The terrible twos.ppt -...
Transcript of The terrible twos.ppt -...
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The Terrible Twos Complications of Twinning
Paula J. Woodward, M.D.
Disclosure
Medical Editor Amirsys Content, Elsevier
Learning Objectives
Thoroughly understand how to determine chorionicity and amnionicity and their impact on pregnancy management and outcomes
Twins
Twins account for 1.1 % of pregnancies in USA but 10% of perinatal morbidity and mortality Perinatal mortality 4 to 6x singleton rate
What matters? Presence of anomalies
Growth discrepancy
Chorionicity!
Probability of 2 live births
Normal scan at 6 weeks Dichorionic 76%
Monochorionic 39%
Normal scan at 12 weeks Dichorionic 96%
Monochorionic 74% Blastocyst “buries” itself
into endometrium
Forms gestational sac
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Decidua is endometrium in pregnancy
Intradecidual sac sign - visualized 4 – 4.5 wks
Decidua is endometrium in pregnancy
Double decidual sac sign Intradecidual sac sign
Decidua is endometrium in pregnancy
Decidua basalis
Decidua capsularis
Decidua parietalis
Double decidual sac sign
DB
Decidua is endometrium in pregnancy
Decidua basalis
Decidua capsularis
Decidua parietalis
Double decidual sac sign
DC
Decidua is endometrium in pregnancy
Decidua basalis
Decidua capsularis
Decidua parietalis
Double decidual sac sign
DP
Decidua is endometrium in pregnancy
Decidua basalis
Decidua capsularis
Decidua parietalis
Double decidual sac sign
DP
DC
DB
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Decidua is endometrium in pregnancy
Double decidual sac sign
DP
DC
DB
DCDP
Chorion is from embryonic trophoblast
Chorionic sac = Gestational sac
Chorion is from embryonic trophoblast
Chorionic sac = Gestational sac Smooth chorion
Villous chorion (chorionic fronduosum)
Smooth chorion
Villous chorion (chorionic fronduosum)
Visualized at 5 – 5.5 weeks
Normal YS < 5 mm
Yolk sac
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Visualized at 5 – 5.5 weeks
Normal YS < 5 mm
Yolk sac Visualized 6 – 6.5 wks
Embryo
Diamond Ring Sign
Embryo Yolk Sac
Visualized 6 – 6.5 wks Embryo
Double Bleb Sign
7-9 Weeks
10-13 wks
Embryo becomes fetus at 10 wks
Organogenesis complete
Many anomalies identifiable
Amnion and chorion not yet fused
Decidua Parietalis Decidua Capsularis
Decidua Basalis Villous Chorion
Smooth Chorion
Amnion
Placenta
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Multiple Gestations
Types of Twinning
Dizygotic (70%) 2 eggs
Incidence increases with age, parity, maternal family history, assisted reproduction, race (African-American > Caucasian > Asian)
Monozygotic (30%) Single egg
Incidence about 1/250, independent of race, age, parity
Multiple Gestations
# of chorions equals # of placentas sharing is bad
risk for twin/twin transfusion
# of amnions equals # of separate sacs sharing is really bad
risk for cord accidents
Multiple Gestations
# of chorions equals # of placentas sharing is bad
risk for twin/twin transfusion
# of amnions equals # of separate sacs sharing is really bad
risk for cord accidents
Multiple Gestations
# of chorions equals # of placentas sharing is bad
risk for twin/twin transfusion
# of amnions equals # of separate sacs sharing is really bad
risk for cord accidents
Multiple Gestations
# of chorions equals # of placentas sharing is bad
risk for twin/twin transfusion
# of amnions equals # of separate sacs sharing is really bad
risk for cord accidents
Multiple Gestations
# of chorions equals # of placentas sharing is bad
risk for twin/twin transfusion
# of amnions equals # of separate sacs sharing is really bad
risk for cord accidents
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Dizygotic TwinsDizygotic must be dichorionic (2 placentas)
and diamniotic (2 amniotic sacs)
4.5 weeks 5.5 weeks
7 weeks 2nd trimester
Twin Peak Sign
When present 94% sensitivity for predicting dichorionicity
If absent may still dichorionic
2nd trimester
1st Trimester
2nd Trimester
