www.elsevier.es/rmuanl
SCIENTIFIC
LETTER
Thrombosed
great
saphenous
vein
aneurysm
accompanied
by
venous
thrombosis
F.G.
Rendón-Elías
a,∗,
R.
Albores-Figueroa
a,
L.S.
Arrazolo-Ortega
a,
F.
Torres-Alcalá
a,
M.
Hernández-Sánchez
b,
L.H.
Gómez-Danés
aaServiceofThoracicandCardiovascularSurgeryofthe‘‘Dr.JoséEleuterioGonzález’’,UniversityHospitaloftheUANL,Mexico bServiceofPediatrics,UMAE
·21,IMSSMonterrey,NuevoLeón,Mexico
Received14May2014;accepted22July2014 Availableonline23June2015
KEYWORDS
Greatsaphenousvein; Venousaneurysms; Vascular
malformations; Venousthrombosis
Abstract Superficialvenousaneurysmsofthelowerextremitiesareconsideredrareandtheir clinicalsignificanceispoorlydefined.Thepurposeofthisarticleistoreportacaseofa 72-year-old womanwithathrombosed greatsaphenousveinaneurysmalongwithdeepvenous thrombosisandreviewitsclinicalpresentation,diagnosisandtreatment.
©2014UniversidadAutónomadeNuevoLeón.PublishedbyMassonDoymaMéxicoS.A.Allrights reserved.
Introduction
Venousaneurysms(VA)areararevascularpathology. First describedbySirWilliamOslerin1913,1VAscanbelocated
anywherethroughoutthevenoussystemanddonothavea preferenceregardingsexorage.2
VAs are usually located in the lower extremities, and can be deep or superficial, depending on whether the affected vein is over or under the muscle fascia. Deep VAs are the most frequent, because the popliteal vein is the most commonly affected (between 60% and 70% of
∗Correspondingauthorat:ServiciodeCirugíaTorácicay
Cardio-vasculardelHospitalUniversitario‘‘Dr.JoséEleuterioGonzález’’, delaUniversidadAutónomadeNuevoLeón,Av.MaderoyGonzalitos S/N,CP.64460,Monterrey,NuevoLeón,Mexico.
E-mailaddress:[email protected](F.G.Rendón-Elías).
the cases), and the most studied because of their high thromboembolismrisk.3
SuperficialVAsarerare,withunder60reportedcasesin medicalliterature4 andaretakenless seriouslythan deep
VAs,due tothefactthatsuperficial VAsareconsidered to havealowriskoflife-threateningcomplications.
Thepurposeofthisarticleistopresentacaseofapatient affectedby athrombosed greatsaphenous vein aneurysm alongwithdeepvenousthrombosis.Additionally,the liter-atureisreviewedwithadiscussiononclinicalimplications andthediagnosticandtherapeuticapproachof thislesser knownvascularpathology.
Clinical
case
The patient is a 72-year-old female, with no positive background of chronic degenerative diseases or of any othertype,andanegativehistoryofsmoking.Thepatient
http://dx.doi.org/10.1016/j.rmu.2015.02.002
Figure1 Reconstructedultrasoundimagewhichshowsaninternalsaphenousveinvenousaneurismof9×5cm,whichcompression
didnotremove.
presentedanincreaseinvolumeoftherightpelviclimb10 daysafterundergoing surgerytocorrect adirect inguinal herniausingtheBassini---Shouldice technique.The subject pointed out that for over 40 years she presented a soft tumorofapproximately10cm,locatedinthemiddlethird ofthemedialpartoftherightthigh22cmfromtheinguinal fold. The tumor was soft, palpable and painless with a variationinvolumedependingonthepositionanddidnot causeanydiscomfort. Subsequenttothe hernioplastythe patientbegannoticingthatthetumorhardenedandcaused pain,alongwithrednessandanincreaseinlocal tempera-ture,consequently thephysiciandiagnosedacellulitisand prescribedantibiotictherapy.Thesymptomatologydidnot improvewiththeprescribedtreatmentandnowpresented anincreaseinvolumeoftheentirepelvicmember.Shewas thereforereferredtoourservice.Duringtheexaminationa differenceof5cminvolumebetweenbothpelvicmembers wasobserved,noticingahard,non-mobile,painfullumpof 9×6cm.The restof the examination wasnormal. A
vas-cular ultrasoundwas performed, showing a non-palpable, anechoic growth,connectedto thegreat saphenousvein, of9×5cm(Fig.1)without the presenceof venous reflex
neitherinthe greatsaphenousnorin thesaphenofemoral junction.Moreover,a posterior tibialveinthrombosis was detected.Withagreatsaphenousveinaneurysmdiagnosis, thepatient wastransferredtosurgery where an aneurys-mectomyandSFJligationwereperformed(Figs.2and3).
