sFGR - MCDA sFGR - DCDA TTTS - MCDA TAPS - MCDA TRAP - … · 2019. 6. 3. · Dear Asma, Thank you...
Transcript of sFGR - MCDA sFGR - DCDA TTTS - MCDA TAPS - MCDA TRAP - … · 2019. 6. 3. · Dear Asma, Thank you...
Asma KhalilSt George’s Hospital, University of London, UK
sFGR - MCDA sFGR - DCDA TTTS - MCDA TAPS - MCDA TRAP - MCDA
Complications in Twins
How should we monitor, diagnose and manage TTTS?
Complications in twins
Complications in twins
Polyhydramnios oligohydramnios
Deepest vertical point:16-20 weeks: >8cm AND <2cm>20 weeks: >10cm AND <2cm
Diagnosis
TTTS: Diagnosis
Modified Diagnostic Criteria of TTTS
• <18 weeks• Oligohydramnios (DVP ≤ 2cm)• Polyhydramnios (DVP ≥ 6cm)
• 18-20 weeks• Oligohydramnios (DVP ≤ 2cm)• Polyhydramnios (DVP ≥ 8cm)
•>20 weeks• Oligohydramnios (DVP ≤ 2cm)• Polyhydramnios (DVP ≥ 10cm)
DVP in MCDA twins
Khalil UOG 2017
I: Bladder donor visibleII: Donor empty bladderIII: Critically abnormal DopplersIV: Hydrops in one or both twinsV: IUFD of one twin
Quintero Staging
TTTS: Staging
Twin Pregnancy: TTTS
What is the treatment of choice for TTTS?
• Laser ablation is the treatment of choice for TTTS atQuintero stage ≥II.
A
• Conservative management with close surveillance orlaser ablation can be considered for Quintero stage I.
B
• When laser expertise is not available, serial
amnioreduction is an acceptable alternative after 26weeks.
A
Twin Pregnancy: TTTS
Evolution of Stage 1 TTTS: Systematic Review and Meta-Analysis
Progression 27%
Expectant
At least 1 survival 87%
Amnioreduction
86%
Overall survival
Double survival
79%
70%
77%
67%
Laser
81%
68%
54%
Khalil et al TRHG 2016
Twin Pregnancy: TTTS
North American Fetal Therapy Network: intervention vs expectant
management for stage I twin-twin transfusion syndrome
Emery et al AJOG 2016
010
20
30
40
50
60
70
80
90
100
Expectant
(n=49)
Amnioreduction
(n=30)
Laser surgery
(n=45)
Su
rviv
al (%
)
Double survival
At least one survival (p=0.02)
No survival (p=0.01)
• Retrospective multicentre cohort study
• Stage I TTTS was associated with
substantial fetal mortality
• Progression in 30%
• Both amnioreduction and laser therapy ↓
the chance of no survivors
• Laser was protective against poor
outcome independent of multiple factors
Twin Pregnancy: TTTS
What is the protocol for screening for TTTS?
• Start at 16 weeks and repeat every 2 weeks thereafter
What is the prognosis for MC twin pregnancies with
amniotic fluid discordance?
• Follow up on a weekly basis for progression to TTTS
• Good outcome (93% overall survival)
• Low risk of progression to severe TTTS (14%)
How should TTTS be followed-up and what is the optimal GA for delivery?
• Weekly ultrasound assessment for the first two weeks
after treatment, reducing to alternate weeks followingclinical evidence of resolution.
• In case of sIUD (post-laser)
• Brain imaging in 4-6 weeks
• Neurodevelopmental assessment at 2 years of age
Twin Pregnancy: TTTS
GA at delivery: 34 weeks
Stirnemann et al AJOG 2012
What to look for at the follow-up after Laser for
TTTS?
