Diagnosed with SIUGR

DIAGNOSED WITH SIUGR

What is SIUGR?

SIUGR, selective intrauterine growth restriction, is a form of TTTS.  It is defined as one of the twins being under the 10% for growth and more than a 20% size difference between the babies.  It basically means that there is an unequal share of the placenta.  Growth charts are usually based on singleton pregnancies.  To get a more accurate percentile you can enter the date into this calculator for a twin growth chart developed after analysis of over 2000 twin pregnancies https://www.twingrowth.co.uk/

The placenta is the babies’ source of nutrition and oxygen. Normally, you are pregnant with one baby and one placenta.  With monochorionic twins, there is one placenta that is shared by two babies.  This takes place 4-6 days after conception when the embryo miraculously splits to create two identical babies in their own amniotic sac (monochorionic, diamniotic).  At this time, their umbilical cords randomly insert into their shared placenta ‘claiming their space’ so to speak.  This random cord insertions play a huge role in monochorionic twin pregnancies because if they both implant in the center of the placenta, you have won half the battle in your pregnancy as they will share the placenta fairly equally.

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OUR LETTER TO PARENTS

Diagnosed with SIUGR

We have been where you are and understand.  We are all here to help you and it is our honor.

Types of Cord Insertions

When there is a discordance in size, it is a warning sign that cord insertion is playing a role.

  • Central-Central Cord Insertion
    When both umbilical cords attach to the placenta in the center of the placenta, it is very promising that they will not have any growth restrictions. You would predict growth to be similar for each baby.  Even with central-central cord insertions, a size difference could occur if TTTS were to develop, but could also improve if the TTTS was treated.The only problem that can happen with central-central cord insertions is if the cords are too close together where a laser doctor feels there is not enough space between the cords to laser the shared vessels. Our advice if this were to happen is to get a second opinion as experience varies among doctors.
  • Central-Marginal Cord Insertion
    When one baby’s cord is attached in the center of the placenta, it has a good placental share.  A  marginal cord Insertion, however, is way to the outer edge of the placenta and will have much less placental share.
  • Marginal-Marginal Cord Insertion
    Both umbilical cords are implanted at the edge of the placenta Central-Velamentous Cord Insertion Even of more concern would be a velamentous cord insertion when the umbilical cord inserts into the amnionic sac or membrane and the blood vessels travel have to travel from that insertion point to the placenta.

Blood Vessels in the Monochorionic Placenta

To understand the blood vessels in the placenta, we use the example of a capital “H”.  The outer lines of the capital “H” represent the independent blood vessels that only go to Baby A and only go to Baby B.  The middle line of the capital “H” represents the shared vessels connecting the babies’ circulations.

The combination of independent blood vessels together with shared blood vessels is unique to every single placenta making each pregnancy with monochorionic twins unique.  Adding to the types of blood vessels would be how they flow (artery to artery, vein to vein and artery to vein).

So, you have the percentage of independent blood vessels that each baby has that determines their placental share, the number of shared vessels that connect their circulations, and the direction of the flow of the shared vessels (artery to artery, vein to vein and artery to vein). 

Figure 2. This is a placenta from a TTTS pregnancy treated with reduction amniocenteses that delivered at 27 weeks’ gestation. The donor and recipient sides of the placenta are labeled, and their umbilical cords are seen with plastic clamps attached. This placenta was equally shared by the twins and contained three artery-to-vein connections (arrows) between the twins which joined in the region of the vascular ‘equator.’ (Photo courtesy of Dr. Julian E. De Lia)

DIAGNOSED WITH SIUGR

For an In-Depth Understanding of the Monochorionic Twin Placenta

 

What is Placental Share and Independent Blood Vessels?

What is Placental Share?

