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ULTRASOUND IN THE EVALUATION OF MULTIPLE GESTATION
Jude P. Crino, M.D.
Gynecology and Obstetrics
OVERVIEWUltrasound in Multiple Gestations
• Complications of multiple gestation• Zygosity and chorionicity
Gynecology and Obstetrics
• Fetal growth assessment• Conditions unique to monochorionic twins• Fetal surveillance• Intrapartum ultrasound• Recommendations for ultrasound evaluation
COMPLICATIONS OF MULTIPLE GESTATION
Gynecology and Obstetrics
MULTIPLE GESTATIONFetal / Perinatal Complications
• “Vanishing” twin
• Congenital anomalies
• Twin-twin transfusion
• Abnormal placentation
Gynecology and Obstetrics
• Prematurity
• Fetal growth restriction
• Fetal demise of 1 twin
or cord insertion
• Cord accidents
• Malpresentation
MULTIPLE GESTATION“Vanishing” and “Appearing” Twins
• “Vanishing” twin – first trimester demise– incidence ~ 20%– more common in DC twins < 8 weeks
Gynecology and Obstetrics
– first trimester bleeding common– prognosis similar to singleton
• “Appearing” twin – undercounting fetuses– up to 14% when evaluated < 6 wks– more common in MC twins, 5.0-5.4 wks
MULTIPLE GESTATIONFetal Anomalies
• Higher incidence than singletons– overall risk in twins 1.2-2x higher– dizygotic – risk per fetus same as singletons– monozygotic – 2-3x higher
Gynecology and Obstetrics
monozygotic 2 3x higher• cardiac (2.3%, 3x higher in TTTS recipients, esp.
pulmonic stenosis)• neural tube defects• facial clefts• gastrointestinal defects• abdominal wall defects
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MULTIPLE GESTATIONFetal Anomalies
• Anomalies in multiples are usually discordant– dichorionic 90%– monochorionic 80%
Gynecology and Obstetrics
• Management of discordant anomalies depends upon:– chorionicity– gestational age at diagnosis– type of defect (lethal vs nonlethal)– potential for pregnancy complications
ZYGOSITY AND CHORIONICITY
Gynecology and Obstetrics
MULTIPLE GESTATIONZygosity and Chorionicity
• Zygosity– number of zygotes (fertilized eggs)
– assessment requires invasive testing
Gynecology and Obstetrics
• Chorionicity– number of chorions (placentas)
– can be assessed sonographically
– main determinant of risk in multiples
MONOZYGOTIC TWINS
33% 65% 2%DICHORIONIC
Fetuses
Sacs
Placentas
Days
CON-JOINED
MONOAMNIOTIC
MONOCHORIONIC
0 3 8 13 15
•• All dizygotic are All dizygotic are dichorionicdichorionic
•• 2/3 of twins are dizygotic2/3 of twins are dizygotic•• 1/3 of twins are monozygotic1/3 of twins are monozygotic
MULTIPLE GESTATIONZygosity and Chorionicity
Gynecology and Obstetrics
ygygRare exception: dizygotic Rare exception: dizygotic monochorionicmonochorionic twinningtwinning
•• 1/3 monozygotic are 1/3 monozygotic are dichorionicdichorionic
•• All All monochorionicmonochorionic are monozygoticare monozygotic•• 90% of 90% of dichorionicdichorionic are dizygoticare dizygotic
MULTIPLE GESTATIONAssessment of Chorionicity
• Sac number & appearance – 6-9 weeks
– dichorionic – thick septum between sacs
• Placental number
i h di h i i
Gynecology and Obstetrics
– one – either mono or dichorionic
– two – dichorionic
• Fetal gender
– same – either mono or dichorionic
– different – dichorionic
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MULTIPLE GESTATIONAssessment of Chorionicity
6-9 weeksmonochorionic dichorionic
Gynecology and Obstetrics
MULTIPLE GESTATIONAssessment of Chorionicity
• Dividing membrane
– thickness• monochorionic – thin
( 1 4 1 0 2 0)
Gynecology and Obstetrics