ThickThin
Hmm…
No definition of “thick” and “thin” at any gestational age Monozygotic Twins
1/3 are Dichorionic/Diamniotic (30%) cleavage by day 3
2/3 are Monochorionic/Diamniotic (60-65%) cleavage day 4-8
Monochorionic /Monoamniotic (5-10%) cleavage >8 days
Conjoined twins (< 1%) cleavage >14 days
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Dichorionic Diamniotic Monochorionic Diamniotic
MonochorionicMonoamniotic
Monochorionic Diamniotic
5.5 weeks
Generally, number of yolk sacs = number of amnions
Generally, number of yolk sacs = number of amnions
5.5 weeks 7.5 weeks
Monochorionic Diamniotic Monochorionic Monoamniotic
Monochorionic Monoamniotic Monochorionic Monoamniotic
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Monochorionic Monoamniotic
2013 literature review on outcome of 228 fetuses (i.e. 114 pairs) with cord entanglement Overall survival 88.6%
Perinatal mortality 11.4%
“Cord entanglement is a minor complication of monoamniotictwin pregnancies“
Prematurity and congenital anomalies have far more significant impact on outcomes
Rossi AC et al: Impact of cord entanglement on perinatal outcome of monoamniotic twins: a systematic review of the literature. Ultrasound Obstet Gynecol. 41(2):131-5, 2013
Monochorionic Monoamniotic
Conjoined Twins
Difficult diagnosis in first trimesterMust follow all monochorionic monoamniotic twins closely
Conjoined Twins
Difficult diagnosis in first trimesterMust follow all monochorionic monoamniotic twins closely
trichorionic, triamniotic dichorionic, triamniotic
monochorionic, triamnioticdichorionic, diamniotic
A bad day in ultrasound
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Monochorionic Issues
Twin-twin transfusion syndrome (TTTS)
Twin anemia polycythemia sequence (TAPS)
Twin reverse arterial perfusion (TRAP)
Fetus-in-fetu
Twin demise
Twin-Twin Transfusion Syndrome (TTTS)
Placental anastomosis result in artery-to-vein shunting
Donor (pump) twin Sending blood to co-twin instead
of to placenta
Less blood to placenta less coming back
Oligemia decreased renal perfusion oligohydramnios
Recipient twin Gets “extra” blood from co-twin
volume overload
Lots of blood to kidneys lots
of urine polyhydramnios
Twin-Twin Transfusion Syndrome (TTTS)
Not all anastomoses created equal
Present in virtually all monochorionic pregnancies
Artery-to-artery Superficial and bidirectional
Protective
Vein-to-vein Superficial and bidirectional
Artery-to-vein Deep and unidirectional
Twin-Twin Transfusion Syndrome (TTTS)
Not all anastomoses created equal
Present in virtually all monochorionic pregnancies
Artery-to-artery Superficial and bidirectional
Protective
Vein-to-vein Superficial and bidirectional
Artery-to-vein Deep and unidirectional
Twin-Twin Transfusion Syndrome (TTTS)
Fluid discordant: oli/poly Polyhydramnios: Deepest fluid
pocket > 8cm
Oligohydramnios: Deepest fluid pocket < 2cm
“Stuck” twin Severe oligohydramnios
Fetus is in fixed position
May not see membrane
EFW discordance >20%
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Twin-Twin Transfusion Syndrome (TTTS)
Fluid discordant: oli/poly Polyhydramnios: Deepest fluid
pocket > 8cm
Oligohydramnios: Deepest fluid pocket < 2cm
“Stuck” twin Severe oligohydramnios
Fetus is in fixed position
May not see membrane
EFW discordance >20%
TTTS: Quintero Staging
Stage 1: Donor bladder visible, normal Doppler
Stage 2: Donor bladder empty, normal Doppler
Stage 3: Donor bladder empty, abnormal Doppler
Stage 4: Hydrops in recipient
Stage 5: Demise of one or both
TTTS: Quintero Staging
Stage 1: Donor bladder visible, normal Doppler
Stage 2: Donor bladder empty, normal Doppler
Stage 3: Donor bladder empty, abnormal Doppler
Stage 4: Hydrops in recipient
Stage 5: Demise of one or both
TTTS: Quintero Staging
Stage 1: Donor bladder visible, normal Doppler
Stage 2: Donor bladder empty, normal Doppler
Stage 3: Donor bladder empty, abnormal Doppler
Stage 4: Hydrops in recipient
Stage 5: Demise of one or both
Tricuspid regurgitation
