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Encapsulating peritoneal sclerosis and other aspects of long-term peritoneal dialysis

Vlijm, A.

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Chapter 3

IMAGING IN ENCAPSULATING PERITONEAL SCLEROSIS

Review

Anniek Vlijm ¹, Joost van Schuppen ², Armand B.G.N. Lamers 3, Dirk G. Struijk 1,4,

Raymond T. Krediet ¹

¹ Division of Nephrology, Department of Medicine, Academic

Medical Center, University of Amsterdam, Amsterdam,

The Netherlands

² Department of Radiology, Academic Medical Center, University of

Amsterdam, Amsterdam, The Netherlands

³ Department of Radiology, Kennemer Gasthuis, Haarlem,

The Netherlands4 Dianet Foundation, Utrecht-Amsterdam, The Netherlands

Submitted

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AbstrActEncapsulatingperitonealsclerosis(EPS)isararebutveryseverecomplicationoflong-

termperitonealdialysis (PD).Sincethefirstreportsonthisdisease intheeighties,

several imagingtechniqueshavebeenused for itsdiagnosis.Becauseof therarity

of this condition,uniformity inmodalityandprotocols for abdominal imaging for

diagnosishasbeenlackingovertime.Nowadays,computedtomography(CT)ismost

oftenused.Inthisreview,weprovideanoverviewofallimagingmodalitiesthathave

beenusedovertimetodiagnoseEPSasalatecomplicationofPD.Imagingfeatures

characteristicforEPSandadvantagesaswellasshortcomingsofallmodalitiesare

discussed. We believe that when EPS is suspected, CT with contrast enhancement

shouldbethemodalityoffirstchoiceinclinicalpractice.

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IntroductionEncapsulatingperitonealsclerosis(EPS),aconditioninwhichafibrouscocoonhassurround-

edthebowel loops1 isanuncommonbutdevastatingcomplicationofchronicperitoneal

dialysis(PD).LongPDdurationandchronicexposuretodialysissolutionsareconsideredrisk

factorsforitsdevelopment2-4.Clinically,patientscanpresentwithsymptomsofabdominal

pain,nausea,vomiting,repeatedbowelobstruction,blood-stainedeffluent,andlossofultra-

filtrationcapacity.ThediagnosisofEPSisbasedonclinicalsymptomsincombinationwith

pathologicalfindingsandabdominalimaging5.

Recently,Stuartet al.describedallimagingtechniquesappliedincharacterizingvarious

complicationsofPD6.Severalimagingtechniqueshavebeenusedoverthepastdecadesto

diagnoseEPS.Casereports,caseseriesandsomelargerstudieshavebeenpublishedover

theyears.Anincreasedawarenessofcomputedtomography(CT)as imagingmodalityfor

diagnosingEPShasdeveloped7.Inarecentpaperthatreviewedtheclinicalsignificanceand

implicationsofEPS,imagingmodalitiesweredescribedinshortandCTscanningwassug-

gestedastheinvestigationofchoiceinpatientswithestablishedEPS8.

ThepresentreviewfocusesonallimagingmodalitiesspecificallyusedtodiagnoseEPS,

nowadaysandinthepast,anddiscussestheirfeatures,qualities,andshortcomings.Itislim-

itedtodiagnostictechniquesforEPSsecondarytoPD.TheMedlinedatabasewassearched

forrelevantreportsandstudieson imagingmodalitiestodiagnoseEPS.Theseparate im-

agingmodalitieswereentered incombinationwith“peritonealsclerosis”, “encapsulating

peritonealsclerosis”,“sclerosingperitonitis”,and“peritonealdialysis”assearchterms.We

restrictedthelanguageofoursearchtoEnglish.

Abdominal radiographyPlainabdominalradiographycanshowair-fluidlevelsandsignsofboweldilation,indicating

obstruction9-11.Anothercommonfeatureisthepresenceofperitonealcalcification6, 12-15.

However,plainabdominalX-rayfilmscanappearnormaleventhoughEPSispresent12, 16.

Nodataonsensitivityandspecificityofplainabdominalfilmsareavailable.Althoughitis

readilyavailableandhelpfulinestablishingbowelobstructionandperitonealcalcifications,

itdoesnotprovideconclusiveorsometimesnotevenadditionalinformationonthepresence

orabsenceofEPS;therefore,weconcludethatwhenEPSissuspected,anabdominalX-ray

hasnoadditionaldiagnosticvalueindiagnosingEPS.

