complications and management of monochorionic twins
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11/17/19
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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
MC twins: single placenta
• ”chorio-angio-pagus” -(placenta-vascular-conjoined)
• Angioarchitecture explains pathophysiology behind unique complications and reasoning for management
• Explains how both are affected by complications
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Diseases associated with unbalanced intertwin blood flow
• Twin-twin transfusion syndrome (TTTS)• Twin anemia polycythemia sequence (TAPS)
Diseases association with unequal partitioning of placenta
• Selective IUGR (FGR)• Can have both TTTS and sIUGR co-existing
IUGR twin
AGA twin
Unbalanced AV anastomoses and partitioning
TTTS TAPS
TTTS and TAPS
sIUGR
TTTS and sIUGR
TTTS and sIUGR TTTS and sIUGR and TAPS
Adapted from Dr Stephen Emery NAFNET
• Majority are uncomplicated
• 10 - 15% have TTTS
• 3% have spontaneous TAPS
• 15% have sIUGR– Unbalanced division of the placenta -> sIUGR
• Can have combination of unbalanced intertwin blood flow and unequal placental share
TTTS
• Accounts for about half of all deaths in MC twins• 70-100% loss rate – esp early severe disease• High neurologic morbidity in survivors (10-30%)
Lewi L AJOG 2008, Berghella V JRM 2001,van Hetern CF, Obstet Gynecol 1998
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TTTSImbalance of A-V anastomoses in one direction – donor “transfuses”
volume to recipient
Dx: twin poly-oligo ( MVP ≤ 2cm, ≥ 8cm)
Recipient: polyuria, hypervolemia, distended bladder, HTN, cardiac hypertrophy and failure, abnormal arterial and/or venous Dopplers
Donor:hypovolemia, oliguria, collapsed bladder, IUGR, abnormal umbilicalartery Dopplers
Ultrasound findings in TTTS
• 1st trimester– CRL discordance– NT > 95th %ile or discordance >20%– Reversal or absence of ductus venosus A wave
• 2nd trimester findings– Abdominal circumference discordance–Membrane folding– Velamentous placental cord insertion in donor
TTTS Quintero Staging
Quintero RA. J Perinatol 1999, Stamillo AJOG 2010, Simpson L. AJOG Jan 2013
Some centers incorporate fetal echocardiography (recipient cardiomyopathy) into staging
TTTS Management• Delivery• Expectant management– majority stage I remain stable or regress– High perinatal mortality in stage III or higher
• Serial amnioreduction• Laser photocoagulation– Superior to AR in RCT– Treatment of choice for dual survival
• Selective termination of one fetus• Pregnancy termination
Senat MV NEJM 2004
TTTS outcomes with laser therapy
• 85% chance 1 survivor• 65% chance 2 survivors• 54% chance donor demise in Stage III with
abnormal Dopplers and sIUGR• 8% chance neurologic morbidity of survivors• Mean GA delivery – 33 weeks
Senat MF NEJM 2005, Chmiat RH AJOG 2011
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NAFNet: what to do about stage I
• Multicenter retrospective observational study• 124 cases stage I TTTS• Expectant mgmt. vs. AR vs Laser• Risk factors for progression• Outcome data– Good: 2 survivors≧ 30 weeks–Mixed: Single survivor or delivery 26-29.9 weeks– Poor: Double fetal demise or delivery < 26 weeks
AJOG 2016
Column1
progress loss regress st able
60%
Progress/lost:50% poor outcome
8%
22%
10%
Regress/stable:Good outcome
Expectant management group
• Average of 11 days until change in status (regress, progress, termination, etc)
• No factors at diagnosis predictive of disease outcome• AR or laser protected against no survivors• Laser protected against poor outcome
TAPS(Twin Anemia Polycythemia Sequence)
• Large inter-twin Hb difference w/o AF discordance
• Small unidirectional unbalanced AV anastomoses near perimeter of placenta
• Incidence:– 3-6% previously uncomplicated 3rd
trimester MC/DA twins vs 13% after laser therapy
– Usually diagnosed > 26 weeks• DX: MCA-PSV dopplers:
• MCA PSV > 1.5 MoM in donor and < 0.8 MoM in recipient
TAPS Staging SystemStage Characteristics
I MCAPSV > 1.5 MOM and < 1.0 MOM, no compromise
II MCAPSV > 1.7 MOM and < 0.8 MOM, no compromise
III Stage I or II + cardiac compromise (severely abnormal dopplers)
IV Hydrops of donor
V Demise of 1 or both fetuses after diagnosis of TAPS
Stagelle f. Feta; Doagm Tjer 2010
Management and Outcomes of TAPS• Depends of timing and severity and
GA– Delivery– Expectant management– Selective termination– Intrauterine transfusion – Partial exchange transfusion– Laser
• Outcomes in spontaneous TAPS– 49 cases mc twins with spont
TAPS– 71% antenatal diagnosis
• 57% fetal therapy (IUT/PET, laser, selective feticide)
– 53% donors also had severe FGR vs 8% recipients
– Long term neurodevelopmental outcomes in 74 TAPS survivor at median of 4 years:• NDI in 44% donors and 18%
recipients• Severe NDI in 9% donors and 3%
recipients• Severe anemia and GA delivery
were independent risk factors for NDI
Tollenaar et al Ultrasound Obstet Gynecol 2019
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Monochorionic complications: sIUGR
• Disproportionate placental partitioning• Incidence: 12 - 25% MC twins• Diagnosis
– EFW < 10th %ile in smaller twin– Significant growth discordancy (≥25%)
even at EFW > 10th %ile– Increase risk adverse outcomes
• Increase in perinatal loss and adverse neurologic complications– 20% fetal demise (smaller twin)– 35% neurologic morbidity (larger twin)
Graticos E UOG 2004
Classification, Outcomes and Management of sIUGR
Type Umbilical artery dopplers
Un-expected IUFD of either
Neurologic complic.
In-uterodeterioration
Average dis-cordance
GA delivery
monitor
I 2-4% <5% rare 29% 35w Weeklydopplers
II 0 – 30% 14% 90% 38% 32w DopplersSel. TermPTD, laser
?
III 15% in smaller
twin
15-40% 11% 36% 31-32w DopplersSel term
Laser?
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
Options
• Expectant• Termination• Umbilical cord occlusion– Bipolar cord coagulation– Radiofrequency ablation (RFA)
RFA
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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
weeks
Thank you
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