Cincinnati system incorporates cardiovascular profiling score DV/UV/UA Doppler
Cardiothoracic ratio
Ventricular systolic function
Atrioventricular valve regurgitation
Strongest predictor of recipient demise is echocardiographic evidence of cardiomyopathy
TTTS: Staging 20 weeks
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TTTS: Natural History
Complicates 10-20% of monochorionic pregnancies
70-90% reported mortality of untreated Donor dies first in 2/3 of cases
Morbidity common in survivors Neurologic (especially in co-twin demise)
Cardiac
TTTS: Treatment
Early delivery
Serial amnioreductions
Septostomy
Laser coagulation of shunt vessels
Laser Coagulation Corrects hemodynamic
imbalance (“dichorionizing”)
Overall survival 66%
70% recipient
60% donor
Complex protocol requiring serial scans, Doppler, echocardiography and MRI
Laser Coagulation Corrects hemodynamic
imbalance (“dichorionizing”)
Overall survival 66%
70% recipient
60% donor
Complex protocol requiring serial scans, Doppler, echocardiography and MRI
TAPS: Twin Anemia Polycythemia Sequence
Most commonly seen after laser for TTTS but can occur spontaneously Residual small artery-vein
anastomsis
No fluid discrepancy
Intertwin shunt
Hb discrepancy tracked by MCA Doppler MCA PSV > 1.5 MoM in one twin
MCA PSV < 0.8 MoM in co-twin
Treatment is supportive
Transfuse for severe anemia
Discordant Twins
Not the same as TTTS No unbalanced vascular anastomoses
> 20% difference in EFW
> 20 mm difference in AC
Ratio of AC < 0.93
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Southwest Thames Obstetric Research Collaborative (STORK)
EFW discordance of >25% is the best predictor of perinatal mortality Irrespective of chorionicity or individual fetal size
(i.e. does not have to growth restriction) Discordant growth in monochorionic twins should
always be considered pathological even if both grow appropriately
D’Antoio etl al. Weight discordance and perinatal mortality in twins Ultrasound in Obstetrics & Gynecology June 2013 Volume 41, Issue 6
Look for marginal/velamentous cordwith discordant twins
Always document placental postion and umbilical cord insertion 17 weeks 32 weeks
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Twin Reversed Arterial Perfusion (TRAP)
Placental anastomosis result in arterioarterial shunting
Recipient twin (acardiac) Perfused with co-twin’s
deoxygenated blood
Blood flow into fetus via arteries (i.e. reversed flow in umbilical cord)
Twin Reversed Arterial Perfusion (TRAP)
Recipient twin (acardiac) UA flow preferentially
into internal iliac and lower extremities
Poor or no development of heart or cranial structures
Dysmorphic edematous mass usually with recognizable torso and lower extremities
Twin Reversed Arterial Perfusion (TRAP)
Recipient twin (acardiac) UA flow preferentially
into internal iliac and lower extremities
Poor or no development of heart or cranial structures
Dysmorphic edematous mass usually with recognizable torso and lower extremities
Acardiac arterial anastomotic vessel
A
B
Arterial flow toward fetus is diagnostic
Pump twin TRAP twin
Also evaluate ductus venosus flow in donor
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Often shows rapid growth
Twin BTwin A
Head
11 weeks
17 weeks
Twin B
Twin A
25 weeks
Twin B
Twin A
Head
Twin B
25 weeks
Twin A
Head
Twin B
Risks to pump (normal) twin High output heart failure
Hydrops
Polyhydramnios – often severe
Growth restriction
Prematurity
Death 10-70% (av. 50% most series)
Neurologic, cardiovascular sequelae in survivors
Treatment Options
Aim is to preserve normal twin Expectant management
Small/ slow growth of acardiac No hydrops/ CHF in pump twin Requires very close monitoring
Interruption of cord blood flow Surgical ligation, laser
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Treatment Options Radiofrequency ablation (RFA)
RFA needle device introduced into acardiac abdomen at level of umbilical artery and vein
RFA energy applied until cessation of blood flow noted in acardiac
