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REVIEW

Twin pregnancy

Emma Ferriman

Stephen Stratton

Vicky Stern

Abstract

Twins account for 2e3% of all births. They carry signicant risks to both mothers and babies. These risks include preterm delivery, intra- uterine growth restriction, and pre-eclampsia. In addition, monochor- ionic gestations confer an even higher rate of perinatal morbidity and mortality arising from a shared placenta due to placental anasto- moses, which may lead to twin-to-twin transfusion syndrome (TTTS) or twin anaemia-polycythaemia sequence (TAPS). It is essential that cho- rionicity is established in the rst trimester in order to initiate the appropriate antenatal management and surveillance. In view of the high risk of both maternal and fetal complications, twin pregnancies are ideally managed in a dedicated clinic according to agreed proto- cols with both obstetric and midwifery input.

Keywords chorionicity; dichorionic; monochorionic; twins

Introduction

Twin pregnancies account for approximately 3% of all live births, but account for 6.3% of stillbirths and 12.7% of neonatal deaths. Twins are more at risk of pregnancy complications (Table 1 ). Monozygotic twin frequency rates remain relatively stable worldwide at 3.5/1000 maternities, but dizygotic twins have a variable rate depending on a number of factors including geographical location, assisted reproductive techniques and increasing maternal age. Rates vary from 1.3 to 49/1000 mater- nities. Monochorionic twin gestations are associated with even higher perinatal risk. Multiple pregnancies have been described as a modern epidemic and carry considerable resource implica- tions for health providers. In order to reduce the numbers of twin pregnancies conceived as a result of assisted conception tech- niques, a number of strategies have been proposed such as elective single embryo transfer, selective fetal reduction and single blastocyst transfer.

Zygosity and chorionicity

Twin pregnancy usually results from the fertilization of more than one oocyte, producing dizygotic or non-identical fetuses.

Emma Ferriman MBChB FRCOG is a Consultant in Feto-Maternal Medicine at The Jessop Wing, Shefeld, UK. Conicts of interest:

none declared.

Stephen Stratton BSc MBChB MRCOG is a Senior Registrar with a special interest in Fetal Medicine at The Jessop Wing, Shefeld, UK. Conicts of interest: none declared.

Vicky Stern MBChB MRCOG is a Fetal and Maternal Medicine Sub-

specialty Trainee at The Jessop Wing, interest: none declared.

Shefeld, UK.

Con icts

of

221

Frequency of fetal complications in twins

 
   
 

Singleton Dichorionic Monochorionic

Miscarriage 11e23 weeks Perinatal death IUGR

1%

2%

10%

0.5%

1.5%

3%

5%

20%

30%

Preterm delivery <32 weeks 1%

5%

10%

Major defects

1%

1%

4%

Table 1

Splitting of a single fertilized oocyte produces a monozygotic twin pregnancy with two genetically-identical co-twins. Non- identical twins develop their own placentae: monozygotic twins may share a placenta according to the time of separation

(Figure 1 ). Dichorionicity occurs in 80% of twins, and genotyp- ing is required to confirm zygosity in these cases ie dichorionic twins can be monozygous. Monochorionicity confers major increases in perinatal morbidity and mortality when compared to dichorionic gesta- tions. Chorionicity may be accurately determined by ultrasound

from between 10 and 14 weeks’ gestation. A number of methods

are widely used, including the presence of the lambda or ’twin peak sign’ for dichorionicity and the ’T sign’ for mono-

chorionicity. In addition, the thickness of the intertwin mem- brane may be determined e a membrane thickness of less than 2

mm is suggestive of monochorionicity. Other indicators in the

second trimester may be the presence of two separate placental masses or discordant fetal sex. All twin pregnancies should be offered an ultrasound scan between 11 and 13 þ 6 weeks to assess viability, determine chorionicity and to screen for Down’s syndrome. In monochorionic twins it is important to exclude acardiac twinning. If a woman with a twin pregnancy presents after 14 weeks, it is important to determine chorionicity at the earliest opportunity by ultrasound using the number of placental masses, the lambda or T sign, the membrane thickness and discordant fetal sex. If chorionicity remains uncertain, even after senior review, the pregnancy should be managed as monochorionic.

