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R E V I E W

A R T I C L E

Monochorionic Twin with Selective


Intrauterine Growth Restriction
Yao-Lung Chang*

A monochorionic twin pregnancy with selective intrauterine growth restriction (IUGR) of


one twin is defined as one twin with an estimated fetal weight below the 10th percentile
for gestational age. Selective IUGR occurs in about 12% of twin pregnancies. The incidence
of IUGR is similar in dichorionic and monochorionic twin pregnancies, but the risk of neuro-
logic damage to the fetus is greater in monochorionic twin pregnancy. Monochorionic
twins have the highest risk of complications, because the well-being of one fetus crucially
depends on that of the other because of vascular anastomoses in the common placenta.
The spontaneous demise of the twin with selective IUGR may result in the concomitant
demise of the twin who is appropriate for gestational age (AGA) in up to 40% of cases or
in neurologic damage of the AGA twin in up to 30% of cases. However, even in the
absence of single intrauterine fetal death, the risk of neurologic damage is still increased
in monochorionic as compared with dichorionic twin pregnancies. Because monochori-
onic twins with selective IUGR lack a definite treatment process as in twin-twin transfu-
sion syndrome, this article reviews the ultrasound diagnosis, etiologies, Doppler findings,
classification, and the management of such high risk pregnancies.

KEY WORDS intrauterine growth restriction, monochorionic twin, umbilical


artery Doppler, placental share

J Med Ultrasound 2008;16(3):194201

Introduction known and unique complications, including twin-


twin transfusion syndrome (TTTS) and twin reversed
Twin gestation is more complex and has increased arterial perfusion (TRAP) sequence [2]. However,
perinatal morbidity and mortality rates compared MC with selective intrauterine growth restriction
with singleton pregnancies [1]. Poor perinatal out- (IUGR), which is also a unique complication in MC,
come is not only the result of prematurity, fetal is less discussed. MC have the highest risk of com-
growth restriction, and structural and chromosomal plications and the well-being of one fetus crucially
anomalies. Monochorionic twins (MC) have well depends on that of the other because of vascular

Received: April 7, 2008 Accepted: July 15, 2008


Department of Obstetrics and Gynecology, Chang Gung Memorial Hospital, Chang Gung University
College of Medicine, Tao-Yuan, Taiwan.
*Address correspondence to: Dr. Yao-Lung Chang, Department of Obstetrics and Gynecology, Chang Gung
Memorial Hospital, Linkou Medical Center, 5, Fu-Shin Street, Kweishan, Taoyuan 333, Taiwan.
E-mail: j12054@cgmh.org.tw

194 J Med Ultrasound 2008 Vol 16 No 3 Elsevier & CTSUM. All rights reserved.
Monochorionic Twin with Selective IUGR

anastomoses in the common placenta. Even exclud- monochorionicity is the T sign which has a sensitivity
ing these unique complications, the prognosis of of 100% and a specificity of 98.2% [9]. The accuracy
MC is still poorer than that of dichorionic twins [3]. is increased when ultrasound is performed before
Twin pregnancy with selective IUGR occurs in 14 weeks gestation [10]. Every twin pregnancy has
about 12% of twin pregnancies [46], and the inci- to be tested for chorionicity in the first trimester.
dence of IUGR is similar in both dichorionic and If two separate sacs are seen in the first trimester,
monochorionic twin pregnancies. Unequal placental then a dichorionic pregnancy is diagnosed.
sharing has been found to be the primary contrib- MC with selective IUGR is defined as a MC preg-
utor to birth weight discordance in MC [5]. MC with nancy with a small fetus, and the estimated fetal
selective IUGR usually presents with significant dis- weight determined by sonography is usually below
cordance in fetal size and, on first impression, can the 10th percentile [11]. To define IUGR in a twin
be assumed to be TTTS. These cases vary from sim- pregnancy is somewhat different from a singleton
ple amniotic fluid volume discordance to isolated pregnancy [12]. However, using the definition of
polyhydramnios and isolated oligohydramnios. IUGR in a singleton pregnancy to predict IUGR in a
The main pathophysiologic basis for MC with twin pregnancy is acceptable [8] because IUGR is a
selective IUGR is the placental territory factor, which poor prognostic indicator for the fetus, and using
arises from unequal sharing of the placenta in MC, a looser definition to predict IUGR in a twin preg-
and is different from the vascular communicating nancy in order to obtain closer fetal monitoring may
factor in TTTS. Since September 2005, there have be acceptable. Some authors have suggested that
been approximately 20 cases of MC with selective intertwine weight discordance should be considered
IUGR transferred to Chang Gung Memorial Hospital, in the definition of MC with selective IUGR. How-
Linko Medical Center, and half of these cases were ever, IUGR could increase perinatal complications,
initially diagnosed as TTTS at other hospitals. Three so we did not use intertwine weight discordance in
of the cases eventually met the criteria for TTTS the diagnostic criteria in this article.
after a few weeks of follow-up. Thus, there is the
possibility that MC with an unequal placental share
and an eventual superimposed vascular factor will Differential Diagnosis of MC with
be diagnosed with TTTS. After years of testing, the Selective IUGR and TTTS
treatment of TTTS has become more definite [7].
However, the optimal management of MC with The etiology of TTTS appears to result from a net
selective IUGR is still lacking [8]. In this article, we unbalanced blood flow between two monochori-
will discuss the unique high-risk MC pregnancy. onic fetuses through placental vascular communica-
tions, which results in a donor twin and a recipient
twin. Actual documentation of this unbalanced
Definition and Diagnosis of MC with blood flow remains elusive [13]. The most unique
Selective IUGR feature of MC with selective IUGR is the lack of the
polyhydramnios/oligohydramnios sequel. Generally,
Before the diagnosis of MC with selective IUGR is the etiology of TTTS is a vascular factor and that of
made, it is necessary to know how to diagnosis MC MC with selective IUGR is a placental territory factor.
using ultrasound. Among the criteria used to predict However, a previous study has shown that when the
MC using ultrasound, the most reliable indicator vascular factor in the etiology of TTTS is removed,
for dichorionicity (to exclude the diagnosis of MC) an unequal placental share would also be found
is the presence of two separate placentas which [14]. In MC with selective IUGR, an unbalanced
have a sensitivity and a specificity of 97.4% and vascular communication would exist, but the vas-
100%, respectively. The most useful test to predict cular factor would not be severe enough to cause

