DMS443Wi2020: Umbilical Cord Masses

Discussion Board Week 3.............

Masses associated with the umbilical cord include the following (not exclusive):

Omphalocele (cord runs through the middle of this mass as it protrudes from the umbilicus)
Gastroschisis (cord usually found to the right of this mass)
Umbilical herniation
Teratoma of the umbilical cord
Aneurysm of the cord
Varix of the cord (may be intraabdominal)
Hematoma of the cord (usually iatrogenic—cordocentesis or amniocentesis)
True knot of the cord
Angioma of the cord (well-circumscribed echogenic mass that may cause increased cardiac failure and hydrops—AFP level is increased; associated with a cyst because of transudation of fluid from a hemangioma)
Thrombosis of cord secondary to compression or kinking, focal cord mass, true cord knots, velamentous cord insertion, cord entanglement in monoamniotic twins (commonly seen with fetal demise)

Discuss what these may look like and their implications-

Hello All,

I am moderating this discussion board this week and look forward to learning with you all. Because the list is long maybe we can each choose a couple and discuss their similarities/ differences and how we could distinguish them sonographically.

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Here's an example of omphalocele versus the umbilical hernia.

An omphalocele is when the abdominal contents fail to return to the abdomen after the normal physiologic midgut herniation. As we know, around the 8th week the midgut herniates out of the abdomen (as it is rapidly growing) and returns by the 12th week. An omphalocele is the result of the intestines, liver, and or other abdominal organs protruding from the abdomen at the base of the umbilical cord contained within a membranous sac. This condition is associated with physical and chromosomal abnormalities and can easily be detected by ultrasound. The umbilical vessels insert directly into the membrane instead of the intact body wall

An umbilical hernia occurs after the normal return of the intestines to the abdominal cavity, but there is a defect that causes a protrusion of a lined sac through the weakened abdominal wall. The difference between an omphalocele and a hernia is that a hernia is covered by subcutaneous tissue and skin.

See the source image

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I got the omphalocele question wrong on the quiz, so I took the liberty of looking up what omphalocele is associated with.

According to Tawain Journal of Obstetrics:

...omphalocele-related disorders: otopalatodigital syndrome type II; Melnick–Needles syndrome; Rieger syndrome; neural tube defects; Meckel syndrome; Shprintzen–Goldberg omphalocele syndrome; lethal omphalocele-cleft palate syndrome; cerebro-costo-mandibular syndrome; fetal valproate syndrome; Marshall–Smith syndrome; fibrochondrogenesis; hydrolethalus syndrome; Fryns syndrome; omphalocele, diaphragmatic defects, radial anomalies and various internal malformations; diaphragmatic defects, limb deficiencies and ossification defects of skull; Donnai–Barrow syndrome; CHARGE syndrome; Goltz syndrome; Carpenter syndrome; Toriello–Carey syndrome; familial omphalocele; Cornelia de Lange syndrome; C syndrome; Elejalde syndrome; Malpuech syndrome; cervical ribs, Sprengel anomaly, anal atresia and urethral obstruction; hydrocephalus with associated malformations; Kennerknecht syndrome; lymphedema, atrial septal defect and facial changes; and craniosynostosismental retardation syndrome of Lin and Gettig. Perinatal identification of omphalocele should alert one to the possibility of omphalocele-related disorders and familial inheritance and prompt a thorough genetic counseling for these disorders.

That's a lot! But medlineplus.gov accessed from the U.S. National Library of Medicine says:

The exact cause for abdominal wall defects is not known.

Infants with an omphalocele often have other birth defects. Defects include genetic problems (chromosomal abnormalities), congenital diaphragmatic hernia, and heart and kidney defects. These problems also affect the overall outlook (prognosis) for the baby's health and survival.

How can we not know what causes omphalocele to occur? Does that mean that all of these "associated" pathologies are just coincidental?

Omphalocele affects the midgut which is mainly stomach and intestines.. so it sounds like the 12th week mark is when things go wrong. Bones are supposed to be ossified developed during this time. So I could see how Melnick-Needles syndrome could be related. Reiger syndrome is a genetic disorder that contributed to underdevelopment of teeth, eyes, etc. Most of the genetic syndromes have to do with underdevelopment. There must be a link somewhere.

I was reading that omphaloceles themselves don't have too many implications and are more worrisome for the genetic syndromes associated. So omphalocele is used as an indication for further global evaluation.

Small omphaloceles can be treated post birth with no intervention prenatally.

Large omphaloceles should be delivered via c-section though at a hospital with an NICU and pediatric surgeon. Natural birth could damage the fetal liver and other vital organs.

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Hello Jacky,

I got the omphalocele question wrong on the quiz as well. =( Although I can identify visually what the difference is between omphalocele and gastroschisis I had a humbling moment during the test where it dawned on me the background on these two did not stick I was confused and picked something else. So I did some research as well, I appreciate you listing all those anomalies associated with omphalocele. I found out it is better for a fetus to have gastroschisis over omphalocele. With gastroschisis the intestines and other organs develop outside the body and never return during normal herniation and are not enclosed in a sac. When the babies are born they are taken immediately to surgery, and all the intestines and organs are put back inside. The babies do not have other related birth defects and eventually grow up normal. However, I found that the fetus that has omphalocele (organs developing outside the body in a sac) is at a high risk for many of the anomalies and disorders you mentioned. Also the sac that the intestines and other organs are in can grow, and cause the mother to have an emergency c-section. Furthermore, having omphalocele (a large one of course) can leave the baby with life long problems such as breathing and feeding restrains due to all the surgeries you typically have with omphalocele.

Here is a video of how the fix gastroschisis by: https://surgery.ucsf.edu/conditions--procedures/gastroschisis.aspx

What will happen after my baby is born?

Preview YouTube video Gastroschisis - Your Operation

Gastroschisis - Your Operation

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Hi Jaz,

That makes sense. I would think that the organs loose during gastroschesis could subject them to impact damage. Or the likely hood of getting twisted or knotted. You said that organs keep growing inside the sac for omphalocele? Doesn't the sac membrane grow too? Otherwise the organs would be constricted.

