11/17/19 1 Complications and management of monochorionic twins Joanne Stone, MD MS Director, Maternal Fetal Medicine Professor, Obstetrics, Gynecology and Reproductive Sciences Icahn School of Medicine at Mount Sinai • I have no financial disclosures Complications and management of monochorionic twins Joanne Stone, MD MS Director, Maternal Fetal Medicine Professor, Obstetrics, Gynecology and Reproductive Sciences Icahn School of Medicine at Mount Sinai Monochorionic complications • Fetal loss • Fetal anomalies • Twin Twin Transfusion Syndrome (TTTS) • Selective Intrauterine Growth Restriction (sIUGR) • Twin Anemia Polycythemia Syndrome (TAPS) • Twin Reversed Arterial Perfusion (TRAP) • Monoamniotic Monochorionic Twins (MA/MC, MoMo) • Conjoined twins • High-order MC multiples • Death of one twin Incidence • Rate of spontaneously-conceived monozygotic twinning is constant: 3-5/1000 deliveries • 30% of spontaneous twins are MZ • 2/3 of MZ twins are monochorionic • 20% of spontaneous twins are MC Dizyotic (2/3): Maternal age (FSH) Genetics ART Monozygotic (1/3): 0.4 – 0.45% following non- stimulated in vivo conception MZ twinning increased after ART: <1% 75% 25%
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11/17/19
1
Complications and management of monochorionic twins
Joanne Stone, MD MSDirector, Maternal Fetal Medicine
Professor, Obstetrics, Gynecology and Reproductive Sciences
Icahn School of Medicine at Mount Sinai
• I have no financial disclosures
Complications and management of monochorionic twins
Joanne Stone, MD MSDirector, Maternal Fetal Medicine
Professor, Obstetrics, Gynecology and Reproductive Sciences
Icahn School of Medicine at Mount Sinai
Monochorionic complications• Fetal loss• Fetal anomalies• Twin Twin Transfusion Syndrome (TTTS)• Selective Intrauterine Growth Restriction (sIUGR)• Twin Anemia Polycythemia Syndrome (TAPS)• Twin Reversed Arterial Perfusion (TRAP)• Monoamniotic Monochorionic Twins (MA/MC, MoMo)• Conjoined twins• High-order MC multiples• Death of one twin
Incidence
• Rate of spontaneously-conceived monozygotic twinning is constant: 3-5/1000 deliveries
• 30% of spontaneous twins are MZ• 2/3 of MZ twins are monochorionic• 20% of spontaneous twins are MC
Dizyotic(2/3):Maternal age (FSH)GeneticsART
Monozygotic (1/3):0.4 – 0.45% following non-stimulated in vivo conceptionMZ twinning increased after ART: <1%
75%
25%
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Dating a twin pregnancy
• Use known date of conception if ART used• Ideally date CRL at 11+0 – 13+6 weeks• For spontaneous twins, larger CRL should be
used
ISUOG guidelines Ultrasound Obstet gGnecol 2016
• CRL discordance ≧ 10% or NT ≧ 20% require MFM discussion, detailed US and karyotype – For MCDA: NT Found in 25% MC twins and risk
early IUD or development TTTS > 30% but poor PPV and NPV
• Establishing Chorionicity– Diagnosis best in 1st trimester
• 98% accurate– Single placenta, T sign, membrane thickness < 1.5-2
mm
*Maruotti et al Eur J Obstet Gynec and Reprod Bio 2016 , **ISUOG guidelines Ultrasound Obstet gGnecol 2016
Follow-up after diagnosing MC twins
• MFM consultation• US every 2 weeks– MVP (maximum vertical pocket) to assess amniotic
fluid– Bladder– Umbilical artery and ductus venosus dopplers as
appropriate– MCA (middle cerebral artery dopplers)– Early and routine anatomy survey– Fetal echocardiograms
Gratacos Ultrasound Obstet and Gynecol 2007, Ishti et al Fetal Diag 2009, Valsky et al Sem Fetal and Neon Med 2010, Johnson, A personal communication
Normaldoppler
PersistentAEDF/REDF
intermittentAEDF/REDF
Intra-uterine demise
Neonatal death
Intact survival
Type I sFGR expectant 3.1% 97.9%
Type I sFGR laser 16.7%
Type I sFGR selective red 0% 100%
Type II sFGR expectant 16.6% 6.4% 89.3%
Type II sFGR laser 44.3% 100%
Type II sFGR selective red 5% 3.7% 90.6%
Type III sFGR expectant 13.2% 6.8% 61.9%
Type III sFGR laser 32.9% 100%
Type III sFGR selective red 0% 5.2% 98.8%
Exp mgmt. best
Laser or SR may be better at previableGA in severe cases to protect survivingtwin from demise or neurologicimpairment
Townsend et al Ultasound Obstet Gynecol 2019
TRAP(Twin Reversed Arterial Perfusion)
Incidence: rare – 1/35,000 deliveriesDue to patent vascular anastomosesDiagnosis: Doppler ultrasound of Acardiac fetus’ umbilical cord shows arterial blood flowing toward the acardiac twin
• Early loss of 1 of a mc twin pair with patent anastomoses perfusing other?