• Biometry + EFW discordance
• Amniotic fluid volume (DVP)
• Doppler (UA and MCA +/- Ductus venosus)
• Anomaly: heart, brain and limbs
Twin Pregnancy: TTTS
A case of missed TTTS resulting in single intrauterine demise
Complications in twins
• 28 years old, P1• Husband is studying medicine in the UK• Spontaneous MCDA twin pregnancy • First scan at 12 weeks: no concern• Next scan booked at 20 weeks
• 20+4 weeks• Twin 1: sIUD, oligohydramnios• Twin 2: alive, polyhydramnios
• Urgent referral to SGH:• Twin 1 (demised) has oligohydramnios• Twin 2 had polyhydramnios + severe
anaemia • Planned for urgent IUT• Fetal brain MRI in 5 weeks
• Follow-up scans: no concern• Fetal brain MRI at 26 weeks
irregularity at the superior aspect of the bodies of the lateral ventricles, representing focal subependymal cystic changes. This could be due to infection or focal white matter injury (due to hypoxia either arterial or venous). Fissuration is lagging 1-2 weeks. We could organize a follow up scan to monitor the changes.
• Follow-up scans: no concern• Repeat Fetal brain MRI at 30 weeks
The repeat fetal brain MRI has confirmed evidence of periventricular white matter injury, which might be associated with neurological disability. However the brain has matured since the previous MRI, with normal sulcation for gestational age.
The parents remain committed to this pregnancy and will not consider the option of termination of the pregnancy. In fact, they are moving to Canada in approximately 10 days. We would be happy to be contacted if there is any concern.
Dear Asma,
Thank you for all the help and support you gave us to give this baby a chance to be with us today. we truly appreciate. like i promised to inform you, the baby is here. I was induced on the 28th of October, was already 2cm dilated as at the time i got to the hospital. The induction started at 11am and he arrived 7.31pm at 7 pounds 4.4ounces. He was checked by the piediatrician and they feel he is meeting all milestones so far but we keep an eye on him. But i truly beleive all is fine with him.
I will bring him in to visit you once we return to London. we are still in the hospital treating Jaundicr with photo therapy and he is getting better. I have attached his picture to this email. Tank you so much once again.
Kind regards,Ndidiamaka
11-14 week• Dating, labelling
• Chorionicity
• Screening for trisomy 21
20-22 week
• Detailed anatomy
• Biometry
• Amniotic fluid volume
• Cervical length
24-26 week
28-30 week
• Assessment of fetal growth
• Amniotic fluid volume
• Fetal Doppler
36-37 week
Delivery
32-34 week
• Assessment of fetal growth
• Amniotic fluid volume
• Fetal Doppler
• Assessment of fetal growth
• Amniotic fluid volume
• Fetal Doppler
• Assessment of fetal growth
• Amniotic fluid volume
• Fetal Doppler
Dichorionic Twin Pregnancy Monochorionic Twin Pregnancy
11-14 week• Dating, labelling
• Chorionicity
• Screening for trisomy 21
20 week
• Detailed anatomy
• Biometry, DVP
• UA PI, MCA PSV
• Cervical length
28 week
30 week
34 week
32 week
16 week• Fetal growth, DVP
• UA PI
18 week• Fetal growth, DVP
• UA PI
• Fetal growth, DVP
• UA PI, MCA PSV
• Fetal growth, DVP
• UA PI, MCA PSV
• Fetal growth, DVP
• UA PI, MCA PSV
• Fetal growth, DVP
• UA PI, MCA PSV
22 week
24 week
26 week• Fetal growth, DVP
• UA PI, MCA PSV
• Fetal growth, DVP
• UA PI, MCA PSV
• Fetal growth, DVP
• UA PI, MCA PSV
36 week• Fetal growth, DVP
• UA PI, MCA PSV
Twin Pregnancy: ultrasound monitoring
Twin Pregnancy: Single fetal death
MC
PN brain abn
Neurological abn
34%
26%
DC
16%
2%
Death of co-twin
Preterm delivery
15%
68%
3%
54%
Senat MV et al. AJOG 2003
Hillman SC et al. Obstet Gynecol.2011
Hillman SC et al. Semin Fetal Neonatal Med 2010
sIUD in MC twin pregnancy
Referral to Fetal Medicine Centre:
• Detailed scan
• Umbilical, MCA PSV, DV Doppler
• Counselling (15% IUD, 25% neurological
morbidity vs 2% in DC)
Fetal biometry + Dopplers /2weeks
Fetal brain MRI 4-6 weeks after sIUD
Delivery at 34-36 wk after steroids
• TOP if abnormal
• PM (fetus + placenta)
Death of one fetus
Twin Pregnancy: Single fetal death
How should we monitor, diagnose and manage TAPS (Twin Anaemia Polycythaemia Sequence)?
