialpainalongwiththe radiologi-calevidenceofceliacarterystenosisfacilitatedadiagnosisofboth MALSandCrohn’sdisease.Withthisjointclinicalscenario,the sit-uationwasthoroughlydiscussedwiththepatientandthedecision toperformsurgicalrelease oftheMALwasagreed.Laparotomy 2210-2612© 2013 Surgical Associates Ltd. Published by Elsevier Ltd.

http://dx.doi.org/10.1016/j.ijscr.2013.01.015

Open access under CC BY-NC-ND license.

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Fig.1. US-Dopplershowingtheincreaseinflowvelocity(A)andstenosisoftheceliactrunk(B).

wasperformed;inspectionoftheileumshowednoCDfeatures. Consequently,wemadeanexplorationandskeletonizationofthe celiactrunk,commonhepaticandleftgastricartery(Fig.3A). Digi-talpalpationofceliacarterybrancheselicitedveryweakpulse.An intraoperativeDoppleroftheceliactrunkconfirmedthestenosis andtheincreasedflowvelocity.Afibroticareacompressingthe celiactrunkattheoriginoftheleftgastricarterywasidentified

andcut(Fig.3B).Subsequently,thearcuateligamentwascutalong its entire length (about 2cm) and sent for a histopathological test.Thenormalizationoftheceliacbloodflowwasconfirmedby intraoperativeUS-Doppler;thepulsewasrestoredinthecommon hepatic,gastroduodenalandleftgastricarteries.The histopatho-logicalexaminationofthespecimenrevealedafibrotictissuewith hypertrophicperipheralnervestructures.Thepatienthadashort

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A.Sturialeetal./InternationalJournalofSurgeryCaseReports4 (2013) 399–402 401

Fig.3.Intraoperativeimageofthemedianarcuateligamentfiberscompressingtheceliactrunk(A).Animageaftercuttingthemedianarcuateligament(B).

hospital stay and was discharged after three days reporting a completereliefofsymptoms,especiallyofpostprandialpain,and relativegoodhealth.Sixmonthsaftertheoperation,shereturned foracomplaintofabdominalpainandsubocclusion.Colonoscopy withretrogradeileoscopyshowedamildterminalileum inflam-mation;fecalcalprotectinwas97mg/kg.Todate,thepatientison medicaltreatmentforCDwithagoodresponse.

3. Discussion

TheMALSisararevasculardisorderwithanincidenceofabout2 casesper100,000patients,withdiffusedandnon-specific abdom-inalpain.Inliterature,itwasfoundthattheincidenceofMALSin asymptomaticpatientsrangesbetween7.3and27%.2,8–10_Suchhigh incidenceinasymptomaticpatientscouldraisetheissuewhether surgicalreleaseofthecompressionwouldrelievethesymptoms ofnon-specificabdominalpain,which couldberelatedtoother causes. It is known that many patients have minimal asymp-tomaticceliactrunkcompression;hence,itisofmajorimportance to discriminate those having a pathological compression.5 _The firstanatomicdescriptionofMALSdatesbackto1917,givenby Lipshutz,11_{butonlyin1965theradiologistJ.D.Dunbarreported} thefirst case seriesfocusing ondiagnosisand treatment.1 _This syndromeisduetotheceliactrunkcompressionbythemedian arcuateligamentinassociationwithganglionicperiaortictissue.9 Although47yearshaveelapsed,thepathogenesisofMALSisstill uncertain.Someauthorssupportthetheorybasedonahigher ori-ginoftheceliactrunkfromaorta,othersmaintaintheexuberant growthofneurofibroustissueoriginatingfromtheceliacplexus causingcompression.12_{Thesymptomsarethetypicalpostprandial} painassociatedwithnausea,vomiting,andunintentionalweight loss.13 _{ThepathophysiologyofMALSis still unknown.Thefirst} hypothesisisbasedonmesentericischemiaevenifthesuperior andinferiormesentericarteriesarenormal.Itcouldbedueto post-prandial“theft”ofblood,whichcausespain.Thesecond,instead,is theconsequenceofdirectorindirectoverstimulationoftheceliac plexus,causedbychronicinflammation,resultingin splanchnic vasoconstrictionwithischemia.5,13_{Despiteseveraltests,the} diag-nosisisbasedontheexclusionofotherabdominaldiseases.Lateral aorticangiographyisthegoldstandardbut thereareotherless invasivetechniquessuchasUS-Doppler,CTorMRA.Ineverycase itisimportanttocorrelateabdominalsymptomswith radiologi-caldata.8,13_{ThetreatmentconsistsinthesectionoftheMAL,and} maybecarriedoutbyanopenorlaparoscopicapproach.Mostof thecasesinliteratureweretreatedwiththeopentechnique.5,8,9,13 Thelongestcaseserieswaspublishedin1985,byReilly,with51 patients,showinggoodlong-termresults.9_{Someauthorsproposed}

alsoanendovasculartreatment,butitdidnotgivegoodresultsdue tothepersistingextrinsiccompression.Conversely,thismaybea usefultreatmentincaseofpersistentsymptomsfollowingthe sur-gicalsectionoftheMAL,duetovesselwallalterationscausedbythe chronicexternalcompression.5,13_{CD,oneofthetwomajorforms} ofinflammatoryboweldiseases,hasanetiologyand pathogene-sisthatincludescomplexinteractionsofgenetic,immunological andenvironmentalfactors,withanimportantrole beingplayed byluminalmicrobialagents.14_{Ithasdifferentclinicalpatternand,} ineverycase,thecommonsymptomsaremainlyabdominalpain associatedwithdiarrheaandfever.Extraintestinalsymptomssuch aserythemanodosum,iritisanduveitis,peripheralartritiscould alsoappear.15_{Asreportedinliterature,thediagnosisofMALSis} mainlybasedonthefusionofsymptomsandradiologicalimages excludingotherabdominaldiseases.AlthoughinourpatientCD wouldhavejustifiedmostofthesymptoms,postprandialpainwas sotypicalthatitleadustothinkofotherpathologiesand, conse-quently,toperformingvascularinvestigations.Aftersurgery,the patienthadacompleteremissionofabdominalsymptoms, espe-ciallypostprandialpain.Itwouldalsobeimportanttoevaluateif thechronicischemia,resultingfromthecompressionoftheceliac trunk,could bea long-term co-factor in thedevelopment of a multiple-sitechronicinflammatorydiseasecompatiblewithCD.

4. Conclusion

ThiscasedemonstratesthattheMedianArcuateLigament Syn-dromecouldbethemajorcauseofsymptoms,eveninexistenceof otherabdominaldisorders.Forthisreasoninpatientswithupper gastrointestinaldisorders,especiallypostprandialpain,that per-sistaftermedicaltherapy,itcouldbeusefultoperformvascular investigationevaluatingthepossibilityofceliactrunkcompression.

Conflictofinterest

Nonedeclared.

Funding

None.

Ethicalapproval

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

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Authors’contribution

Allauthorscontributedequallytothiswork:Alessandro Sturi-ale, Giovanni Alemanno and Francesco Bellucci collected the data,AlessandroSturialeandGiovanniAlemannoanalyzeddata, Alessandro Sturiale, GiovanniAlemanno, Francesco Giudici and RamiAddasiwrotethemanuscript,FrancescoTonellisupervised allthemanuscript and obtainedaninformed consentfromthe patient.

Acknowledgement

Prof.MariaRosariaBuri,ProfessionalTranslator/AIICConference Interpreter,UniversityofSalentofortheEnglishlanguageediting. References

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