Screening for abnormality in twins

Aneuploidy screening

The risk of Down’s syndrome (Trisomy 21) is 1 in 700 preg-

nancies. The risk for monozygotic twins is the same as for sin- gletons, but for dizygotic twins this risk is doubled as each twin has its own individual risk. The screening test of choice for twins is combined first trimester screening at between 11 and 13 þ 6 weeks’ gestation with a calculated risk for the pregnancy in monochorionic twins and an individual risk per baby in dichor- ionic twins. Combined screening uses the measurement of the

nuchal translucency combined with first trimester measurements of pregnancy associated plasma protein A (PAPP-A) and human chorionic gonadotrophin (HCG). The detection rate varies ac- cording to the chorionicity of the pregnancy: in monochorionic twins the detection rate should be the same as for singletons (80% with a 3% false positive rate), however, in dichorionic twins where one baby is affected with aneuploidy, the detection

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REVIEW

The relationship between zygosity and chorionicity

The relationship between zygosity and chorionicity DIZYGOTIC MONOZYGOTIC (non-identical) (identical) DICHORIONIC
DIZYGOTIC MONOZYGOTIC (non-identical) (identical) DICHORIONIC MONOCHORIONIC
DIZYGOTIC
MONOZYGOTIC
(non-identical)
(identical)
DICHORIONIC
MONOCHORIONIC

Figure 1

rate may fall to between 40 and 50% with a 3% false positive rate. Detection rates can be improved further using additional ultrasound markers such as the presence of the nasal bone and ductus venosus and tricuspid Doppler waveform analyses. Additional biochemical markers, alpha-fetoprotein (AFP) and oestriol, can increase the detection rate further (up to 87%). For women who present beyond 14 weeks the quadruple test may be offered up to 20 weeks gestation. Finally, the advent of Non-Invasive Prenatal Testing (NIPT) offers a further choice in screening for women with twin preg- nancies. Detection rates are between 98 and 99% in singletons with false positive rates of < 0.2%, however data for NIPT in twins is still being collected. There seems little doubt it will perform in twins much more highly that the combined or quadruple tests. Following a high-risk screening result, or the detection of a fetal abnormality including an increased nuchal translucency, the option of invasive testing should be discussed. Both amniocen- tesis and CVS are possible in twin pregnancy, but these proced- ures should be performed in a specialist fetal medicine unit to ensure that the pregnancy is mapped correctly and the samples taken are correctly attributed to each of the fetuses. The risks of miscarriage and other procedure-related complications are quoted as around 1 in 50 in twin pregnancies. Both amniocen- tesis and chorionic villus sampling are valid options, but there is some evidence to suggest that a double amniocentesis has a lower risk of mistakenly sampling the same fetus twice.

Anomaly screening The frequency of fetal abnormality in dizygotic twins is compa- rable to that of singleton pregnancies (2 e3%). This contrasts with the increased frequency of anomalies seen in monozygotic pregnancies where rates of up to 10% have been reported, or 2 to 3 times those which occur in dizygotic twinning. Several different

types of anomaly are thought to be more commonly seen in twin pregnancies, including neural tube defects and congenital heart disease. In monozygotic twinning, abnormal vascular connec- tions predispose to limb reduction defects and bowel atresias. Disorders of laterality occur when embryonic migration has begun prior to zygotic splitting and may explain the increased incidence of cardiac anomalies in monozygotic twin pregnancies. A fetal echocardiogram is ideally offered at 20 e 22 weeks gestation. While the majority of monozygotic twins appear to be almost identical, there are monozygotic offspring who are genetically and phenotypically dissimilar. Mechanisms may include unequal allocation of blastomeres between the two embryos, disrupted embryonic migration, somatic mosaicism or chimerism, and variations in penetrance of single gene disorders producing phenotypic discrepancy. The type of discordance varies from genetic and chromosomal abnormalities through to isolated structural anomalies. Discor- dant single gene disorders, imprinting defects and aneuploidy have all been reported in monozygotic twins. Case reports detail a range of discordant structural anomalies found in monozygotic twin pairs, from neural tube defects and holoprosencephaly to lateral and ventral body wall defects, and anomalies related to the VATER association.