Complications in twins
Diagnostic Criteria
TAPS
POSTNATAL
• Intertwin Hb difference >8.0 g/dl
and
• at least one of the following:
• Reticulocyte count ratio >1.7
• Placenta with only small (<1mm)
vascular anastomoses
ANTENATAL
• MCA-PSV >1.5 MoM in the donor
and
• MCA-PSV <1.0 MoM in the recipient
Incidence• Spontaneous: 3-5% MC twin pregnancies
• Post-laser: 2-13% TTTS cases
Placenta: minute (<1mm) AV anastomoses
Slaghekke et al, Fetal Diagn Therap 2010Lopriore et al, Prenat Diagn 2010
AN staging
Stage 1: Donor MCA-PSV >1.5 MoM and recipient MCA-PSV <1.0 MoM,
without other signs of fetal compromise
Stage 2: Donor MCA-PSV >1.7 MoM and recipient MCA-PSV <0.8 MoM,
without other signs of fetal compromise
Stage 3: Stage 1 or 2 and cardiac compromise in donor
(UA AREDF, UV pulsatile flow, DV increased or reversed flow)
Stage 4: Hydrops of donor
Stage 5: Death of one or both fetuses preceded by TAPS
Stage Intertwin Hb
difference (g/dL)
1 >8.0
2 >11.0
3 >14.0
4 >17.0
5 >20.0
PN staging
Slaghekke et al, Fetal Diagn Therap 2010Lopriore et al, Prenat Diagn 2010
TAPS
Management
• Expectant
• Delivery
• Intrauterine transfusion
• Selective feticide
• Fetoscopic laserLopriore et al, AJOG 2008
Herway et al, UOG 2009Slaghekke et al, Fetal Diagn Ther 2010
Lopriore et al, Placenta 2007
Genova et al, Fetal Diagn Ther 2013 Slaghekke et al, UOG 2014
Lopriore et al, Prenat Diagn 2010
TAPS
• Screening: MCA PSV should be measured in all MC twins andduring the follow-up of treated TTTS cases
• Prevention: Solomon fetoscopic laser ablation technique
TAPS
A case of post-Laser TAPS
Complications in twins
37 years old
1 previous uncomplicated singleton pregnancy
Spontaneous MCDA twin pregnancy
18 weeks
TTTS
Laser
One week post-Laser
One week post-Laser
One week post-Laser
Post-Laser TAPS
Post-Laser TAPSRepeat Laser
Follow-up scansNo recurrenceFetal brain MRI 28 weeksNormal
OutcomeLivebirth x 2
How should we monitor, diagnose and manage FGR in twins?
Complications in twins
• Estimate fetal weight discordance at each scan from 20 wk.
• Do not scan more than 28 days apart.
• Consider a ≥25% difference in size as clinically important
and refer woman to a 3ry level fetal medicine centre.
NICE 2011
The Fetal Medicine
Foundation sFGR Delphi
Diagnostic features
Solitary: EFW <3rd centile
Contributory: at least 2/3
• EFW <10th centile
DC twins MC twins
• EFW discordance ≥25%
• Umbilical PI >95th centile
Solitary: EFW <3rd centile
Contributory: at least 2/4
• EFW <10th centile
• EFW discordance ≥25%
• Umbilical PI >95th centile
• AC <10th centile
NEW
Khalil et al UOG 2018
What is the management of sFGR?• DC twins: sFGR can be followed as in FGR singletons
• MC twins: limited evidence to guide themanagement
Twin Pregnancy: sFGR
sFGR - MCDA sFGR - DCDAOptions include:
• Conservative management
followed by early delivery
• Laser ablation
• Cord occlusion
What is the follow-up protocol for sFGR?