• Twin with absent heart (acardiac) is perfused by co-twin (pump)
• Deoxygenated blood from pump twin leads to variable growth of acardiactwin
• Acardiac twin – high-flow, low resistance vascular bed
• Pump twin at risk of cardiac decompensation and demise (50%)
Management• Sonographic markers for poor
prognosis– Ratio of acardiac twin to pump twin
• L x W x H X 0.52 (formula for volume of a sphere) of acardiac/wt pump > 50%
– Polyhydramnios– Pump twin with cardiac failure with
abnormal dopplers– Increase in size of pump twin (AC of
acardiac/pump >1.0)• Expectant
• 30% loss rate between 1st trimester diagnosis and 2nd trimester intervention
• Early intervention– Occlusion of vascular connections
(RFA, laser)
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Monoamniotic (MA) twins
• 1 in 10,000 pregnancies
• Greater number of superficial and deep
anastomoses = ?protective
• Risks:
– Cord entanglement
– Congenital anomalies
– PTD
– TTTS (rare)
• Perinatal Mortality
§ Past - PNM 30-70%
§ Recently - PNM 10-20%
§ 4% > 24 weeks if no structural anomalies,
TTTS, TRAP
• Inpatient management?
• Delivery 32-34 weeks – C/S
• Mode of delivery = cesarean
Ultra Obstet Gyn 2000;16(3):223, Acta Obstet Gyn Scand 2005;84(5):432, ltra Obstet Gyn 2006;28:681 Prefumo et al Pren Dx 2015
• Multinational cohort study 2010-2017• Non-anomalous uncomplicated MO/MO twins with 2 live
fetus at 26 weeks included• 10 centers inpatient, 12 centers outpatient• Primary outcome IUFD• 195 women (290 fetuses)• Results
– Overall perinatal loss rate 10.8%– 4 women (5/3%) inpt and 15 women (12.5%) outpt IUFD– Peak fetal death rate 4.3% occurring at 29 weeks– From 32 – 36 +6 weeks no fetal/neonatal deaths– No difference in in-patient or out-patient groups
Conjoined twins
• Very rare: 10.2/million births
• 18% prenatally-diagnosed fetuses survive
• Increase rate of structuralanomalies
• Outcomes depend on which organs are shared
Discordant anomalies• Structural anomalies more common in MC twins
(6-8%)• Only 20% are concordant for anomaly• Monozygotic twins are NOT identical– Post-zygotic mutation– Variations in gene expression– Asymmetric x-chromosome inactivation– Parental imprinting– Discordant gene methylation– Vascular accidents
RFA: technique• IR suite• IV sedation• US guidance• Skin prep• Bilateral grounding pads• Local anesthesia• Skin/fascia incised with 11 blade
scalpel• LeVeen needle inserted into fetal
abdomen just at/above cord insertion
• Prongs deployed• 60W energy delivered for about
60seconds; power increased by 20W in 60 second intervals to 120W or when impedance dropped
• Cessation of vascular flow within cord confirmed
• Pt observed post-op for several hours
RFA outcomes – for various etiologies
• About 15% PPROM (up to 25%)• Miscarriage survivor about 5%• Neurologic morbidity survivor about 5%• Live birth rate about 80%• Mean GA delivery 33-36 weeks
Kumar et al AJOG 2014, Lee et al Fetal Diagn Ther
Death of one twin
• Bleeding of surviving twin into demise twin– Hypotension, hypovolemia, anemia, hypoxia, acidosis– 15% risk demise of co-twin– 25-35% risk severe neurologic morbidity in survivor
• Management– Immediate deliver after unwitnessed twin death – no
benefit– Expectant management– Fetal brain MRI’s of survivior
Demise of co-twin
• Retrospective observational study at UCSF• 21 MC twins (none had laser/RFA)• Mean GA demise: 19 6/7 w (12 4/7 – 26 6/7)• Interval to MRI: 4 3/7 w (0-12 1/7)• 41% associated with TTTS• Abnormal findings in 7 cases (33%)• Majority had normal ultrasound
Jelin et al AJOG 2008
conclusions
• Establish chorionicity early• Every 1-2 week surveillance• Anatomy surveys and echocardiography• Deliver uncomplicated MC twins around 36