Management of twins discordant for fetal anomaly The diagnosis of discordant anomaly in twins creates significant dilemmas for parents, and careful counselling is required in centres with expertise in this area. Accurate diagnosis and determination of chorionicity is critical for subsequent manage- ment. Depending on the anomaly detected, parents may be faced with a choice of continuing the pregnancy and delivering both a normal and an affected baby, or of terminating the affected fetus and risking the viability of the healthy co-twin. Invasive testing

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for chromosomal abnormality in dichorionic twins requires dual puncture in most cases. In general, monochorionic twins require a single puncture but in cases of discordant anomaly, both fe- tuses should be sampled. Selective feticide using intracardiac lignocaine or potassium chloride injections of the affected fetus is only possible in dichorionic twin pairs due to their separate inter-twin circula- tions. It is associated with an increased risk of pregnancy loss, and if not performed in the first trimester, is usually delayed until the third trimester when viability of the normal twin is more certain. This must be balanced against the risk of spon- taneous premature labour, especially in cases complicated by polyhydramnios such as anencephaly. Selective feticide in monochorionic twins requires a cord occlusive technique such as intrafetal laser or radiofrequency ablation of the umbilical cord insertion, or bipolar diathermy to the cord. These tech- niques also carry a significant risk of loss of the entire pregnancy.

Pregnancy complications speci c to twins

Complications specific to both mono- and dichorionic twin pregnancies include vanishing twin and fetus papyraceous. Se- lective fetal growth restriction (sFGR) is also possible in both however it is more frequent in monochorionic pregnancies and given the nature of a shared placenta poses a greater challenge to manage. Abnormalities unique to monochorionic pregnancies are twin-to-twin transfusion syndrome (TTTS); twin anaemia- polycythaemia sequence (TAPS) and the twin reversed arterial perfusion syndrome (TRAP). Monoamniotic pregnancies will also be discussed.

Vanishing twin and fetus papyraceous Up to 21% of twin pregnancies are complicated by either miscarriage or loss of one twin in the early stages. This ‘van- ishing twin’ phenomenon is increasingly detected by high- resolution ultrasound and it is suggested that the miscarriage rate in these pregnancies is about five times higher than that of normal twins. No increased monitoring should be necessary if the baby appears structurally normal, as the pregnancy is most likely to progress as expected for a singleton. Care should be taken where pregnancies have begun as multiple gestations in women undergoing screening for chromosomal abnormality as the non-viable fetus may cause false positive results. The loss of a co-twin in the second or third trimester carries a risk of preterm delivery, neurological sequelae or death to the remaining fetus. It may result in the phenomenon known as fetus papyraceous, where the anatomically-preserved demised fetus can be identified at the later delivery of the surviving twin.

Selective fetal growth restriction Selective Growth Restriction (sFGR) complicates 10 e 15% of monochorionic twin pregnancies. The natural history and path- ophysiology of this condition is different between monochorionic and dichorionic twins. Growth in dichorionic pregnancies reflects both genetic potential and placental function, but monochorionic twin growth is also subject to the effects of unequal blastomere separation, velamentous cord insertion and placental vascular communications.

Selective fetal growth restriction is defined as an estimated fetal weight (EFW) discordance of > 20%. The diagnosis may be confused with TTTS as the smaller twin may have reduced li- quor, but the larger twin will tend to have normal liquor volume (not increased) and both bladders are visible. sFGR is an inde- pendent risk factor for an increase in perinatal mortality and morbidity. Once identified, sFGR in monochorionic twins should be referred to a fetal medicine centre for further management. Three types of sFGR have been identified; type I where there is discordant growth but normal Dopplers, type II where there is absent or reversed EDF in the UA Doppler and type III where there is alternating positive, absent and reversed EDF in the UA Doppler. Type I should be delivered between 34 and 36 weeks gestation whereas type II and III should have planned delivery by 32 weeks. Management of this condition prior to twenty-four weeks may include selective termination of pregnancy using vaso-occlusive techniques in order to protect the appropriately grown twin. After 24 weeks frequent monitoring of the growth, umbilical artery Doppler, middle cerebral artery peak systolic velocity and ductus venous waveform is essential, and consideration must be given to elective delivery. Paradoxically, the vascular anasto- mosis between monochorionic twins which predispose to TTTS can actually benefit the smaller twin in sFGR because artery eartery connections can compensate for the placental insuffi- ciency. This means that fetal demise may occur later in a mon- ochorionic twin pregnancy complicated by fetal growth restriction (average 10 weeks from onset) than in dichorionic twins (3e 4 weeks from onset of the growth restriction).