Therewerenocomplicationsduringtheprocedure,the evolutionwasgoodandthepatientwasdischargedonthe secondpostoperativedaywithacompressiveand anticoag-ulantDVTtreatmentforaperiodof3months.
Discussion
The terminology used to describe venous dilatations can cause confusion. The terms phlebectasia, varicose vein and/or venous aneurysm are considered synonyms in the medicalcommunity;however,theymeandifferentthings. Phlebectasiais defined asa fusiform and diffusely dilated vein.Theassociationofdilatedandtortuousveinsisknown as varicose veins.5 There is no precise criteria regarding
sizeandwhenavenousdilationisconsideredananeurysm; however,Mateo6 andMcDevitt7established thatwhenever
Figure 2 Surgical excision of the internal saphenous vein venousaneurisms.
thevein’sdiameteristwiceaslargeasthenormal diame-ter,thenitisconsideredtobeananeurysm(thesaphenous vein’snormalsizeat thesaphenofemoraljointis3---5mm, 2---4mmatthethighand1---3mmattheankle).8
.
Neverthe-less,inordertoconsideritaprimaryvenousaneurysmsize is notthe onlyfactor considered. Itmust alsobe a local-izeddilation,conformedbythreehistologicallayerswhich constitutethenormalvenouswall.Thiscouldbesaccularor
fusiform (animportant distinctionbecauseofits hemody-namicimplicationsthatinfluencethecourseoftreatment) andshouldonlycommunicatetothecorrespondingveinina proximalanddistalmannerandneitherbesecondarytoan arteriovenousfistula,9norberelatedtoavaricosevein.10
Hilschercoined the term primaryvenous aneurysm, mak-ingacomparisontoarterialaneurysms.Abbottwastheone whointegratedthecriteriatodefineVAs.11Ourpatientmet
therequired criteriatoclassifyhervenous pathologyasa primaryvenousaneurysm.
PrimaryVAsoccurintheheadandneck,thoracicand vis-ceralveinsaswellasveinsofthe extremities.Aneurysms of the deep venous system are characteristic on the extremities,whereagreaterincidenceofthromboembolic complicationsisreported.
Theincidenceofsuperficialvenousaneurysmsis approx-imately0.1%,12withaprevalenceof1.5%,13equallyinboth
sexesanditmayoccuratanyage.
Thepathogenesisofvenousaneurysmsisunknown; sev-eralmechanismshavebeenimplicatedinitsformation,such ashemodynamicchanges,arteriovenousfistulae, inflamma-toryprocesses,infections,trauma,congenitalweaknessof the venous wall and degenerative changes.5,14 The most
accepted theory states that it is a result of the loss of connective tissue components of the venous wall, which can be caused by a congenital defect or secondary to a degenerative process due toaging.15,16 In a recent study,
tissue from the wall of venous aneurysms was examined anditwasreportedthatstructuralchangesofthe aneurys-matic wall can be related tothe increased expression of metalloproteinase,17whichcanbetranslatedtoareduction
ofthemusclelayer,fragmentedelastictissue,anincrease inthefibroustissueandinfiltration ofinflammatorycells. SchatzandFine,18believedthatedophlebohypertophywas
a majorfactor in the formation of VA, which begins with anincreaseinvenousflowleadingtoahypertrophyofthe venouswall, thendilation andsclerosis.Pascarellaetal.4
informedthatsuperficialVAsonlowerlimbsweregenerally locateddistaltoanincompetentvenousvalve,whichcauses venousreflex,hittingthevenouswallandproducinga tur-bulentflow,causing structuralchanges onthe wallof the spleen,whilethoseindeepveinsaresecondarytointrinsic changesofthevenouswall.
