Wednesday, November 10, 2010

Welcome: An Introduction to Mullerian Anomalies

This is a blog dedicated to informing people about Mullerian Anomalies - what they are, how they are diagnosed, treatments, and the personal stories of the women who have them. The information you read here has been compiled by a group of courageous women who have a wide variety of Mullerian Anomalies. They are not doctors, but are experienced and pro-active patients of a medical system that rarely seems to have a grasp of this very special condition.

If you have recently been diagnosed with a Mullerian Anomaly, please consider joining the MA Support Group on Yahoo forums. The women who write the boards there are the same women who have contributed here. Their knowledge and expertise can be a great help to you as you navigate through your diagnosis and treatment.

Good Luck on your journey!

Saturday, August 30, 2008

Advice for after a loss

If I can give you any advice right now, it would be to take time for yourself and let yourself grieve and lean on others.

Seek counseling if you think you need it.

Everyone grieves differently and at a different pace, so be gentle with yourself and have patience. It will hurt intensely all the time for awhile, and then you'll get some times of peace in between times of sadness. Gather all the memories you can now, while they're fresh--save everything.

You'll always miss your baby, but it does get better, and your memories will become more comforting.

By K, who lost her first baby at 18 weeks.

Saturday, June 28, 2008

Your questions about MA answered here!

Have a specific question about Müllerian Anomalies? Chances are your question may have already been answered by the the Müllerian Anomalies Yahoo Group volunteer consulting physician. Below are a list of questions you can read the answer to by clicking HERE:

1. Miscarriage causes
2. Incompetent cervix, Labor induction
3. Predicting incompetent cervix in the MA patient
4. UU and placing a cerclage
5. Bicollis: septate cervix versus true duplication
6. When in the cycle to have a laparoscopy/hysteroscopy
7. MA complications and the type of physician to see
8. Post-resection pregnancy monitoring in the septate uterus
9. Leaving lower septum segment intact; Resection techniques
10. Distinguishing between SU and BU using Doppler ultrasound
11. Diagnosing and resecting a vascular septum
12. Monitoring septum resection
13. UU and L-shaped cervix: cerclage?
14. Small cervix—is it a MA?
15. Treating endometriosis
16. When to measure cervical length in pregnancy?
17. Length of septum: absolute vs. relative measurement
18. Monitoring for incompetent cervix
19. Can HSG fail to show a septum?
20. Transabdominal versus transcervical resection
21. “Regrowth” of septum, effect of pregnancy on septum
22. C-section techniques for MA, Resection during birth?
23. Infertility questions
24. Late ovulation
25. Polycystic ovarian syndrome (PCOS)
26. Incompetent cervix guidelines
27. Ectopic pregnancy
28. Use of Clomid
29. Balloon catheters and progesterone following resection of SU

Tuesday, April 8, 2008

What does müllerian mean and what causes these anomalies?

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.

In the male embryo, in the presence of anti-müllerian hormone (AMH), the müllerian structures disintegrate during early development. They persist in the female because she does not produce AMH.

In the embryo, the müllerian ducts act as scaffolding for the mesonephric ducts, which give rise to the kidneys. Because of this parallel structural relationship, it is common for a kidney or other urinary anomaly to be present with a müllerian anomaly.

What causes müllerian anomalies?
To date, there is no singular cause for müllerian anomalies. Some may be hereditary , others result from an insult to the fetus while in the womb (the T-shaped uterus of fetuses exposed to DES, for example), and still others may be attributed to random mutation. It is important to remember that in our grandmothers and mothers’ generations, many women with this problem were not diagnosed; while up to 4% of women may have a müllerian anomaly, it may be far more common than physicians realize. Only as diagnostic technology improves and women become more aware of their reproductive health will science get a better understanding as to how common these differences really are. In future years, our honest communication with our children will help build a larger base for understanding the causes of this uniqueness.

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.

Tuesday, April 1, 2008

Diagnostic Methods

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.
  • MRI - 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.

Saturday, March 29, 2008

Surgeries associated with MA's (preconception and in pregnancy)

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.

In deciding whether or not to have it repaired, take into account your own pregnancy history, your complications, your age, and, possibly, your investment. Are you spending thousands per cycle on IVF treatments? Then you may want to get the septum out of the way. If you have no trouble getting pregnant and have had only one miscarriage, some doctors recommend a more conservative approach, treating with surgery only if another miscarriage happens. Resecting a septum, although not without risks, is a relatively minor surgery compared to the open, abdominal metroplasty once performed.

