Twin-twin transfusion syndrome | Acog TTTS

Home»Publications&Guidelines»Twin-twintransfusionsyndromeTwin-twintransfusionsyndromeDiagnosis/definition:ThediagnosisofTTTSrequires2criteria:(1)thepresenceofamonochorionicdiamniotic(MCDA)pregnancy;and(2)thepresenceofoligohydramnios(definedasamaximalverticalpocket<2cm)inonesacandpolyhydramnios(definedasamaximalverticalpocket>8cm)intheothersac.TheQuinterostagingsystem(Table1)appearstobeausefultoolfordescribingtheseverityofTTTSinastandardizedfashion.Epidemiology/Incidence:TTTScomplicatesabout8-10%ofMCDApregnancies.TheprevalenceofTTTSisapproximately1-3per10,000births.Pathophysiology:TheprimaryetiologyofTTTSisthoughttoinvolveintertwinvascularconnectionswithintheplacenta(Figure4).Animbalanceofbloodflowthroughplacentalanastemosesleadstovolumedepletioninthedonortwin,witholiguriaandoligohydramnios,andtovolumeoverloadintherecipienttwin,withpolyuriaandpolyhydramnios.Complexinteractionsoftherenin-angiotensinsysteminthetwinsalsoappeartobeinvolvedinthedevelopmentofthisdisorder.Riskfactors/associations:Thereareseveralsecond-andevenfirst-trimestersonographicfindingsthathavebeenassociatedwithTTTS(Table3).Complications:ThepresentationofTTTSishighlyvariableandoftendoesnotprogressinapredictablemanner.SingletwinsurvivalratesinTTTSvarywidelybetween15-70%,dependingongestationalageatdiagnosisandseverityofdisease.Twothirdsofrecipienttwinsshowdiastolicdysfunction,asindicatedbyaprolongedventricularisovolumetricrelaxationtime,whichisassociatedwithanincreasedriskoffetaldeath.Scoringsystemsthatincludecardiacdysfunctionhavebeendeveloped,buttheirusefulnesstopredictoutcomeinTTTSremainscontroversial.Thenaturalhistoryofadvanced(eg,stage>III)TTTSisbleak,withareportedperinatallossrateof70-100%,particularlywhenitpresents<26weeks.Evenlaser-treatedTTTSisassociatedwithaperinatalmortalityrateof30-50%(Table7),anda5-20%chanceoflong-termneurologichandicap(Table8).Althoughtheriskofmembranerupturemaybeaslowas10%inexperiencedcenters,thereremainsa10-30%procedure-associatedfetallosswithlaser.Management:(Figure10)S


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