• In DC sFGR fetal Dopplers should be assessed every two
weeks depending on the severity. In MC sFGR
pregnancies fetal Dopplers should be assessed at least
weekly.
Twin Pregnancy: sFGR
How should sFGR in MC twins be classified?• depends on the pattern of the end-diastolic velocity
in the umbilical artery Doppler.
Type 1 Type 2 Type 3
When should we deliver twins with sFGR?
• In DC sFGR: avoid delivery <30-32 weeks
• In MC sFGR:
If there is a substantial risk of fetal demise of the smaller
twin (e.g. reversed a-wave in DV)
• >26 weeks: consider delivery
• <26 weeks: consider selective termination
D
Delivery
• sFGR type 1: 34-36 weeks
• sFGR type 2 and 3: 32 weeks or earlier if deterioration
Twin Pregnancy: sFGR
How should we manage discordant anomaly in twins?
Complications in twins
Discordant anomaly in twins
How common are structural or genetic anomaly in twins?6% in total birth vs 3% newborn
Most of the anomalies affect only one twin (>80%)
Do anomalies in twins differ according to chorionicity?• 1% DC vs 4% MC twins• MC twins 4 x singletons
The Fetal Medicine
Foundation Congenital anomalies in twins
What is the detection rate of anomalies in twins in the 1st trimester? 27%
What are the risk factors? • Monochorionicty• CRL discordance • NT discordance
Discordant anomaly in twins
Which anomalies affect twins?• Heart and CNS (Brain and NTD)• Midline anomalies in MC twins
The Fetal Medicine
FoundationCongenital anomalies unique to twins
Midline structural defects (twinning process) Conjoined twins
The Fetal Medicine
Foundation Congenital anomalies unique to twins
• Brain abnormalities • PVL• Ventriculoemegaly• Cerebral atrophy
• Bowel atresia• Renal dysplasia• Limb amputation
Malformations resulting from vascular event (hypotension and/or ischaemia with vascular anastomoses)
The Fetal Medicine
Foundation
Management of Discordant Anomaly
Expectant TOP entire pregnancy
Counselling• Risk of intrauterine death• Risk of miscarriage• Risk of preterm birth
Discordant Anomaly
Selective TOP
The Fetal Medicine
Foundation Discordant Anomaly
What should we consider when we see a twin pregnancy with discordant anomaly?• Chorionicity• Amnionicity• Type of anomaly• Gestational age• Parents’ wishes• Technical and legal matters
Twin Pregnancy: Discordant anomaly
How should twin pregnancies discordant for fetal anomaly
be managed?
• Twin pregnancies discordant for fetal anomaly should
be referred to a regional fetal medicine center.
• lethal abnormality with a high risk of
intrauterine demise:
• DC twins: conservative management
• MC twin: selective termination to
protect the healthy cotwin against
the adverse effects of spontaneous
demise.
Twin Pregnancy: Discordant anomaly
Selective Feticide in Twin Pregnancies
• Dichorionic: intracardiac or intrafunicular injection of
KCl or lignocaine, preferably in the first trimester.B
• When the diagnosis is made in the second trimester,
women might opt for late selective termination in the
third trimester, if the law permits.
MC pregnancies: cord occlusion, intrafetal coagulation (laser or
radiofrequency ablation)
• Survival >80%
• Premature rupture of the membranes and PTB <32 weeks 20%
• Adverse neurological sequelae
Khalil et al UOG 2016
Trisomy 21 – Meta-analysis
NIPT in Twin Pregnancies
Lau et al 2013; Huang et al 2014; Benachi et al 2015; Sarno 2016; Tan 2016; Gil et al UOG 2017
all12
Total DR (%)
Lau et al 2013 100
Huang et al 2014 9 100
FPR (%)
0
1 0
all12Benachi et al 2015 100
Sarno et al 2016 8 100
Tan et al 2016 4 100
0
0
2 0
Pooled analysis 24 100 0
Non-Trisomy 21
Study
12
Total
180
10
12
409
506
5
1110
Trisomy 21
Trisomy 18/13
NIPT in Twin Pregnancies
Detection Rate False Positive Rate
67% (2/3) 0% (0/254)
Sarno et al UOG 2016
Study
Sarno et al 2016
63% (5/8) 0.15% (1/658)Published studies
Singletons
Twins
Log fetal fraction
Fre
qu
en
cy
Fre
qu
en
cy
Median Fetal fraction
11% 8%
Singletons Twins
Sarno et al UOG 2016
Although the total fetal fraction
in twins is 1.6 x singletons, the
average fetal fraction per twin is
lower.