After the embryo splits, the umbilical cords for each baby randomly attached to their shared placenta.  It is at this time the placental share to each baby is determined.  Placental share is the percentage of blood vessels that travel from the placenta independently to just Baby A, and the percentage of blood vessels that travel from the placenta independently to just Baby B.  You hope that the placental share is 50-50 to each baby, but it is rare for it to be split perfectly equal.  There are varying degrees of unequal placental share, but with SIUGR, the share can be much more unequal.  As a result, one baby becomes much smaller and slower in growth than the other.  Usually, a size difference between the babies of 20% or less is considered normal.  SIUGR can be a reason that the size difference would be higher.  You can’t change placental share, but sometimes the shared vessels in the placenta can help direct more blood flow from the larger baby to the smaller baby to help it get to a gestational age that is safer for delivery.

What are the shared vessels?

These are the vessels that do not make up the placental share, but are vessels that cross the placenta connecting the vein of one baby to the vein of the other (V-V0, the artery of one baby to the artery of the other (A-A), these are the connections that benefit the smaller baby and give it more blood, and the artery of one baby to the vein of the other.

These are called shared blood vessels, connections or anastomoses all meaning vessels that create a situation where the twins are circulating blood between each other.  All monochorionic twins have shared vessels, but not all twins are negatively affected if there is a balance to the back and forth of the blood between the babies.

With TTTS, the shared blood vessels are the connections that are cauterized or sealed shut during laser surgery for TTTS, and the goal is always to cauterize all the connections (you cannot leave the artery to artery that benefit the smaller baby).  With SIUGR, this same surgery can be done in more serious situations when there is consistent abnormal  blood flow in the umbilical cord.

What is crucial to understand with SIUGR, however, is that after laser surgery, the babies revert back to their placental share only, meaning they only have their independent blood vessels to continue to get oxygen and nourishment to grow.  The risk is whether or not the baby will have enough placental share after the surgery to grow to a safe gestational week to deliver.

Whether to decide to do laser surgery involves serious consideration and knowledge of their babies’ situation as every placenta is unique.  Some moms decide not to do the surgery for fear of losing the smaller baby and opt for hospital monitoring and early delivery.

Some moms decide to move forward with the surgery if the smaller baby is very affected and it may also be also affecting his or her twin.  If so, the specific prayer at this point is for the smaller baby to have enough placenta share afterwards to get to a safer gestational week to deliver.

Create your Medical Plan of Action Today

DIAGNOSED WITH SIUGR

How is SIUGR Diagnosed?

SIUGR is Diagnosed by Ultrasound

As stated in the pregnancy section, ultrasounds should take place between 6-8 weeks of pregnancy to determine if there is one placenta or two.  By 16 weeks, the ultrasounds must be weekly with a high-risk perinatologist or maternal-fetal medicine specialist (MFM) to look for the signs of SIUGR, TTTS, TAPS and TRAP.

Many doctors may feel every two weeks is sufficient, but it is not.  Two weeks is appropriate to get a good weight estimate, but it is a lifetime to wait to monitor for the warning signs of TTTS including TAPS and  SIUGR.  TRAP should be diagnosed or ruled out by 16 weeks if not much earlier.

What are the Warning Signs of SIUGR?

The warning signs of SIUGR include a weight difference of the twins of 20% or more together with the smaller baby in the 10th percentile or under for average size for that gestational age. They types of SIUGR are determined by the differences in the blood flow patterns of the smaller baby as seen by doppler ultrasound of the umbilical cord. Depending on the results of the warning signs seen, SIUGR can be diagnosed and is staged in Types 1-3.

You might also see the smaller baby have decreased amniotic fluid. When a baby becomes very malnourished from lack of placental share, his or her body will focus on all nutrients (blood) to go to the heart and brain of the baby and to the kidney last.

Amniotic fluid is basically the urine of the baby, so seeing this warning sign triggers more attention to diagnose SIUGR and make a plan of action to help the baby.  When one twin has normal amniotic fluid and the other below 2cm, you can rule out TTTS, but with a size difference, it would appear to be SIUGR.

You have to continue monitoring several times a week to watch for symptoms to change is not every day in some situations.

What are the Types of SIUGR?