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Hello Jacky, Yes the sac from the omphalocele is what is growing the organs are just trapped inside. Their is also a series of surgeries that follow omphalocele unlike gastroschisis.

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Hello Ladies,

I love the correspondence between you two and the clarity that came about. I was googling a little bit more just about ompahlocele because it has so many correlations with other disorders and managed to find a video about one families journey with the condition. It is a series of surgeries, but according to his parents in this case the child is genetically normal and doing just fine. I find that encouraging considering all that we are learning about ompaloceles.

https://www.bing.com/videos/search?q=omphalocele+video&view=detail&mid=2F289C7764D78433C48E2F289C7764D78433C48E&FORM=VIRE

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Omphalocele and Gastroschisis are two masses associated with the umbilical cord that I find could be easy to get mixed up. First to differentiate the two, I think of Omphalo-’seal’ having that ‘seal’ around the hernia. The seal is made up from the epithelium of the umbilical cord. Also, with Gastroschisis it is important to know that the cord is found to the right of the mass and that this mass has no seal, causing the AFP levels to be elevated. This would be a useful screening test to differentiate the two masses.

Sonographically, these will both be masses protruding from the umbilicus of the fetus, yet one will have a seal and the other will not. I think color might be helpful here as well to help see where the umbilical cord courses through the mass... is it through the middle or more to the right of the fetus?

Here are two correlating images:

What do you guys think the bottom right (unlabeled) image is depicting?

Image result for ultrasound of omphalocele and gastroschisis

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From this image I could see that our right side is the fetus's body. You can see the three ossifications of the bone. The umbilical cord is in the center and the gastroschisis is on our far left to the side of the umbilical cord.

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If I haven't learned anything else, I have learned that you have to correlate lab values with the findings to create the bigger picture. Today I was gently reminded to do so when I measured a 80 year old's CBD at .4 mm. That in it of itself isn't that abnormal, however, like I said 80 year old patient AND she had a cholecystectomy. Those numbers just don't add up. Her actual CBD measurement was .9 mm...which is completely normal for the circumstances named. This isn't completely correlated to OB but the take away is to consider all things when scanning and even in the moment use critical thinking skills. It is important to always consider the whole picture, lab values, symptoms and how do they fit in with the images taken or the story being told?

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Perresha great reminder! We had a follow up pt that was initially seen Sunday. HCG levels were elevated, irregular shaped gest sac. There was no fetal pole or yolk sac in her gest sac. Her gestational sac measured 15 mm. She was about 5 weeks. She was seen again today. We delayed seeing her because we needed current HCG levels to determine what's going on before we started the exam. Long story short; we found out that she has two healthy kids and this was going to be her 3rd. HCG levels were declined. Her clinical sx- bleeding alot. When I scanned her, there was no trace of a gest sac. She must have passed it. All that I found was an involuted corpus luteal cyst. I thought it was something bad but my CI informed me that it was involuted that's why it appeared that way.. It didn't look like the textbook images we have seen so far, threw me off. Interesting case though. My point was yes labs create a turning point on your next move. My Ci concluded that it was a spontaneous abortion.

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Thank you so much for sharing that personal experience. I think that sharing the things we go through and see help us to realize that it is more than just theory and actually becomes practicum.

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I am selecting varix of the cord versus aneurysm of the cord.

Varix of the cord is a focal dilatation of the umbilical vein. It exceeds 9 mm. This is at the level of the cord insertion. Varix of the cord is when the right umbilical vein persists and the left umbilical vein has become occluded. There is lack of blood flow causing developmental concerns. This is not common. The associations include intrauterine fetal death, chromosomal abnormalities, and hydrops.

See the source image

Aneurysm of the umbilical cord is rare and has high mortality & morbidity. It is a abnormal widening or blood filled bulge in the artery. It also has decreased blood flow. This causes fetal distress. Its associations include fetal demise, aneuploidy, and IUGR. According to "Intechopen" result of compression of the dilated artery on the umbilical vein causes fetal demise.

(https://www.intechopen.com/books/congenital-anomalies-from-the-embryo-to-the-neonate/abnormalities-of-the-umbilical-cord)

See the source image

I think it's helpful using Color flow to differentiate umbilical artery, umbilical vein in reference to the cord insertion. This will enable you to see what is truly enlarging: the dilated / bulging artery versus an abnormal widened diameter of the umbilical vein.

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Hey Charissa, I think you're absolutely right in using color flow to differentiate the vessels, but the color only indicates the direction. I think it would be more important to rely on the pulse wave as a final determinant of vein versus artery. What do you think or how would you differentiate only using color doppler?

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Hey Perresha I would definitely use PW to differentiate my low resistive versus my high resistance index and this would confirm what I am visualizing; vein versus artery.

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Great differentials! A varix of the cord would present with color and flow in only 2/3 vessels where an aneurysm is bulging with decreased blood flow. Both are very serious and thankfully rare anomalies. The umbilical cord is another aspect of the anatomy survey that is easy to breeze by. The cord insertion to the placenta and umbilical are documented along with prooving both arteries around the bladder and then we move on. The free floating cord is more difficult to conisistently see and document. This week I was able to practice doing cord dopplers for IUGR and per our protocol we have to take three points. At both insertions and one in free floating. If either of these anomalies would have been present it would have causes abnormal flow in the cord doppler samples.

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Thanks Heather! Yeah I feel that we need to be on our toes with visualizing the abnormal. Being thorough on sweeps and work overall, especially with a potential varix or aneurysm of the umbilical cord not being common anomalies. I think that's awesome you were able to challenge yourself with the cord dopplers! Definitely, bidirectional flow.

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True knots are knots of the umbilical cord and occur in 1% of pregnancies. Risk factors are polyhydramnios and having a long umbilical cord. Being a small fetus or one that moves a lot also increases the chances of forming a true knot. A monoamniotic twin gestation also increases the risk of true knots due to proximity.