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UltrasonographyUltrasonography(US)hasbeenusedinthepastwhenEPSwassuspected.UScharacteristics

ofEPSarebestappreciatedwithperitonealfluidin situ.Inonestudy,USfindingsof14EPS

patientswerereviewed16.Abnormalsmallbowelactivitywaspresentin12patients,tether-

ingofboweltotheposteriorabdominalwallin10,intraperitonealechogenicstrandsin�,

andmembraneformationin5.InanotherstudybyKrestinet al.disturbedmotilityduring

real-timeUSwasobservedinall13patients,signsofintestinalobstructionin�,andbowel

wallthickeningin511.Campbellet al.reviewedUSimagesof5patientsthatdiedfromEPS,

4patientswithEPSsuspicion,and6patientsconsideredtobeatanincreasedriskforEPSdue

toprolongedPDtherapy.Theyfoundacharacteristicappearanceinseveralpatientsconsist-

ingofanechogenicmembraneinthebowelwall12.Calcificationscanalsobedetectedwith

US11, 15.USisnon-invasiveandhasnoradiationburden.Amajorlimitationisthattheinter-

pretationoftheimagesisverydependentontheradiologist.Therearenodataonsensitivity,

specificityandreproducibility.

Computed tomography

TheuseofCTinEPSdiagnosticswasintroducedin1���17.AbdominalCTscansof2patients

withaclinicalsuspicionofEPSrevealedloculatedascites,adherentbowelloops,narrowing

ofbowellumen,andathickenedperitoneum.SeveralothercasereportsdescribedsimilarCT

findingsandotherfeaturessuchasboweldilation9, 18andthepresenceofperitonealcalcifi-

cation13-15, 18, 19.Krestinet al.describedCTfindingsin�EPSpatients.Inallcases,signsof

disturbedmotilityindicatedbydilatedbowelloopsandair-fluidlevelswereseenandinhalf

ofthecasesloculatedfluidandcontrast-enhancedthickeningoftheperitoneumwerepres-

ent11.Campbellet al.alsoreviewedCTscansof5EPSpatients,4patientswithEPSsuspicion,

and6patientsconsideredtobeatanincreasedriskforEPSandfoundperitonealthickening

andcalcificationsinsomecases12.

ThreestudiescomparedCTscansofEPSpatientstothoseofotherPDpatients.Inthe

firststudy,CTfindingsof10EPSpatientswerecomparedtothoseof�1controlPDpatients20. Peritoneal calcifications, peritoneal thickening, fluid loculation and tethering of small

bowel loops were considered diagnostic for EPS. In the second study, abdominopelvic CT

scans of 2� patients with EPS were compared to CT scans of 15 HD and 20 PD patients

byusingaseverityscoringsystem21.Scoringparametersincludedperitonealcalcification

andthickening,bowelwallthickening,boweltetheringanddilation,andfluidloculation.

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12Figure1–Anexampleofacomputedtomographicscanofapatientwithencapsulatingperitonealsclerosis.Itshowsasci-

tes,bowelloopsthataredrawnintothecenteroftheabdominalcavityindicatingadhesions,andanenhanced,thickened

peritoneumwithcalcificationsbothvisceralandparietal.

AhighlysignificantdifferencewasfoundbetweentotalCTscanscoresofEPSpatientsand

scores of controls. The authors concluded that CT scans could not be used for screening

purposesbecauseEPSpatientshadonlymildabnormalitiesin�outof13casesonCTscans

thatwereperformedmorethan4monthsbeforethediagnosis.Inthethirdstudy,performed

byourowngroup,CTfindingscharacteristicforEPSwereinvestigated.Westudied15EPS

patientsand16long-termPDcontrolpatients22.Wefoundthatconstrast-enhancedCThad

asensitivityof100%andaspecificityof�4%fordiagnosingEPSwhenexperiencedradiolo-

gistsappliedacombinationofspecificCTfindings.Acutoffpointforapositivetestwassetat

positivelyscoringthreeofthesixfollowingitems:peritonealenhancement,thickeningand

calcifications;adhesionsofbowelloops;signsofbowelobstruction;andfluidloculation/sep-

tation.ArepresentativeexampleofaCTscanofanEPSpatientisshowninFigure1.

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CTperitoneography,atechniqueinwhichaCTscaniscombinedwithperitonealcontrast

mediuminsertedthroughtheperitonealcatheter,candemonstratescartissueandpatho-

logicalperitonealrecesses23.However,calcificationscanbeoverlookedbecausetheycanbe

obscuredbyhighattenuationofcontrastmedium24.Itmightbevaluabletoevaluatethe

presenceofEPSwiththistechniquebuttoourknowledgenostudieshavebeenpublished.

MajoradvantagesofCTarethatitiswelltoleratedbypatientsandreadilyavailablein

mosthospitals.ShortcomingsofCTareradiationburdenandriskof lossofresidualrenal

functionduetocontrast-inducednephropathy.Despitetheseshortcomings,itisconsidered

asafetechnique.Whenusedintherightclinicalsettinginsymptomaticpatients,dangerof

radiationexposureofCTingeneralisoutweighedbythemedicalneedandbeneficialeffect

of anaccuratediagnosis 25. Toprevent contrast-inducednephropathy,patients shouldbe

wellhydratedbefore,during,andaftertheprocedure.Althoughtheincidenceisrelatively

lowinwellhydratedpatients,theriskisincreasedinpatientswithaseverelydecreasedkid-

neyfunction.Inarecentstudy,�outof5�patientswitharesidualrenalfunctionofbelow

30 mL/min developed contrast-induced nephropathy 26. If a long-term PD patient has no

residualrenalfunctionanymore,itisofnoconcern.Inanyothercase,CTwithoutcontrast-

enhancementshouldbeconsidered.