If you have a septate uterus and suffer from painful menstrual periods, there is a good chance that having your septum resected will cure the painful periods.

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.

Sometimes a well developed unicornuate uterus has a rudimentary bud (anlage) that is capable of supporting a pregnancy for time, but then ruptures, because of its tiny size. This tiny half-uterus may need to be removed in what is known as a “hemihysterectomy.” The surgery eliminates the possibily of an ectopic pregnancy in the rudimenary horn, lessens pain during menses and reduces the possiility of endometroisis caused by retrograde menstruation. The surgery is not common, but a few members have had it done.

Women with UD or with a completely septate uterus may also have a vaginal septum, sometimes expending to the perineum, creating two vaginal openings. This may make intercourse difficult or painful, or using tampons impractical, and surgery to lyse the vaginal septum is fairly simple. There is some controversy about severing the septum within a septate cervix, since there is a possibility of creating an incompetent cervix. Some surgeons prefer to spare the cervical segment of a septum.

How long to wait to TTC following surgery?
The standard advice is to wait either two cycles or 8–10 weeks following hysteroscopic metroplasty surgery, to allow time for the inflammatory response at the wound site to go away. Local inflammation is a normal response to an injury, but may increase miscarriage or hinder conception—opinions and studies vary. Sometimes, the hormones prescribed before or after surgery can interfere with conception as well.

Sometimes a surgeon will shorten the recuperation time to one cycle, depending in part on the scale of the surgery. Some list members have become pregnant even without an intervening menstrual period and the pregnancies have gone well.

Recuperation time for an abdominal metroplasty will be longer; 3–6 months' waiting is a common recommendation, because of the full-thickness wound in the uterine wall.

It is prudent to have a follow-up HSG to confirm the results of surgery before trying to conceive. An HSG can be done after the first menstruation, ideally before the next ovulation, while the lining is thin and compact. It will show not only the changed configuration of the uterine cavity, but may reveal perforations.

What is surgery like?
Hysteroscopic metroplasty (uterine septum resection via the vagina) ; a.k.a. septoplasty, septotomy
During this procedure, a uterine (and also a vaginal septum, if applicable) can be incised to open the uterus up for a better pregnancy result. This surgery is usually done at the same time as a laparoscopy (see below) and is often referred to on the board as a “lap/hyst.” It can be done with scissors, an electrocautery tool or a laser tool, depending on what the surgeon is most experienced with.

"Resection" is not an accurate term for what happens, since no tissue is removed from the uterus; instead, the surgeon parts the septum, like cutting a swatch of material in half. On parting, the septum retracts close to the normal uterine wall on each side, where it is covered with endometrium within days or weeks.

It is a day surgery in most cases. Recovery is different from woman to woman, but most agree that 3–5 days are required. It is important to know that some women require more than one surgery to adequately resect a septum.

You prepare for laparoscopy by fasting and discontinuing meds as ordered (aspirin or metformin, for example). You may or may not be ordered to do a bowel prep (laxative, enema, clear liquids), and wash your abdomen with special soap such as Phisoderm for a period of time preceding the surgery, as well as trim back or shave the pubic hair.

When you are under anesthesia (usually general), you’ll be put in the lithotomy position (a lot like a Pap smear), draped and prepped. The surgeon will make two to four puncture wounds in your abdominal wall—one inside the navel (for the laparoscope) and one to three in the bikini line fold just above the pubic hair, for other implements. Sometimes a minilaparotomy incision, about an inch wide, is made on the bikini line at the midline, though this is not usually the case. The surgeon will then inflate your abdominal cavity with carbon dioxide to improve visibility, and have a look with the lighted scope at your pelvic organs.

The main purpose of a laparoscopy is to monitor the outside of the uterus for imminent or actual perforations as the surgery goes on inside the uterus, but other fertility-related surgery may be done as needed: lysing adhesions, removing fibroids or endometriosis, or ovarian laser drilling.

After the surgery, you can expect to feel sleepy, forgetful and possibly nauseated from the anesthesia and the pain medication, and that may last over 24 hours. You may feel pain in your abdomen, under the ribcage and even referred to your shoulders from remaining carbon dioxide within your abdomen. Expect to have a sore abdomen for the next week or so. Many women compare the feeling to that of having done too many sit-ups.