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Y.L. Chang

marked amniotic fluid discordance as in TTTS. We Vascular factors


have had experience of cases initially diagnosed as In our series of MC with selective IUGR [18], all
MC with selective IUGR even without discordant the AGA twins had the largest placental share.
amniotic fluid (AF), which progressed to TTTS However, in MC without IUGR, there was one case
weeks later. where the smaller twin had the larger placental
share. It has been hypothesized that in such cases
(larger twin with the smaller placental share),
Etiologies of MC with Selective IUGR there may be net arteriovenous anastomosis from
the larger twin to the smaller twin [19,20]. There-
Placental factors fore, we cannot rule out the possibility that there
The placenta has a striking functional reserve capac- is net flow from the twin with a larger placental
ity, which has been shown in studies where fetal territory to perfuse the twin with a smaller placen-
oxygen consumption has been artificially increased tal mass. However, the incidence of the small twin
[15,16]. It was thus concluded that the placenta is having a larger placental share in MC has been
usually small because the infant is small, but its small reported to be only 1.37% (7/509) and the type of
size does not act as a contributory factor to fetal vascular anastomosis has been proven not to be
growth restriction [15]. Quintero et al found that significantly associated with the birth weight dis-
the individual placental mass could be determined cordance in MC [19]. So, the communicating vas-
in MC [17], so we also used the same concept to cular factor possibly plays a role in the etiology of
estimate the placental share in MC with selective IUGR in MC, but the role is less important than the
IUGR. In our previous publication [18], the placental placental share factor.
share was severely unequal in MC with selective
IUGR (Fig. 1). The discordance in estimated fetal Genetic factors
weight detected by ultrasound before birth was As the twins are identical, genetic factors would
less than the degree of discordance in unequal pla- not be involved in MC.
cental share in such cases. We confirmed that the
placental territory factor plays a major role in MC
with selective IUGR. Doppler Findings in MC with
Selective IUGR

Umbilical artery (UA) Doppler


MC with selective IUGR has been classified into
three types using UA Doppler of the IUGR twin [21].
These three types of MC with selective IUGR are
based on UA Doppler findings: (1) normal, defined
as positive diastolic velocity; (2) persistent, absent
or reversed end-diastolic velocity (AREDV); and (3)
intermittent AREDV. Doppler assessment of the UA
is an established test of fetal well-being in high-risk
Territory of AGA twin Territory of IUGR twin
pregnancies. In singleton pregnancies, AREDV has
been attributed to increased downstream placen-
tal resistance because of a reduction in the number
Fig. 1. Unequal placental sharing in the monochorionic twin
with selective intrauterine growth restriction (IUGR). The of small muscular arteries [22,23]. AREDV in TTTS
IUGR twin had a much smaller-than-normal placental share has also been associated with poor perinatal out-
for appropriate-for-gestational age twin. come [24,25]. The etiology of AREDV in the donor