Twin-to-twin transfusion syndrome (TTTS) Twin-to-twin transfusion syndrome (TTTS) complicates 10 e 15% of monochorionic twin pregnancies. Feto-fetal transfusion occurs via multiple vascular anastomoses between the circulations of each co-twin, such that there is a net flow of blood from one twin (the ‘donor’) to the other (the ‘recipient’). This results in hypo- volaemia and oligohydramnios in the donor twin and hyper- volaemia and polyhydramnios in the recipient. Progression of the syndrome in the donor leads to growth restriction and in severe cases, absent or reversed end-diastolic frequencies in the umbilical artery. The recipient may develop organomegaly, with abnormal ductus venosus Doppler fre- quencies related to polycythaemia and hydrops. Tricuspid regurgitation is an ominous sign of cardiac dysfunction in the recipient and is associated with significant postnatal cardiac dysfunction (Figure 2 ). Twin-to-twin transfusion syndrome ac- counts for about 20% of stillbirths in multiple pregnancies.

Pathophysiology : both superficial and deep placental vascular connections are present in the monochorionic placenta. Deep anastomoses occur between arteries and veins. These arteriove- nous (AV) connections are unidirectional and require the pres- ence of ‘balancing’ superficial anastomoses to prevent TTTS. Superficial anastomoses are bi-directional and are commonly found between arteries (arterio-arterial anastomoses) and veins (veno-venous anastomoses). Bidirectional flow allows compen- satory activity in the event of vascular flow differences across the deep anastomoses of the placenta, and if it is reduced or absent then TTTS may develop.

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REVIEW

REVIEW Figure 2 Shows the enlarged bladder of the recipient twin with poly- hydramnios in twin-to-twin

Figure 2 Shows the enlarged bladder of the recipient twin with poly- hydramnios in twin-to-twin transfusion syndrome.

Diagnosis of TTTS : most commonly, the diagnosis of TTTS is made in the second trimester following the detection of discor- dant growth or discrepant liquor volumes. A ‘stuck twin’ may be visible, compressed against the uterine wall, where the donor is constricted by anhydramnios and the tense sac of the poly- hydramniotic co-twin. Acute TTTS may present as the sudden onset of maternal discomfort and increasing girth, following rapid development of polyhydramnios. Mortality is extremely high usually as a consequence of premature delivery, either spontaneous or iatrogenic. A diagnostic staging system proposed by Quintero describes a progression from early (stage I) to late (stage IV) disease (Table 2 ). A high stage at diagnosis is associated with increased neurological morbidity and mortality, but progression of the disease from early to a more advanced stage is also important for prognosis. Uncertainty exists regarding the optimum manage- ment of early (stage I) disease, where there is some evidence that aggressive treatment may confer little benefit.

Management options for TTTS : several management options exist for the treatment of TTTS, however there is now

The Quintero classification system

 

Stage

Classification

1

There is a discrepancy in amniotic fluid volume with oligohydramnios of a maximum vertical pocket (MVP 2 cm) in one sac and polyhydramnios in the other sac (MVP 8 cm). The bladder of the donor twin is visible and Doppler studies are normal

II

The bladder of the donor is not visible, but Doppler studies are normal

III

Abnormal Doppler studies in either twin characterised by reversed EDF in the umbilical artery, reversed flow in the ductus venosus or pulsatile umbilical venous flow

