R a d i o l o g y C a s e R e p o r t s 1 5 ( 2 0 2 0 ) 1 4 9 6 – 1 5 0 1
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Case Report
Torrential bleeding of arteriovenous malformation in hand post-ethanol sclerotherapy: A case report ✩
Jacub Pandelaki, M.D., Ph.D.
a,∗, Theddeus Octavianus Hari Pr asetyono, M.D .
b, Prijo Sidipratomo, M.D., Ph.D.
a, Heltara Ramandika, M.D.
aaDivisionofInterventionalRadiology,DepartmentofRadiology,CiptoMangunkusumoHospital/FacultyofMedicine UniversitasIndonesia
bDivisionofPlasticSurgery,DepartmentofSurgery,CiptoMangunkusumoHospital/FacultyofMedicine UniversitasIndonesia
a r t i c l e i n f o
Articlehistory:
Received1June2020 Revised5June2020 Accepted5June2020 Availableonline3July2020
Keywords:
ArteriovenousMalformations Embolism
Ethanol Sclerotherapy
a b s t r a c t
Embolizationorsclerotherapyisconsideredasthefirst-linetherapyforthemanagement ofarteriovenousmalformations(AVM)andcanbeperformeddirectlytargetingthenidus.
Ethanolisaneffectiveembolicagent;however,somecomplicationsmayarise.Thispaper illustratesacaseoftorrentialbleedingfollowingethanolsclerotherapyinapatientwithpro- gressivehandarteriovenousmalformationswithapoorprognosisandwassuggestedtobe amputated.Directpressure,tourniquetappliance,andsplit-thicknessskingraftprocedure wereperformedtostopthebleedingsuccessfully.Norecurrenceofbleedingwasreported;
andcompletealleviationofpainwasachieved.
© 2020TheAuthors.PublishedbyElsevierInc.onbehalfofUniversityofWashington.
ThisisanopenaccessarticleundertheCCBY-NC-NDlicense.
(http://creativecommons.org/licenses/by-nc-nd/4.0/)
Introduction
Embolization is considered as the first-line therapy for ar- teriovenousmalformations (AVMs) [1].Other treatmentop- tions include pharmacological, surgical, and radio surgical approach. These treatments could producea more benefi- cialoutcomeincertaincircumstances,butembolizationbe- comesthepreferabletreatmentoptionasitprovidesaccept- ablenidusocclusionandalowerrateofbothcomplications and recurrence. [2,3] Disease classification, which is based onangioarchitectureofAVM[4,5],providesembolizationap-
✩ DeclarationofCompetingInterest:Theauthorsdeclarethereisnoconflictofinterestregardingthepublicationofthispaper.
∗Correspondingauthor.
E-mailaddress:[email protected](J.Pandelaki).
proachoptionsthatcouldhelpformulatethetreatmentfor thepatient.
Absoluteethanolisacommonliquidembolicagentused to treat vascular malformation, despite its known toxicity becauseit isvery effectiveinoccluding vessels[6].Dosage adjustment is highly recommended as major and minor complications could arise from its use [7]. Although rare, majorcomplicationssuchaslimbamputationandcardiovas- cularcollapsemaytakeplace[8].Torrentialbleedingwasnot reportedasoneofthemajorcomplicationsfollowingethanol sclerotherapy [6]. Minor complications may include local bullae,localtissuenecrosis,andtransientlocalneurological
https://doi.org/10.1016/j.radcr.2020.06.017
1930-0433/© 2020TheAuthors.PublishedbyElsevierInc.onbehalfofUniversityofWashington.Thisisanopenaccessarticleunderthe CCBY-NC-NDlicense.(http://creativecommons.org/licenses/by-nc-nd/4.0/)
Fig.1– InitialDopplerultrasoundexamination.Dopplerultrasoundidentifiesthevascularnidus,witha“bagofworm”
appearance,atthedorsalregionoftherighthand.
deficits [9–11]. This report illustrates a case of torrential bleeding followingethanol sclerotherapy in a patient with progressivehand AVM witha poorprognosisandwassug- gestedtobeamputated.Nevertheless,the patientprovided consenttopossibleemergencyamputation.
