Fetal Conditions Treated

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Selective Intrauterine Growth Restriction (SIUGR)

Although most pregnancies with Selective Intrauterine Growth Restriction (SIUGR)

also called monochorionic twins (twins that share a common placenta) are uncompli-
cated, the presence of a common placenta does pose a relatively increased risk to the
welfare of the fetuses. The single placenta contains blood vessels that link the blood
flow between the twins. Unbalanced flow of blood from one twin to the other twin may
lead to a cascade of events that result in twin-twin transfusion syndrome (further infor-
mation regarding this syndrome is detailed herein: Twin-Twin Transfusion Syndrome).
Another potential problem that may occur in monochorionic twins is the dispropor-
tionate distribution of placental mass between the twins. This factor may result in poor
nourishment of one of the twins, resulting in subsequent poor overall fetal growth.
Because this problem typically affects only one of the fetuses, this condition has been
coined selective intrauterine growth restriction (SIUGR). SIUGR is estimated to occur
in approximately 10% of monochorionic twin pregnancies.

Severe cases of monochorionic twins with SIUGR show ultrasound evidence of ab-
normal blood flow through the umbilical artery of the poorly grown twin. In this cir-
cumstance, spontaneous death of this baby within the womb may occur in up to 40%
of cases. Because of the blood vessels that link the twin’s circulatory system together,
death of one twin may result in severe drop in blood pressure of the other twin and
subsequent brain damage (up to 30%) or death (up to 40%). This complication results
from the hemorrhage of blood from the appropriately grown twin into the demised
SIUGR twin.

Because the adverse effects to the appropriately grown twin is mediated through the
blood vessels that link the circulations of the twins, it has been suggested that oblit-
eration of these vascular communications may result in improved outcomes for the
normally grown twin. Separation of the circulations may be done using the surgical
techniques which were originally developed for the treatment of twin-twin transfusion
syndrome. In order to test this theory, CHLA-USC Fetal Therapy Program is part of
a multicenter clinical trial spearheaded by Dr. Ruben Quintero in Tampa, Florida, to
evaluate the outcome of this condition managed expectantly as compared to laser
therapy.

Diagnosis
The in utero diagnosis of SIUGR is established by ultrasound. First, the presence of
a monochorionic twin gestation should be confirmed. Usually ultrasounds performed
earlier in the pregnancy may be useful in establishing the chorionicity (number of pla-
centas). Ultrasound findings such as a single placenta, same fetal sex, and a “T-sign”

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 Selective Intrauterine Growth Restriction (SIUGR)

in which the dividing membrane inserts perpendicular to the placenta are helpful in diagnosing a
monochorionic twin gestation.

Once a monochorionic placentation has been established, the diagnosis of SIUGR requires the pres-
ence of two important ultrasound findings:

1. The estimated fetal weight (EFW) of one twin measures less than the 10th percentile for the as-
signed gestational age. The EFW is calculated by measuring standard fetal biometric components
via ultrasound. Because prior studies have shown negligible differences between growth curves for
singleton and twin gestations in the second trimester, standards as established by Hadlock (1991)
for singletons are used to assign the growth percentile.

2. Persistent absent or reversed flow in the umbilical artery of the growth-restricted twin.
Finally, the diagnosis of twin-twin transfusion syndrome (TTTS) must be excluded. TTTS is diag-
nosed by assessing the discordance of amniotic fluid volume on either side of the dividing fetal
membranes; the maximum vertical pocket (MVP) of amniotic fluid volume must be greater than or
equal to 8.0 centimeters in the recipient’s sac, and less than or equal to 2.0 centimeters in the do-
nor’s sac to secure the diagnosis of TTTS.

Management Options and Outcomes

The treatment options along with expected pregnancy outcomes are listed below:

1. Expectant Management: Prior to the development of the laser therapy outlined below, the treat-
ment of this condition has been traditionally one of expectant management. This entails at least
weekly ultrasound assessments of fetal well-being, amniotic fluid volume assessment, and Dop-
pler studies of the umbilical artery, as well as sonograms to assess fetal growth about every three
weeks. After 24 weeks’ gestation, parents traditionally discuss with their physicians whether there is
a need for increased fetal surveillance, such as fetal heart rate monitoring, and if a course of steroids
is required for fetal maturation therapy. Early delivery may be decided if fetal status is deemed non-
reassuring based on fetal heart rate monitoring or ultrasound parameters. The challenge that this
condition presents to parents and physicians alike is in regards to the timing of delivery. On the one
hand, delay of delivery will reduce the complications associated with premature birth. On the other
hand, prolongation of the pregnancy in this setting, particularly if findings suggestive of a nonreas-
suring fetal status are present, may result in the demise of one twin in the womb. This may occur in
up to 40% of monochorionic twins with SIUGR. As described above, the death of one twin while in
the womb may result in the concomitant demise of the other twin in as high as 40% of cases. If the
other twin does survive, there is up to a 30% risk of subsequent neurologic handicap. The demise of
a twin results in these adverse effects on the other twin because of the blood vessels on the surface
of the placenta that connect the circulatory systems of the babies – essentially linking the livelihoods
of each baby to one another.