We can recognize it by seeing a tortuous bulging or knotting. Putting on color can help us differentiate a true knot from a false knot which only looks knotted. Part of the cord has to be surrounded by another part of the cord.

I was reading that most knots are loose and will not impact the fetus's health, but if the knot gets tight blood flow will decrease and can lead to fetal distress, fetal asphyxia, and or fetal demise.

Other implications of an umbilical cord knot are focal dilatation of the umbilical vein and or umbilical vein thrombosis which can worsen prognosis towards the above implications.

Here are images of true umbilical cord knot that I got from:

Ultrasound Obstet Gynecol 2004; 23: 99–100 Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/uog.9

In this image above, the top right picture of a loose knot. A loose knot is known as the "hanging noose" sign,

In this image, again you can see the hanging noose sign in the top left. On the middle top image there is an arrow demonstrating how color doppler can indicate a true knot. You can see that the horizontal vein is turbulent inside and not all one color. From this you can deduce that there is pressure acting on the vein. Therefore it must be from a knot.

I was thinking that it can't be from thrombus because the cord is full of color so it must be due to extrinsic factors. We just learned about "jets" in vascular. Same principles apply when dopplering the umbilical cord.

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Hello Jacky,

Great images and explanation on true cord knots! It is kinda scary to think if the knot gets tight blood flow will decrease and than lead to fetal distress, fetal asphyxia, or fetal demise. I noticed you brought up false cord knots, and how we should not get them confused with one another, so I thought I would elaborate on false cord knots to help our class differentiate the two variants.

False cord knots are important to differentiate from a true knots. The actual loops of the vessels in false cord knots are only seen in some ultrasound planes. Exaggerated loops appear as a “bulge,” just like (Ashley mentioned) in the umbilical cord or a knob like protuberance on it. It can be caused from a couple different things such as focal accumulation of Wharton’s jelly or focal cord dilatation. There is not much clinical significance if a fetus should have a false cord knot just another common variant of the umbilical cord. Some health professionals refers to it as the “pseudoknot”. While others describe it as kinking of the umbilical cord. Either way here is a image of a false cord knot in ultrasound.

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Thank you Charissa and Jackie for breaking down true knots versus aneurysm of the umbilical cord. It is helpful to have the sonographic appearance of each, and to think of the "hanging noose" as a sonographic appearance for something that can cause fetal demise is harrowing. Can either of you ladies think of a reason why we would try to use color doppler instead of power doppler in the above mentioned circumstances?

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Hi Jaclyn,

You are right that true umbilical cord knots are formed mostly when the fetus is active, the umbilical cord is long, or there is polyhidramnios. In addition, umbilical cord entanglement can happen with monochorionic monoamniotic pregnancy. In his report, Francois Duchatel, MD represented a case of monochorionic monoamniotic (mono/mono) twins with a big cord entanglement. Both fetuses were eutrophic. “Resistant indexes of the umbilical arteries in both fetuses were normal: fetus A = 0.78, fetus B = 0.63” (Duchatel, 2007). Fortunately, during follow-ups ultrasound evaluations, alterations in the fetal heart rates or changes in the Doppler resistant indexes of umbilical arteries were not seen. Two healthy girls were delivered by a C-section at 34 weeks of gestation.

Duchatel, Francois (2007). Cord Entanglement. Retrieved from https://sonoworld.com/Fetus/page.aspx?id=2459

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Ellen,

Amazing photos of cord entanglement! It's so interesting to see, and easy to understand why and how this could happen with twin gestations. Look at the size of that entanglement, its insane. Thank you for finding this and sharing

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Thrombosis of the umbilical cord can lead to fetal demise so I thought this was a good one to elaborate on.

First let’s discuss causes:

The causes of thrombosis of the umbilical vessels include some sort of inactivity in blood usually caused by true and false knots, cord compression, or even velamentous cord insertion.

Next background:

Thrombosis in the umbilical cord is rare abnormality and research shows the thrombosis usually forms in the umbilical vein.

As a fetus having a thrombosis in your umbilical cord can cause you severe intrauterine growth restriction (IUGR). Because if you are not getting enough oxygenated blood from the umbilical vein due to the thrombosis you will not grow at the optimal rate. Most babies born with thrombosis in the umbilical cord are born in the 5th percentile or experience demise before birth. One of the things you can do is Doppler the umbilical artery and vein when there is a thrombosis in the umbilical cord to abcess the S/D ratio and level of resistance. Hopefully make the mother come in for more frequently ultrasound exams. They also start corticosteroids therapy to help the lungs mature. Here are some examples of thrombosis in the umbilical cord.

Thrombosis in umbilical vein

Thrombosis in umbilical artery

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5785950/

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Hey Jasmine,

I like the topic you chose of thrombosis and how fetal demise is relevant to this abnormality. I didn't realize that is usually forms in the umbilical vein. It makes sense there would be restricted growth and such severe problems affecting the fetus because it's highly dependent on receiving its oxygenated blood from its umbilical vein. I think of it as clamping a portion of a hose (vein) and the water (oxygen) is unable to come through due to the occlusion (thrombosis).

Your topic reminded me also of Intervillous Thrombus, but it affecting the placenta. Thought I'd share an image :)

See the source image

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That thrombus portion is so small it would be easy to miss. We could mistake it for our own user error/ artifact if we think we're not getting the right angle or having appropriate color fill settings. Knowing our machine is important. I can think that it would be easy to mistake thrombus for maybe fingers even. If the fetus were holding the cord I think the impedance of the fingers would block our color readings. I don't know. I've never see it. But I think it would be an issue. And if the cord is moving a lot while in free float..or due to baby's movement...how do we get an accurate reading? OB scanning is no joke!

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The two pathologies that I will showcase are hemangiomas of umbilical cord ( I couldn't find angiomas) if its the same great, if they differ well its a bonus. I will also describe thrombus of the umbilical cord.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5079340/

Accordind to the article above hemangiomas of the umbilical cord are extremely rare. Since 1951 on 44 cases have been reported. Differentials include, but are not limited to, Omphalitis, umbilical hernia, thrombus, and other abdominal wall defects. May present with increased AFP, fusiform swelling of umbilical cord, hydramnios, fetal hydrops, and is linked to increased morbidity. Hemangiomas are benign endothelial cell neoplasms that are often found on the skin, in the liver/spleen and other organs. They put the fetus at high risk during pregnancy.