Case Report
A27yearsoldmalewasintroducedwithamassontheright palminvolvingthethirdandfourthdigits.Thelesionstarted todevelop20yearsbeforehisvisitandhad progressedrig- orouslyforthepast1year.Thepatienthadahistoryofsur- gicalmassremoval11yearsagoandclaimedthatthemass wasnolongervisibleafterthesurgery.Themassthenslowly grewbackinsizeaccompaniedbypain(numericratingscale) reached8in the ring fingerfor 10 years afterward.Subse- quently,thetipoftheringfingerdevelopedanecroticwound, which was spreading slowly proximally. A surgery by an- otherteamwasfollowedtodebridethenecrotictissue.The first computertomography angiography (CTA) examination showedAVMwithmultiplenidiatthirdandfourthdigitswith feedingarteriesfromtheradialandulnararteryandwascon- firmedundertheDopplerultrasoundexamination.(Fig.1)
Thepatientthenunderwentthefirstdigitalsubtractionan- giography(DSA)oftherighthand,followedbysclerotherapy usingabsoluteethanolandiohexolsolutionforinjection(Om- nipaque,GE Healthcare,Shanghai,China)with8to2ratios viadirectpuncture.Sclerotherapytargetedthenidibetween third-andfourth-handraysatthepalmregion.Posttreatment DSAshowedsomedevascularizationonthenidi(Fig.2)Clin- icalsymptomwasalleviatedwithasignificant reductionof
pain(numericratingscale2).Afterthe embolization,spon- taneousbleedingoccurredinthedistalfourthfinger.Direct pressureatbleedingsiteandfingertourniquetwereappliedto stopthebleeding.Surgicalhemostasisanddebridementpro- cedurewereperformedonthefourthfingertip.
Second sclerotherapy guided by ultrasound and fluo- roscopyusing20mLofabsoluteethanolandOmnipaque(GE Healthcare,Shanghai,China)mixturewasperformedinthe followingmonth.Onedayaftersclerotherapy,multiplebul- laeappearedatthemainsitesofinjection.(Fig.3)Drainage ofmultiplebullaewasperformedonthepatient’srighthand.
Thepatientreportedcomplete alleviationofpainfollowing theprocedure.
Incidentally, spontaneous massive bleeding occurred at thesamesiteasthelocationofveinvarixduringguidedscle- rotherapy3weeksafter(Fig4,supplementaryvideoavailable ononline version).Thebleedingwastorrential,continuous arterialbleeding,andunresolvedwithdirectpressureusing layersofgauze.Itwasmanagedbypressuredressingunder thetemporaryapplicationoftourniquet(onlyduringdressing for 5 minutes). Constant direct pressure with the elastic bandage was applied successfully to prevent rebleeding.
Following this occurrence of torrential bleeding, patient’s hand wasplannedtobeamputated,howeverthecasewas postponeduntilfurtherangiographyevaluation.Subsequent dressingchangeswereconductedonceeveryweekusingthe sametechniqueoftourniquetapplication.
After one-month, multiple granulation tissues were formed.Nobleedingoccurredduringadressingchange,even without the needto apply a tourniquet. DSA showed that theniduswasfoundtobemuchlessinextensioncompared topreviousDSA.(Fig.5)Amputationprocedurewasdecided as unnecessary and further limb-preserving management
1498
R a d i o l o g y C a s e R e p o r t s 1 5 ( 2 0 2 0 ) 1 4 9 6 – 1 5 0 1Fig.2– Angiogramafterfirstandsecondsclerotherapy.A:FirstdirectpuncturesclerotherapyunderDSAusingabsolute ethanolwastargetedonthenidus.Postsclerotherapyangiogramshowsadequatedevascularization.B:Second
sclerotherapyamonthafterthefirstsclerotherapy,guidedbyultrasoundandfluoroscopy,targetedthenidusaroundthird andfourthdigitsuntildevascularizationwasvisualized.
Fig.3– Multiplebullaeafterthesecondsclerotherapy.One dayafterthesecondsclerotherapy,multiplebullae developedasseenonthevolarside.
was conducted. Split-thickness skin graft (STSG) was per- formedtopreventfurtherbleedingcomplicationsandcover the granulationtissue.Thegraft worked well.Fig. 6shows 3weeksaftersurgery.Thepatientwasscheduledforaddi- tionalsclerotherapywhileembarkingonpassiveandactive range of motion exercise for his thumb, index, and little
finger. All procedures were performed with the patient’s consent.
