Refractory hepatic lymphorrhea after total pancreatectomy. Case report and literature review of this uncommon complication.

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

Refractory

hepatic

lymphorrhea

after

total

pancreatectomy.

Case

report

and

literature

review

of

this

uncommon

complication

Michele

Bartoli

,

Gian

Luca

Baiocchi,

Nazario

Portolani,

Stefano

Maria

Giulini

DepartmentofMedicalandSurgicalSciences,SurgicalClinic,UniversityofBrescia,BresciaCivilHospital,P.leSpedaliCivili,1,25123Brescia,Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received20August2015 Receivedinrevisedform 14September2015 Accepted18September2015 Availableonline26September2015 Keywords:

Refractoryhepaticlymphorrhea Chylousascites

Lymphadenectomy Hepatoduodenalligament Lymphangiography

a

b

s

t

r

a

c

t

INTRODUCTION:Afterextendedabdominallymphoadenectomy,lymphaticvesselinjurymaycause lym-phorrhea thatusuallydisappears spontaneously.However, intractableascitessometimes develops. Althoughtherearemanyreportsdescribingpersistentchylousascitesfromintestinallymphorrhea,little isknownabouthepaticlymphorrhea,notcontainingchyle.Itiscausedbyinjuryofthelymphaticvessels duringhepatoduodenalligamentlymphadenectomy.

Wepresentacaseofmassiveascitesduetohepaticlymphorrheaaftertotalpancreatectomyand extendedlymhoadenectomyforAmpullaradenocarcinoma.Wesuccessfullytreateditwithprolonged medicaltherapyaftersurgicalrelaparotomy.

PRESENTATIONOFCASE:A65-yearoldmanunderwenttotalpancreatectomywithextendednodal dis-section.Massiveclear-coloredascites(2000–9000mLperday)developedsincethesecondpostoperative dayandpersisteddespiteconservativetherapy.Atre-laparotomynolymphaticleakagewasfound. Sim-ilarlylymphangiographywasshowednocontrastspreading.Wetreatedthishepaticlymphorreawith intermittentopeningoftheabdominaldrainageuntilspontaneousresolution.

DISCUSSION:Thestandardtreatmentofhepaticlymphorrheaisanaggressivemedicaltreatment.After suchapproachthemosteffectivetherapyseemstobesurgicalexploration.Otheroptionare peritoneove-nousshuntorintraperitonealadministrationofOK-432.

CONCLUSION:Inourexperiencetheintermittentabdominaldrainageuntilspontaneousresolutionisan usefulapproachtohepaticlymphorrhea.

©2015TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Duringabdominalsurgery,especiallyafterextended lymphoad-enectomyforgastricorpancreaticcancer,lymphaticvesselinjury causeslymphorrhea.Thepostoperativelymphorrheausually dis-appearsspontaneouslyinafewdays.However,intractableascites may develop especially in patients with liver cirrohosis, heart failureorrenalfailure.Althoughtherearemanyreports describ-ing the diagnosis, causes and treatment of persistent chylous ascites fromintestinallymphorrhea, littleis knownabout hep-atic lymphorrheafollowing abdominal surgery. It is caused by injuryofthelymphaticvesselsespeciallyduringthe hepatoduo-denalligmanetlymphadenectomy.Usuallyconventionalmedical treatmentssuchascessationoforalintake,totalparenteral nutri-tion,diuretictherapy,albuminsupport,intravenousreinfusionof ascites,ortherapeuticparacentesisareunsuccessful.Wepresent acaseofintractablemassiveascitesduetohepaticlymphorrhea aftertotal pancreatectomyand extended lymhoadenectomy for

∗ Correspondingauthor.

E-mailaddress:michelebartoli83@gmail.com(M.Bartoli).

Ampullaradenocarcinoma,successfullytreatedwithconservative approachafterineffectivesurgicalrelaparotomy.