Fetal fraction
NIPT in Twin Pregnancies
Risk factors for Failed
NIPT:
• Maternal BMI (dilutional)
• IVF (impaired placentation)
Twins ----
Singleton
IVF
Non-IVF
MA 35 years
Caucasian
CRL 55 mm
Maternal BMI (Kg/m2)
cfD
NA
failu
re r
ate
(%
)
Sarno et al UOG 2016
NIPT in Twin Pregnancies
How should we diagnose and manage Twin Reversed Arterial Perfusion?
Complications in twins
Ultrasound findings• Absence of normal cardiac structure • Cardiac movement • Variable structural abnormalities
Twin reversed Arterial Perfusion
Heart failureIntrauterine demisePolyhydramniosPreterm birth
TRAP sequence
Controversy on timing of intervention
• Risks: demise of the co-twin; preterm birth
• Spontaneous cessation of the flow in theacardiac twin
• High loss rate (54%) between diagnosis in thefirst trimester and planned intervention at 16weeks
• Prenatal intervention: 80% survival rate
• Lower very preterm birth rates with early vs lateintervention
Lewi L, AJOG 2010; Chaveeva P, Fet Diagn Ther 2014
Pagani G, UOG 2013
A: Spontaneous demise at 13-16 weekB: Spontaneous arrest of flow at 14-17 weekC: Pump twin alive at 16-18 week with reversed flow
Management of TRAP• The chances of survival of the pump twin are increased by the
use of minimally invasive techniques (preferably <16 weeks)
• Intrafetal Laser
• Cord coagulation/ligation
• Laser photocoagulation of the anastomoses
D
Twin Pregnancy: TRAP
Take-home Messages
Thank you
• DC twins: US every 4 weeks after 20 weeks• MC twins: US every 2 weeks after 16 weeks
• sFGR in DC twins: avoid delivery <30 weeks• sFGR in MC twins: Gratacos classification
•TTTS: Polyhydramnios • TAPS: MCA PSV discordance
• TTTS: US every 2 weeks from 16 weeks• TAPS: MCA PSV from 20 weeks and follow-up after Laser (TTTS)
• TTTS: Laser surgery (Soloman) at 16-26 weeks (≥stage 2)• TAPS: Individualised management options (no guidance)
Complications in Twins
Twin reversed Arterial Perfusion
Radiofrequency ablation
Twin reversed Arterial Perfusion
Intrafetal interstitial Laser
35 years old
IVF MCDA twin pregnancy
TRAP mass; 50% size of the pump twin
Normal NT and ductus venosus Doppler
Prognosis
Size of the TRAP mass
NT and ductus venosus Doppler of the pump twin
Twin reversed Arterial Perfusion
Intrafetal interstitial Laser
Laser at 13 weeks
Follow-up scans
Follow-up scan at 28 weeks
Follow-up scan at 28 weeks
Fetal brain MRI 29 weeksNormal
TRAP mass
Follow-up scan at 29 weeks
Weekly monitor
Delivery by CS
35+ weeks
Take-home Messages
Thank you
• DC twins: US every 4 weeks after 20 weeks• MC twins: US every 2 weeks after 16 weeks
• sFGR in DC twins: avoid delivery <30 weeks• sFGR in MC twins: Gratacos classification
•TTTS: Polyhydramnios • TAPS: MCA PSV discordance
• TTTS: US every 2 weeks from 16 weeks• TAPS: MCA PSV from 20 weeks and follow-up after Laser (TTTS)
• TTTS: Laser surgery (Soloman) at 16-26 weeks (≥stage 2)• TAPS: Individualised management options (no guidance)
Complications in Twins
Twin reversed Arterial Perfusion