Hysteroscopy (HSC)
A hysteroscopy involves distending the uterus with a fluid or gas, inserting a scope and then doing one of two things: either operating (e.g. removing a polyp or fibroid, resecting a septum) or just documenting any abnormality and then withdrawing the instrument. You prepare for laparoscopy by fasting and discontinuing meds as ordered (aspirin or metformin, for example). You may or may not be ordered to do a bowel prep (laxative, enema, clear liquids), and you may be on progestins, danazol or GnRH analogs in order to thin the uterine lining prior to surgery. Surgeons' preferences vary widely. It seems to be most useful in improving visibility with a wide septum or a complete septum (one that extends to the cervical os or beyond).

While it is a minor surgery, any instrumentation of the uterus creates the risk of injury or infection, and HSC carries with it about a 1% risk of perforating the uterine wall. Most perforations are minor in nature, but if the wound is large or bloody, or injures other abdominal structures, such as the intestines, complications can be serious. Perforation also increases the likelihood of rupture during pregnancy or childbirth. HSC also creates a risk of fluid overload and serious electrolyte imbalance as the distending medium enters the bloodstream, although fluid status is continually monitored during surgery. There is also the risk that the surgery will not accomplish its purpose and may need to be repeated.

The advantage of HSC, of course, is avoiding abdominal metroplasty, a major surgery with all the attendant risks.

Hysteroscopy itself does not seem to cause pain during recuperation, although the accompanying laparoscopy tends to give a sore abdomen. Expect to bleed for a day or two, then to see the flow lighten to a watery pink. After a few days, the flow should change to a watery, slightly yellowish serous fluid, and disappear at roughly two weeks post surgery. Foul-smelling discharge or a fever warrants an immediate call to the surgeon because of possible infection.

Some surgeons like to leave an inflated Foley catheter in place to hold the sides of the uterus apart, or to leave some other adhesion barrier in there. Barriers tend to increase bleeding and cramping beyond a few days, and some surgeons believe that barriers increase the risk for intrauterine adhesions and ascending infections.

An experienced surgeon may choose to waive the laparoscopy during HSC, but this is the exception, not the rule.

As often as not, an operative HSC is followed up with a course of conjugated estrogens to help promote regrowth and proper healing of the endometrium, especially if the patient has taken premedication to thin the uterine lining. Studies increasingly show postoperative estrogen to make little difference in re-epithelialization of the cut septum.

Antibiotics during or after surgery are also commonly given, although risk of endometritis is low.

An exploratory (rather than operative) hysteroscopy is an office procedure, frequently done while the patient is awake. Since the uterus needs to be expanded with saline, glycine or carbon dioxide, this can be quite painful, similar to having a hysterosalpingogram (HSG).

Cerclage is a stitch placed around the opening of the uterus, usually the cervix, to hold the uterus closed as pregnancy advances, and is done in cases where there has been a loss due to painless prolapse of the membranes in the second trimester of pregnancy. The McDonald stitch is the most common form of cerclage done. It is a suture done with mersilene or proline suture material, under local anesthesia (usually a low, short-acting spinal block). It takes only a few minutes to place, and recovery and monitoring following the procedure take only a few hours. Bedrest for 48 hours after the procedure is recommended

The patient is placed in the lithotomy position following the spinal or epidural anesthesia, and the area is draped and swabbed with antiseptic. A kind of caliper called a tenaculum is used to pull the cervix closer to the mouth of the vagina and manipulate the cervix as the surgeon makes the sutures. The patient may feel a painless tugging sensation as this is done. Sedation is optional; some surgeons feel that tranquilizers or general anesthesia pose an unnecessary risk to the developing fetus.

The patient is monitored closely for signs of bleeding, contractions and ruptured membranes and then usually discharged the same day, after she is able to void her bladder into the toilet. Sometimes an overnight stay is needed. The surgeon may or may not prescribe bed rest beyond 2–3 days, depending on individual circumstances. "Pelvic rest," or refraining from intercourse and orgasm, is also frequently advised, because of the infection risk and tendency for a cerclaged uterus to be irritable. At minimum, pelvic rest is advised for one wekk prior to and one week following surgery.