(mean 1.4 mm, range 1.0-2.0)
• dichorionic – thick
(mean 2.4 mm, range 1.2-4.0)
– number of layers• two – monochorionic
• three or four – dichorionic
MULTIPLE GESTATIONAssessment of Chorionicity
• Dividing membrane
– appearance of basemonochorionic – “T” dichorionic – “twin peak” or “lambda”
10-14 weeks
Gynecology and Obstetrics
monochorionic T dichorionic twin peak or lambda
MULTIPLE GESTATIONAssessment of Chorionicity
Fused placenta
Separate placenta
Total
Frequency of lambda sign in dichorionic pregnancies
Gynecology and Obstetrics
placenta placentaN 67 34 101
10-14 weeks 100% 100% 100%16 weeks 100% 91% 97%20 weeks 93% 74% 87%
Obstet Gynecol 1999;94:450
MULTIPLE GESTATIONAssessment of Chorionicity
Gynecology and Obstetrics
Ultrasound Obstet Gynecol 2002;19:350
MULTIPLE GESTATIONAssessment of Chorionicity
Gynecology and Obstetrics
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MULTIPLE GESTATIONAssessment of Amnionicity
• Number of yolk sacs in 1st trimester does not predict monoamnionicity in MC twins
– 15% of MC/DA twins have 1 yolk sac
Vi li ti f di idi b
Gynecology and Obstetrics
• Visualization of dividing membrane
– serial scans if not visualized
• Other findings in monoamniotic twins
– umbilical cord entanglement
– proximity of placental cord insertions
MULTIPLE GESTATIONAssessment of Amnionicity
Umbilical cord entanglement
Gynecology and Obstetrics
MULTIPLE GESTATIONAssessment of Amnionicity
Proximity of placental cord insertions
Gynecology and Obstetrics
FETAL GROWTH ASSESSMENT
Gynecology and Obstetrics
MULTIPLE GESTATIONFetal Growth Assessment
• incidence of IUGR in multiples– MC risk 4x higher than DC
M i f t d t i i th
Gynecology and Obstetrics
• Main factors determining growth –genetic potential & placental function– usually the same in monochorionic
– may be different in dichorionic
• IUGR vs discordance
MULTIPLE GESTATIONFetal Growth Assessment
• Multiples and singletons grow similarly to 28-30 weeks, then multiples slow– triplet growth curve deviates from twin
t 35 k
Gynecology and Obstetrics
curve at ~ 35 weeks
• Normal growth curves affected by:– chorionicity
– fetal gender
– ethnicity
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MULTIPLE GESTATIONFetal Growth Assessment
Gynecology and Obstetrics
Obstet Gynecol Clin N Am 2005;32:39
MULTIPLE GESTATIONFetal Growth Assessment
monochorionicmonochorionic
dichorionic
dichorionicAnanth et al 1998
Gynecology and ObstetricsObstet Gynecol 1998;91:917
Ananth et al 1998
Naeye et al 1966
• IUGR – diagnosis– EFW below 10th percentile for ga
• Discordance – diagnosis
MULTIPLE GESTATIONIUGR and Discordance
Gynecology and Obstetrics
Discordance diagnosis– 20 mm difference in AC after 24 wks
• 83% PPV for 20% BW discordance
– 20% difference in EFW
EFW larger twin – EFW smaller twinEFW larger twin
% Discordance = 100X___________________
MULTIPLE GESTATIONCauses of Discordance
• Structural anomalies
• Chromosomal abnormalities
• Genetic syndromes
f
Gynecology and Obstetrics
• Discordant congenital infection
• Unfavorable placental implantation
• Unfavorable cord insertion site
• Placental abruption
• Complication of monochorionic placentation
• Greater morbidity than dichorionic– overall fetal survival 78%
• Distinguish from TTTS by normal AFV in sac of appropriately grown fetus
MULTIPLE GESTATIONDiscordance in MC Fetuses
Gynecology and Obstetrics
pp p y g• Principal factors influencing MC growth:
– degree of placental sharing– implantation of each placental portion– angioarchitexture