Onto Thrombus of the umbilical cord

Thrombus of the umbilical cord is more commonly found in the vein, but can also compromise the umbilical arteries. Ultrasound may show focal areas of increased echogenicity with an absence of flow with color doppler. A fetus with arterial thrombosis has a poor prognosis since they are no longer recieving the oxygen they need/able to get rid of toxins. Thrombus can be caused from knots, compression or other trauma to the cord. Commonly found following Varix of the cord.

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I wonder if maternal blood thinners could help with cord thrombosis.

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I keep wondering the same thing. There is too much advancement in medical technology and really in medicine in general for there not to be a treatment for these conditions. If the medications we take can cross the placenta, I don't know or understand why we couldn't do something like that. I am thinking about the illegal drugs that women take and have children addicted to them (cocaine) why not use therapeutic drugs to treat them too?

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Hi Heather,

No wonder that thrombosis of the umbilical cord can occur after Varix of the cord because of blood stasis, which is one of the condition for thrombogenesis.

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The umbilical cord has many masses that include:

- omphalocele where the babies intestines are outside the baby covered by a thin layer of tissue.

sono: will look like a round mass next to the belly.

- Gastroschisis where the intestines are floating outside of the fetus stomach. sonographically you will see intestines floating with the baby.

- umbilical herniation is when the fetus intestines protrude the abdominal muscle.

Sono: you will see small bowel extending from the naval area.

-Teratoma of the umbilical cord is a tumor that can grow in the umbilical cord.

Sono: we will see a mass on the umbilical cord.

-aneurysm of the cord is a rare condition but it is associated with aneuploidy and fetal demise.

Sono: will have a thicker appearance where the aneurysm is located.

-Varix of the cord is when they veins diameter is larger near the cord insertion to the fetus.

Sono: umbilical vein will appear large in the abdominal portion compared to the gallbladder.

-hematoma of the cord is blood inside Wharton’s jelly. This can be dangerous because it can result in fetal demise.

-True knot of the cord is when the cord twists on itself.

Sono: will see the umbilical cord in a knot, best seen when color is on.

-Thrombosis of the cord happens when blood is stasis. This can happen with a true knot. Not sure how it will look sonographically.

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Hi Lauren,

You did a good job giving a brief synopsis of each of the conditions. Do you have a way that you would be able to differentiate all of these from one another? What do you think a good method would be to organize these so that it sticks. I am also looking for a way to really cement them in my mind as well.

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I'll be discussing Teratomas of the umbilical as well as Varix of the cord:

Teratomas of the umbilical are considered rare cases, yet they are the most common congenital tumors. According to an article I read online, "Umbilical cord teratomas derive from ectopic germ cells that migrate out of the wall of the invaginated gut into the connective tissue of the cord, they rarely contain malignant tissue components, they have both a solid and cystic nature, and are covered with skin." In an ultrasound this may reveal a heterogeneous, echogenic mass along the umbilical cord (Figure 1, a and b), with a differential that includes angiomyxoma, myxoma, hamartoma, or acardiac twin. Teratomas can have adverse outcomes for the fetus, such as;

Image result for umbilical teratoma"

https://www.degruyter.com/view/j/crpm.2014.3.issue-2/crpm-2013-0056/crpm-2013-0056.xml

https://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2019-0161-RA

As for Varix of the cord, it is a complication of the intra-abdominal umbilical vein (FIUV) varix, it is defined as "focal dilatation of the umbilical venous diameter at the level of cord insertion." According to, Obstetrics and Gynecology Science journal, " FIUV varix has been reported to be associated with intrauterine fetal death (IUFD), chromosomal abnormalities, fetal hydrops and other adverse pregnancy outcomes." It goes to explain that FIUV is a developed lesion opposed to a fetal congenital anomaly.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5785950/

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Teratomas are incredibly interesting to me and I cannot begin to imagine seeing one within the cord!

I was curious, so I found an additional image:

The "E" represents an exomphalos and the "AS" is acoustic shadowing from bone found in the teratoma within the exomphalos. (https://obgyn.onlinelibrary.wiley.com/doi/full/10.1002/uog.11124)

An article called, "Abnormalities of the Umbilical Cord" by Sidonia Catalina Vrabie et al in 2017 states that there are only 12 cases of UC teratomas in literature! (https://www.intechopen.com/books/congenital-anomalies-from-the-embryo-to-the-neonate/abnormalities-of-the-umbilical-cord)

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I agree that teratomas are incredibly interesting. I was fascinated by them when we first learned about them in the gyn class. I think that the body is fascinating that these undefined cells can grow fatty and sebaceous material, hair. soft tissue, calcifications, and teeth. I honestly think that each of us walking around "normal" is a bigger anomaly than the abnormals at this point. The list of things that can go wrong just seems to go on and on. Great pictures Beth and Maria

https://www.ajronline.org/doi/pdfplus/10.2214/ajr.167.5.8911163

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Hi Maria,

Thank you for the great explanation why teratomas form in the umbilical cord. I just would like to add that sonographically they might look like disorganized, heterogenous mass (Stephenson, 2012). It can grow pretty big, up to 9 cm in diameter, have calcifications, and appear at any spot along the cord. You made a good point that we need to differentiate it from hamartomas and acardiac twin. Teratomas can also be differentiated from hematomas and hemangiomas of umbilical cord.

Stephenson, Susan (2012). Obstetrics and Gynecology. 3^rd ed.

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UC Cysts (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4554503/)

"Cystic segment of UC in cross section (a und b), and in longitudinal section (c und d). Cysts can be differentiated from perfused vessels using Doppler ultrasound (b und d). Despite the cystsʼ segmental occurrence and irregular shape, both characteristic of pseudocysts, differentiation is only possible on histology. This fetus also had agenesis of the septum pellucidum, schizencephaly and atrioventricular valve incompetence."