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Monochorionic Twin with Selective IUGR

twin has also been studied in cases receiving laser has no obvious role in MC with IUGR if there is no
therapy; small placental territory and intertwined twin intrauterine demise. In MC, the MCA PSV has
artery to artery anastomoses have been recognized been reported to be unstable because of the vas-
as the cause [14]. However, there have been very cular anastomoses [37]. The role of MCA PSV may
few cases of MC with selective IUGR which have be related to the detection of the severity of ago-
been treated with laser therapy, so other than the nal transfusion in IUGR twin intrauterine fetal death.
small placental territory, the type of intertwined If MCA PSV is significantly elevated after one twin
anastomoses has been suspected but has not been intrauterine fetal death, the possibility of severe
proven as the cause of AREDV in the IUGR twin. anemia in the surviving twin is high.
Interestingly, intermittent AREDV in TTTS was not
recognized as an abnormal Doppler finding in the Amniotic fluid (AF) level
Quintero staging system [26]. However, in MC with MC with selective IUGR could present with discor-
selective IUGR, intermittent UA AREDV has been dance in AF level between the twins but is not nec-
recognized as a subgroup with an elevated risk of essary for the diagnosis. AF is produced by fetal
intrauterine demise in the smaller twin and neuro- urine after 20 weeks of gestation. In the case of MC
logic damage in the larger twin. This latter finding with extreme AF discordance, this condition was
is not restricted to this condition but has also been categorized as TTTS. Usually the IUGR twin would
found in cases of intrauterine fetal death of the have a lower level of AF than the AGA counterpart
cotwin [27]. Therefore, UA Doppler of the IUGR in MC with selective IUGR. Currently, there are no
twin has the strongest prognostic role in MC with studies comparing the outcomes of MC with selec-
selective IUGR. tive IUGR with or without AF discordance. However,
in MC with selective IUGR, those with discordance
Umbilical venous (UV) Doppler in AF level and UA AREDV would have an adverse
UV blood flow reflects the placental circuit of fetal perinatal outcome compared with those with nor-
circulation and is crucial for intrauterine develop- mal IUGR twin UA Doppler [38]. Therefore, UA
ment [28]. Placental blood flow, which represents Doppler findings may be a stronger prognostic
approximately 30% of the biventricular fetal car- marker than AF level.
diac output [29], is reported to be reduced in IUGR
[3032]. Although the significant fetal weight dis-
cordance can be estimated in twin pregnancy Classification
before birth [33], the degree of unequal placental
share cannot be predicted before delivery. In a The classification of MC with IUGR mainly depends
previous study of TTTS, the donor twin had a sig- on the pattern of UA Doppler in the IUGR twin
nificantly smaller UV flow before laser therapy [21,39]. The reason for the classification of MC with
[34,35]. In our experience, the UV flow was also IUGR is that the spontaneous demise of the twin
decreased in the IUGR twin compared with the AGA with selective IUGR may result in the concomitant
counterpart in MC with selective IUGR. This decrease demise of the twin who is AGA in up to 40% of
in UV flow has been detected as early as 16 weeks cases or in neurologic damage in the AGA twin in
gestation. up to 30% of cases [39]. AREDV of the UA in the
IUGR twin also poses an increased risk of fetal [21]
Middle cerebral artery (MCA) Doppler and neonatal death [18]. The MC with selective
MCA peak systolic velocity (PSV) in singleton preg- IUGR were subsequently classified into one of three
nancies has been widely used in the detection of types according to the characteristics of UA Doppler
fetal anemia and has also proved useful in TTTS flow as evaluated on the first examination: type I
following laser therapy [36]. Currently, MCA PSV (positive end-diastolic flow in the UA), type II

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Y.L. Chang

(AREDV constantly observed during the examination) placental share in order for the F/P ratio to reach
or type III which was defined as the clear observation statistical significance. In these IUGR twins, their
of AREDV alternating with a short period of positive birth weights are low, but their placental weights
diastolic flow, in the absence of fetal and maternal are even lower. The discordance in estimated fetal
breathing. This classification may be of help in clin- weight by ultrasound detected before birth did not
ical decision making and when comparing clinical reflect the degree of discordance in placental shar-
studies [21]. However, in our experience, only AREDV ing in such cases. So, by studying the F/P ratio in
in the IUGR twin has prognostic value [18]. MC with selective IUGR, we have found that the
etiology of IUGR in one twin can mainly be attri-
buted to the small placental factor. However, a
Individual Fetal/Placental Ratio in smaller placenta territory does not necessarily make
MC with Selective IUGR the twin smaller. If the placental territory is small
enough, then the affected twin would have IUGR.
The placenta, as the maternalfetal interface, is In MC with selective IUGR, the F/P ratio of the
responsible for the exchange of oxygen and all IUGR twin is significantly higher than that of the
nutrients between the mother and the fetus, and is, AGA counterpart, so the fetal weights are not pro-
thereby, a principal determining factor in fetal birth portional to the placental masses of the twins. The
weight. Because placental weight and birth weight discordance in estimated fetal weight by ultrasound
are available on patient records, these parameters detected before birth does not reflect the degree
are accessible. In general, placental size and fetal of discordance in placental share.
size are directly related and experimental reduction
of placental size by uterine carunclectomy prior to
pregnancy has been shown to result in smaller Management of MC with Selective IUGR
fetuses in sheep [40]. In MC, the placental share
plays a role in the ratio of intertwine birth weight; Current management of MC with selective IUGR
the exact placental share between the two fetuses involves expectant management and early delivery
can only be detected after delivery by placenta ex- (if warranted), termination of pregnancy, selective
amination. Therefore, prenatal diagnosis of unequal laser photocoagulation of communicating vessels
placental sharing is not possible, but the signifi- or umbilical cord occlusion [39]. Most cases of MC
cant intertwined birth weight discordance can be with selective IUGR are managed by expectant
predicted by sonographic examination [33]. The management. The key steps are close monitoring
weight of placentas from small-for-gestational age and prompt delivery. There have been reports that
infants has been reported to be reduced when the use of daily biophysical profiles to monitor such
compared with those of AGA infants. However, cases can prolong pregnancy with an acceptable
changes in the fetal/placental (F/P) ratio in single- perinatal outcome [42]. In our hospital, MC with
tons depends on gestational age at delivery, and selective IUGR are managed conservatively; for those
even at the same gestational age, the ratio varies MC with selective IUGR and normal IUGR twin UA
quite widely [15,41]. We have found that the indi- Doppler, follow-up visits in the clinic at 2-week
vidual F/P ratios between the twin fetuses in MC intervals and patient education to self-monitor
with selective IUGR were significantly different [18]. fetal movement and check daily body weight are
The F/P ratio in the IUGR twin was significantly encouraged. If fetal movement decreases or the
larger than that in the AGA twin. The fetal weights UA Doppler shows abnormal results, then hospital
were not proportional to the placental mass in the admission is required. In cases with abnormal IUGR
twins; the degree of birth weight discordance has twin UA Doppler, these cases are admitted to hospi-
to be smaller than that of the discordance in the tal after 2426 weeks of gestation. After admission,

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Monochorionic Twin with Selective IUGR

than in TTTS, owing to a lack of adequate operat-


ing field. In unfamiliar hands, this could make the
condition worse.
If the diagnosis of MC with selective IUGR is
detected relatively early in the gestational period,
Repeat deceleration of the fetal and the IUGR twin faced impending death, then
heart rate of the IUGR twin
selective feticide of the IUGR twin can be per-
formed. This would reduce the risk of exsanguina-
tion in the AGA twin and could be performed by
bipolar cord coagulation, ligation of the cord or
Fig. 2. The fetal heart beat monitor in the monochorionic twin laser photocoagulation. Laser photocoagulation to
with selective intrauterine growth restriction (IUGR). The fetal occlude the cord can usually only be performed at
heart beat of the IUGR twin showed repeat variable deceleration. a relatively early gestational age because of the
cord size. At a later gestational age, cord occlusion
patients are monitored by continuous fetal moni- by bipolar coagulation or ligation under fetoscopic
toring, if repeated fetal heart beat deceleration is guidance can be performed.
detected (Fig. 2) (usually detected in the IUGR The management of MC with selective IUGR is
twin), then corticosteroids are given to improve currently still a dilemma; however, close monitor-
fetal lung maturity followed by prompt delivery. In ing and prompt delivery are the key steps in man-
our preliminary report [18], MC with selective IUGR agement. The differential diagnosis between MC
with normal IUGR twin UA Doppler had a 100% with selective IUGR and TTTS is important, because
survival rate and had a perinatal death rate of 20% TTTS has more agreeable management methods
when the UA Doppler of the IUGR twin was abnor- [7]. Laser therapy of the communicating vessels
mal. The perinatal deaths in these cases of abnormal between the two fetuses in MC with selective IUGR
IUGR twin UA Doppler were not confined to the is more difficult than in TTTS, and can only be
IUGR twin. Some of the MC with selective IUGR performed by experienced personnel.
would progress to TTTS after only weeks of follow-
up. These fetuses usually had abnormal IUGR twin
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