Introduction • Managing twins with ultrasound Ultrasound plays an important role in the management of twin pregnancies. Ana Monteagudo, MD Outline • • • • Assessing chorionicity & amnionicity Establishing gestational age Confirming viability R/o congenital anomalies – Management of complications/anomalies unique to multifetal pregnancies. • Antenatal surveillance Essential Embryology • The major difference between the dizygotic and monozygotic twins involves the placenta. • All dizygotic twins have – dichorionic placentas Chorionicity & Amnionicity Spontaneous Twins • ~80% are dizygotic and 20% are monozygotic. After ART • Most twins are dizygotic • Increased rate of monozygotic twin (~ 2.5 x) Dizygotic Dichorionic (100%) • But monozygotic twins may have either – dichorionic or monochorionic placentas. Diamniotic (100%) 1 4 to 6 weeks 6 to 7 weeks • Count gestational or chorionic sacs • Count the embryos in the sacs Number of embryos = Fetal number Number Chorionic sacs = Chorionicity 1 sac 6 to 7 weeks 11 weeks • Count the number of yolk sacs Number yolk sacs = number of amnions 12 weeks DiDi Twins 11 wks 2 Monozygotic Twins 1/3 Dichorionic Diamniotic (~ 99%) 4 to 7 weeks Monochorionic Twins Placentation 2/3 Monochorionic Monoamniotic (~ 1%) Monochorionic-Diamniotic • Single sac with two embryos in the sac Number of Yolk Sacs Can Predict Diamniotic Twins »Benirschke K. NY St J Med 1961;61:1499 4 to 7 weeks • Single sac with 2 embryos and 2 yolk sacs 8 weeks ...“the sonographic identification of two yolk sacs in monochorionic twins enables us to make the diagnosis of diamniotic twins early in the first trimester, before the amniotic membrane can be imaged” Bromley B and Benacerraf BR . J Ultrasound Med. 1995 Jun;14(6):415-9 3 Monochorionic-Diamniotic 9 weeks 8 weeks Monochorionic-Monoamniotic 11 weeks • Single sac with 2 embryos and one yolk sac 11 weeks Monochorionic-Monoamiotic 4 Are all twins that have a single placental mass monochorionic? Of Course NOT!! • Correct 65 out 67 cases or 97% Twin Peak Sign vs. T-Sign Twin Peak Sign vs. T-Sign • In MoDi pregnancies a single placental mass is seen • In dichorionic fused placentas – placental tissue may extend into the base of the intertwin membrane – the thin amniotic membranes are seen apposing each other • Seen easily during the 1st trimester • Seen easily during the 1st trimester Diagnosis of Chorionicity & Amnionicity Second Trimester 100% • 70 patients with histologic correlation 5 2nd and 3rd trimesters ultrasonographic evaluation chorionicity & amnionicity Opposite Sex “λ” Diamniotic Sexing Membrane Origin Placental Site Separate Separate >> 2mm 2mm 4 Same Sex Membrane Thickness Membrane layers (#) Versus “T” Versus Versus Monoamniotic Diamniotic << 2mm 2mm 2 Monochorionic Monteagudo Monteagudo A, A, Timor-Tritsch Timor-Tritsch IE.. IE.. JJ Reprod Reprod Med Med 2000;45:476-80. 2000;45:476-80. See it less well since here the membrane is parallel to the US beam Axial resolution: the minimum reflector separation along the sound beam, so separate reflections are produced (In simple terms: two point discrimination) Fused Fused No No Membrane Membrane Dichorionic Axial and lateral resolution Why don’t you see the membrane uniformly well over it’s entire length? See it BETTER since here the membrane is at a RIGHT ANGLE to the US beam Axial and lateral resolution • Both can be increased by increasing the frequency (among others) • The axial resolution of transducers is always better than the lateral resolution Lateral resolution: The minimum reflector separation in the direction perpendicular to the sound beam producing separate reflections when the sound beam scans across them The membranes: Di/Di twins Ch Am 1 Horizontal orientation of membranes Ch Am Single Twin Death • In the first trimester – ‘Vanishing twin’ may occur in 5-10% of twins – In IVF associated