IV

The presence of hydrops in the recipient

V

Death of one or both twins

Table 2

overwhelming evidence that laser ablation of the communicating placental vessels has better outcomes. Other options are serial amnioreduction with or without septostomy, and occlusive feticide. Endoscopic placental laser ablation aims to coagulate the vascular anastomoses contributing to TTTS and may be selective or non-selective. Non-selective coagulation destroys all vessels crossing the intertwin membrane, including the healthy circula- tion, and may increase mortality in the donor twin. This alter- native is to selectively ablate only specific vascular connections (the Solomon Technique). This carries the risk of incomplete treatment and a recurrence of the condition. Amnioreduction is performed prior to laser ablation in most cases. The Eurofetus randomised trial demonstrated increased sur- vival of one or both twins following laser (76%) compared with serial amnioreduction (56%). The median gestational age at delivery was increased in the laser group (33 weeks vs 29 weeks) and laser was associated with a reduced incidence of periven- tricular leukomalacia. As live birth rates were similar in both groups, this survival advantage may reflect the differences in gestation rather than a consequence of the therapy. In addition, early stage disease was not well-represented in this study, leav- ing persistent doubt about the benefit of laser in early disease. A systematic Cochrane review in 2008 included only two randomised controlled trials (including Eurofetus) with similar results. Long term neurological sequelae have a reported inci- dence of 13% e17% and do not appear to be reduced following laser compared with amniodrainage. Following successful laser ablation, the incidence of intra- uterine death is reported to be 13 e 33%, and that of ruptured membranes approaches 10%. High stage disease is more likely to result in mortality. Late complications of laser are increasingly reported, and mainly relate to the presence of persistent communicating vessels causing recurrent TTTS or reversal of flow (reverse TTTS). Despite this, since the Eurofetus study, laser has been considered the first-line treatment for TTTS. Amnioreduction aims to reduce liquor volume in the recipient twin and to prevent premature delivery. It is likely to require repeated procedures and does not treat the underlying cause of feto-fetal transfusion. Associated risks include premature labour, ruptured membranes, chorioamnionitis and placental abruption. Septostomy aims to disrupt the inter-twin membrane allowing normalisation of liquor volume between the two sacs and may be followed by amniodrainage as an adjunctive treatment. A randomised controlled trial comparing amnioreduction with septostomy in TTTS before 24 weeks of gestation found that the rate of survival of at least one twin was similar in both groups (78% vs 80%), with no significant advantage of septostomy with amnioreduction over amnioreduction alone. Possible disadvantages of septostomy include the fact that the resulting chorioamniotic separation may hinder subsequent laser ablation. Amnioreduction is now most commonly utilised at later gestations or where laser ablation is not feasible and is useful in stage I disease where the evidence for laser ablation is less robust.

Selective feticide The termination of one fetus by disruption of the cord is an op- tion, particularly in the presence of discordant anomaly. Laser

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ablation, radiofrequency ablation and cord occlusion are the techniques possible. Laser is usually reserved for the first trimester, radio frequency 22 e24 weeks gestation and cord occlusive techniques at gestations greater than 24 weeks. Parents may choose to terminate a severely affected twin to increase the survival chances of the other, less affected twin and reduce the risk of losing both babies. From the limited available evidence, singleton survival rates after the procedure would appear to be about 67%, ruptured membranes in 5 e10% and severe neuro- logical sequelae in the region of 6%.

Twin anaemia-polycythaemia sequence (TAPS) This syndrome has a similar pathology to TTTS however it is thought to occur by much smaller ( <1 mm), hair like arterio- venous connections, which allow insidious flow of blood from one twin to the other. The result is one twin becomes anaemic (donor) whilst the other twin becomes polycythaemic (recip- ient). It is thought to complicate 5% of monochorionic twin pregnancies, increasing to 13% following laser ablation therapy.

Diagnosis prenatally is by the measurement of the peak systolic velocities (PSV) in the middle cerebral artery (MCA). The difference

is considered significant where the donor twin has an MCA-PSV

greater than 1.5 multiple of the median (MoMs) and the recipient twin has an MCA-PSV less than 1.0 MoMs. Similar to TTTS, there is

a staging system. At present there is no clear consensus on the

frequency of measurement of the MCA-PSV in monochorionic twin pregnancy with some authorities recommending screening every two weeks whilst others not recommending any routine screening at all. Given the significant increase in its occurrence after laser therapy, MCA-PSV is considered an essential part of surveillance after the procedure has been performed. The outcome in TAPS can vary from delivery of healthy twins which need treatment for anaemia/polycythaemia in the

neonatal period, through to a twin suffering severe hydrops or fetal demise. There is also evidence that this condition has im- plications on neurodevelopment, with up to 20% of twins having some degree of impairment. There is no consensus on how to manage TAPS, but the op- tions should be discussed on an individual basis and are heavily dependent on gestational age. These include conservative man- agement, early delivery, fetoscopic later ablation (as discussed above), intrauterine blood transfusion for the anaemic twin and partial exchange transfusion of the polycythaemic twin.

Twin reversed arterial perfusion sequence (TRAP) Acardiac anomaly is a rare complication of monochorionic twin pregnancies, occurring in approximately 1 in 35,000 cases. In this condition, arterial blood flows in a retrograde fashion from the pump twin towards the affected twin via a single arterio-arterial anastomosis; hence the synonym twin reversed arterial perfusion syndrome (TRAP). The poorly oxygenated blood entering the circulation of the affected twin preferentially perfuses the caudal structures rather than the cephalad, resulting in abnormal development of all organ systems. The head and the heart are commonly absent, with a preserved central trunk and rudimen- tary spine. The lower limbs may be more preserved due to the improved blood supply. Acardiac twins are frequently hydropic due to their abnormal lymphatic and vascular drainage.

The diagnosis of TRAP usually follows the detection of a grossly abnormal co-twin within a monochorionic pair. The absence of cardiac pulsation in the acardiac twin is usually evident, although rudimentary cardiac tissue or transmitted pulsations may produce the appearances of normal cardiac function. Paradoxical blood flow may be visualised by colour Doppler ultrasound to confirm the diagnosis. Once diagnosed, the primary aim of management is to improve survival chances for the structurally normal pump twin. Poor prognostic features include increasing size of the acardiac twin with signs of cardiac insufficiency in the donor secondary to increased demand. Management options for intervention include cord occlusion techniques, or an intrafetal approach to ablate the vasculature in the acardiac twin.

Selective feticide The termination of one fetus by disruption of the cord is an op- tion, particularly in the presence of discordant anomaly. Laser ablation, radiofrequency ablation and cord occlusion are the techniques possible. Laser is usually reserved for the first trimester, radio frequency 22 e24 weeks gestation and cord occlusive techniques at gestations greater than 24 weeks. Parents may choose to terminate a severely affected twin to increase the survival chances of the other, less affected twin and reduce the risk of losing both babies. From the limited available evidence, singleton survival rates after the procedure would appear to be about 67%, ruptured membranes in 5 e10% and severe neuro- logical sequelae in the region of 6%.

Monochorionic, monoamniotic twins Monoamniotic twinning occurs in 1 e2 % of monochorionic gestations (1 in 3000 e6000 pregnancies) as a result of zygotic separation beyond eight days of conception. The diagnosis is usually made following the first trimester ultrasound, showing a single placenta and two freely moving fetuses with no inter-twin membrane evident. These pregnancies are associated with the highest perinatal loss rate of all twins, at around 30 e 60% in most series. Umbilical cord accidents and prematurity account for much of this loss rate, along with higher rates of congenital anomaly (20e25%) and fetal growth restriction. More recent series suggest a fall in perinatal mortality, possibly associated with earlier diagnosis and intensive surveillance in these cases. Despite the shared placenta, chronic TTTS appears to be less common in these gestations (5%). Up to 60% of the antenatal fetal deaths occur prior to 32 weeks’ gestation. This is thought to be related to cord entangle- ment and occlusion, although cord entanglement will be present at almost all gestations. Consequently, this complication is not preventable and cannot be predicted by cardiotocographic monitoring. Strategies have been reported to reduce amniotic fluid levels, limiting fetal movement to prevent tightening of the tangled cords. Medical amnioreduction with oral prostaglandin synthase inhibitors has been described with 100% survival of forty fetuses (twenty pairs). However, the majority of mono- amniotic pregnancies undergo intensive surveillance with serial ultrasound monitoring in an attempt to detect impending cord occlusion.

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Maternal risks associated with twins

Maternal risks associated with multiple pregnancy

Hyperemesis Increased mechanical symptoms of pregnancy Gastro-esophageal reflux Hypertensive disorders Gestational diabetes mellitus Anaemia Operative delivery Post-partum haemorrhage Perinatal mental health disorders

Table 3

Elective delivery at 32 weeks’ gestation following adminis- tration of steroids is usually advocated, since at this point neonatal survival is comparable to term survival in most cen- tres. Meta-analyses of perinatal loss report a rate of around 10% in monoamniotic pregnancies c ontinuing beyond this point. Vaginal delivery of monoamniotic pairs has been achieved successfully but is associated with the risks of cord prolapse and fetal impaction in the materna l pelvis. Vaginal delivery is usually reserved for the extremely premature or non-viable fetuses.

Conjoined twins Incomplete division of the embryo may result in conjoined twins. The classification of this anomaly is largely descriptive and dependent on the anatomical areas joined. Conjoined thorax (thoracopagus) and conjoined thorax and abdomen (thoraco- omphalopagus) are the commonest subtypes with conjoining at the pelvis and head (ischiopagus and craniopagus) being less common. With the advent of improved ultrasound techniques, most cases are identified in the first trimester, and in view of the sig- nificant mortality and morbidity, a significant number of parents

will opt for termination. Survival depends on the organs joined. 50% are stillborn and of the survivors up to 75% may have inoperable defects. Elective caesarean delivery is usually advo- cated, but there are reports of vaginal deliveries occurring.