Cerclages work well (up to 90% success rate), but some do fail. In that case, a second stitch can be placed, and the patient is put on strict bed rest, which involves taking meals lying down and using a bedpan. One alternative to cerclage is strict bed rest by itself, beginning in the early second trimester; some studies show it to be just as effective as cerclage. The advantage of cerclage is, of course, avoiding the health risks of lying in bed for several months.

One contraindication for having a cerclage is active labor, since this may cause the stitch to tear through the cervix. Consequently, most cerclages are removed around 37 weeks, to allow effacement and dilation to occur naturally. Some women with very weak cervices experience labor and birth almost immediately after cerclage removal, but not always. Sometimes the cervix develops scar tissue from the cerclage and does not thin out or open up easily (cervical dystocia), which may necessitate a c-section.

Opinions vary as to when it is appropriate to place a cerclage. Current trends point to earlier placement—at 10 weeks or as soon as the embryo looks viable on ultrasound. Prior conventional wisdom indicated placement at 12–14 weeks, after it becomes 97% certain that the pregnancy will continue. However, ultrasound technology can predict a positive outcome with 95% certainty at 10 weeks, LMP.

A cerclage done to salvage a threatened pregnancy, after the membranes have begun to prolapse, is called an emergent or rescue cerclage. A rescue cerclage requires bed rest, and the prognosis is not as good as that of a cerclage done well before cervical shortening begins.

For patients with underdeveloped cervices or repeated cerclage failures, an abdominal cerclage promises some success. Prior to conception, the suture is placed around the lower segment of the uterus, deep to the uterine arteries, and is done through a laparotomy. Delivery must be by c-section, and the cerclage can be left in place for the next pregnancy.

If your c-section is planned, you will be asked to take nothing by mouth for several hours prior to surgery. At the hospital, you will be hooked up to a fetal monitor for a time and an IV of normal saline or Ringer's lactate will be started in your non-dominant hand. You may be shaved, prepped (enemas are passé!) and catheterized prior to the spinal anesthesia. The nurses will put tight anti-embolic stockings on your legs to reduce the risk of blood clotting while you are immobile.

Regional anesthesia is increasingly the method of choice for controlling pain. Shortly before surgery, the nurse anesthetist or anesthesiologist will start a spinal anesthetic of a fairly short-acting substance, such as lidocaine. You will be asked to lean forward and "shove" your back towards the anesthetist, to make room for the needle to enter the spinal space. You will feel a small prick right about at kidney-level, slightly off-center. When the anesthetic is introduced, you will lose a sense of having a lower body from the nipple line on down, and a warm, not unpleasant feeling may overtake you. Assistants help you lie back on the operating table and one or both arms are strapped at 90° angles to your body. Someone will apply a pulse oximeter, EKG leads and an oxygen mask or nasal cannula. You may begin to feel tingling or numbness in your thumbs and a difficulty swallowing, and while disconcerting, this is seldom a problem. A medication may be introduced into your IV to help with any feelings of anxiety.

Meanwhile, nurses expose your abdomen, scrub it with antiseptics and drape it with sterile drapes. Your surgeon will probably make a 10-cm. horizontal incision just superior to the pubic bone, above the hair line—a pfannensteil incision. Working very quickly, the surgeon will part and retract the underlying tissues, exposing the uterus. The uterus is opened with (usually) a horizontal incision, and the amniotic sac incised. Amniotic fluid is allowed to drain away. In many cases, the patient may not even have realized that surgery has begun.

While an assistant presses downward on the fundus, the surgeon reaches into the uterus and delivers the fetus' head. Taking gentle traction on the head, he or she delivers the baby rapidly. You may feel a pressure or rocking as they work.

Then comes the joyful part, as the baby is assessed and begins to cry. You may be able to touch, kiss or hold the baby, or even nurse the baby immediately following birth (this is something to work out beforehand in your birth plan with your obstetrician).

Meanwhile, the uterus itself is often delivered outside the laparotomy incision, inspected, suctioned, massaged and sutured, then replaced into the pevlic/abdominal cavity. This may be a good time to have a tubal ligation performed, if you do not want any more children. And your surgeon may ask if you want any septum present to be resected. One school of thought holds that childbirth is a risky time for a metroplasty because of increased vascularity and risk of infections and adhesions, but there are two documented cases of septa being successfully resected during a c-section.

