Pylephlebitis and Crohn's disease: A rare case of septic shock

CASE

REPORT

–

OPEN

ACCESS

ContentslistsavailableatScienceDirect

International

Journal

of

Surgery

Case

Reports

jo u r n al ho me p a g e :w w w . c a s e r e p o r t s . c o m

Pylephlebitis

and

Crohn’s

disease:

A

rare

case

of

septic

shock

Stefano

Scaringi

_,

_Francesco

_Giudici

_,

_Giacomo

_Gabbani

_,

_Daniela

_Zambonin

_,

Marco

Morelli

_,

_Rossella

_Carrà

_,

_Paolo

_Bechi

a_{DigestiveSurgeryUnit,DepartmentofSurgeryandTranslationalMedicine,CareggiUniversityHospital,LargoBrambilla3,50134Florence,Italy} b_{DiagnosticandOperativeRadiology,DepartementofEmergency,CareggiUniversityHospital,Italy}

c_{IntensiveCareMedicine,OspedaledelMugello,AziendaSanitariaFirenze,Italy}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory: Received18June2017

Receivedinrevisedform29June2017 Accepted8August2017

Availableonline10August2017

Keywords: Crohn’sdisease Pylephlebitis Septicshock Gastroenterology Surgery

a

b

s

t

r

a

c

t

INTRODUCTION:Troncularpylephlebitis,definedassepticthrombophlebitisoftheportalvein,isusually

secondarytosuppurativeinfectionfromtheregionsdrainedbytheportalsystem.Therefore,pylephlebitis

canoccurfromtheportalveinmaintributaries.TheoccurrenceofmesentericpylephlebitisinCrohn’s

diseaseisextremelyrare.

PRESENTATIONOFCASE:Wedescribeacaseofsepticshockduetomesentericpylephlebitisina47years

oldmaleaffectedwithCrohn’sdisease.Thepatientwasadmittedtotheemergencydepartmentafter

hehadbeencomplainedfrom3hofaperi-umbilicalabdominalpainassociatedtofeverandshivering

quicklyfollowedbyaseverehypotension.Hismedicalhistoryincludedhistologicallyconfirmedileal

Crohn’sdiseasediagnosed4yearsbeforeandtreatedwithmesalamineonly.Computedtomographyscan

confirmedthemesentericpylephlebitisdiagnosis.Aftermedicaltherapywithantibioticsandsystemic

nutrition,thepatientwassuccessfullyoperatedtotreathisilealCrohn’sdisease.

DISCUSSION:Inourcase,thequickonsetofasepticshockwasnotduetoaperitonitiscomplicatinga

Crohn’sdisease,buttoarareconditionnotneedinganurgentsurgicalresolution.Thisreportshows

that,eveninCrohn’sdisease,oncediagnosisisperformed,antibiotictherapyassociatedtoenteraland

parenteralnutritioncanleadtoacompleteclinicalremissionofmesentericpylephlebitis,mandatoryto

performanelectivesurgery.

CONCLUSION:Thiscasehighlightstheimportanceofpromptlyconsiderateandtreatmesenteric

pyle-phlebitisinpresenceofasepticshockinaCrohn’sdiseasepatientwhoisnotshowingclinicalsignsof

peritonitis.

©2017TheAuthors.PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopen

accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Pylephlebitis,defined asseptic thrombophlebitisof the por-tal veinsystem, is usually secondary toa suppurative process developed in the region drained by the portal vein (troncular phylephlebitis)or byitsmaintributaries(mesentericorsplenic phylephlebitis)[1].Pylephlebitis wasdiagnosed at autopsy and describedforthefirsttimebyWallerin1846inapatientaffected withappendicitis[2].Inthepastitsprognosiswasextremelypoor, butbroad-spectrumantibioticsandsurgicalremovalofthe infec-tivefocuswereabletodecreasebothitsincidenceandmortality.

∗ Correspondingauthor.

E-mailaddresses:stefano.scaringi@unifi.it(S.Scaringi),

francesco.giudici@unifi.it(F.Giudici),giacomogabbani@gmail.com(G.Gabbani),

daniela.zambonin@gmail.com(D.Zambonin),marco.morelli@uslcentro.toscana.it

(M.Morelli),rossella.carra’@uslcentro.toscana.it(R.Carrà),paolo.bechi@unifi.it

(P.Bechi).

PylephlebitiswasdescribedasaCrohn’sdiseasecomplicationin 1946byTaylor[3]and,tothebestofourknowledge,onlyother8 caseshavebeenreportedsofar[2,4–8].Furthermore,onlyinthree ofthemthesuperiormesentericveinwasdescribedtobeprimarily involved.Wereportacaseofsepticshockduetomesenteric pyle-phlebitisinapatientaffectedwithrecentlydiagnosedilealCrohn’s disease.ThisworkhasbeenreportedinlinewiththeSCAREcriteria [9].