The difference among findings in the reports suggests thattheetiologyofVAsisdiverseanddependsontheir loca-tionandnaturalhistory.16,19---22Inourcase,theetiologymay
come asaresultof acongenitaldefect in theconnective tissuestructure,whichgeneratedaweakvenouswall sus-ceptibletodilation,hencecontributingtotheonsetofthe inguinalhernia.
Accordingtotheetiopathogeny,VAscanbedividedinto primary (congenital) or acquired. Based on the Hamburg classificationfor congenital vascular malformations,23 VAs
correspondtoavenousmalformationofthetronculartype, which can be presented as aplasia, hyperplasia, stenosis (i.e.the leftiliac veinin May-Turnersyndrome),dilations oraneurysms(themostcommonbeingthepoplitealvein).24
CongenitalVAsarelesscommonbutcanoccurinanyvein ofthebody.Inastudy,Guillespieetal.,2reportedthat77%
of VAs were located in the lower extremities(57% in the deepvenoussystem),10%intheupperextremitiesand13% intheneck.
The clinical presentation of superficial VAs in lower extremitiesis that of a palpablesoft lump. It can change itssizeandlocationwiththebody’spositionortheValsalva maneuver;theycanbecompletelyasymptomaticorpainful withedema,andhavesignsandsymptomssimilartothose describedinourcase.
The diagnosis can be made by the clinical history and physical examination in most cases,but it is usually con-firmed by image studies. Within image studies, vascular ultrasoundis themethodofchoice forthestudy ofVAs.25
Intheultrasound,VAsarepresented asananechoic cystic structure,withwell-definedwalls,whichcanbesecularor fusiformandwithalowflowvolume;also,theyprovideus withinformationonvascularconnections,theexistenceof thrombosis,or if thereis an associated arteriovenous fis-tulaorany other pathologies,26 in additiontoguiding the
therapeuticapproach.TheCATscan,theMRIandthe phle-bography are studies which can be performed in case of diagnosticdoubtorwhenmoreexactinformationisrequired (size,extension, associatedlesionsand vascular origin).27
Clinicalhistoryandexaminationarethebasisfor reaching any diagnosis, but in the case of venous problems, vas-cular ultrasound evaluation is fundamental to reaching a correctdiagnosis,andthuschoosingthepropertreatment. Itis only in case of doubt,where further imagingstudies arerequired.Inthedifferentialdiagnosis,wemustconsider varicoseveins,softtissuetumors,hygromas,hemangiomas and, depending on location, inguinal hernias. Vascular aneurysmcomplicationsare:thrombophlebitis,deepvenous thrombosis, pulmonary embolism and bleeding caused by rupture.
CoagulationdisordersassociatedwithVAsare character-izedby blood stasisin thedilated vesselsandwitha low bloodflow,whichactivatesthecoagulationcascadewiththe subsequentproduction of thrombin andthe conversion of fibrinogenintofibrin.28,29Thenthefibrinolysisprocessbegins
which is reflected in the rise in fibrinogen derived prod-ucts,including D-dimer. This is the simplified description oflocated intravascularcoagulopathy whichcharacterizes coagulopathy associated with venous malformations.30,31
Newlyformed microthrombusattachtocalciumand phle-boliths are formed.32,33 These can be detected during
physicalexamination andverifiedby imagingstudies. The presenceofphlebolithsinVAscanindicatetreatmentwith anticoagulants, especially in large and extensive VAs.34
Localized intravascular coagulopathy is of utmost impor-tancebecause it is linked to the presence of local pain, thrombophlebitis,deep venous thrombosisand pulmonary embolism.
tothepresenceofsuperficialanddeepvenous thrombosis describedinourreport.
RegardingtheriskofVArupture,itrepresentssomething theoreticalsincetherearenoreportedcasesofthis compli-cation.
SuperficialVA treatment canbeconservative, endovas-cular or surgical. If the VA is not toolarge and does not causesymptomsit maybetreated conservativelythrough compressive therapy and prophylaxis in order to avoid thrombophlebitis or deep venous thrombosis. The indica-tions for surgical treatment in superficial VAs are: the presence of symptoms, the risk of thrombosis, compres-sion of nearby structures and, more commonly, esthetic problems.35 Surgical treatment36 consists of ligation and
total excision of the aneurysm. However, in some cases, endovascular methods can be used, like foam, laser or radiofrequencysclerotherapy.37 Inthecaseofpatientswho
present superficial venous thrombosisabove the knee (as inourcase)or deepvenousthrombosisatanylocation,it is important to treat with anticoagulants for 3---6 months in patients with normal thrombophilic profiles, otherwise theanticoagulantisprescribedindefinitely.Inthepresented case,thereisnodoubtthatthechosensurgicaltreatmentis adequateandthetreatmentwithanticoagulantsduetothe presenceofdeepthrombosisforaperiodofthreemonthsis acceptable.