Expect to spend an hour or two in recovery, being assessed and reassessed, and having your fundus massaged. With luck, you will be able to bond with your baby during this time. You will remain in bed, catheterized, until the anesthesia wears off, possibly with pneumatic sleeves on your feet or lower legs that periodically inflate to stimulate circulation in your legs. Once the catheter is out and you are able to void, you will be encouraged to stand, sit and walk around as soon as possible.

The level of discomfort following a c-section varies widely, and you may or may not require much pain medication. If it is painful to nurse your baby, a pillow over the incision may distribute pressure more comfortably. You will have lochia (postpartum flow), but it tends to be lighter than that of a vaginal birth. A typical hospital stay following a c-section is 2-5 days. Following hospital discharge, you will be asked not to lift anything heavier than your baby, or to drive, to minimize the risk of adhesion fomration immediately following the surgery. The stitches or staples closing the incision usually come out within a few days following surgery, and are replaced with steri-strips or other dressing. The scar gradually thins and fades in color.

Vaginal birth following a lower segment incision is definitely possible, with the right provider.

An emergency c-section is done after the mother goes into labor and it becomes apparent that she needs a section. A crash c-section is done when the fetus is at risk of hypoxia or death. In both these cases, anesthesia may be by epidural block or general.

Thursday, March 20, 2008

What are some of the pregnancy complications associated with MA's?

Before reading this list of possible complications, it is important to remember that most babies born to women suffering from MAs encounter none of these problems. These are simply possibilities to keep in mind, and most of them come from the potential poor attachment/blood supply of the placenta, or the lack of space.

a. Miscarriage: Spontaneous abortion is very common, especially with the septate uterus, because of blood flow disruptions and possibly hormonal receptor abnormalities. The normal miscarriage rate for a woman with no fertility or anatomical problems is 20%, or 1 out of 5 pregnancies lost. In the septate uterus, which has the worst pregnancy outcomes, some studies show a miscarriage rate approaching 90%.

b. Incompetent cervix: As many as 20% of anomalous uteri may have additional problems, such as incompetent or weak cervix. Such cervices tend to give way between 16 and 22 weeks’ gestation. A woman with a known MA should ask her OB to check for this possibility. Cerclage—sewing a purse stitch around the cervix to keep it closed—is the most common remedy.

c. Placenta previa: Because of the tight quarters in an abnormal uterus, it is a little more common for the placenta to ride low and cover the inner cervical os. This condition poses a bleeding risk, but may correct itself as the pregnancy advances and the uterus stretches upward.

d. Abruptio placenta: Because of the abnormal configuration, contractions and vasculature within an abnormal uterus, problems with its attachment to the uterine wall may arise.

e. Premature labor: Although no one knows all the causes of premature labor, expansion restrictions may be one reason why an anomalous uterus is more prone to it. Another may be disorganized contractions of the abnormal muscle in a septum.

f. Abnormal fetal lie: Breech position is very common in an abnormally shaped uterus, possibly because there is more room for limbs at the bottom, or because the fetus loses its ability to roll 180° earlier than a fetus in a normal uterus.

g. Intrauterine growth restriction (IUGR): Again, because of the possible placental vascular insufficiency, the fetus may not get all the nutrients it needs, and may be small for dates. One form of IUGR tends to spare the brain, and the fetus, if born prematurely, does quite well despite the small size.

Should I see a regular OB-GYN during pregnancy?

While it should be remembered that many women with uterine anomalies have no trouble at all during pregnancy, one can never go wrong erring on the side of caution. If the MA is a severe one (a deep SU or BU, or any UU), it might be wise to at least consult with a high-risk specialist (perinatologist) early in the pregnancy, or have one co-manage your case along with your OB-GYN. Or you might simply interview your OB-GYN and find out how much MA experience he or she has, what his or her philosophy of care is, and whether or not you feel comfortable with it.

Expect to have your cervix length evaluated (sometimes a cervix weakens when the baby is crowded) and possibly treated with a purse stitch (cerclage) if it begins to open up in the second trimester. You may also expect to be monitored for premature labor, intrauterine growth retardation and abnormal fetal lie during your pregnancy; these pregnancy complications are more common with müllerian anomalies.

