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Information leaflet on

Twin–Twin
Transfusion
Syndrome

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What is Twin–Twin Transfusion Syndrome?
Twin–twin
What transfusion
is Twin–Twin syndrome (TTTS)Syndrome?
Transfusion complicates 1 in 8
(10 –15%) of monochorionic (MC) twin pregnancies.
Twin–twin transfusion syndrome (TTTS) complicates 1 in 8 (10–
In
monochorionic twins (identical twins), the twin pregnancies
15%) of monochorionic (MC) twin pregnancies. In monochorionic
‘share’ the placenta (afterbirth) and there are often blood
twins (identical twins), the twin pregnancies ‘share’ the placenta
vessels within and
(afterbirth) on the
and there are surface of vessels
often blood the placenta connecting
within and on the
both surface
twins.of the placenta connecting both twins.
umbilical artery

umbilical vein

shared lobule

This isThis is a very


a very seriouscomplication
serious complication of of
multiple pregnancies
multiple and
pregnancies and
can
can present:present:
a) In the first trimester with discordant nuchal translucencies
a) In the(collection
first trimester
of fluid atwith discordant
the back nuchal
of the baby’s neck)translucencies
in MC
(collection of fluid at the back of the baby’s neck) in MC
twins. But this is not a reliable screening test for this
twins.condition.
But thisAtispresent
not a only serial,screening
reliable two weekly test
scansfor
from 16
this
weeks can screen for TTTS.
condition. At present only serial, two weekly scans from 16
weeks cananscreen
b) With excessivefor TTTS.of amniotic fluid around one
amount
twin (>8cms deepest pool depth) (recipient = receives a
b) Withhigh
an excessive
perfusion ofamount
blood fromofthe
amniotic
placenta)fluid
and aaround
decreasedone
twinamount
(>8cmsofdeepest poolaround
amniotic fluid depth) the(recipient
other (<2cms)= receives
(donor = a
highunder perfusion
perfusion of of bloodfrom
blood from the
theplacenta).
placenta) and a
decreased
c) Differing sizes of twins (>20% differencearound
amount of amniotic fluid the other
in fetal weights).
(<2cms) (donor = under perfusion of blood from the
placenta). 2

c) Differing sizes of twins (>20% difference in fetal weights).


In 60% of fetal ‘donors’ there is selective growth
restriction.
2
The ultrasound diagnosis is made when one twin
has increased
The ultrasoundamniotic fluid
diagnosis is made and
when onethe
twin other has
has increased
reduced amniotic fluid in the twin’s amniotic sacs.
amniotic fluid and the
twin’s amniotic sacs.
other has reduced amniotic fluid in the

polyhydramnios

hypervolemia
Recipient

<20 wk >8cm. polyuria


>20 wk >10cm.

oligohydramnios
(<2cm)
oliguria
Donor hypovolemia

Key to diagnosis is amniotic fluid discordance

Without treatment, this condition will lead to death or


Without treatment, this condition will lead to death or
miscarriage in excess of 90–95% of these MC twin
miscarriage in excess of 90–95% of these MC twin pregnancies.
pregnancies.
In addition In
thisaddition this
is a condition is can
that a condition thatBabies
damage babies. can damage
babies. Babies may have complications of poor
may have complications of poor blood supply that can cause blood supply
that can cause ‘strokes’ and ischaemia (poor blood supply) of
‘strokes’ and ischaemia (poor blood supply) of the limbs. It is
the limbs.
thus, aIt is thus,
serious a serious
problem problem
effecting twins, witheffecting
up to 30% twins,
long with
term handicap.
up to 30% long term handicap.
Forty to fifty cases are treated annually (one per week on
Forty average)
to fiftyatcases
the Fetal
areMedicine
treated Centre at Birmingham
annually (one per Women’s
week on
Hospital, predominantly by Laser ablation of placental vessels.
average) at the Fetal Medicine Centre at Birmingham
The patient usually presents at between 16–26 weeks with
Women’s Hospital,
abdominal predominantly
pain, contractions by Laser
and occasionally ablation
vaginal of
bleeding
placental vessels.
and the diagnosisThe patientbyusually
is confirmed ultrasoundpresents
scan. at between
16–26 weeks with abdominal pain, contractions and
occasionally vaginal bleeding 3and the diagnosis is confirmed
by ultrasound scan.

There is a high risk of miscarriage (death <24 weeks), death


soon after birth (stillbirth) and chronic handicap (cerebral
palsy) in survivors (20–25%) due to poor placental function,
abnormalities of fetal growth and risk of pre–term delivery.
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Over the last ten years interest has focused upon both survival
of babies with this condition, as well as the long–term
handicap of survivors. This has led to the development of
new management strategies of this condition with the goal
of significantly reducing long–term handicap and damage in
survivors.