with LBW and SGA • Single twin demise after 1st trimester – Less common – The odds of IUFD and neurological impairment is higher in monochorionic vs. dichorionic twins 6 IVF Pregnancies Vanishing Twin & SGA Fetal Loss • Monochorionic twins have higher loss rates than dichorionic twins. Dichorionic Monochorionic At least one fetal loss < 24 wks > 24 wks 2.50% 2.80% 12.70% 4.90% Sebire NJ et al BJOG 1997;104:1203 IVF Pregnancies Vanishing Twin & SGA & LBW • ~6% of all singleton deliveries after IVF/ICSI originated from a vanishing twin pregnancy • A higher risk for LBW and being SGA for survivors • The survivors vs. control: – LBW 26.1% vs. 12.0% – SGA 32.6% vs. 16.3% • In 10% of live born IVF singletons • IVF singletons from vanishing twin gestations have a higher risk of being SGA than singletons from a single gestation. • The higher the gestational age ( > 22 wks) at the time of vanishing, the higher the risk that the surviving infant is being SGA. Pinborg A et al Vanishing twins: a predictor of small-for-gestational age in IVF singletons. Human Reproduction Vol.22, No.10 pp. 2707–2714, 2007 Prognosis for the co-twin following single-twin death • Systematic review - 28 articles • The risk of greater for monochorionic vs. dichorionic co-twin Demise Dichorionic 12% 4% 18% 1% 68% 57% (95% CI 7– 7–11) Neurological abnormality Preterm delivery Shebl O et al. Birth weight is lower for survivors of the vanishing twin syndrome: a case-control study. Fertil Steril 2008;90:310–4. Monochorionic (95% CI 2– 2–7) (95% CI 11– 11–26) (95% CI 0– 0–7) (95% CI 56– 56–78) (95% CI 34– 34–77) Ong SSC et al. Prognosis for the co-twin following single-twin death: a systematic review.BJOG 2006;113:992–998. Placentation and Mortality Monochorionic Twins Distribution of placentation Di = 62% Mo = 38% Mortality with placentation Mo = 76% 2% • Higher antenatal complications and mortality vs. dichorionic twins Di = 24% 11% 27% 36% 13% 44% 32% 35% Di Di fused Di Di separate Mo Di Mo Mo From Benirschke K Ultrasound and Multifetal Pregnancy, 1998 7 Perinatal Mortality • In monochorionic twins PM is ~ 3-4 x higher than in dichorionic twins Derom R et al. Eur J Obstet Gynecol 1991;41:25 » Machin G et al Am J Genet 1995; 55:71 • Risk of in utero death for Mo/Di twins is 4X than for Di/Di. Preterm Delivery 24 - 32 weeks • Singleton pregnancy: 1-2% • Twins* – Monochorionic:9.2% Monochorionic:9.2% – Dichorionic: 5.5% 14.7% • Median gestational age at delivery* • Even among “apparently normal twins” in utero survival was lower for Mo/Di vs. Di/Di – Monochorionic: 36 weeks – Dichorionic: 37 weeks *Sebire NJ et al BJOG 1997;104:1203 Growth Restriction • Twins – Risk of delivering growth restricted baby is ~ 10X greater than in singletons*. • Chorionicity** Mo Di One Fetus Monochorionic 34% Dichorionic 23% Both 7.50% 1.70% *Luke B, Keith LG. J Reprod Med 1992;37:661 **Sebire NJ et al BJOG 1997;104:1203 8 Selective Intrauterine Growth Restriction (sIUGR) Selective Intrauterine Growth Restriction (sIUGR) • Definition: One twin’s EFW is less than 10th or 5th percentile for the gestational age while the other has normal growth. • Associated with increased risk of fetal death for one or both twins • Reported to occur in 12-25% of all MC twins • Incidence of structural heart defects in the general population is 8:1000 • In MC twins the incidence is increased – Overall risk 9.1% – MC/DA 7% – MC/MA 57.1% • Selective IUGR and monochorionicity increases the risk of fetal loss • Conclusions: The increased rate of CHD is independent of TTS Twin-Twin Transfusion Syndrome • Systematic literature review – 9-fold increase in CHD – If complicated by TTS 13 to 14-fold increase compared to the general pop. – Most common: VSD & pulmonary stenosis • ?? Fetal echocardiography for all MC/DA • Complicates ~15% of monochorionic twins • There are multiple theories on the