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Umbilical Artery Thrombosis (http://www.fetalultrasound.com/online/text/34-026.htm)

Small, slightly echogenic area of cord.

Umbilical artery aneurysm in the fetus of a 28-year-old woman with a prior ectopic pregnancy who presented with contractions at 19 weeks 4 days of gestation. Transverse color Doppler US image at the level of the fetal pelvis shows focal dilatation of the umbilical artery in the left pelvis (arrow). Turbulent flow is noted within the area of fo-cal dilatation, a finding that denotes umbilical artery aneurysm. (https://pubs.rsna.org/doi/pdf/10.1148/rg.341125127)

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Hi Beth,

I like that you put grey scale next to the color images. This shows that color is essential when trying to locate the aneurysm. How would you think a spectral waveform would look if you used pulsed Doppler on the artery? I would think there would be an absence or reverse flow of diastolic flow.

I scanned a limited OB today and had to do cord Doppler because the baby had IUGR. With mom breathing and baby moving and kicking the cord, obtaining a spectral waveform can be tricky at sometimes. I thought it would've been a lot easier!

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Yes, Amanda - super challenging to get those cord dopplers when there is so much movement in that belly! According to the Journal of Clinical Diagnosis & Research (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4740657/), UA aneurysms are the most rare cord defect (with UV aneurysms being more common) and are at "high risk of fetal aneuploidy and fetal demise." 1 image illustrates turbulent flow:

An external file that holds a picture, illustration, etc. Object name is jcdr-10-QD01-g001.jpg

I believe there would definitely be reverse flow.

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Beth, I imagine this would be difficult to document with ultrasound because the cord is so long and to visualize it in trans along its 60 cm would be very hard when it's floating around baby..

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I completely agree, Elona! So much of the cord is hiding around inside...

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HEMATOMA of the Umbilical cord!

Image result for hematoma of the umbilical cord"

A hematoma of Umbilical Cord is a very uncommon condition. This condition is the accumulation of blood within the umbilical cord. It is associated with certain iatrogenic procedures such as cord blood sampling procedure (cordocentesis) and potentially an amniocentesis to determine if there is blood in the amniotic fluid.

The signs and symptoms typically include excessive bleeding and decreased blood supply to the fetus resulting in fetal distress. Because the hematoma may cause bleeding or even compression of the umbilical cord, the condition results in fetal fatalities in about half of the cases, during labor or after birth.

Trauma or infection of the umbilical cord and cord abnormalities (during its formation) may play a role in some cases. However, because the cause is unknown, there are currently no preventative measures.

Unfortunately, the umbilical cord masses can be difficult to diagnose. Just like the placenta, the umbilical cord is another area of the exam that I don't spend very much time on. Also, because it's free floating, it can make the visualization of the entire cord very difficult. The good this (for us, not the baby/mother), is that if there is something wrong in the umbilical cord, it will most likely reflect in the baby (measurements, bleeding, etc.).

If we see something wrong with a baby, it's important to keep in mind that the placenta AND mom might be perfectly fine, but it's the umbilical cord that is having difficulties. Al we can do is start ruling things out one at a time.

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Hi Paris,

Thanks for sharing, this is interesting that the hematoma happens inside the umbilical cord and not outside. I guess, I was thinking that, if something gets poked it would form some type of blood collection outside the hole as well as within. Another thought I had when I read this was, why would they be doing a cordocentesis, wouldn't this interrupt the flow of the umbilical cord and what are they sampling? The arteries or the vein?

I looked it up and read that "umbilical cord hematoma is caused by extravasation of venous blood into the Wharton jelly that surrounds the umbilical cord vessels, which is believed to be a result of compression of the umbilical arteries or from fetal anemia due to blood loss within the cord." It also explained that babies who are asymptomatic with a hematoma are able to have no complications and mother can have a normal delivery.

Here's a photo of a post delivery hematoma:

https://www.consultant360.com/articles/spontaneous-umbilical-cord-hematoma

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Hey Maria,

That poor baby! To answer your question, I don't think it will interrupt the flow to and from the baby to do a cordocentesis. It's no different than drawing blood from our arms. It doesn't interrupt anything.

Cordocentesis, also sometimes called Percutaneous Umbilical Cord Blood Sampling (PUBS), looks for chromosomal abnormalities and blood disorders. It's more rare to do this, however. They really only do this if there is information they need that cannot be gathered from an amniocentesis, CVS, or an ultrasound.

You are right though! There are definite risk factors involved. Miscarriage, the main concern, happens 1-2 of every 100 patients. Other potential side effects include blood loss from the puncture site, infection, drop in fetal heart rate, and premature rupture of membranes. These potential side effects should speak to the necessity of this exam if it is being used.

Image result for cordocentesis"

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After seeing your post about cordocentesis, Maria, I had to go look it up.

Here's an article from Mayo Clinic: https://www.mayoclinic.org/tests-procedures/percutaneous-umbilical-blood-sampling/about/pac-20393638

It says risks are:

Fetal bleeding, Cord hematoma, Slowing of the baby's heart rate. Infection. fetal-maternal bleeding. Passing maternal infection. Pregnancy loss.

I was interested to find out which part of the umbilical cord they take a sample from. The article says they do it to determine if the fetus has anemia, so they take a sample from one of the veins. This makes sense! Because the vein is carrying oxygenated blood that will go to the fetus. And there are two of them, so if something bad happens to the site there is another vein to carry on.

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Hi Paris, I now Better understand a hematoma of the umbilical cord. It is interesting to read that it happens for unknown reasons and that it can be fatal for the fetus. I do have a question, sonographically would it look anechoic with some echos within the cord or how?

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Hi Lauren,

According to https://radiopaedia.org/articles/umbilical-cord-haematoma?lang=us, it appears as follows:

focal cord expansion with a heteroechoic to hyperechoic mass in the umbilical cord.
discordant umbilical artery Doppler waveforms
normal placenta

I couldn't find very many images as most images are taken after the baby is born. However, I did post one above.