Monday, March 10, 2008

Müllerian anomaly myths

This section deals with misinformation. Many women with müllerian anomalies have been horrified by the predictions of doctors who have not had a lot of exposure to women with müllerian anomalies. Some have been told they will only have miscarriages and preemies. A few have even been told to consider adoption or surrogacy, and that trying to conceive would be foolish. If you have experienced this, request a referral to a Reproductive Endocrinologist (RE), who can better assess your prognosis. Please read on.

A. You Cannot Have Children
  1. You will not be able to get pregnant because of your MA — FALSE. In most women, having a müllerian anomaly alone does not affect their fertility. If there are other fertility concerns (e.g. damage to the single connecting fallopian tube in a Unicornuate uterus) an MA is one more factor to consider during fertility treatment.
  2. You will never carry a baby safely — FALSE. Many women find this web site after one or more devastating losses. The thing to remember is that with proper medical supervision, most women can carry their pregnancies to a safe, live birth. After conception, a woman with a müllerian anomaly should consult a high-risk OB/GYN (also called Perinatal Specialists or Maternal-Fetal Specialists) to discuss how their pregnancies will be monitored. A high-risk OB will monitor your cervix for signs of incompetence and preterm labor, your baby for signs of stress and your amniotic fluid for lower levels. These sound scary but can be managed, and if someone is watching you from the start, often prevented. Some of our mothers went overdue. Others have had twins. In fact, a number of mothers on this board have been discharged from their high-risk OBs because they were doing so well.
B.Your mother took DES (or was otherwise responsible) — Not always
  • It is true that some mothers who were given a drug called DES while they were pregnant gave birth to daughters who had distinctive T-shaped uteri. However these young women make up just a fraction of women who are diagnosed with müllerian anomalies. The fact is, the cause of MAs is simply not completely understood.
  • From time to time an ignorant person may assume that a müllerian anomaly is the result of venereal disease or abortion. The answer is always false; MAs are always a birth defect.
C. You can not have a vaginal birthNot always
Studies and anecdotal evidence support the notion that the uteri of women with müllerian anomalies may be prone to stretching differently than a normal uterus while a baby grows. This can result in a breech or transverse fetal lie towards the end of pregnancy. In these cases, a cesarean birth may be scheduled in advance. This is just a tendency, however, and a number of our members have had uncomplicated vaginal births. Many women on this board feel cheated and mourn the loss of the vaginal birth experience. If you feel this way, you aren’t alone.

D. One surgery can remove your septumSadly, this is not always true
While many of our members have had one surgery “do the trick”, quite a few have been shaken to find out a second, and perhaps even a third surgery may be necessary to remove as much septum as possible. In any case, most postoperative HSG's do reveal an arcuate-looking uterus, and a residual septum of less than 1 cm. apparently does not affect pregnancy outcomes. Some researchers even recommend leaving a residual "stump," to guard against rupture. Please read on:
“A surgeon could leave too much septum behind if he only cut the septum at the cervix end of the uterus, but stopped too soon, before he got anywhere near the fundus. If a surgeon does this, only some part of the septum snaps back, but some of it is left intact… This can happen if the surgeon is not experienced, or, if there is poor visibility in the scope due to too much blood or endometrial tissue.”

E. Your "tipped" or "tilted" uterus is causing your infertilityFalse
A tipped uterus refers to the very common condition of the uterine fundus' pointing somewhere besides slightly forward, toward the navel. Instead, it may be pointing straight up, backward, or may be flexed forward more than usual. This is almost never a problem, beyond the potential for causing painful intercourse. In short, the tipped uterus is not a müllerian anomaly, does not cause infertility, and usually "cures" itself by the 10th week of pregnancy.