• anastomoses• placental cord insertions
MULTIPLE GESTATIONManagement of IUGR & Discordance
• Serial sonograms for growth & AFV• Fetal surveillance
NST BPP modified BPP Dopplers
Gynecology and Obstetrics
– NST, BPP, modified BPP, Dopplers
• Steroids for fetal lung maturity• Timing of delivery depends upon:
– chorionicity– gestational age– fetal surveillance results
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MULTIPLE GESTATIONManagement of IUGR & Discordance
• Monochorionic more problematic
– Dopplers may have lower prognostic value
– IUFD of 1 twin may cause twin
Gynecology and Obstetrics
“embolization” syndrome
• selective feticide by cord occlusion may be appropriate for imminent IUFD
– selective photocoagulation of vascular anastomoses under investigation
CONDITIONS UNIQUE TO MONOCHORIONIC TWINS
Gynecology and Obstetrics
MULTIPLE GESTATIONConditions Unique to MC Twins
• Twin “embolization” syndrome
• Twin-twin transfusion syndrome (TTTS)
Gynecology and Obstetrics
y ( )
• Twin reversed arterial perfusion (TRAP)
• Monoamniotic twins
• Conjoined twins
MULTIPLE GESTATIONTwin “Embolization” Syndrome
• Tissue necrosis or death of living fetus after IUFD of monochorionic co-fetus in 2nd or 3rd trimester
• Occurs in up to 25% of surviving co-fetuses
• Mechanism – hemodynamic shifts from live to dead
Gynecology and Obstetrics
yfetus causing acute severe hypotension in survivor (“embolization” is misnomer)
• Manifestations most common in brain (ventriculomegaly, porencephaly, cerebral atrophy, cystic encephalomalacia, microcephaly), kidneys, bowel (atresias), abdominal wall (gastroschisis)
MULTIPLE GESTATIONTwin “Embolization” Syndrome
Survivor at 18 Weeks
Gynecology and Obstetrics
MULTIPLE GESTATIONTwin “Embolization” Syndrome
Survivor at 23 Weeks
Gynecology and Obstetrics
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MULTIPLE GESTATIONTwin-Twin Transfusion Syndrome (TTTS)
• Occurs in up to 15% of monochorionic twins
• Caused by chronic imbalance in net flow of blood across placental vascular
Gynecology and Obstetrics
anastomoses
• Donor twin develops hypovolemia, oliguria and oligohydramnios
• Recipient twin develops hypervolemia, polyuria and polyhydramnios
MULTIPLE GESTATIONSonographic Predictors of TTTS
• NT in at least 1 twin at 11-14 weeks
– sensitivity 28%, PPV 33%
• Folding of dividing membrane at 15-17 weeks
– sensitivity 28%, PPV 33%
Gynecology and Obstetrics
• Absence of arterioarterial anastomoses
– detected by color flow mapping & pulsed Doppler from 12 wks, most detected 18 wks
• Velamentous cord insertion
– postnatal studies – 60% MC twins with velamentous insertion developed TTTS
Curr Opin Obstet Gynecol 2003;15:177
Natural History of MC Twins
n=83 11-14wks
donor
recipient
Gynecology and Obstetrics
donor
n=23 (28%)
15-17wks
n=12 (14%)
Sebire et al 1997
MULTIPLE GESTATIONPlacental Anastomoses
Gynecology and Obstetrics
Obstet Gynecol Clin N Am 2005;32:105
MULTIPLE GESTATIONZygosity and Chorionicity
• Zygosity– number of zygotes (fertilized eggs)
Gynecology and Obstetrics
– assessment requires invasive testing
• Chorionicity– number of chorions (placentas)
– can be assessed sonographically
– main determinant of risk in multiples
MULTIPLE GESTATIONDiagnosis and Evaluation of TTTS
• Diagnosis
– 16-26 weeks, monochorionic
– polyuric polyhydramnios in recipient
8 0 DVP b f 20 k
Gynecology and Obstetrics
• 8.0 cm DVP before 20 weeks
• 10.0 cm DVP after 20 weeks
– oliguric oligohydramnios in donor
• < 2.0 cm DVP
• Fetal condition assessed by staging
