Tuesday, March 11, 2008

Müllerian Anomalies Explained

I am not a doctor. But if I were, I probably wouldn't know much about Müllerian anomalies, so I don't mind sounding like I know what I'm talking about. As a reminder: my doctor told me immediately after my D&C that I have something in my uterus that may have caused my miscarriage. It could be a septum or I may have a bicornuate uterus. At the follow-up appointment, she did not say the words "septum" or "bicornuate," but when I mentioned a septum, she hinted that that's what she thinks it is. I await further diagnosis through HSG.

Someone on the Nest pointed me to this Yahoo! Health Group: Müllerian Anomalies. It has, by far the most comprehensive information on the web on MAs. Rather than putting it all into my own words, I've decided to copy/paste from their FAQ page, and put in bold the statements I find to be important.
1. What does müllerian mean?

The uterus, fallopian tubes and upper vagina are made up of two partially fused tubes, which, in the embryo, are known as müllerian ducts, named for physiologist Johannes Peter Müller, who first described them in 1830. They are also known as the paramesonephric ducts, and are at first present in embryos of both sexes.
Normally, these ducts run down vertically from flank to pelvic floor in the young embryo and eventually fuse into a double-barreled tube with two loose ends, known as the uterovaginal primordium, or UVP. The double UVP will eventually merge into a single-barreled uterus, cervix and upper vagina, while the loose ends develop into the fallopian tubes. In adulthood, these organs are referred to as the müllerian tract and congenital malformations of this tract are called müllerian anomalies, or MAs, as we call them on this list.

2. What are the different types of müllerian anomalies?

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.

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.

Obviously, there are other types of MA, such as unicornuate, arcuate, and didelphys, but SU and BU are the most common and seem to be the most relevant to me at this point.


7. Is HSG alone good enough for diagnosis?

First of all, in an infertility work-up, a hysterosalpingogram (HSG) should be done to either rule out or assess the presence of a two-chambered uterus, the depth of the division, as well as tubal patency. But an HSG alone cannot differentiate between septate and bicornuate uterus. Misdiagnoses of BU by HSG are very common.

Other methods have better levels of reliability:

• Transvaginal ultrasound is nearly 100% successful in detecting a bifid uterus, but only 80% successful in differentiating between SU and BU. It can be a helpful tool in the diagnostic process, but should not be relied upon alone.
• Three-dimensional ultrasound (3DUS), is 92% accurate in differentiation of BU from SU, according to one 1997 study, but not widely available at the time of this writing. It should not be relied upon alone, with an 8% margin of error.
• According to two studies done in 1994 and 1995, MRI can reliably differentiate between BU from SU, with an accuracy of 100% in comparison with laparoscopy/hysteroscopy. More recent studies cast some doubt on this. Proceed with caution after an MRI.
• Concurrent laparoscopy and hysteroscopy are considered the "gold standard” of BU/SU differentiation. This test is invasive, but if needed, corrective hysteroscopic metroplasty can be done at the same time.

8. Should I have my uterus surgically repaired?
Hysteroscopic metroplasty, abdominal metroplasty, hemihysterectomy, vaginoplasty

The quick, reflexive party line here is that if you have a septate uterus, yes, you should have it fixed. Studies tend to show a poor pregnancy outcome in the uncorrected SU, but a near-normal pregnancy outcome in the surgically corrected SU. Of course there are exceptions, and we should not forget that most women with a septate uterus are never diagnosed, and may indeed have no trouble with reproduction.

Bicornuate, arcuate and didelphic uteri are generally thought to do well in pregnancy, but some studies show correlation to miscarriage and other problems. Again, take your history into account, first and foremost, and weigh the benefits versus the risks. Surgical correction of a bicornuate uterus involves an open (laparotomy) procedure in which the surgeon cuts through the uterine wall of each horn and then sews them together. Unlike most septum surgeries, abdominal metroplasty is a major procedure and carries greater risks of bleeding, infection, adhesions, infertility and rupture during pregnancy. Recuperation takes at least twice as long, and greater pain control is required.

The FAQ page continues with details about surgeries and diagnostic tests as well as success stories. In the past few days, I've also been reading the huge backlog of posts on the message boards. It's refreshing to see so many people who live and deal with these problems, most of them able to have children. I'm finally to the state, emotionally, where I can deal with reading so much information, accepting the risk of getting very worried about the type of care I will receive. Will my doctors be familiar with these problems? Why am I wasting time waiting for an HSG when it doesn't even show the outside of the uterus? How long will I have to wait to see a specialist, if indeed I will have to see one? How long will it be before I can have the surgery, if I have a SU? Is it realistic to hope I can get pregnant again before the end of the year?

It's frustrating that no one can answer my questions, though I am tempted to call my doctor and share my concerns about the HSG. If my safe window for HSG falls while I'm overseas in a few weeks, it will be very hard to wait another month. I will still know nothing! On the other hand, I may be premenstrual right now and don't yet have reason to give up hope that I will be able to have the HSG done in the next few weeks, in which case I can be patient. I suppose.

For the first time, I feel like God is trying to tell me something. Maybe this wasn't the right time to have a baby, and from this experience I will learn the very great importance of patience.

No comments: