What is selective intrauterine growth restriction (also called selective fetal growth restriction)?
Monochorionic twins are twins that share a single placenta. Selective intrauterine growth restriction (sIUGR), also called selective fetal growth restriction (sFGR), occurs when there is unequal placental sharing which leads to suboptimal growth of one twin. In cases of sIUGR, the estimated fetal weight of the smaller, growth-restricted twin usually falls below the 10th percentile. This will usually result in more than a 25 percent weight difference between the twins.
sIUGR is estimated to occur in approximately 10 percent of monochorionic twin pregnancies. There are three types of sIUGR that are determined by the blood flow pattern in the umbilical artery of the growth-restricted twin.
Causes of sIUGR
The principle cause for the development of sIUGR in monochorionic twins is unequal placental sharing. The growth-restricted twin has a smaller share of the placenta, which over time results in abnormal blood flow and less growth. This could lead to death of the growth-restricted twin. Because the shared placenta also contains shared blood vessels between the twins (vascular communications), sIUGR can also impact the development of the brain and nervous system in the normal twin (neurodevelopment).
Symptoms of sIUGR
There are no physical symptoms of sIUGR that you, as a mother, would feel. sIUGR is a diagnosis made exclusively through ultrasound examination.
Evaluation and diagnosis of sIUGR during pregnancy
Accurate diagnosis is extremely important in distinguishing sIUGR from other diagnoses such as twin-twin transfusion syndrome (TTTS). TTTS and sIUGR both involve a shared placenta, but are differentiated by amniotic fluid levels.
The differences between the two can be very subtle. In TTTS, there is a characteristic and progressive series of changes that happens to each twin. In sIUGR, the distribution of blood is more balanced, but because the growth-restricted twin has a much smaller portion of the placenta, the resistance in the umbilical artery is higher than normal, resulting in suboptimal fetal growth.
Watch this video to learn more about the differences between TTTS and sIUGR and how they are diagnosed.
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Mark P. Johnson, MD, MS: Sometimes families come to us with the diagnosis of TTTS, and it turns out to be something more like Selective Intrauterine Growth Restriction or sIUGR. TTTS and sIUGR are related because they involve the same common shared placenta between the two babies. What differentiates them is the vascular connections between the two babies.
Jack Rychik, MD: Often the differences between sIUGR and Twin-to-Twin Transfusion Syndrome can in fact be very subtle. Through the use of Doppler ultrasound, we can help tease out the facts that support one particular diagnosis versus another. And through Doppler echocardiography, we can identify the direction of blood flow the velocity of blood flow, and also determine patterns of blood flow.
Mark P. Johnson, MD, MS: One of the things that we look at, of course, is cardiac changes. In TTTS, there is a very characteristic progressive series of changes that happens in the larger twin, due to the extra volume that comes from the smaller twin to the larger twin.
Jack Rychik, MD: Blood is exchanged from one twin, the donor, into the recipient, and then as a consequence, there's a huge cascade of hormonal changes that take place, that then brings about the cardiovascular manifestations in the recipient.
Mark P. Johnson, MD, MS: In selective IUGR, there isn't this transfer of volume. You know, there's not this net shift of volume from one twin to the other. It's much more balanced. But that balance, you know, can be a tenuous balance.
Jack Rychik, MD: In the smaller twin, there is an abnormality of placental sharing.
Mark P. Johnson, MD, MS: The smaller baby has a much smaller portion of the placenta.
Jack Rychik, MD: And the resistance in the umbilical artery of that particular twin, is much higher than normal, resulting in alterations in growth in the smaller twin.
Mark P. Johnson, MD, MS: Selective intrauterine growth restriction has been recognized for a long time, but it's only been in the last decade that we've been able to see that there are different forms of it.
There's a milder form, which we call type 1. There's a more severe form that we call type 2, and then there's a new entity that's really only becoming understood, that's come out in the last few years. It's the type 3, selective IUGR. So if we were to look at a normal placenta, there would probably be a large number of connections between both fetuses.
There's kind of a sense of balance there, so that shifts in blood in one direction would be offset by shifts in blood in the other direction. In type 1 selective IUGR, what you notice is perhaps a 60/40 distribution of placental area, but you see a decrease in the number of connections. That means that there can't be as dynamic a shift in blood volume between the two twins.
When you go to the type 2 selective IUGR, you start to see a much smaller portion of placenta for the one that develops the intrauterine growth restriction. You also notice that the number of vessels, again, decreases even more. They tend to be balanced so that the number of artery to vein connections from the smaller to the bigger is still offset by artery to veins in the other direction, but the numbers are much, much fewer.
And so again, it's this idea of dynamic sharing that is more restrictive, and that forces the fetus with the smaller portion of placenta to really try to survive on what it's got as far as placental mass. And the less volume of the placenta it has, the more it struggles. In the type 3 selective IUGR, they have just small, small proportion of the placenta.
There tends to be a higher proportion of arteries connecting to veins, from the normal baby to the smaller baby, but it's a very small placental area, and that's led to the concept of rescue transfusion, the larger baby being able to send blood to the other side of the placenta. And so those connections are absolutely vital for the smaller baby's survival.