Antenatal management of twin pregnancies

Women with twin pregnancies should be given the same advice about diet, lifestyle and nutritional supplements as in routine standard care. There is a higher incidence of anaemia in women with twin pregnancies therefore a full blood count should be performed at 20 e24 weeks to identify women who need sup- plementation with iron or folic acid. This should then be repeated at 28 weeks as in routine antenatal care. It is vital to offer antenatal care in an appropriate setting aiming to provide standardised care to all women with multiple pregnancies. Clinical care for women with twin pregnancies should be provided by a nominated multidisciplinary team con- sisting of named specialist obstetricians, specialist midwives and ultrasonographers, all of whom have experience and knowledge of managing twin pregnancies. In addition, women should have access to a perinatal mental health specialist, women’s health physiotherapist, an infant feeding specialist and a dietician. A dedicated clinic allows the close surveillance required by this population along with the specialised care they may need in terms of preparation for birth and psychological support. Mothers with twin pregnancies are at higher risk of all obstetric complications and should be counselled appropriately (Table 3 ). In general, maternal mortality associated with multiple births is 2.5 times higher than singleton births. NICE and the RCOG consensus document has recommended two distinct care pathways for monochorionic and dichorionic twins (Table 4 ).

Hypertension Women with twin pregnancies may be at higher risk of hyper- tension. NICE suggest that women with multiple pregnancies should take 75 mg of aspirin daily from 12 weeks until the birth

Schedule of antenatal scans and timing of delivery

 
   

Type of pregnancy (uncomplicated)

Scans

Delivery

Monochorionic diamniotic twins

Approximately 11 weeks 0 days e13 weeks 6 days (viability, chorionicity, 1st trimester screening) and 16, 18, 20, 22, 24, 28, 32 and

From 36 þ 0 weeks following antenatal steroids.

34

weeks e fetal growth and TTTS Fetal

Dichorionic, diamniotic twins

echo at 20 weeks Approximately 11 weeks 0 dayse13 weeks 6

days (viability, chorionicity, 1st trimester screening) and 20, 24, 28, 32 and 36 weeks e fetal growth 11 weeks to 13 þ 6weeks (viability, chorionicity, 1st trimester screening) and 16,18,20,22,24,26,28,30 weeks e fetal growth, polyhydramnios, TTTS Fetal echo at

From 37 þ 0 weeks. Consider steroids for elective caesarean sections

Monoamniotic monochorionic twins

From 32 þ 0 weeks following antenatal steroids. Delivery by elective caesarean section

20

weeks

Table 4

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of their babies if they have one or more of the following risk factors for hypertension:

First pregnancy Age 40 years or older Pregnancy interval of greater than 10 years BMI of 35 kg/m 2 or more at first visit Family history of pre-eclampsia

Preterm birth Twin pregnancies are at high risk of spontaneous or iatrogenic preterm delivery. The incidence of preterm delivery prior to 37 weeks can be up to 50 %. Delivery at less than 32 weeks appears to vary with the type of twinning, ranging from 5% for dichor- ionic twins and 10 % for monochorionic twins, compared with 1% for singleton pregnancies. Current evidence suggests that cervical length assessment, progesterone supplementation and cervical cerclage do not prevent early preterm labour in twin pregnancies and therefore the routine measurement of cervical length is not recommended. Studies into mechanical devices to prevent preterm labour in twins such as the Arabin pessary are ongoing. The use of untargeted single or multiple courses of corticosteroids is also not recommended.

Intrauterine growth restriction Twin pregnancies are known to be at a significantly increased risk of intrauterine growth restriction (IUGR) and this is partly due to the increase in risk of placental dysfunction, with rates varying from 20% in dichorionic twins to 30% in monochorionic pairs. Two thirds of unexplained stillbirths in multiple pregnancies are associated with a birthweight below the tenth centile. It is important to estimate fetal weight discordance using two or more biometric parameters at each ultrasound scan from 20 weeks. The optimum surveillance for IUGR in twins less than 32 weeks with abnormal Doppler studies has not been defined. The timing of delivery at very early gestations is a balance between the risks of prematurity and the risk of exposing the fetus to prolonged hypoxaemia. Surveillance of growth-restricted twins will include monitoring of fetal Doppler waveform analyses (umbilical artery, MCA and ductus venosus), liquor volume and computerised cardiotocography.

