Lymphangiomas, also known as lymphatic malformations, are multi-cystic, localized malformations that involve the lymphatic and vascular ...CreateaccountLoginMainpageArticlesGettingStartedHelpRecentchangesMyPortalPageDiscussionViewformViewsourceHistoryOrbitalLymphangiomaFromEyeWikiJumpto:navigation,searchEnrollintheResidentsandFellowscontestEnrollintheInternationalOphthalmologistscontestResidentsandFellowscontestrules|InternationalOphthalmologistscontestrulesOriginalarticlecontributedby:AnneBarmettler,MD, SheelR.PatelAllcontributors:AnneBarmettler,MD, MichaelTYen,MDAssignededitor:AnneBarmettler,MDReview:AssignedstatusUptoDate byAnneBarmettler,MDonApril27,2021.addContributingEditors:addContents1DiseaseEntity1.1Definition1.2Prevalence1.3Classification1.4Pathophysiology1.5Histology2Diagnosis2.1ClinicalPresentation2.2DiagnosticProcedures2.3Differentialdiagnosis3Management3.1Observation3.2Sclerotherapy3.3Medicaltherapy3.3.1SildenafilTherapy3.3.2SirolimusTherapy3.4Surgery4ReferencesDiseaseEntityDefinitionLymphangiomas,alsoknownaslymphaticmalformations,aremulti-cystic,localizedmalformationsthatinvolvethelymphaticandvascularsystems,mostcommonlyoccurringintheheadandneckregionofpediatricpatients.PrevalenceTheprevalenceoflymphangiomashasbeenestimatedtobe1.1to5.3casesper10,000livebirths.However,theprevalenceappearstobehigherwhenthestudiesincludelive-births,terminatedpregnancies,andstill-births:then,theestimateis30casesper10,000.[1]Lymphangiomasrepresentaround4%ofallvasculartumorsandapproximately25%ofbenignpediatricvasculartumors.[2]Orbitallymphangiomashaveaprevalenceof1.1to5.3casesper10,000livebirthswithanequalincidencebetweenmalesandfemales.[1][3]ClassificationLymphangiomascanbeclassifiedinthreeways:a)bloodflowtype,b)vascularlesiontype,c)cystsizeanddepth.Thesecharacterizationsarehelpfulinpredictingwhatformsoftreatmentmaybesuccessful.ThebloodflowclassificationsystemwasdevelopedbytheInternationalOrbitalSociety(Table1).Inthisclassificationsystem,vascularmalformationsareseparatedin