Discussion
AVMmayoccurinanybodypart,whichbarelyeasytoidentify atitsearlystage[11].Itisevidentinthemassoftherightpalm whichwasdetectablewhenthepatientwasalready8years ofage.Typically,AVMisstagedwithSchobingerclassification [11,12].Inthiscase,anupstagingfromstageIItostageIIIwas depictedwhenthepatientstartedtosufferfromseverepain, andthefourthfingertipdevelopedanecroticwound.Thispro- gressivityandnecrosisareassociatedwitharterialstealsyn- dromeoftheAVMthatreducestissuenutritiveflow[13].
AVM isdiagnosedprimarilybyultrasound andmagnetic resonanceimaging(MRI).Ultrasoundisusefulforanalyzing flowpatternstodiagnosefast-flowmalformationsandtocon- firmarteriovenousshuntingshowninthepatient[12,14].Dy- namicMRIisuseful foridentifying inflowarteries,outflow veins,andthelocationofshunting.Despiteitslimitedutiliza- tioncomparedtodynamicMRI,CTAwithhighspatialresolu- tioncouldbebeneficial,asinthiscase,tomapfeedingand drainingcompartmentsandevaluatebonyinvolvement[14]. CTAofthepatientshowedcomplexAVM,withextensivein- volvementofthepalmandthethirdandfourthdigits,with corticalerosionofthemid-phalanxofthefourthdigit.Multi- pledilatedshunts,identifiedasacomplexvascularnetwork, anddilatedveinsinCTAwereconfirmedbyconventionalar- teriographybeforethefirstsclerotherapy.
TheAVMshouldbefurtherclassifiedaccordingtoitsan- giographicpattern,ascouldbeseenbyCTA.Thepresenceof multipledilatedshunts correspondstotype IIIbofCho-Do classificationandtypeIIIbofYakesclassificationlesion[12]. Thisangiographicpatternclassificationwouldbeneededto determinefurthertreatment.Then,combinedwiththeSchob-
Fig.4– Torrentialbleedingduringguidedsclerotherapythreeweeksaftersecondsclerotherapy.Thebleedingwastorrential, continuousarterialbleeding,andunresolvedwithdirectpressureusinglayersofgauze.Itwasmanagedbypressure dressingunderthetemporaryapplicationoftourniquet(onlyduringdressingfor5minutes).Constantdirectpressurewith theelasticbandagewasappliedsuccessfullytopreventrebleeding.[JP1] [JP1]Addedadditionalpicturetorepresentthe torrentialbleedingoccurred.(Includingastillimagefromthevideo(atabout5secintothevideo)wouldbehelpfulforthose whodonotwanttodownloadandviewtheentirevideo).)
ingerclassification,as inthe presentedcase,percutaneous sclerotherapy,endovascular embolization,or surgerywould beindicated[12,13,15].However,thesurgicalapproachalone maycauselife-threateningbleedingandalsocouldresultin AVMexplosivegrowthduetoincompleteresection[15].There- fore,curativeembolizationoraspreoperativedevasculariza- tionbeforesurgery[15,16]wastheconcernforthiscase.
Amongthe3liquidagentscommonlyused,ethylene-vinyl- alcohol-copolymerandn-butyl cyanoacrylatehasless local and systemic complication than ethanol [17]. Ethanol was chosenbecause it wasmore cost-effectivethan the others inregardstothiscaseandyieldedahighresponserate,yet thefrequentcomplications[6]. Directpuncture injectionof ethanolisalsoreferredtoasethanolsclerotherapyduetoits sclerosingproperties[14,18].Ethanolsclerotherapywasmost effectiveintypeIIandIIIbofCho-Doclassification,withcom- pleteocclusionrateof83%[4].