Single intrauterine fetal death The death of one twin carries an increased risk to the remaining fetus, which is greater in monochorionic pregnancies. The sur- viving twin of a monochorionic pair has a 15% risk of death, with neurological sequelae in 26% and preterm delivery of 68%. In dichorionic pairs, the risk of death is just 1%, with 4% developing neurological disability and less than 50% subse- quently delivering prematurely. This significant difference in risk has been attributed to substantial haemodynamic shifts within the shared placenta following the death of one fetus of a mono- chorionic twin pair. These patients should be carefully coun- selled regarding the prognosis for the surviving twin, and an MRI of the fetal brain is advocated in monochorionic gestations.

Delivery NICE guidance recommends delivery from 37 to 38 weeks gestation for uncomplicated dichorionic twins and from 36 to 37 weeks gestation for uncomplicated monochorionic diamniotic

twins. There is marked variability in practice. It should be remembered, however, that 58% of twins will deliver sponta- neously before 37 weeks. There is significant evidence that perinatal mortality rates increase after 38 weeks even in un- complicated twin pregnancies. Additionally, intervention at 37 weeks does not appear to be associated with a significant dif- ference in mode of delivery or maternal complications when compared to expectant management. For women declining de- livery, weekly monitoring should occur. Retrospective cohort data suggested that, when compared to the presenting twin, the second twin is at higher risk of intrapartum mortality due to the complications of vaginal delivery. However, the publication of the Twin Birth Study in 2013 has refuted this. The study was a large, prospective, randomized, controlled trial comparing planned Caesarean section to planned vaginal birth for twins delivered between 32 and 38 weeks gestation where the presenting twin was cephalic. It found that planned caesarean section did not reduce the risk of fetal or neonatal death or serious neonatal morbidity when compared with planned vaginal delivery. There was a higher risk of adverse perinatal outcomes for the sec- ond twin, but this was not reduced by a planned caesarean birth. Current practice supports the policy of planned vaginal birth in uncomplicated pregnancies with a cephalic first twin. On a more practical level, delivery should be conducted in a unit where continuous electronic fetal monitoring is available and there is access to early recourse to caesarean section. An experienced operator should be present at delivery to enable expert management of the second twin, in particular with regard to vaginal breech delivery. Overall, there is a higher risk of emergency caesarean section in labour for twin pregnancies, with rates approaching 50% overall and between 3 and 5% for the second twin following vaginal delivery of the first twin.

Conclusions

Multiple pregnancy is a common cause of morbidity for both mothers and babies. Antenatal care focuses on screening for anomalies and for early signs of complications such as fetal growth restriction, TAPS and TTTS. Accurate diagnosis of cho- rionicity in the first trimester is essential and allows appropriate surveillance to be planned. Following the results of the Twin Birth Study there is no evidence to support a policy of elective caesarean section for all twins. Current practice in the UK would be to support vaginal delivery in uncomplicated dichorionic twins in which the first baby has a cephalic presentation. For monochorionic twins, this practice is less clear-cut due to the incidence of acute twin-to-twin transfusion occurring in labour (up to 10%), but no national guidance currently recommends routine birth by caesarean section in this group as yet. A

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REVIEW

2017.

Practice points

C

Early assessment of chorionicity is essential for the planning of subsequent management.

C

First trimester combined screening by nuchal translucency and first trimester biochemistry should be offered between 11 weeks and 13 weeks and 6 days.

C

A fetal anomaly scan should be offered between 18 and 22 weeks gestation.

C

For twins discordant for fetal anomaly referral to a specialist fetal medicine centre is recommended.

C

Clinicians should appreciate the increased risk of congenital anomaly, in particular congenital heart defects in monochorionic twins.

C

Discordant fetal growth with abnormal Doppler assessment should be referred to a specialist centre when diagnosed.

C

The appropriate antenatal pathways should be followed with increased surveillance of monochorionic twins.

C

Uncomplicated dichorionic twins should be offered delivery be- tween 37 and 38 weeks gestation and uncomplicated mono- chorionic twins should be offered delivery between 36 and 37 weeks gestation.

C

Monoamniotic twins should be delivered by elective caesarean section at 32 weeks gestation

C

In twins planning a vaginal delivery, an experienced practitioner must be present or easily accessible during the intrapartum

 

period.

228

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