2. Presentationofcase

A65-yearoldmanwashospitalizedduetoobstructivejaundice. AnadenocarcinomaofVaterpapillawithnodalinvolvementwas diagnosedbyabdominalTCscanandERCP.Preoperativestaging wasT2N+.ASAgradewas3.Intra-operativefindingofsoft pan-creasandlittleWirsungduct(<3mm)promptedustoperformtotal pancreatectomy.Suspectednodalinvolvementtillinteraortocaval nodeswasfound,soextendedlymphnodedissectionwascarried out(overD2).PostoperativestagingwasT3N1withinvolvementof 2of27nodes.Massiveascites,rangingfrom2000to9000mLper day,developedsincethesecondpostoperativeday.Theasciteswas unresponsivetoalbuminandelectrolytesupport,administration ofmaximaldiureticsdose,diet,totalparenteral nutrition(TPN), continuousdrainage,andoctreotideinfusion.Cytological examina-tionrevealednomalignatcells,andculturewasinitiallynegative. Theascites wasclear-coloredand wasdiagnosedbiochemically asnonchylouslymphorrheawithtotal proteinconcentrationof 3g/dL,albuminconcentration1,5g/dLandserum-ascitesgradient http://dx.doi.org/10.1016/j.ijscr.2015.09.023

2210-2612/©2015TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http:// creativecommons.org/licenses/by-nc-nd/4.0/).

CASE

REPORT

–

OPEN

ACCESS

M.Bartolietal./InternationalJournalofSurgeryCaseReports16(2015)134–136 135

Fig.1. Lymphangiographyshowingnocontrastspreading.

1g/dL.Themainconstituentoftheasciteswashepaticlymph.It derivedfromsurgicalinjuryofthelymphaticsinthe hepatoduo-denalligament.Inthe19thpostoperativeday(POD)wedecided toperformsurgicalre-exploration.Howevernoobviouslymphatic leakagepointwasfoundevenafteradministrationoffattymeal throughnaso-gastrictubeandLipofundin(BBraun; Melsungen; Germany)injectioninlymphaticvessels.Meticulousexplorationof hepatoduodenalligamentwasperformed;eveninabsenceofclear leakage,surgicalligationofinteraortocavaltissuesand Atossis-clerol(ChemischeFabrikKreussler&Co.;Wiesbaden;Germany) application in hepatoduodenal ligament were carried out. Also Tachosil(Takeda; Linz;Austria) wasappliedtotreat lymphatic leakages.

Aftersurgicaltreatmenttherewasnochangein theamount of the drainage (about 4000–9000mL/day). The patient’s daily activitiesworsened,duetodisturbanceofmobilityandfever com-promisingrespiratoryfunctiontillacuterespiratoryfailure.

Inthe34thPOD,duetotheincreasingoflymphorrhea, lym-phangiographywasperformedinanattempttoidentify(andtreat withlymphaticembolization)anunrecognizedleakage.However nocontrastspreadingwasfound(Fig.1).

The patient developed sepsis sustained by Pseudomonas Aeruginosaatfirst,andthenbymulti-resistentKlebsiella Pneu-moniae and Candida Albicans. He also suffered from septic chorioretinitis that required surgical treatment (vitrectomy) in additiontotargetedantibiotictherapy.

The postoperative course was characterized by progressive weightloss,hypoalbuminemia,electrolyteimbalanceanddiffuse edema. We decided toclose theabdominal drainage for a few days.Thisoption caused asthenia,appetite loss,nausea, vomit-ing,fullnesssensation,abdominaldistention,peripheraledemaand requiredparenteralsupportandcarefulenteralnutritionwithnose gastrictube.

Theintermittentopeningofthedrainage,causedthe progres-sivedecreaseofthelymphorrhea.After132dayswecouldremove thetube. The patient wasdischarged in the 181th POD.At 12 monthsfollow-up,thepatientisalive,diseasefree andhasfair generalconditionswithoutlymphorrhearecurrence.

3. Discussion

Intraabdominallymphpathwaysaremanlyclassifiedinto hep-aticandintestinal.both Thesepathwaysdrainintothecisterna

chyliand subsequently intothecirculatorysystemthrough the thoracicduct.Thehepaticlymphaticsystemhastwomajor path-ways(ascendinganddescending)ofdrainage.Viatheascending pathways,lymphfromthesurfaceoftheupperpartoftheliver flows along thediaphragm intothecisterna chyli whilelymph fromtheliverbedandintheliverflowsalongthehepaticveins. Thedescendingpathwayrunsthroughthehepatoduodenal liga-mentwithportalvein,hepaticarteryandbileduct.Intestinallymph drains50–75%ofintraabdominallymphandcontainsmainlylipid dropletsoflongchainfattyacids;thusitscolorismilky.Ontheother hand,hepaticlymphdrains25–50%ofintraabdominallymph.This lymphcontainsproteinsofthesamedensityasplasmawithout lipiddropletsandisclear-colored[1,2].