HemorrhageinAVMcoulddevelopduetoitsnaturalpro- gressionorasaposttreatmentcomplication.Duringitsnat- uralcourse,astheAVM enlarges,theinabilitytoadjustthe bloodflowwouldweakenthevascularwall.Theseareasare weakpointswithahighrateofbleedingandshouldbeelim- inatedbyendovasculartreatment[19].Nevertheless,ifhem- orrhageoccursafterendovasculartreatment,bleedingdueto posttreatmentcomplicationsshouldbeconsidered.Ethanol toxicitycouldcausetransmuralvascularnecrosisextending tosuperficialskinandincreasestheriskofhemorrhage[18]. Inhighflowmalformations,dilutionoftheethanolcouldalso resultinunintended infarctionsoftissuedownstream[20]. Shouldvenousoutflowiscompromisedorthrombosedpar- tiallyduringembolizationwhilethearterialbloodsupplyis preserved,theincreaseofshearstresswouldalsoincreasethe riskofhemorrhage[20].Suchexamplescouldbeseeninthe casewithhighflowbleeding1monthafterthesecondscle-
Fig.5– FollowupevaluationbyDSAonemonthafter secondsclerotherapy.Multipletortuousvessels,indicating vascularnidus,werevisualizedaroundthemetacarpal region,multiplefeedingarteries,fromtheradialartery, interosseousartery,andulnararterywasalsoidentified.
Vascularniduswasfoundtobesmallerinsize(redarrow).
1500
R a d i o l o g y C a s e R e p o r t s 1 5 ( 2 0 2 0 ) 1 4 9 6 – 1 5 0 1Fig.6– ThewoundthreeweeksaftertheSTSGprocedure.
Thegrafttakes,notorrentialbleedingoccurred.Aminor rawsurfaceonthetipoftheforthdigithealssecondarily.
rotherapy.Asthelocationofbleedingisinthe samesiteof venousvarixidentifiedpreviously,whichwaspunctureddur- ingtreatment,hemorrhageofthispatientmostlikelyrelated toendovasculartreatment,mainlyduetoethanoltransmural thrombosis.
Hemorrhageisanabsoluteindicationofprompttreatment forAVM[21].Nevertheless,inourcase,emergencyendovascu- lartreatmentcouldnotbeperformed.Withoutadoubt,treat- mentbytourniquetusealongwithdirectpressure,andcon- stantpressurewiththeelasticbandagewaseffectivetocurb the emergencyproblem.Despite the planforfurthertreat- ment,the hemorrhagewas foundto stop completely,with AVMnidifoundtobesmallerinsizecomparedtoprevious DSA.Whiletourniquetanddirectcompressionbyitselfarea bleedingcontrolmethod,thecompletecessationofthebleed- ingwouldalsobeduetotheeffectofthedelayedsclerosing activityofethanol1monthbeforethevasculardamagepro- cess[14].Atransientposttreatmentinflammatoryresponse hasbeenreportedinthiscase,inwhichfullresponsetothe treatmentcouldbeevaluated.
Inthefinalpresentationofthecase,thehemorrhagehas stopped with no more complaint of pain being reported.
Wound dressing followed bysplit-thickness skingraft was undertakentocover granulationtissueandpreventfurther complications. In consideration of the case, multimodality treatment was administered as it is crucial for high-flow hemorrhage following AVM sclerotherapy. Apart from the necessitytotreatfurthertheAVMpathology,thetechnique
reportedherehadbeensuccessfulinmanagingthehighflow ofhemorrhage.Whilecompletepainreductionwasachieved, thepatientwasnotcompletelyhealedfromtheAVMandwas scheduledforfurthertreatmentsessions.
Thiscaseshowedthepotentialofhighflowhemorrhage followingAVMethanolsclerotherapywhichthenlatershowed completecessationwithconstantpressureusingelasticban- dages.Thiscasealsoshowedethanolsclerotherapyclinical benefit in alleviating pain completely. It would encourage ethanolsclerotherapyforAVMdespitethefearofitssideef- fect,whichcouldbetreatedbyproperpressureapplianceus- ingtemporaryaidoftourniquet.
Acknowledgment
Theauthors thank Albert Owen, M.D.,Samuel Dominggus ChandraSiahaan,M.D.,andEdwinSuharlim,M.D.fortheiras- sistancewiththemanuscriptpreparation.
Supplementary materials
Supplementarymaterialassociatedwiththisarticlecan be found,intheonlineversion,atdoi:10.1016/j.radcr.2020.06.017.
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