pathogenesis of this syndrome. • Regardless of the pathogenesis untreated TTS (before 28 wks) carries a mortality rate of ~ 80% for one or both twins*. *Fisk NM, Taylor MJO. The fetus(s) with twin twin transfusion syndrome. In: Harrison M et al, eds. The unborn patient. WB Saunders Co, 2000:341-55 9 Mono-Di 14 wks Nuchal Translucency Early Prediction of Severe Twin-Twin Transfusion Syndrome • In 287 monochorionic • In 132 twins the likelihood monochorionic ratio of increased NT twins at 10 – 14 at 10-14 wks for the wks increased NT subsequent in one or both development of severe fetuses results in TTS was 3.5. a 4x increased risk of TTS Sebire NJ et al UOG 1997;10:86 Sebire NJ et al UOG 2000;15:2008 Outcomes of TTTS Treatment (observational studies) • Expectant management – Survival rate of 20% to 30% – Neurological morbidity of 25% • Laser coagulation vs. amnioreduction • Serial amnioreduction – Survival rates of 37% to 60% – Neurological morbidity between 17-33% – Less overall death (48% vs. 59%) – Survival rates of 55-73% – Neurological morbidity of 4.2% – At 6 months more babies alive without neurological abnormality ( 52% vs. 31%) • Laser photocoagulation • Septostomy • Less perinatal death (26% vs. 44%) • Less neonatal death (8% vs.26%) • Amnioreduction vs. septostomy – Survival rates up to 83% – Neurological morbidity: no data – No difference in perinatal outcomes Roberts D et al. Interventions for twin-twin transfusion syndrome: a Cochrane review. UOG 2008:31:701-711 Mono/Mono twins Cord Entanglement • Present from the first trimester. • In 42 to 80% of the cases Cord entanglement at 9 weeks 4 days ??? Color enhancement 9 wks 10 Cord Entanglement EGA =154/7 wks Cord Entanglement ‘Galloping FHRs’ FHRs’ Cord Entanglement 25 6/7 wks Malformations • Prevalence of structural defects per fetus in dizygotic twins is the same as in singletons. • In monozygotic twins rate is 23 x higher • Concordance is uncommon – ~ 10% in dichorionic – ~ 20% in monochorionic pregnancies Burn J. Ciba Found Symp 1991;162:282 Baldwin VJ . Pathology of Multiple Pregnancy NY: Springer Verlag: 1994;169 Conjoined Twins Conjoined Twins 106/7 wks 11 Hearts Livers Bladders Cephalothoracopagus syncephalus Twin Reversed Arterial Perfusion Sequence (TRAP) “Acardiac Twin” • Absence of TRAP Sequence cardiac pulsations • Poor definition of the head, trunk, and upper extremities • Marked tissue edema • Deformed lower extremities • Two vessel cords • Hydramnios of the acardiac fetus TRAP Sequence TRAP Sequence 12 TRAP: Color Doppler Findings TRAP • 40 y o G1 presented for NT screen of an IVF twin gestation at US: Mono-Di twins 11 4/7 w • B: Acephalic, had cystic hygroma, no heart & 1 UA • A: NL (NT of 0.7mm) • A-to-A anastomosis • Reversed arterial flow was confirmed 3D Surface Rendering at 14 Weeks This modality provided additional sonographic evidence of TRAP sequence, further enhancing the extent of the malformations 3D multiplanar & surface rendering modes • Detection of deformed lower limbs and rudimentary upper limb that were barely visible on 2D ultrasound Mono/Mono twins Discordant for Anencephaly Twin A Antenatal Surveillance • Dichorionic Diamniotic – Serial growth scans q 4 weeks – Antenatal testing starting at • Monochorionic Diamniotic – Serial growths scans q 4 weeks – If discordance q 2 weeks – Antenatal testing from 32 weeks Entangled Cords Twin B 13 In Summary…. • Dizygotic Twins – All are Dichorionic-Diamniotic (DiDi) • Monozygotic Twins – 1/3 are Dichorionic-Diamniotic – 2/3 are Monochorionic-Diamniotic • ~1% are Monochorionic-Monoamniotic Conclusion • Routine ultrasound is an essential component of prenatal care for twin gestations. • It plays an important role in assessing not only amnionicity and chorionicity, but in diagnosing abnormalities, as well as providing fetal surveillance throughout the duration of gestation. 14