2. Presentationofcase

A47-year-oldmanwasadmittedtotheemergencydepartment becauseofasepticshock.Hehadbeencomplainedfrom3hofa peri-umbilicalabdominalpainassociated tofeverandshivering. Thesesymptomswerequicklyfollowedbyaseverehypotension. HismedicalhistoryincludedhistologicallyconfirmedilealCrohn’s diseasediagnosed 4 years beforeand treated withmesalamine only.Hewas187cmtalland92kgweight.Physicalexamination revealeda fixedperi-umbilicalmass withoutabdominal guard-http://dx.doi.org/10.1016/j.ijscr.2017.08.009

2210-2612/©2017TheAuthors.PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http:// creativecommons.org/licenses/by-nc-nd/4.0/).

CASE

REPORT

–

OPEN

ACCESS

S.Scaringietal./InternationalJournalofSurgeryCaseReports39(2017)106–109 107

Fig.1.Thickeningandalterationoftheintestinalwall(1a);smallairbubbleswerevisibleinthemesentery(1b).Fatstrandingwithobstructionoftheinferiormesenteric vein(1c);peripherallinearcollectionsofgasintheliver(1d).

CASE

REPORT

–

OPEN

ACCESS

108 S.Scaringietal./InternationalJournalofSurgeryCaseReports39(2017)106–109

ing.Blumbergsignwasnegative.Initialworkuprevealedsevere leukopenia (300 white blood cells/mcL) and highprocalcitonin levels(453␮g/L).Abdominalx-rayexcludedanintestinal perfora-tion.Abdominalcomputedtomography(CT)scanwasperformed (Fig.1):itconfirmedthepresenceofaCrohn’sdiseaseinvolvingthe terminalileumforalengthofabout70cm.Alongthissmallbowel tractthemesenterywasextremelyinflamedasinaphlegmonous conditioncausingtheobstructionoftheinferiormesentericvein. Airbubbleswerevisiblebothinthemesenteryandintheliver. Thesefindingsweresuggestiveformesentericpylephlebitis com-plicatingilealCrohn’sdisease.Thepatientwasinasevereseptic shockbutwithouthavingclinicalsignofabdominalperforation andperitonitis.Bothleukopeniaandhighprocalcitoninvalue sug-gested the presence of Gram-negative bacteremia. Patientwas promptlysupportedbymechanicalventilationandvasopressors agents. A broad-spectrum antibiotic therapy (intravenous car-bapenem:meropenem1grper3times/day)wasadministeredand thecatabolicstatecorrectedwithbothenteralandparenteral nutri-tions.PeripheralbloodcultureshowedthepresenceofKlebsiella oxytocaandEscherichiacoli.Antibioticsandfluidsresuscitation broughtthepatienttoaquickimprovementofhisgeneral condi-tionsandafter3weeksintheintensivecareunitthepatientwas discharged.Heunderwentelectivesurgeryabouttwomonthslater. Atsurgerythemesenteriumoftheterminalileumwasextremely retractandincreasedinitsthickness(morethan8cm),withsome diseasedilealloopstangentiallyattachedtoit.Anileo-colic resec-tion(80cm+7cm)wasperformed,followedbyanileo-colicside tosideanastomosis.Post-operativecoursewasuneventfulandthe patientsdischargedatday10.HistologyconfirmedCrohn’disease oftheterminalileumassociatedtoanobliterativevasculopathy (Fig.2).Biologicaltherapywasstartedandafter6monthsfollow-up thepatientisingoodgeneralconditionsandfreefromrecurrence.

3. Discussion

Mesentericpylephlebitisisaninfectivesuppurativethrombosis ofthemesentericveinoritsbranches.Itisusuallysecondarytothe developmentofintra-abdominalinfectivefocitypically complicat-ingdiverticulardisease,appendicitisandnecrotizingpancreatitis [2].Thiscriticalclinicalconditioncanariseininflammatorybowel diseasestoo[2–8].However,inCrohn’sdiseaseitisextremelyrare. Furthermore,asshowedbythisreport,theoccurrenceofsucha complicationseemsnottobedirectlyrelatedtotheseverityofthe Crohn’sdiseased:ourpatientwasaffectedbyamildformofCrohn’s disease,recentlydiagnosedandtreatedwithmesalamineonly.

Thepatientexperiencedsepticshockwithoutclinicalsignsof peritonitis:webelieveinCrohn’sdiseaseitisextremelyimportant mesentericpylephlebitistobeconsideredinpresenceofsuch clini-calpresentation,sincenowadays,asshowedbythisreport,through aresuscitationtherapyassociatedtobroad-spectrum antibiotics andenteralandparenteralnutrition,itisacurableconditioneven inpresenceofconcomitantCrohn’sdisease.Twomonthsafterthe patienthadbeendischargedfromtheintensivecareunit,we indi-catedsurgery,performingtheileo-colicresectionwithileo-colic side toside anastomosis. We believe thestrict clinical evalua-tionby surgeons, gastroenterologists and anesthesiologistswas extremelyimportantallowingthepatienttobeelectively oper-atedina“window”periodcharacterizedbyagoodglobalhealth status,startingfromanobtimalnutritionalrepletion,auspicable expeciallyinpatientsaffectedwithCrohn’sdisease,toobtainan uneventfulpostoperativeoutcome,aswellastominimizetherisk ofpost-operativestoma[10].Interestingly,histologicalanalysison thesurgicalspecimenconfirmedthepresenceofanobliterative vasculopathy,eveninabsenceoftheinitialclinicalsymptoms.