TWIN A: Pump twin
TWIN B: “Acardiac” RFA of reverse-perfused twin at 18 weeks
Treatment Options Radiofrequency ablation (RFA)
RFA needle device introduced into acardiac abdomen at level of umbilical artery and vein
RFA energy applied until cessation of blood flow noted in acardiac
A
B
Mono-mono twins stillborn at 24 weeks; demise of twin B (acardiac)
noted clinically at 16 weeks
Twin A Twin B
30 cm, 455 g 12 cm, 46 g
Mono-mono twins stillborn at 24 weeks; demise of twin B (acardiac)
noted clinically at 16 weeks
Fetus-in-Fetu Historically regarded as well-differentiated teratomas
Current thinking is parasitic twin Monochorionic, diamniotic twin incorporated into the body of
other twin early in development
Anastomoses between vitelline vessels
Surrounding capsule formed by amniotic membrane
Forms fluid-filled cavity
Axial skeleton surrounded by synchronous organ development around this axis
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AnteriorLeft foot with 6 toes, 3 fused
Right Foot with5 toes
Possible arm bud
3-vessel umbilical cord
Vertebral column
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Monochorionic Twin Demise
Twin “embolization” syndrome
More likely acute hypotensive episode in survivor causing hypoxic injury Brain
Kidneys
Heart
Brain Injury
November 23rd December 1st
Myocardial Injury
November 23rd December 1st
Take Home Points
The 1st person to scan the patient MUSTdetermine chorionicity and amniocity
Monochorionic pregnancies should be followed closely for complications
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References – The Terrible Twos: Complications of Twinning
Carter EB et al: The impact of chorionicity on maternal pregnancy outcomes. Am J Obstet Gynecol.
213(3):390.e1-7, 2015
Henry A et al: Pregnancy outcomes before and after institution of a specialised twins clinic: a
retrospective cohort study. BMC Pregnancy Childbirth. 15:217, 2015
Blumenfeld YJ et al: Accuracy of sonographic chorionicity classification in twin gestations. J Ultrasound
Med. 33(12):2187-92, 2014
Ratha C et al: An analysis of pregnancy outcome in dichorionic and monochorionic twins given special
antenatal and intranatal care: a four-year survey. J Obstet Gynaecol India. 64(4):255-9, 2014
Mcnamara HC et al: A review of the mechanisms and evidence for typical and atypical twinning. Am J
Obstet Gynecol. ePub, 2015
American College of Obstetricians and Gynecologists et al: ACOG Practice Bulletin No. 144: Multifetal
gestations: twin, triplet, and higher-order multifetal pregnancies. Obstet Gynecol. 123(5):1118-32, 2014
Stahr N et al: In utero and postnatal imaging findings of parasitic conjoined twins (ischiopagus
parasiticus tetrapus). Pediatr Radiol. 45(5):767-70, 2015
Burans C et al: 3-dimensional ultrasound assisted counseling for conjoined twins. J Genet Couns.
23(1):29-32, 2014
Mone F et al: Intervention versus a conservative approach in the management of TRAP sequence: a
systematic review. J Perinat Med. ePub, 2015
van Gemert MJ et al: Twin reversed arterial perfusion sequence is more common than generally
accepted. Birth Defects Res A Clin Mol Teratol. 103(7):641-3, 2015
Chaveeva P et al: Optimal method and timing of intrauterine intervention in twin reversed arterial
perfusion sequence: case study and meta-analysis. Fetal Diagn Ther. 35(4):267-79, 2014
Halling C et al: Neuro-developmental outcome of a large cohort of growth discordant twins. Eur J
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monochorionic diamniotic twin pregnancies? J Ultrasound Med. 33(9):1573-8, 2014
Johansen ML et al: Crown-rump length discordance in the first trimester: a predictor of adverse
outcome in twin pregnancies? Ultrasound Obstet Gynecol. 43(3):277-83, 2014
Rossi AC et al: Impact of cord entanglement on perinatal outcome of monoamniotic twins: a systematic
review of the literature. Ultrasound Obstet Gynecol. 41(2):131-5, 2013
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