Wednesday, March 5, 2008

A list of Terms and Abbreviations

Müllerian Anomalies: Terms
This is a short list of abbreviations and specialized terms used when discussing Müllerian Anomalies on this site.
  • AF: "Aunt Flow" or menstrual period.
  • Anlage: The name for the undeveloped or rudimentary müllerian duct.
  • Arcuate: AU A variation of normal uterine development in which the top of the uterus has a slight dip. Rarely a problem.
  • AU: "Arcuate uterus"
  • BD: "Baby-dancing," or sex intended for conception.
  • BFN: "Big Fat Negative," on a home pregnancy test. "Fat" may be substituted by the F-word of one's choice.
  • Bicollis: Meaning "2 cervices." Many septate, bicornuate and didelphys uteri can have a double cervix. Eg. "uterus bicornis bicollis."
  • Bicornuate: BU Meaning "two horns," it describes a uterus with a distinct division (> 1 cm.) visible from the outside, caused by incomplete fusion of the two müllerian ducts. A.k.a. "uterus bicornis."
  • BMS: "Baby-making sex"
  • BU: See: Bicornuate uterus.
  • CC: See: Clomid
  • Clomid: Brand name of "clomiphene citrate," an oral fertility drug. A.k.a. CC.
  • Cornua: Term for the two horns of a uterus, normally united to form a cavity shaped like an upside-down triangle. The cornua are more separate and pronounced in müllerian anomalies.
  • DD: "Dear daughter."
  • DES: See: Diethylstilbestrol.
  • DH: "Dear husband."
  • Diethylstilbestrol: DES. Artificial hormone given to pregnant women in cases of threatened abortion, especially during the 1960s and early 1970s. DES was found to cause many reproductive abnormalities in the fetus, including a small uterus with a T-shaped cavity in the female.
  • DS: "Dear son."
  • DW: "Dear wife."
  • Dysmenorrhea: Painful menstrual cramps. Women with uterine anomalies frequently have painful menstruation, typically from associated endometriosis, outlet obstruction, retrograde menstruation and the disorganized muscle contractions caused by an intrauterine septum.
  • hCG: "Human chorionic gonadotropin." This is the hormone made by the embryo's placenta. The pregnancy hormone.
  • Hemihysterectomy: Removal of one uterine horn, commonly done for the tiny, undeveloped horn opposite a unicornuate uterus.
  • Horn: Common term for the hemi-uterine cavities found in bicornuate and septate uteri.
  • HPT: Abbreviation for "home pregnancy test."
  • HSC: "See: "Hysteroscopy"
  • HSG: See: "Hysterosalpingogram"
  • Hypoplastic: Term meaning small, or underdeveloped. The small uterus of a DES daughter is said to be hypoplastic.
  • Hysterosalpingogram: The x-ray "dye test" good for showing the shape of the uterine cavity and whether or not the oviducts are open.
  • Hysteroscopy: A minimally invasive surgery in which a lighted scope is inserted through the cervix of the uterus. It can be done just to check the uterine cavity ("diagnostic hysteroscopy") or to correct polyps, septa, adhesions, etc. ("operative hysteroscopy"). General anesthesia is used for the latter.
  • IC: See: incompetent cervix.
  • Incompetent cervix: IC. A condition in which the cervix opens up under the weight of a growing pregnancy, and and very common in women with müllerian anomalies. Sometimes the lack of uterine volume forces open an otherwise normal, competent cervix, but it is also the case that a cervix can be malformed or congenitally weakened, as in the case of DES daughters.
  • Intrauterine insemination: Placement of live, washed sperm inside the uterine cavity with a catheter. A.k.a. IUI.
  • Intravenous pyelogram: "IVP." This is an x-ray dye test of the kidneys, done in women with müllerian defects, most especially those with asymmetrical defects, such as uterus unicornis. Since the urinary and reproductive tracts form at the same time, it is not unusual for a woman with only one uterine horn to lack a kidney or ureter on the opposing side. Likewise, it is sometimes possible to have a "horseshoe kidney," that is a single large kidney extending from one side to the other, caused, much as a septum is, by the failure of some embryonic structure to deteriorate at the right time.
  • IUGR: "Intrauterine growth retardation," a condition common when uterine volume is diminished, in which the fetus does not obtain sufficient nutrition from a rapidly aging placenta and is small for gestational age. The placenta deteriorates more rapidly because it is overly compressed as the pregnancy progresses. IUGR also has other causes, such as autoimmune disorders and diabetes.
  • IUI: See: Intrauterine insemination.
  • IVP: See: "Intravenous pyelogram"
  • Jones & Jones metroplasty: A type of alteration of the uterus done through a laparotomy.