I hope this helps!

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Paris

You have made such a good point. I have wanted to say all throughout this thread exactly what you have written. The cord is "free floating, it can make the visualization of the entire cord very difficult." I have been thinking to myself that really the only parts of the cord that we have visualized or taken pictures of are the cord insert on baby and on the placenta. I am thinking that if there was a problem with the baby because of the cord, it would be at that point that the portion of the cord with the problem would become easier to visualize. What are your thoughts? Have you seen any other portion of the umbilical cord other than the inserts?

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Hey Perresh,

You’re right for the most part we are only seeing the umbilical cord at the placenta and at the abdominal cord insertion area. However, throughout the exam there are times where you see the cord free-floating. For instance when you get the three vessel cord image that kind of looks like the smiley face (two umbilical arteries and an umbilical vein).

I guess all we can do as sonographers is pay attention to the cord when we see it. Even if we are going after a different image, if we happen to pass a section of the umbilical cord, then we should make sure that everything looks normal.

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I would like to review a bit more in depth about the true knot and false knot of the umbilical as those two can be easily confused with each other.

Alrighty so what's the difference between a true and false knot? Per our book a true knot is formed when the cord itself loops and creates knots upon itself. I think of baby inside with ample amount of space so its moving, flipping exc,( mom does not have this kid on a short leash, can do what they want ) however once baby grows less space so that umbilical cord that is forming into a knot is more taught/ tightened due to constriction of space thus forming a knot.

factors that can lead to a true knot are the following: long cord, polyhydramnios, monoamniotic twins
this knots may be multiple
so umbilical cord vessels are important due to fact since they are essential for oxygen and important nutrients for fetus if there is a true knot impairing any exchange this situation is deadly the tyke is not is not getting oxygenated blood nor exchange of the venous blood to the placenta ( role of the vessels within the umbilical cord) high mortality rate!!!!
facts: true knots occur every 1 out of 2000 deliveries

FALSE KNOT AKA pseudoknots

with false knots it is what is sounds like. Like a real knot, it consist of any sort of bulging or protuberance in the baby’s umbilical cord formed by swollen vessels or excessive covering of Wharton’s jelly. Per Birth injury safety article" Small pseudoknots are relatively common in pregnancies. They can’t be untangled (because it’s a formation inside the cord, not outside), but luckily, they have no clinical significance and do not present a danger to the baby."

false knots seen when the blood vessels are longer than the cord itself
they are only folded on themselves
produce a nodular like appearance on the surface looking like a real knot
very common

How can you differentiate these two?????

use your color with true your going to have absences of flow ( true is impeding blood from flowing ) with false you should still be able to see blood flow

take home : true knots : loops of the whole umbilical cord its self into a knot and false knot dilation of the vessel within the cord causing protrusion on the surface of cord , or loops of the vessels within each other due to fact they are longer than the cord

my sources are the following : http://www.pathologyoutlines.com/topic/placentaknots.html

https://www.birthinjurysafety.org/birth-injuries/causes-of-birth-injuries/umbilical-cord-knots.html

few images :

By Tbsdy lives - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=9390666

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Hi Liz, I like how you gave examples of how a true knot can come about. It is true that if the baby has a long cord or there is polyhydramnios that the baby can move so much and tangle itself. I was unaware that false knots can not be untangled because it is inside the cord.

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hey lauren!

yes after looking more into it I have a clearer understanding. it makes sense because since the vessels are within the cord and they are longer in length that the cord that capsulate it they are bound to kind of shrivel up and tangle or protrude appearance on the outside

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Hi Liz,

Thanks for including the pictures, they really help with the bullet points you provided for both true and false knots. It seems that color Doppler is the number 1 way to determine if a knot is true or false. I would think to also use pulse wave before and after the suspected knot. Would you agree? Maybe adding a pulse wave at the level of the fetal cord insertion would be beneficial as well. This could tell us if baby is getting enough oxygen from the blood.

That picture of the baby with the true knot is nerve-wracking, look at that blood collection distal to the knot! I hope there were no serious growth restrictions with this little one.

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hey Amanda,

I completely agree use of your pulse doppler to make a final confirmation for you and your rads it most defiantly a way to go nicely with confirming absence of flow! great thinking ! that image is pretty gnarly you can see the blood collecting at portion of the cord

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Thank you for explaining it so clearly Liz. I think a really tight knot can also lead to an umbilical cord thrombosis with no flow. I think this would be hard to see on a quick anatomy scan unless there is some indication to investigate the cord.

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hey Elona, that's completely right! in the first article I referenced it as well listed complication that may go with have true knot and umbilical cord thrombosis was one of them along with IUGR, depending on how severe the knot is it can unfortunately lead to even a fetal demise sadly

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Great post Liz,

I read that a false knot is the most common variant of the umbilical cord and can also be due to the length of the umbilical vein being different than the umbilical arteries. I thought that was interesting. Also, in terms of true umbilical cord knots, in addition to the hanging noose sign there is the clover leaf pattern and "four leaf clover" sign when there are three knots for identifying different true knot variants.

http://www.fetalultrasound.com/online/text/4-022.HTM

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Hey all!

I decided to focus on angiomas of the cord as this hasn't been touch on too much yet.

An angioma of the cord will image as a well-circumscribed, echogenic mass that may cause increased cardiac failure and hydrops.

Clinical signs will be an increased AFP level.

Umbilical cord angiomas consist of an angiomatous nodule containing and encompassed by edema and myxomatous degeneration of Wharton's jelly, which is often cystic. The angiomatous nodules range from 0.2 to 7 cm in largest dimension, whereas the associated edema may measure up to 15 cm. The tumors tend to occur at the placental end of the cord and presumably arise from one or more of the major umbilical vessels.

As of now there is no apparent association between cord hemangiomas and maternal age, race, or gravidity nor does one sex predominate. Unlike placental hemangiomas, cord hemangiomas are not associated with maternal hydramnios.

Cord angiomas tend to be associated with fetal morbidity.