Intra abdominal lymphorrhea, without chylous ascites, is a rarecomplicationafterabdominalsurgery.Itresultsfrominjury ofthelymphaticvesselsparticularlyinthehepatoduodenal liga-ment.Althoughsmalllymphleaksarecommonlyleftopenduring lymphadenectomy,theyrarelycauselymphorrheabecauseofthe abundanceofcollaterallymphaticchannelsandtheregeneration ex-novo oflymphatics. Therefore theyusually remainclinically asymptomaticorunrecognized.Lymphorreabecomesintractable incases ofinjurytomajorlymphaticvesselsand when repara-tivemechanismsbecomeinsufficient. Littleis knownaboutthe managementofpostoperativehepaticlymphorrea.Toour knowl-edge,thereisonlyonereportofhepatic lymphorrheafollowing abdominalsurgeryintheWesternmedicalliterature[3],andless thantwentycasesinEasternmedicalliterature[4].After diagno-sisofpostoperativehepaticlymphorrhea,allpatientsweretreated conventionallybydiet,TPN,diureticsandtherapeuticparacentesis. We performed an aggressive medical treatment since the presentation of lymphorrhea by TPN, massive diuretics ther-apy,continuous drainage, albuminand electrolyte supportand octreotideinfusion.Themechanismby whichoctreotideaffects lymphorreaisunknown.Ithasalsobeenspeculatedthat somato-statininhibits lymph fluid excretion through specific receptors foundinthenormallymphaticvessels.

After conventional medical treatment, various regimens are usedtotreattheintractablehepaticlymphorrhea(Table1).The mosteffective therapyappears tobesurgicalligationoflymph fistulawithsclerotherapyand/orfibringluesprinkleevenifthe pre-operativeimagingstudydoesnotshowanobviousleakagepoint

[4].

Wecarriedoutsurgicalligationearlierthanotherauthors,even inabsenceofobviousleakagepoint,becauseofamountof lymphor-reaandresistancetoallconservativetherapies.Noauthorreported ascites more than4500mLper day.Atthe time ofreoperation thepatientsbotheredlymphorrhearangingfrom4000to8000mL perday,drainedbytheabdominaldrainagetube.Itcausedsevere andpotentiallylife-threateningipokalimia,albumindepletionand dehydrationdespitemedicaltherapy.

Inoue[5]reportedtheuseofperitoneovenousshunt(PVS)for intractableascitesduetohepaticlymphorrhea.Theplacementof aPVSismainlyusedforintractableascitesduetodecompensated livercirrhosisandforrefractorychylousascites.Itisasimpleand cost-effective procedureeven ifit is linkedtohighmorbidities suchasobstruction,vascularthrombosis,infections,sepsisand dis-seminatedintravascularcoagulopathy.Henceithasbecomealess commontechnique.Moreoverourpatientshowedsignsofsepsis (leukocytosis, fever,highPCR andprocalcytonine value) associ-atedwithP.AeruginosaandKlebsiellapneumoniacolonizationof theascites.ThisconditionhighlycontraindicatedPVSplacement.

Fewauthors[4,6]reportedeffectivetreatmentofhepatic lym-phorrhea using local intraperitoneal administration of OK-432 whichisaheatandpenicillin-treatedpreparationofstreptococcus pyogenes A3. It is largely used in the treatment of lymphat-ics malformations and for malignant ascites. OK-432 promotes

CASE

REPORT

–

OPEN

ACCESS

Table1

Chracteristics,therapiesandclinicaloutcomeofthepatientswithhepaticlymphorrhea.