Magnetic Resonance ImagingTwocasereportsdescribedmagneticresonanceimaging(MRI)findingsinEPSpatients.Small

boweldistensionandcircumscribedfocalwallthickeningwasdescribedinonepatient27

andmassivelobulatedascitesintheomentumwithwallenhancementofthelobulatedas-

citesandcompressionofthebowelinanother28.AnadvantageofMRIisthatthereisno

radiationburden.Magneticresonanceperitoneograhyhasbeenusedtodetectcomplications

ofPD 29, 30buttoourknowledgeithasneverbeenusedforthepurposeofdiagnosingEPS.

Gadolinium-containingMRcontrastmediaareassociatedwithnephrogenicsystemicfibrosis

andshouldthereforebeavoidedinpatientswithrenalfailure31-33.Also,MRIisatimecon-

sumingandrathercostlytechnique,whichisnotyetasavailableasCT,makingwidespread

uselessappealing.

Colon transit studies Follow-through examinations of small and large bowels have been performed in EPS pa-

tients.Inonecase,asmall-bowelfollow-throughrevealedbowelwallthickeningofadistal

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jejunal loopfollowedbya“cauliflower-like” formationof ileumloops18. Inanothercase,

smallbowelfollow-throughwithbariumshowedboweldilationandencapsulatedloops10.

InthestudybyKrestinet al.11anuppergastrointestinalfollow-throughexaminationwas

performedwithbariumin3patientsandwater-solubleDiatrizoatein5beforesurgicalin-

terventiontookplace.Allcasesdemonstratedadelayedtransittimebutnoclearevidence

ofcompressingintraperitonealbandswaspresent.InthestudybyCampbellet al.12all10

livingpatientsunderwentacolontransitstudy.Theyswallowedcapsulescontainingradi-

opaquemarkerson3successivedays.Onday4,aplainabdominalfilmwasmadeandthe

amountofmarkerswascounted.Fourpatientshadincreasednumbersofcolonicmarkers

indicatingsignificantlyslowedcolonicmotility.

Follow-throughexaminationscanprovideinformationonbowelfunctionandmaybe

helpfulinlocatingtheobstructionsite.However,theyareinvasive,timeconsuming,andre-

quirepreparationsthatcouldinterferewithfluidrestrictionsofdialysispatients.Nowadays,

theyarelessfrequentlyusedinclinicalpractice.

Imaging techniques using radioactivityTheusefulnessoffluordeoxyglucosepositronemissiontomography(PET)indiagnosingEPS

wasstudiedin3EPSpatientsand5asymptomaticlong-termPDpatients34.Forthistech-

nique,radioactivelylabelledtracerwasadministeredintravenously;thereafter,aPETscan

wasdone.Theauthorsshowedthatthistechniquedetectstheinflammatoryphase,ifpres-

ent,of“sclerosingperitonitis”becauseofanincreasedtraceruptakeintheperitoneum.How-

ever,apositivescancouldalsooccurasaresultofanacuteperitonitis;therefore,theclinical

presentationshouldbetakenintoaccountinitsinterpretation.Recently,acasereportwas

describedinwhichradiolabeleddialysatewasinsertedintheperitonealcavityafterwhich

aperitonealscintigraphywasperformedbecauseperitonealadhesionsweresuspected35.

Nonuniformdistributionofthedialysateincombinationwithloculatedtraceraccumulation

confirmedthepresenceofadhesions.Itmightbepossiblethatthatthistechniquecouldbe

effectiveindetectingEPSbutnostudieshavebeenpublished.Anobviousdisadvantageis

theuseofradioactivematerialforthesemodalities.

ConclusionEPSisararebutlifethreateningcomplicationoflong-termPD.Biomarkersinperitonealef-

fluenthaveapotentialroleinearlydiagnosingEPS36but,untilnow,noimagingscreening

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methodsareavailable.Accurateimagingtechniquesfordiagnosingthisseverediseaseare

ofgreatimportance.Avarietyofimagingtechniques,invasiveaswellasandnon-invasive,

havebeenusedandstudiedtodiagnoseEPS.Inthisreviewwehaveprovidedanoverviewof

thesemodalitiesanddiscussedtheirspecificfindings,advantagesandlimitations.

CTisthemostfrequentlystudiedimagingtechniquefordiagnosingEPS.Itistheonly

technique thathasbeen investigated in case-controldesigns 20-22 and forwhichdataon

sensitivityandspecificityisavailable22.Althoughwehavediscussedseveralshortcomings,

CThasshowntoaccuratelydiagnoseEPS.WeadvocatethatCTwithcontrastenhancement

shouldbe themodalityoffirst choicewhenEPS is suspected.Evaluationof theCTscans

shouldpreferablybeperformedbyexperiencedradiologistswithknowledgeofPDandEPS.

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