I have included a photo to help with visualizing the sonographic guidleines.

Ultrasound of Umbilical Cord Hemangioma

fetalultrasound.com

In comparison, umbilical cord teratomas are rare tumoral lesions of umbilical cord which have challenging antenatal diagnosis. The cord teratomas contain tissue from all three germ layers and have both cystic and solid components.

Umbilical cord teratomas tend to grow rapidly and may cause cardiac failure and fetal hydrops. If the teratoma is associated with the umbilical vessels or omphalocoele it may lead to rupture of the fetal cord.

exomphalos with umbilical cord teratoma ...

obgyn.onlinelibrary.wiley.com

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Hi Ashley this is great! I referred to this article https://www.dovemed.com/diseases-conditions/hemangioma-umbilical-cord/, and it as well stated that there isn't really any predisposing factors that can indicate a umbilical cord hemangioma. I like the fact that as your stated as there is no predisposing factors however we can correlate our labs as you mention you'll get elevated AFP level as well as your scanning to fully confirm. Per the article it stated typically they are benign and asymptomatic however once they increase in size it can be questionable as well presenting hemorrhage within the gestational tract, and decreased blood flow to the fetus. I like how you stated the differentials such as teratoma that way you can know really nail down any other possibilities and work it.

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The umbilical cord is a link for the exchange of gas, and the supply of nutrients for the baby. It is the fetus’s lifeline. The umbilical cord consists of 1 umbilical vein and two umbilical arteries protected by Wharton’s jelly. I picked Thrombosis of the cord as my topic because thrombosis is not something I think about when I think of a small baby and because it is associated with fetal morbidity and mortality, although it is a rare pregnancy complication.

Thrombosis can happen in the circulation of umbilical vein or artery but thrombosis of the vein occurs more frequently. Thrombosis of the artery is associated with more serious risks in comparison with the vein. It may be cause by mechanical injury or abnormal anatomy of the umbilical cord like twisting, compression, abnormal attachment, tieing, and excessive helix. Umbilical thrombosis can also result due to abnormal fetal coagulation function, abnormal maternal plasma glucose profiles, diabetes, smoking, infection, increased blood coagulation and hemodynamic abnormalities, but I will focus on the mechanical aspect.

The mechanical injury to the cord may cause blood stasis which can lead to the thrombosis.

An ultrasound examination may show the cord to have increased echogencity in this condition. Doppler ultrasound may show turbulent blood flow, absent blood flow, and increased peak systolic velocity in one artery if the other is occluded.

A symptom of this condition are bleeding and decreased blood supply to the fetus. It can occur to women of all ages without racial or geographic differences. This condition is an emergency with the goal of saving the fetus.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4270709/

Image result for thrombosis of umbilical cord

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Hi Elona, I saw this description of thrombosis in a description of umbilical anomalies on SonoWorld, it says that thrombosis of the vein is rare and fatal, but thrombosis of the artery is not necessarily fatal when there is a normal 3 vessel cord. This makes sense to me. I wonder which it is?

"Artery

Thrombosis of an umbilical artery may result from extension of an aortic clot such as may happen in aortic aneurysms⁸⁹. The occluded artery will appear echogenic and at pathology emboli can be seen in the placenta. Calcified mural thrombi have even been identified in an acardiac fetus⁹⁰. The anomaly is not necessarily fatal⁹¹ in fetuses with 3-vessel cord, but is for fetuses with 2-vessel cords.

Vein
Thrombosis of the vein⁹² is rare and fatal⁹³. It has been reported to occur more frequently in the offspring of diabetic mothers as well as chorionic vessel and perinatal renal venous thromboses⁹⁴.
As expected, the appearance is that of an echogenic umbilical vein. If fetal death does not occur immediately, nonimmune hydrops fetalis can develop⁹⁵."

Article: https://sonoworld.com/Fetus/page.aspx?id=1149

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I just noticed that Jazmine had the same images for Cord Thrombosis as I posted just now so I wanted to add these as an example:

Case 1 of umbilical artery thrombosis. (A) The routine ultrasound scan showed 1 umbilical vein and 2 umbilical arteries. (B) Single umbilical artery blood flow imaged using Doppler ultrasound at the level of the bladder.

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Hello Elona, its okay hun, that was a good example. There was limited thrombus photos with color actually Heather, Beth, and I all had the same photo's. =)

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oops! I guess because it's so rare there aren't many photos floating around.

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I was having a similar issue finding images of an umbilical cord hematoma. Most of the images I could find were taken after the baby was born. As Perresha and I mentioned earlier, It seems that problems with the umbilical cord are less documented because during our exams we don’t spend so much time focusing on the umbilical cord. It’s free floating and sometimes difficult to see it in its entire ready. And if we do for the most part it’s normal.

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Umbilical Cord True knots can happen due to: Long umbilical cords, Polyhydramnios, a small fetus due to IUGR, and Monoamniotic twins. This is when the fetus has more space to move in the amniotic fluid resulting in a knot in the umbilical cord. They are more common around 9-12wks when the fetus is smaller. And they are also more common in males than females, but can happen to either gender. A true knot can be confused with a false Knot which gives a knot like appearance due to Larger blood vessels that fold on themselves due to differences in length or too much Wharton's Jelly. With true knots complications occur when the amniotic fluid is low and the cord is no longer free to float in the amniotic fluid. This can cause a decrease in umbilical cord blood flow a possible outcome of which is fetal asphyxia and demise.

The sonographic appearance is:

“Focal dilatation of umbilical vein or umbilical vein thrombosis near the umbilical cord knot, giving a false appearance of an umbilical cord cyst.
Tortuosity of cord at the level of knot
A segment of the umbilical cord that is closely surrounded by another loop of umbilical cord is considered to be highly specific of a true knot and is known as hanging noose sign.”