Theresultsofthetreatment ofthesetypes of patholo-giesareexcellent aslongastheyarenotassociated with othervascular malformationsor pathologies;thereareno reportsofmortalityinsuperficialVAsurgicalinterventionin thelowerextremitiesandthemorbidityisthesameasany venoussurgicalprocedure.36
In our case, the primary care physician diagnosed the increaseinvolumeasasofttissuetumor(firstdifferential diagnosis whichshould bemade) andsince itwas asymp-tomatic,theydidnotgiveitmuchimportancedespitethe fact that the patient reported that the tumor was grow-ing progressively. As a result of the inguinal hernioplasty thepatientwasonbedrestfortwoweeks.Surgicaltrauma andprolongedbedrestareriskfactorsforthrombosis.The patientdidnotreceiveprophylaxisforthrombosis.
At first, and because the tumor looked red and was causing severe pain, it was treated as if it was cellulitis (thrombophlebitis, which is commonly confused withsoft tissueinfections).Itwasnotuntilthesizeofthepatient’s legbegantoincreasethattherewasasuspicionofvenous thrombosisandthecasewasreferredtoourservice.
Thus we conclude that it is important to: (1) perform theclinicalhistoryandathoroughphysicalexaminationin allof ourpatients, (2)we must keepin mindall possible differentialdiagnosesbeforegivingadefiniteone,(3)one mustneverforgetprophylaxis fordeepveinthrombosisin surgicalpatients,(4)notallskinrednessequalsinfection, (5)venous thrombosis must beruled out in every patient whopresentsasuddenincreaseinlegvolume,(6)vascular ultrasoundisanessentialtoolfor diagnosisand(7)venous aneurysmsdoexistandtheyarenotjustvaricoseveins.
Conflict
of
interest
Theauthorshavenoconflictsofinteresttodeclare.
Funding
Nofinancialsupportwasprovided.
References
1.OslerW.Anarterio-venousaneurysmoftheaxillaryvesselsof the30years’duration.Lancet.1913;1:1248---9.
2.Gillespie DL, Villavicencio JL, Gallagher C, et al. Presen-tation and management of venous aneurysms. J Vasc Surg. 1997;26:845---52.
3.SessaC,PerrinM,PorcuP,etal.Poplitealvenousaneurysms.A twocenterexperiencewith21casesandreviewofthe litera-ture.IntJAngiol.2000;9:164---70.
4.PascarellaL,Al-TuwaijriM,BerganJJ,etal.Lowerextremity superficialvenousaneurysms.AnnVascSurg.2005;19:69---73. 5.RodriguezHE,PearceWH.Themanagmentofvenousaneurysm.
In:GlovickiP,DalsingMC,EklofBG,editors.Handbookofvenous disorders.London:HodderArnold;2009.p.604---16.
6.MateoAM,MateoMartínezM.Aneurismasvenosos.In:Estevan SolanoJM,editor.Tratadodeaneurismas.Barcelona:Uriach; 1997.p.589---97.
7.McDevittDT,LohrJM,MartinKD,etal.Bilateralpoplitealvein aneurysms.AnnVascSurg.1993;7:282---6.
8.HamperUM,DeJong MR,ScouttLM.Valoración ecográficade lasvenas de las extremidades inferiores.Radiol Clin NAm. 2007;45:525---48.
9.Sessa C, Perrin M, Nicolini P. Anévrismes veineux. EMC-Chirurgie.2005;2:317---31.
10.CalligaroKD,AhmadS, DandoraR,et al.Venousaneurysms: surgical indications and review of the literature. Surgery. 1995;117:1---6.
11.Hilscher WM. Zur Frage der venösen Aneurysmen. Fortscher Röntgenstr.1995;82:2444---7.