There are three potential options for treatment


1. Amniodrainage
This treatment of TTTS involves the performance of a series of
up to 10 amniodrainage (amniotic fluid volume removals of
up to 5 litres) throughout the pregnancy. Each of these
drainages is performed under ultrasound guidance and
involves a needle being inserted into the amniotic sac (bag of
fluid surrounding the baby) and fluid is drained in order to
reduce the amount and thus reduce the tension of the
mother’s abdomen. This procedure is now used for very early
stage disease or mild disease at a later gestation.

This treatment increases survival in these twins from


5% –10% to between 50 – 60%. However, the long–term
handicap (cerebral palsy) associated with this form of
treatment is very high at between up to 20–25%. For this
reason it is rarely offered unless presentation is atypical.

chorion
amnion of
amnion of fetus ‘b’
fetus ‘a’

1
a
2
b

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2. Laser ablation of placental vessels
A relatively recent development in the management of TTTS
is the use of fetoscopy (special micro–telescope allowing the
baby to be seen while still in the uterus) and laser therapy to
separate the placental blood vessels between the twins. This
is now the treatment option of choice.

This procedure would usually be undertaken once during the


pregnancy (although rarely it may need to be repeated).

The treatment is carried out under local anaesthetic (usually


with local anaesthetic and sedation but more rarely using a
spinal anaesthetic). Under ultrasound guidance a 2–3 mm
micro–telescope is passed into the amniotic sac of the larger
(recipient) twin. This allows visualization of the placental,
blood vessels connecting the babies.

A laser fibre may be passed down the operating channel and


the abnormal placental blood vessels coagulated or ‘blocked’
(as they run from the donor to the recipient twin baby. The
treatment takes approximately 30–40 minutes and is usually
performed between 16 – 26 weeks.

In the largest RCT trial reported of this procedure 142


pregnancies were looked at. 72 of these pregnancies were
allocated to laser treatment (therapy). 73% of these had at
least one twin survive compared with 51.3% treated with
amniodrainage. Of those pregnancies treated with laser
there were 6.9% of deaths within the first week of life
compared with 22.8% in those pregnancies treated with
serial amniodrainage. At the age of one year neurological
handicap was suspected in approximately 5% of the surviving
babies compared to 20–25% in those babies who had been
treated with amniodrainage.

In our own series >85% of procedures have at least one


survivor and this breaks down into:
i) 50% of two survivors.
ii) 40% one survivor only.
iii 10% twin fetal demise.
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i) 50% of two survivors.
ii) 40% one survivor only.
iii 10% twin fetal demise.

Copyright: From Thesis by Dr Femke Slaghekke, University of Leiden, Holland

The majority, international body of opinion would advocate


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treatment of severe, early onset TTTS ((presenting before 26


weeks) using fetoscopically directed laser ablation, as first
resort. The rational for this is not so much to increase fetal
survival but to reduce the significant risk of cerebral palsy in
the survivor(s) of this condition.

The advantages are:

1. The procedure only needs to be performed once in the


majority of cases.
2. There are improved survival rates of at least one twin over
compared with amnioreduction.
3. The risk of long term handicap in survivors appears to be
significantly reduced (approximately 5% each fetus).

The disadvantages and complications of this technique are:

1. There is a risk of pre–labour ruptured membranes 5 – 7%


(waters breaking), associated with a risk of miscarriage.
2. There is a small risk of bleeding into the amniotic fluid
(bleeding within the womb/uterus in <1%).
3. A small risk of infection (<1% and reduced by using
antibiotics and performing the procedure in operating
theatre).
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3. Diathermisation of the umbilical cord: selective
termination of pregnancy.

This is performed when there is evidence that one of the


monochorionic twins has severe heart failure and is close to
death. Often, this is associated with ultrasound detected fetal
anomalies indicating brain damage.

This often presents as ‘fluid within the body cavities’ of one


of the babies, commonly the recipient. In such cases, the cord
of the baby close to death can be coagulated (blocked off) by
diathermy (heating the vessel) to save the other twin. This is
usually only an option in rare cases.

These procedures will be discussed with you in detail and the


one most appropriate for your pregnancy will be discussed.
Please ask the doctor any questions that you may have.

4. Termination of the pregnancy.

This is an option not pursued by the majority but none the


less is an option for parents and is discussed. This is because
this is a morbid condition of twin pregnancy associated with a
risk of brain damage.

If you have any questions or concerns please do not hesitate


to contact the Fetal Medicine Centre on 0121 335 8252.

If you are put through to an answer service please leave your


contact details.

Your call will be returned.

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protection legislation (General Data Protection Regulation
(GDPR)/Data Protection Act 2018. For more information
about how we use your personal data please visit our website
at:

https://bwc.nhs.uk/privacy-policy

Birmingham Women’s Hospital


Mindelsohn Way
Birmingham B15 2TG
Website: www.bwc.nhs.uk

Author: Professor Mark Kilby


Produced: 09/2018
Review Date: 09/2021
Version 1.0
Ref: 0140

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