4. Conclusion

Multidisciplinaryapproachismandatoryforthedecision mak-ingprocessinpresenceofmesentericpylephlebitis,toobtainbotha correctdiagnosisandaprompttreatment.Infact,althoughsurgical approachisoftennecessary,timingforsurgeryisveryimportantin ordertooperatethepatientinanelectivesetting,whichis manda-torytoperformabowelsparingsurgery,minimizingtheintraand post-operativecomplications. Conflictofinterest None. Funding None. Ethicalapproval Notneeded. Consent

Itwasobtainedfromtheinvolvedpatient.

Authorcontributions

All Authors contributed equally to conception and design, and/oracquisitionofdata,and/oranalysisandinterpretationof dataforthiswork;allAuthorsparticipatedindraftingthearticleor revisingitcriticallyforimportantintellectualcontentandgavefinal approvaloftheversiontobesubmittedandanyrevisedversion. Acquisitionofdata:Scaringi,Giudici,Gabbani,Zambonin,Morelli, Carrà;Analysisandinterpretationofdata:Scaringi,Gabbani, Giu-dici,Bechi;Draftingof manuscript:Scaringi,Giudici,Zambonin, Bechi;Criticalrevision:Scaringi,Gabbani,Giudici,Morelli,Carrà.

Guarantor

Dott.StefanoScaringi.

Acknowledgment

Thisresearchdidnotreceiveanyspecificgrantfromfunding agenciesinthepublic,commercial,ornot-for-profitsectors.

References

[1]J.J.Drabick,F.J.Landry,Suppurativepylephlebitis,S.Med.J.84(1991) 1396–1398.

[2]J.W.Baddley,D.Singh,P.Correa,N.J.Persich,Crohn’sdiseasepresentingas septicthrombophlebitisoftheportalvein(pylephlebitis):casereportand reviewoftheliterature,Am.J.Gastroenterol.94(1999)847–849.

[3]F.W.Taylor,Regionalenteritiscomplicatedbypylephlebitisandmultipleliver abscesses,Am.J.Med.7(1949)838–840.

[4]A.R.Shin,C.K.Lee,H.J.Kim,J.J.Shim,J.Y.Jang,S.H.Dong,B.H.Kim,Y.W.Chang, SepticpylephlebitisasararecomplicationofCrohn’sdisease,KoreanJ. Gastroenterol.61(2013)219–224.

[5]M.Aguas,G.Bastida,P.Nos,B.Beltrán,J.L.Grueso,J.Grueso,Septic

thrombophlebitisofthesuperiormesentericveinandmultipleliverabscesses inapatientwithCrohn’sdiseaseatonset,BMCGastroenterol.7(2007)22.

[6]J.Y.Tung,J.L.Johnson,C.A.Liacouras,Portal-mesentericpylephlebitiswith hepaticabscessesinapatientwithCrohn’sdiseasetreatedsuccessfullywith anticoagulationandantibiotics,J.Pediatr.Gastroenterol.Nutr.23(1996) 474–478.

CASE

REPORT

–

OPEN

ACCESS

S.Scaringietal./InternationalJournalofSurgeryCaseReports39(2017)106–109 109

[7]B.Lerman,J.H.Garlock,H.D.Janowitz,Suppurativepylephlebitiswith multipleliverabscessescomplicatingregionalileitis:reviewof literature-1940–1960,Ann.Surg.155(1962)441–448.

[8]S.Kluge,K.E.Hahn,C.H.Lund,A.Gocht,G.Kreymann,Pylephlebitiswithairin theportalveinsystem.Anunusualfocusinapatientwithsepsis,Dtsch.Med. Wochenschr.128(2003)1391–1394.

[9]R.A.Agha,A.J.Fowler,A.Saeta,I.Barai,S.Rajmohan,D.P.Orgill,SCAREGroup, TheSCAREstatement:consensus-basedsurgicalcasereportguidelines,Int.J. Surg.34(2016)180–186.

[10]R.R.Cima,B.G.Wolff,ReoperativeCrohn’ssurgery:tricksofthetrade,Clin. ColonRectalSurg.20(2007)336–343.

OpenAccess

ThisarticleispublishedOpenAccessatsciencedirect.com.ItisdistributedundertheIJSCRSupplementaltermsandconditions,which permitsunrestrictednoncommercialuse,distribution,andreproductioninanymedium,providedtheoriginalauthorsandsourceare credited.