The other characteristic feature in type 3 selective IUGR is a very big artery to artery connection. There can be rapid shifts in blood in either direction. These high volume shifts of blood from one baby to the other, result in the blood pressure going up and going down and going up and going down, and that appears to result in injury.
So while this connection is vital to keeping the smaller baby alive, it can potentially result in a brain injury to the normal baby, and it can actually kill the smaller baby because of this just sudden rapid shift in blood pressure and volume. So in type 3 selective IUGR, it's a very, very dependent and very, very dynamic relationship between the two twins and the connections in the placenta.
Jack Rychik, MD: The science and the practice of managing twin complications such as twin to twin transfusion syndrome or sIUGR perhaps, in effect has only existed for about a decade.
Mark P. Johnson, MD, MS: Understanding the TTTS story has been vital in our understanding of how to differentiate that problem from the selective IUGR problem.
Jack Rychik, MD: High volume exposure to these patients is critical in being able to learn what to expect.
Mark P. Johnson, MD, MS: That high volume, that experience, and that collaboration is what makes us a really good place and allows us to really tailor management for each individual patient to optimize and try to achieve the best outcome possible.
If you are referred to CHOP’s Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment (CFDT), you will be scheduled for a comprehensive, one-day evaluation. During this first visit, you will meet with multiple team members, including a genetic counselor, nurse coordinator and maternal-fetal medicine specialist. You’ll also meet with a research coordinator to discuss ongoing research studies.
Your evaluation will include a high-resolution fetal ultrasound and fetal echocardiogram (a focused ultrasound that provides a detailed view of the fetal heart, performed by our fetal cardiology experts) to identify the direction, velocity and patterns of blood flow in specific blood vessels. This detail can help our experts distinguish between TTTS and sIUGR and confirm your diagnosis.
Your test results and treatment options will be discussed in detail on the day of your evaluation. If fetal intervention is recommended, you can also expect to meet with an advanced practice nurse to complete preoperative testing, and a social worker to arrange your temporary accommodations. Our team is here to help and support you and your family throughout this experience.
“When we came to you, we had been told that our little girl had no chance of survival. We wanted to thank you for giving us a bit of faith that our little girl could make it through. Sydney was born on May 8, one of the many lives I’m convinced you have saved. Words cannot express how much we owe our happiness to you!” — Jamie, about her sIUGR pregnancy
Types of sIUGR
sIUGR has been classified into three types based on specific blood flow patterns in the umbilical artery of the growth-restricted twin.
Type 1
- Consistent forward flow in the umbilical artery of the growth-restricted twin
- Average age at delivery is 34-35 weeks gestation
Type 2
- Either persistent absent blood flow or persistent reversal of blood flow in the umbilical artery of the growth-restricted twin
- Average age at delivery is 26-28 weeks gestation
Type 3
- Unpredictable pattern of intermittent blood flow in the umbilical artery of the growth-restricted twin (forward, absent and reversed)
- Average age at delivery is 30 weeks gestation
- In up to 15 percent of cases, the growth-restricted twin may not survive, which can also impact the neurodevelopment of the normally growing twin
Treatment for selective intrauterine growth restriction
Management of sIUGR may include continued observation with ultrasound surveillance or fetal therapy. Your recommended treatment will depend upon the type of sIUGR your twins are diagnosed with.
Type 1 sIUGR
You will undergo continued weekly observation with your local maternal-fetal medicine specialist. Weekly or twice weekly ultrasounds closely monitor the blood flows of both twins and watch for progression to Type 2 or Type 3 sIUGR which may indicate prompt referral for fetal therapy. Interval growth scans are also performed locally.
Because preterm deliveries are common in pregnancies affected by sIUGR, consultation with neonatology and a specialized delivery center can help you plan for this possibility.
Type 2 and Type 3 sIUGR
Along with at least weekly observation by your maternal-fetal medicine specialist, we may offer fetal therapy in the form of selective cord occlusion.
Selective cord occlusion is a minimally invasive surgical procedure that seeks to improve the outcome for the normally growing twin by stopping the blood flow to the growth-restricted twin in a way that minimizes the impact on the neurodevelopment and survival of the normally growing twin. Selective cord occlusion can be performed using bipolar cord coagulation (BCC) or radiofrequency ablation (RFA) procedures.
Volumes & outcomes
Our team at the Wood Center for Fetal Diagnosis and Treatment cares for a high volume of monochorionic twin pregnancies each year. Since 1995, more than 4,978 complicated multiple gestation pregnancies have been referred to the Center for Fetal Diagnosis and Treatment.
This experience helps to continuously expand our understanding of these complex pregnancies and our ability to differentiate between conditions such as sIUGR/sFGR, TTTS, twin anemia polycythemia sequence (TAPS), and twin reversed arterial perfusion (TRAP) sequence.
Tour our Fetal Center
The Wood Center for Fetal Diagnosis and Treatment has cared for many families and will help you through your journey, too.
What to expect
From the moment of referral through delivery and postnatal care, your family can expect a supportive experience when you come to us with a diagnosis of a birth defect.
Resources to help
Selective Intrauterine Growth Restriction (sIUGR) Resources
Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment Resources
Learning your baby has a birth defect is a life-changing experience. We want you to know that you are not alone. To help you find answers to your questions, we've created this list of educational health resources.