Source: https://radiopaedia.org/articles/true-umbilical-cord-knot?lang=us

Source: https://sonoworld.com/TheFetus/page.aspx?id=3644

Source: http://www.fetalultrasound.com/online/text/34-021.htm

Source: http://www.fetalultrasound.com/online/text/4-022.HTM

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nice images Serina! At first glance I thought the first images were of a nuchal cord. It took me a minute to realize it was a knot! The nuchal cord will look similar when taken in a transverse view at the level of the cervical spine.

Of course a nuchal cord may seem like life threatening it typically isn't. Its fairly common and usually resolves on its own.

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You gave a really great range of sonographic images to illustrate knots well. I like how included the "hanging noose sign." I haven't heard that before.

Thank you!

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Umbilical cord tumors are exceedingly rare. The most common is the umbilical cord hemangioma. Sonographic appearance of hemangioma is “heterogenous mass surrounded by multiple peripheral cystic areas” (Rumack, 2018, p.1483). It consists of angiomatous nodule encompassed by edematous or myxomatous cystic degeneration of Wharton’s jelly. The nodule is usually sharply demarcated from the surrounding stroma and can range in size from 0.2 to 7 cm, while the surrounding edema can be as large as 15 cm. The tumors arise from one or two umbilical vessels usually close to the placental end. Even though hemangioma is invariably cytologically benign, it is closely associated with fetal morbidity and mortality. There is no known association between umbilical cord hemangiomas and maternal age, race, and gravidity, as well as fetal gender.

Umbilical cord hemangioma might cause a serious complication when it compresses the vasculature. In some cases, hemangioma could completely block blood flow in the umbilical cord, which will result in high output cardiac failure and fetal demise.

An umbilical cord hemangioma should be differentiated from a placental hemangioma (or chorioangioma). The main difference is that cord hemangimas are NOT associated with polyhydramnios and it has myxematous degeneration of Wharton’s jelly.

Reference:

Aby, Janelle. Umbilical Cord Hemangioma (n.d.). Retrieved from https://med.stanford.edu/newborns/professional-education/photo-gallery/umbilical-cord.html#umbilical_cord_hemangioma

Heifetz, S.A., Rueda_pedraza M.E. (1983). Hemangiomas of the Umbilical Cord. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/6687288/

Rumack, Carol, Levine, Deborah (2018). Diagnostic Ultrasound, 5^th ed. Elsevier, 2018.

Atlas of Obstetric Ultrasound/Editor Prof. Pilu Glanluigi. Retrieved from https://www.glowm.com/ultrasoundAtlas#us691

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We have discussed many umbilical cord masses, but I would like to add one more pathology of an umbilical cord. It is absence (acordia), or very short umbilical cord. I think it is very important to know about this pathology because we might see it in clinics. It happens often when mothers are drug addicts, especially with cocaine abuse. Usually it is associated with limb-body wall complex, a syndrome with severe fetal structural abnormalities. Sonographic appearance: no cord seen and extraembryonic membranous sac in direct opposition to the chorionic plate.

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Thank you, Ellen for bringing up short cords! I couldn't remember what they were associated with, so I appreciate you giving that information. I don't think I remember ever discussing how they can be due to maternal drug use such as cocaine.

I also couldn't recall what a short cord could cause as far as complications. I found some information stating that normally, umbilical cords are between 55 and 60 cm long. An umbilical cord is considered short if it measures under 35 cm in length.

Usually, when a baby moves around, the tension on the cord promotes growth and development, lengthening the cord to cater to this growth as the pregnancy progresses. If the cord is too short, it means that the baby might not be moving and growing enough, signaling a potential health issue. Short cords can also pose a risk for numerous delivery complications and birth injuries. Short umbilical cords appear in roughly 6% of deliveries.

The complications that arise from short cords can have very dangerous implications for baby and mom and include:

For baby:

Hypoxic-ischemic encephalopathy (HIE)
Cerebral palsy
Umbilical cord rupture
Breech Presentation
Miscarriage/stillbirth
IUGR
Placental disruption

And for mom:

Placental abruption
Inversion of the uterus
Prolonged labor or need for C-section
Retained placenta
Hemorrhage (caused by placental abruption)

Besides drug use, other associated factors with short cords include:

A woman who is of average weight or less
A woman who is pregnant for the first time
A female fetus
Oli/Pohydramnios
A fetus that is small for gestational age
Lack of fetal movement during the first half of pregnancy/intrauterine constraint/conditions that limit the baby’s movement in the womb
History of smoking and alcohol consumption during pregnancy
Pre-Eclampsia

If there is a concerning fetal heart rate or other signs of fetal distress, we might suspect a short cord.

Ultrasound image at 32 weeks. Observe short umbilical cord.

Sources:

Umbilical cord conditions. (n.d.). Retrieved January 17, 2020, from https://www.marchofdimes.org/complications/umbilical-cord-conditions.aspx
Short Umbilical Cord. (n.d.). Retrieved January 17, 2020, from https://www.dovemed.com/diseases-conditions/short-umbilical-cord/
Infant Mortality Rate Doubles with Short Umbilical Cord. (2004, October 01). Retrieved January 17, 2020, from https://www.aafp.org/afp/2004/1001/p1366.html

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To wrap up the week,

We've done a lot of great learning together through various images and descriptions. We have deciphered through the various pathologies including:

Omphalocele
Gastroschisis
Umbilical herniation
Teratoma of the umbilical cord
Aneurysm of the cord
Varix of the cord
Hematoma of the cord
True knot of the cord
Angioma of the cord
Thrombosis of cord secondary to compression or kinking, focal cord mass, true cord knots, velamentous cord insertion, cord entanglement in monoamniotic twins (commonly seen with fetal demise)
Acordia (missing or short umbilical cord)

We have agreed upon the importance of finding and paying special attention to the cord as well as the placenta. We have also agreed that the use of color, and Doppler when necessary to determine flow. We know that many of these conditions have very serious consequences for both the mother and the baby so early detection and monitoring are both key. It is also important to distinguish between the various pathologies as certain ones have more implications and a worse prognosis than others. There have been many great images and resources on the following websites

www.radiopaedia.com

www.sonoworld.com

www.ncbi.nlm.nih.gov

www.fetalultrasound.com

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