Case Author Age/sex Operation Treatment Timetocompleteresolution

1 Miyagawa,1983 65/M TG Surgicalligation 13

2 Nakashima,1985 58/M DG Surgicalligation+sclerotherapy 30

3 Nakano,1987 49/M TG Surgicalligation 14

4 Kawata,1989 52/M DG Surgicalligation+fibringlue+sclerotherapy 37

5 Umehara,1989 59/M TG Surgicalligation 28

6 Kaneko,1991 44/M DG Surgicalligation+PVS 30

7 Imai,1992 34/M TG Reoperation+sclerotherapy 7

8 Shimizu,1992 62/M DG Surgicalligation 30

9 Ota,1993 70/M DG Surgicalligation+fibringlue 50

10 Mitsuno,1993 42/M DG PVS ND

11 Kawahira,1994 58/M DG Surgicalligation+fibringlue+OK-432sclerotherapy 10 12 Matsumoto,1995 44/M DG Re-re-surgicalligation+fibringlue 14 13 Tanaka,1998 49/M DG Surgicalligation+fibringlue+OK-432sclerotherapy 12 14 Tanaka,2004 66/M TG Surgicalligation+fibringlue+OK-432sclerotherapy 67

15 Inoue,2011 73/M HR PVS 12

TG:totalgastrectomy;DG:distalgastrectomy;PVS:peritoneovenousshunt;HR:hepaticresectionND:notdescribed.

reabsorptionofasciticfluidincreasingcapillarypermeabilityand inducing local inflammatory action which causes occlusion of injuredlymphatics.We havenoexperienceofthis kindof scle-rotherapyandtheethicsCommitteedidn’tauthorizetheuseofthis kindofdrugs.

Ourstrategywastoopenandcloseintermittentlytheabdominal drainage.Atfirstthisoptioncauseddiscomforttothepatientbut allowedagradualreductionofspontaneouslymphorrheauntilits resolution.

4. Conclusion

Inourexperience,thisapproachincreasesintraabdominal pres-sureand promotes trans-peritoneal reabsorption. This solution maybeusefulwhen theotheroptions mentioned byliterature provetobeineffectiveorcontraindicated.

Conflictofinterest None. Funding None. Ethicalapproval Notneeded. Consent

Writteninformconsentwasobtainedfromthepatientfor pub-licationsofthiscasereportandaccompanyingimages.Acopyof thewrittenconsentisavailableforreviewbytheEditor-in-Chiefof thisjournalonrequest.

Authorcontribution

MicheleBartoli wastheprincipalinvestigator andwrotethe paper.StefanoMariaGiulini,NazarioPortolaniandGianLuca Baioc-chitreatedthepatient.Allauthorscriticallyreviewedthepaperand approvedthefinalversion.

Guarantor MicheleBartoli. Acknowledgements

None. References

[1]H.Ota,T.Miyazawa,I.Hiizu,etal.,Acasereportofintractableascitesdueto hepaticlymphorrheafromhepatoduodenalligamentafterradicalgastrectomy forgastriccancer,Jpn.J.Gastroenterol.Surg.26(1993)1115–1119.

[2]Y.Kawahira,K.Nakao,M.Nakahara,etal.,Acaseofintractablehepatic lymphorrheaaftergastrectomyforgastriccancer,Jpn.J.Gastroenterol.Surg.27 (1994)117–120.

[3]D.Guez,B.S.Gregory,J.Nadolski,etal.,Transhepaticlymphaticembolizationof intractablehepaticlymphorrhea,J.Vasc.Interv.Radiol.25(1)(2014)149–150.

[4]K.Tanaka,Y.Ohmori,Y.Mohri,etal.,Successfultreatmentofrefractoryhepatic lymphorrheaaftergastrectomyforearlygastriccancer,usingsurgicalligation andsubsequentOK-432(Picibanil)sclerotherapy,GastricCancer7(2004) 117–121.

[5]Y.Inoue,M.Hayashi,F.Hirokawa,etal.,Peritoneovenousshuntforintractable ascitesduetohepaticlymphorrheaafterhepatectomy,WorldJ.Gastrointest. Surg.3(1)(2011)16–20.

[6]Y.Inaba,Y.Arai,K.Matsueda,etal.,Intractablemassiveascitesfollowing radicalgastrectomy,treatmentwithlocalintraperitonealadministrationof OK-432usingaunifiedCTandfluoroscopysystem,Australas.Radiol.47(2003) 465–467.

OpenAccess

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