12.PascarellaL,Al-TuwaijriM,BerganJJ,etal.Lowerextremity superficialvenousaneurysms.AnnVascSurg.2005;19:69---73. 13.Gillespie DL, Villavicencio JL, Gallagher C, et al.
Presen-tation and management of venous aneurysms. J Vasc Surg. 1997;26:845---52.
14.SianiA,AccroccaF,GabrielliR,etal.Anisolatedaneurysmof thethighanterolateralbranchofthesaphenousveininayoung patient.ActaPhlebol.2010;1:27---9.
15.Lev M, Saphir O. Endophlebohypertrophy and phlebosclero-sis: II. The external and common iliac veins. Am J Pathol. 1952;28:401---11.
16.FriedmanSG,KrishnasastryKV,DoscherW,etal.Primaryvenous aneurysms.Surgery.1990;108:92---5.
17.IrwinC,SynnA,KraissL,etal.Metalloproteinaseexpressionin venousaneurysms.JVascSurg.2008;48:1278---85.
18.IrwinJ,SchatzMD,GeraldFineMD.Venousaneurysms.NEngl JMed.1962;266:1310---2.
19.MatsuuraY,HigoM,YamashinaH,etal.Acasereportofvenous aneurysmoftheneckvein.JpnJSurg.1981;11:39---42. 20.GulbertMG,GreenbergLA,BrownWT,etal.Fusiformvenous
aneurysm ofthe neckin children: a reportof four cases.J PediatrSurg.1972;7:106---11.
21.Gruber HP, Amiri MA, Fraedrich G, et al. Multiple venous aneurysmsofthesaphenousvein:reportofanuncommoncase. VascSurg.1990;108:92---5.
22.Regina G, Rizzo S, ImpedovaG. Aneurysm ofexternal jugu-larvein:casereportandreviewofliterature---acasereport. Angiology.1992;108:92---5.
24.ZamboniP,CossuA,CarpaneseL,etal.Theso-calledvenous aneurysms.Phlebology.1990;5:45---50.
25.LapropoulosN,VolteasSK,GiannoukasAD,etal.Asymptomatic poplitealveinaneurysms.VascSurg.1996;30:453---8.
26.WoloskerN,ZeratiAE,NishinariK,etal.Aneurysmofsuperior mesentericvein:casereportwith5-yearfollow-upandreview oftheliterature.JVascSurg.2004;39:459---61.
27.WatanabeA, KusajimaK,AisakaN,etal.Idiopathic saccular azygosveinaneurysm.AnnThoracSurg.1998;65:1459---61. 28.HermansC,DessommeB,LambertC,etal.Venous
malforma-tionsandcoagulopathy.AnnChirPlastEsthet.2006;51:388---93. 29.RedondoP,AguadoL,MarquinaM.Angiogenicand prothrom-boticmarkersinextensiveslow-flowvascularmalformations: implicationsforantiangiogenic/antithromboticstrategies.BrJ Dermatol.2010;162:350---6.
30.DompmartinA,AcherA,ThibonP,etal.Associationoflocalized intravascular coagulopathy with venous malformations.Arch Dermatol.2008;144:873---7.
31.MazoyerE,EnjolrasO,BisdorffA,etal.Coagulationdisordersin patientswithvenousmalformationoflimbsandtrunk:astudy in118patients.ArchDermatol.2008;144:861---7.
32.Hein KD, Mulliken JB, Kozakewich HP, et al. Venous malformations of skeletal muscle. Plast Reconstr Surg. 2002;110:1625---35.
33.EnjolrasO,WassefM,MazoyerE,etal.Infantswith Kasabach-Merrittsyndromedonothave‘‘true’’hemangiomas.JPediatr. 1997;130:631---40.
34.MazoyerE,EnjolrasO,LaurianC,etal.Coagulation abnormal-ities associated withextensive venousmalformations ofthe limbs: differentiationfrom Kasabach---Merritt syndrome. Clin LabHaematol.2002;24:243---51.
35.SessaC,NicoliniP,PerrinM,etal.Managementofsymptomatic andasymptomaticpoplitealvenousaneurysms:aretrospective analysisof25patientsandreviewoftheliterature.JVascSurg. 2000;32:902---12.
36.Roh YN,DoYS,ParkKB,et al.The resultsofsurgical treat-mentfor patientswithvenousmalformations.AnnVascSurg. 2012;26:665---73.