2. Laser Therapy: This surgical approach utilizes an operative fetoscope to deliver laser energy
that then seals off the offending blood vessels on the surface of the common placenta. Because the
vascular connections between the two fetuses are sealed, no further blood exchange between the
fetuses takes place. It has been theorized that elimination of the vascular communications may de-
crease or prevent harm to the surviving twin in the case of the demise of one twin. The magnitude of
 Selective Intrauterine Growth Restriction (SIUGR)

this potential benefit is unknown. A preliminary study comparing the outcomes of patients followed
with expectant management versus those that underwent laser therapy did not show a difference in
survival or in complications of the babies. However, this study was small and did not involve patients
equally. Because the best treatment approach for this condition remains unclear, a multicenter group
has set out to determine the best approach by conducting a prospective randomized trial of expect-
ant management versus laser therapy. Thus, patients identified as meeting criteria (see below) will
be asked to participate in this study. Eligible patients will be randomized (arbitrarily chosen) to either
expectant management versus laser therapy by the Data and Safety Monitoring Committee (DSMC)
site at Wayne State University, Detroit.

3. Umbilical Cord Occlusion: This procedure utilizes an operative fetoscope to interrupt the flow of
blood through the umbilical cord of one of the fetuses. This fetus dies and remains inside the uterus
for the duration of the pregnancy. The CHLA-USC Fetal Therapy Program does not offer this proce-
dure for this condition.

4. Interruption of the Pregnancy: Pregnancy termination may be chosen as an option up to 24

weeks gestation in California. The CHLA-USC Fetal Therapy Program does not offer this procedure.

Candidacy for Treatment

To qualify for participation in this study, the following conditions must be met:

Inclusion Criteria:

1. Gestational age 16-26 weeks

2. Sonographic evidence of monochorionicity
3. Diagnosis of IUGR present in one twin (fetal weight at or below the 10th percentile for gestational
age (Hadlock et al 1991))
4. Absent or reverse-end diastolic flow in the umbilical artery in the SIUGR twin

In Utero Fetal Weight Standards at Ultrasound Percentiles (g)

 Selective Intrauterine Growth Restriction (SIUGR)

Exclusion Criteria:
1. Presence of twin-twin transfusion syndrome defined as a maximum vertical pocket (MVP) of =2
cm in one sac and MVP of =8 cm in the other sac

2. Presence of major congenital anomalies (anencephaly, acardia, spina bifida) or intracranial find-
ings in either twin: IVH, porencephalic cysts, ventriculomegaly or other findings suggestive of brain
damage

3. Unbalanced chromosomal complement.

4. Ruptured or detached membranes

5. Placental Abruption

6. Chorioamnionitis

7. Triplets

8. Refusal to be randomized or to participate in the study

9. Otherwise eligible, but not able to make financial arrangements

Details of Procedure and Outline of Care During Pregnancy

This is a prospective randomized clinical trial. Patients identified as meeting criteria for the study will
be counseled about the different management alternatives and will be asked to participate in the
study. Eligible patients will be randomized to either expectant management vs. SLPCV at the Data
and Safety Monitoring Committee (DSMC) site at Wayne State University, Detroit.

1. Expectant management. Patients randomized to expectant management will be advised to un-

dergo weekly ultrasound examinations including Doppler studies of the umbilical artery and amniotic
fluid volume. Fetal growth will be assessed every 2-4 weeks. After 24 weeks, patients may undergo
frequent ultrasound examinations or fetal heart rate monitoring to assess fetal well-being. At ap-
proximately 26 weeks, steroids may be administered for enhancement of fetal lung maturity. A regi-
men of bed rest and oxygen therapy will also be suggested either as an inpatient or on an outpatient
basis. Early delivery may be decided by the respective obstetricians if either ultrasound or fetal heart
rate monitoring assessments are not reassuring of fetal well being. Of note, if the ultrasound findings
consistent with twin-twin transfusion syndrome (TTTS) develop prior to 26 weeks’ gestation, then
laser therapy as outlined in the TTTS section of this web site will be offered.

2. Laser Therapy. Patients randomized to laser therapy will be treated will undergo selective laser
photocoagulation of communicating vessels. After appropriate local anesthesia, intravenous seda-
tion, and maternal antibiotics are provided, a 3.8 mm trocar will be inserted under ultrasound guid-
ance through a 2-3 mm skin incision into the amniotic cavity of the normally grown twin. The com-
municating vessels will be identified endoscopically and photocoagulated with YAG laser energy.
Patients will remain hospitalized for 24-48 hours. Follow-up ultrasounds will be scheduled every
week for the first month to detect possible intrauterine fetal demise, and monthly thereafter. Delivery
will be decided based on obstetrical indications.