Saturday, April 5, 2008

What are the different types of müllerian anomalies?

a. Agenesis & hypoplasia: Mayer-Rokitansky-Kuster-Hauser syndrome is most common. All or part of the müllerian tract fails to form, or is extremely underdeveloped. For example, a cervix may be a tissue-thin membrane rather than a tough, fibrous "donut" several centimeters thick. Most women suffering from agenesis or extreme hypoplasia have severe fertility problems, simply by lacking sufficient tissue to support a growing pregnancy. A common diagnosis used to be "infantile uterus," but it simply means a smaller-than-average uterus and does not refer to the MA described above. The old "infantile uterus" is typically capable of supporting a pregnancy very well, since a uterus easily grows during pregnancy. The "infantile" term has fallen by the wayside in recent years.

b. Unicornuate uterus (UU): When one müllerian duct is underdeveloped or fails to develop, a banana-shaped half-uterus is formed. It may or may not be accompanied by a rudimentary horn, and that other horn may or may not have an endometrial cavity or communicate with the main uterine cavity. A missing kidney or other kidney problems accompany this asymmetric anomaly more than they do other MAs. Frequently, the ovary on the rudimentary side is found in an odd place, further up by the ribs. Adverse pregnancy outcomes are common with UU. SEE FIGURE BELOW.

Unicornuate uterus



c. Uterus didelphys (UD): The müllerian tract fails to fuse along all or most of its length. There may be complete duplication of the vagina, cervix and uterus, and the two halves may be divided by a ligament of connective tissue. UD is reported to have the best pregnancy outcomes of all the MAs. SEE IMAGE BELOW.

Uterus didelphys



d. Bicornuate uterus (BU): The uterine fundus fails to fuse and a myometrial division extends down to the cervix in a complete bicornuate uterus, or part way to the cervix in a partial bicornuate uterus. The division is visible on the outside of the uterus, evidenced by a groove or cleft in the uterine dome exceeding 1.5 centimeters. Cervix and vagina are usually single but may be septate or duplicate. BU has relatively few pregnancy complications when compared to SU or UU, with breech presentation being one of the most common. SEE IMAGE BELOW.

Bicornuate uterus



e. Septate uterus (SU): The müllerian tract has fused properly and the uterus looks single from the outside, but the inner duct wall (i.e. the median septum) has failed to dissolve around 20 weeks of gestation, and the uterus retains a double cavity. There may or may not be a shallow groove of 1.5 centimeters or less on the outer uterine dome, and sometimes even a whitish triangle of tissue, the septum itself, is visible. The somewhat fibrous inner septum extends to the internal cervical opening or beyond in a complete septate uterus, and extends only part of the way down in a partial septate or subseptate uterus. The inadequate blood supply and progesterone receptors of the median septum may cause problems in pregnancy, giving the SU the worst pregnancy outcomes of all the MAs. SEE IMAGE BELOW.

Septate uterus



f. Arcuate uterus (AU): The fundus of the uterus may be indented slightly both inside and outside. This shape has been variously defined as slightly bicornuate and slightly septate (and may be either one), and is so slight that it is considered a variation of normal. However, a few studies suggest that increased incidence of adverse pregnancy outcomes are associated with an arcuate uterus.

g. DES-related uterus: A T-shaped uterine cavity, dilated horns and malformed cervix and upper vagina may characterize this anomaly. Unlike the other anomalies, a T-shaped uterus is sometimes caused by maternal ingestion of DES, although sometimes the cause is unknown. When caused by DES, there are often other problems, such as incompetent cervix, infertility and abnormal tissue in the cervix and vagina. DES use is associated with high rates of female cancers, including cancer of the vagina.

11 comments:

vincent said...

NP diagnosed bicornate uterus and I was never shown the sonogram is this normal procedure?

Jessmoor7 said...

Vincent,

You have every right to request a copy of your sonogram. Fill out a medical release of records form in order to get copies.

I was misdiagnosed as having a bicornuate uterus and lost my son at 16 weeks into the pregnancy. I went to an RE after this and he performed an HSG, then a laparoscopy/hysteroscopy. He found that my uterus was indeed a septate uterus and he resected the septum at this time. It was a simple outpatient scope surgery. Now, we are getting ready to deliver our baby daughter after 4 miscarriages. A proper diagnosis is vital. Please find an RE or RS (Reproductive Surgeon) who has experience treating MA's.

LauraS said...

Yes, I agree with the suggestion to find a surgeon with experience. If you are in Ontario (Canada), I strongly suggest getting a referral to Dr. George Vilos at London Health Sciences Centre. I had my SECOND surgery with him and was very pleased with my care. He does a lot of research in this area and sees about 1 per week...my first surgeon saw 2-3 per year.

Question for jeesmoor7...were your 4 miscarriages before or after the septum resection? I also had a septate uterus and they removed as much as possible but not the entire septum because my uterus has a slight "dip" in it's shape so they didn't want to pierce through. I am just recovering from a miscarriage from a "chemical pregnancy", which has really been discouraging.

me said...

I was originally diagnosed with a DU, then they decided it was a SU. (It sounds odd, but I became pregnant in one side and had a blighted ovum. They couldn't find a cervix during the DNC procedure).
After having a CT scan, several ultrasounds, and an MRI results were still inconclusive. A HSG/ laparoscopy was scheduled and it was determined that I have a combination of a DU/BU. Complete duplication, but one fundus. My uterus looks normal on the from the outside, but I have two, with two cervixes. They also removed hemi-vaginanal tissue during this procedures. It took over two years to get to this diagnosis. It just goes to show that tests for MAs can still be inconclusive. Sometimes the only way to get the correct answer is by the HSG. Ask questions, ask to be refferred to an RE! Don't hesitate. A good OBGYN will understand your concerns!

SHM said...

Hello - I really need some help -- feeling very confused and upset. I decided to schedule for a May 1 resection procedure for a SU. I have miscarried three times in the last 1 1/2 years, all at abaout 7-8 weeks. The septum measures at 1.06 cm. I am reading and trying to research thse issues. My question: opinions on whether this small a septum should be resected?
Should my husband and I just try again to become pregnant and risk -for 4x? My feelings are conflicted and I'm unable to concentrate on anything else but these issues. I would value your response(s). Thank you. - shaynes11@hotmail.com

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little_red said...

I just had surgery two weeks ago. A laparoscopy, hysteroscopy and resection of a vaginal septum. When they went in to do the resection, they found that I did not have a septum at all, but what I had was two completely separate vaginas, which led to their own cervix and to their own uterus. However, from a laparascopic view, it looked as though I had just one uterus, so there is this kind of balloon-type 'uterus' surrounding these two 'actual' uteri. I don't really know how to explain it, but I hop that makes sense! Also, I have two ureter's on my right side. I also have PCOS (Polycystic Ovarian Syndrome). Does anyone have anything remotely similar to this? All of this mullerian anamoly stuff is very new to me. I only figured out I was anatomically abnormal at the end of last year...

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