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TTTS STAGING:CURRENT SYSTEM
• Stage I:– polyhydramnios in recipient (dvp > 8 cm)– oligohydramnios in donor (dvp < 2 cm)
Gynecology and Obstetrics J Perinatol 1999;19:550
– donor bladder visible• Stage II: stage I criteria plus:
– donor bladder not visible at any point during preoperative evaluation
– umbilical artery diastolic flow present– forward flow in ductus venosus
TTTS STAGING:CURRENT SYSTEM
• Stage III: stage II criteria plus:– critically abnormal Doppler studies (at
l t )
Gynecology and ObstetricsJ Perinatol 1999;19:550
least one)• absent or reversed end-diastolic
velocity in the umbilical artery• reversed flow in the ductus venosus• pulsatile umbilical venous flow
TTTS STAGING:CURRENT SYSTEM
• Stage IV:it h d (fl id ll ti i t
Gynecology and ObstetricsJ Perinatol 1999;19:550
– ascites or hydrops (fluid collection in two or more cavities) in either fetus
• Stage V– demise of either fetus
TTTS STAGING:CURRENT SYSTEM
• Sub-staging of stages III and IV
Gynecology and Obstetrics
– atypical presentation: donor bladder visible
– Sub-staging by affected fetus
• III-donor, III-recipient, III-donor-recipient
• IV-donor (rare)
MULTIPLE GESTATIONTTTS Treatment Modalities
• Expectant management– Up to 90% mortality
• Septostomy
Gynecology and Obstetrics
• Amnioreduction
• Endoscopic laser ablation of vascular anastomoses
• Selective feticide
MULTIPLE GESTATIONTTTS Treatment Modalities
• Laser therapy is the best first line therapy for TTTS < 26 weeks
• Management after 26 weeks should
Gynecology and Obstetrics
• Management after 26 weeks should be individualized– expectant, serial amnioreduction,
laser, delivery
• Management of stage I debated
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MULTIPLE GESTATIONTwin Reversed Arterial Perfusion (TRAP)
• AKA acardiac twinning
• Extreme form of TTTS
– paired A-A and V-V placental anastomoses
lack of heart development in acardiac twin
Gynecology and Obstetrics
– lack of heart development in acardiac twin
– retrograde perfusion of acardiac twin by pump twin through anastomoses
• Incidence 1/35,000 pregnancies
– 1% of monochorionic twins
– 1/30 monochorionic triplets
MULTIPLE GESTATIONTwin Reversed Arterial Perfusion (TRAP)
Acardiac twin• Spectrum of malformations &
d i li
Pump twin• 90% structurally normal
Gynecology and Obstetrics
reduction anomalies (acephalus, anceps, acormus, amorphus)
• Single umbilical artery (66%)
• 100% mortality
• Mortality rate 35-50%
– congestive heart failure
– polyhydramnios
– preterm delivery
MULTIPLE GESTATIONPrenatal Assessment of TRAP
• Poor prognostic factors
– acardiac to pump twin weight ratio > 70%
– CHF or hydrops in pump twin
– polyhydramnios
Gynecology and Obstetrics
polyhydramnios
– umbilical artery RI difference < 0.20
– delivery < 32 weeks
• Calculation of acardiac weight:
weight (g) = (-1.66 x length) – (1.7 x length2)length = longest linear measurement (cm)
MULTIPLE GESTATIONManagement of TRAP
• Expectant management if twin weight ratio < 70% and no evidence of compromise
Gynecology and Obstetrics
• Consider interruption of vascular supply to acardiac twin if poor prognostic factors present– funicular (cord) approach
– intrafetal approach
MULTIPLE GESTATIONManagement of TRAP
Funicular Approach Intrafetal Approach
Gynecology and Obstetrics
Rodeck et al. NEJM 1998Deprest et al. AJOG 2000
MULTIPLE GESTATIONMonoamniotic Twins• 1-2% of twins• Fetal mortality rate 30-70%
– recent series lower (10-20%)– most common causes – cord