What is crucial to understand with the staging of SIUGR is that most understand staging to become more serious as the numbers progress.  With SIUGR, Type 2 is worse than Type 3!  For now, it is Type 2 where surgery is considered as an intervention.  The types are classified using the results of the doppler (wave form) in the umbilical artery of the growth restricted twin.

There is tremendous hope for your babies, even if they are diagnosed.  It is going to take a lot of support, endless patience, and tortuous days that feel like years, but keep going.  You are not alone, and we will help you!

  • Type 1 SIUGR
    The ultrasound will show a positive end-diastolic flow, consistent forward  flow, in the umbilical artery of the growth-restricted twin.  Increase ultrasounds are needed to monitor the babies blood flow patterns.  Babies with Type 1 can do very well, and remain stable with most likely an earlier delivery due to the smaller share of the placenta, but a small percentage will progress to abnormal dopplers and might need more intervention.  The babies probably have a placental share such as 60-40 and there are probably have less shared vessels in the placenta that could benefit the smaller baby than in an undiagnosed monochorinic placenta.
  • Type 2 SIUGR (the most serious type)
    The ultrasound will show persistent, all the time, absent end or reversed end diastolic flow in the umbilical artery of the growth-restricted twin. The blood flow is either persistently absent in the artery or persistently flowing in a reverse direction, away from the smaller baby.  Laser surgery may be an option to try and reverse the abnormal blood pattern, but it is not to be taken lightly as the baby may not have enough placental share after the shared connections are cauterized.Type 2 has less placental share than Type 1.  Doctors consider offering laser surgery at Type 2.  With or without laser surgery frequent monitoring and eventual bed rest in the hospital until delivery, given steroid shots and other help for a premature birth, is what many moms choose to do.

    The goal is to deliver at the moment the smaller baby runs out of its share of the placenta and goes into distress and you need to be in the hospital to do that.  You never want to try and ‘buy time’ if you see that a baby has stopped growing.

    If a baby is not getting enough blood, it is not getting enough oxygen and that is how cerebral palsy can happen or the baby could pass away.  The baby in Type 2 SIUGR will have even less shared connections between babies in the placenta that might have helped compensate for the smaller placental share.

  • Type 3 SIUGR
    The ultrasound will show intermittent, or occasional, absent or revered end-diastolic flow in the umbilical artery of the growth restricted twin, and there is some forward flow.  With placental analysis, it shows that the smaller baby has the smallest placental share of all types, but there is an increase in the artery to vein connections from the larger twin’s side of the placenta to the smaller baby to help increase blood flow to the baby.There may also be a very large artery to artery vessel that can make sudden shifts in blood pressure in either directions in the placenta that is of concern and again, the babies need to have increased monitored several times a week with eventual admission to the hospital until delivery to determine the moment the babies need to be delivered.
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What to look for

Fetoscopic view of the collision front within a placental arterio-arterial anastomosis between monochorionic twins. Here shows a visual depiction of one cause of umbilical artery intermittent absent end diastolic flow in these types of twins.  This video is courtesy of Dr. Ramen Chmait, a Medical Advisory Board Member of The TTTS Foundation

The TTTS Foundation’s Most Important Questions

We have developed the most important questions to ask your doctor at each ultrasound combining TTTS, including TAPS, SIUGR and TRAP. The answers to these questions should give you information about all forms of TTTS. Ultrasounds should start by 6-8 weeks and become weekly at 16 weeks through delivery.

This is when everything is perfect. If there is any concerns about the weights, fluid levels or dopplers, you must be seen several times a week, if not in some cases be admitted for 24 hour monitoring in the hospital. Take these questions extremely seriously and fight for weekly ultrasounds. You may hear over and over that every 2 weeks is sufficient, but it is not. We have helped too many families who have lost one or both babies in that time period. We are fighting to help save your babies lives.

Change will only happen when parents go back to their doctors and demand the care their babies need.  We all want to hear that everything is fine and there is no need to do this or that. But, the truth is that the only way to have that reassurance at that moment is through the answers to these questions and getting the measurements that all the specialist in laser surgery will want to know.  We want you to know what a laser doctor wants to know.

Always ask for your medical records, get copies of your ultrasound videos and especially if you have laser surgery, get a copy of the surgery.  You own your medical records, and we want to bring this to your attention, so you have all the information you need to help you communicate with and have a record what your babies are going through.

Dr. De Lia Conferernce

DIAGNOSED WITH SIUGR

Read The TTTS Foundation's 15 Most Important Questions You Need To Ask Your Doctor Today

What are the Treatments of SIUGR?

The TTTS Foundation highly stresses weekly ultrasounds during a monochorionic pregnancy when everything is perfect.  When there is an unequal placental share, ultrasounds may need to be more frequent to monitor the fluid levels and dopplers to see if there is progression of growth discordance and/or  movement toward TTTS.

Laser surgery for SIUGR has to be taken very seriously for fear the smaller baby may not have enough placental share to grow after the shared vessels are all sealed.  If both babies start to become affected and the smaller baby has continuous reverse flow, the laser surgery may become an option to correct the abnormal dopplers. Laser surgery would always be to try and save both babies.

Twins with unequal placental share often have artery to artery (AV) connections  which can help bring more blood flow to the smaller baby and be a protection against TTTS.  It is a reason to be very careful in a decision of laser surgery.  The symptoms in the baby must be very serious, because all vessels must be cauterized to completely disconnect the babies during this procedure including the AA connections that provide extra blood.

Differences between TTTS and SIUGR?

SIUGR is an unequal placental share causing growth restriction in one of the babies.  TTTS is an unequal sharing of volume of blood through the babies shared placenta causing an amniotic fluid difference.  TTTS can cause a size difference of the babies, but with SIUGR the baby is much smaller under the 10th percentile for growth.

With TTTS, the babies could have a size difference due to one getting more blood volume and the other too little blood volume, yet still have a fairly equal placental share.  In that situation, once the TTTS could be treated, the babies would then get normal blood flow and the size difference could improve.

You can absolutely have SIUGR and TTTS at the same time and experience TAPS as well.  This is why The TTTS Foundation is education you on ALL forms of TTTS.

It's Important to Have Your Placenta Analyzed

Important Guidelines in Your Pregnancy with SIUGR

  1. Contact The Foundation
    Whether you need guidance from our board of medical directors, who are top physicians in sIUGR and TTTS, or financial assistance for travel to gain better access to care, we are here to help! You are not alone in your journey; we offer a community of other families who have experienced this type of pregnancy are able to offer emotional support.  Read more about our SIUGR Coordinator Carolyn here.
  2. Gather Information
    It is important to gain as much knowledge as you can on the topics of TTTS and sIUGR so that you are able to advocate for your family and your babies. Read through out site, join our Facebook Group and reach out to us personally for help and guidance!
  3. Learn About Questions To Ask Your Doctor
    You may feel overwhelmed and anxious with the news of this prenatal diagnosis. Download our Most Important Questions and bring it to your next appointment so that can ask the right questions and learn more about your current condition.
  4. Define The Stage Of SIUGR
    This will better prepare you for what to expect in terms of prognosis as well as give you a starting point for monitoring. Click *Here* to download a summary of the stages to take to your appointment.
  5. Monitor
    Make sure that you are being followed frequently as subtle changes can occur at any time. You don’t want to miss an opportunity to intervene if possible.
  6.  Discuss Delivery Plan
  7. Talk to your Maternal Fetal Medicine (MFM) specialist about what parameters will be the safest to deliver according to size and gestational age.Typically, the longer the babies stay in utero, the safer the delivery, however, there are certain circumstances where it is safer for babies to be delivered and the mothers to be hospitalized until birth.

It is important to have a plan so that you are prepared to advocate for your family and informed on what to expect in the coming days. We are here to support you in any way you may need.

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Dominick and Damian

Carolyn went through TTTS and SIUGR and is now The TTTS Foundation’s SIUGR Coordinator providing hope and compassion to newly diagnosed families out of love for her twin sons.