Gynecology and Obstetrics
most common causes cord accidents, acute TTTS
• Diagnosis based upon– non-visualization of dividing membrane
on serial scans– cord entanglement (> 80%)– proximity of placental cord insertions
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MULTIPLE GESTATIONMonoamniotic Twins – Management
• Patient counseling about risks– consider pregnancy termination
• Serial sonograms
Gynecology and Obstetrics
g
• Intensive fetal surveillance 26 wks
• Indomethacin to reduce AFV
• Antenatal steroids for lung maturity
• Delivery at 32-34 weeks
MULTIPLE GESTATIONConjoined Twins
• Late division of conceptus ( 13 days)
• Incidence 1/50,000 – 1/200,000
• Joined at identical anatomic points
Gynecology and Obstetrics
Joined at identical anatomic points
• Name based upon site of union
• Classification:– typical or symmetric
– atypical or asymmetric
MULTIPLE GESTATIONConjoined Twins
• Typical or symmetric – 8 types– Thoracopagus 40%
– Xipho- or omphalopagus 34%
Gynecology and Obstetrics
– Pygopagus 18%
– Ischiopagus 6%
– Craniopagus 2%
– Parapagus, rachipagus, cephalopagus
MULTIPLE GESTATIONConjoined Twins
• Atypical or asymmetric
– AKA heteropagus or parasitic
Gynecology and Obstetrics
p g p
– dependent portion (parasite) attached to or included in host (autosite)
• fetus in fetu – imperfect fetus contained completely within body of sibling
MULTIPLE GESTATIONConjoined Twins
Parasitic Twins
Gynecology and ObstetricsUltrasound Obstet Gynecol 2002;20:192
MULTIPLE GESTATIONConjoined Twins
Epigastric Heteropagus
Gynecology and Obstetrics
Ultrasound Obstet Gynecol 2001;17:534
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MULTIPLE GESTATIONConjoined Twins
• Prognosis
– 40% stillborn
– 35% liveborns die within 24 hours
60% i i l i
Gynecology and Obstetrics
– 60% survive surgical separation
• Management
– consider pregnancy termination
– prenatal imaging – 2-D & 3-D sono, MRI
– team approach for continuing pregnancies
– Cesarean delivery
INTRAPARTUM ULTRASOUND
Gynecology and Obstetrics
MULTIPLE GESTATIONIntrapartum Management
• Twins – presentation in labor
– vertex / vertex – 45%
• vaginal delivery
Gynecology and Obstetrics
– vertex / nonvertex – 35%
• external cephalic version (60% success)
• breech extraction if EFW 1500-3500 gm
– nonvertex / vertex or nonvertex – 20%
• Cesarean delivery
MULTIPLE GESTATIONIntrapartum Ultrasound
• On admission:
– presentation and lie
– estimated fetal weight
Gynecology and Obstetrics
• At delivery:
– reassess presentation of twin B
– assist with external cephalic version
– assist with breech extraction
– monitor heart rate of twin B
RECOMMENDATIONS FOR ULTRASOUND EVALUATION
Gynecology and Obstetrics
MULTIPLE GESTATIONRecommendations for Ultrasound Evaluation
(weeks) Monochorionic Dichorionic6-9 viability, number, dating, chorionicity
ExaminationGA
Gynecology and Obstetrics
11-14
15-26 q2 wks: TTTS predictors and signsq4 wks: fetal growth
q4 wks: fetal growth
15-28
nuchal translucencyviability, number, dating, chorionicity
modified from Sperling & Tabor 2001
assessment of cervical length
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MULTIPLE GESTATIONRecommendations for Ultrasound Evaluation
(weeks) Monochorionic Dichorionic18-20 screen for structural anomalies
ExaminationGA
Gynecology and Obstetrics
20-22 fetal echocardiogram26 to
delivery
Intra-partum
modified from Sperling & Tabor 2001
as indicated
serial growth scans(normal q4 wks, abnormal q2-3 wks)fetal surveillance as indicated