  • Lap/hyst: Abbreviation "Laparoscopy/hysteroscopy"—a combination of two operations in which the uterus is inspected inside and out, to determine the extent of the malformation. A lap/hyst is the `gold standard' of diagnoses in differentiating between a septate or bicornuate uterus. It is also commonly done during hysteroscopic septoplasty to monitor the operation and verify whether or not a uterine perforation has occurred in the course of the hysteroscopy.
  • Laparoscopy: The inflation of the abdomen with carbon dioxide gas and the insertion of a lighted scope through the navel, through a half-inch incision. Additional incisions for manipulating instruments may be made at the pubic hairline. Laparoscopy may be done to diagnose a uterine anomaly, to operate within the abdomen, or to monitor a hysteroscopic procedure. A laparoscopy is classified as major surgery, but recovery time is considerably shorter than that of a laparotomy. "Keyhole surgery."
  • Laparotomy: a surgical incision in the wall of the abdomen large enough to admit conventional surgical instruments.
  • MA: See: Müllerian anomaly. A.k.a.: Müllerian duct anomaly.
  • Magnetic resonance imaging: MRI A noninvasive test useful in seeing the contours of the uterus and differentiating between a septate and bicornuate uterus.
  • Malpresentation: Common in women with decreased uterine volume, malpresentation is the position of a fetus in the uterus such that some other part besides the head will be coming out first. "Breech presentation," in which the feet or buttocks present first, is the most common malpresentation in women with uterine anomalies, and a common reason for birth by c-section.
  • M/c: "Miscarriage."
  • Metroplasty: A general term for the surgical alteration of the uterus, be it surgery to remove a septum or to unite the two horns of a bicornuate uterus. A subset of metroplasty is septoplasty.
  • MDA: Abbreviation for müllerian duct anomaly. a.k.a. müllerian anomaly.
  • MRI: See: Magnetic resonance imaging
  • Müllerian anomaly: A developmental abnormality of the internal female sex organs resulting from the failure of the müllerian ducts to either fuse and/or resorb properly. Müllerian anomalies include hypoplastic uterus, arcuate uterus, septate uterus, bicornuate uterus, unicornuate uterus, T-shaped (DES) uterus, uterus didelphys, Rokitansky Syndrome, and others.
  • Müllerian ducts: Two long tubular structures found in both the male and the female embryo. In the male, these ducts dissolve, but in the female, they unite to form the uterus and oviducts.
  • OPK: Abbreviation for the home test kit that predicts ovulation.
  • Retrograde menstruation: The reversal of menstrual flow; it goes from within the uterus to the pelvic cavity by way of the Fallopian tubes. It is thought to be one cause of endometriosis, which is more common in women with MAs. Retrograde menstruation is also more common in cases of MA.
  • Rokitansky Syndrome: The congenital absence of a uterus and upper vagina—the most severe form of Müllerian defect. Also known as "müllerian agenesis."
  • Rudimentary horn: the small, undeveloped horn of a unicornuate, didelphic or bicornuate uterus. A.k.a. anlagen.
  • Septate: SU. Adjective describing a uterus with an extra fibrous/muscular band in the middle of its cavity, giving it two horn shaped hemi-uterine cavities. This is the most common müllerian anomaly, and results from the partial or total failure of the wall between the united müllerian ducts to dissolve. A.k.a. "uterus septus." Subcategories of the septate uterus include "total," or "complete," in which the septum involves the cervical canal and even the vagina, and "subseptate," in which the septum's lower end stops short of the cervical canal.
  • Septoplasty: Surgery to remove a uterine septum, usually done by operative hysteroscopy.
  • Septum: The name for the fibrous wall dividing the cavity of a septate uterus.
  • SHG: See: Sonohysterogram
  • Sonohysterogram: SHG. Ultrasound of the uterus and its cavity, aided by the distention of the uterine cavity with saline solution.
  • SSU: "Subseptate uterus"
  • Strassman metroplasty: A type of alteration of the uterus done through a laparotomy.
  • SU: See: Septate uterus.
  • Subseptate: SSU. A form of septate uterus in which there has been a partial dissolution of the embryonic structure dividing the uterine cavity in two. The septum does not run the full length of the uterus.
  • T-shaped uterus: The characteristic shape of the cavity of a uterus affected by diethylstilbestrol.
  • Tompkins metroplasty: A type of alteration of the uterus done through a laparotomy.
  • TTC: "Trying to conceive"
  • UD: See: Uterus didelphys
  • Ultrasound: "US." A tool which can reveal the inner and outer contours of solid bodily organs such as the uterus and kidneys. Sometimes useful in distinguishing between septate and bicornuate uteri. Synonymous with "sonogram."
  • Unicollis: Term meaning "one cervix." Most müllerian anomalies involve the presence of only one cervix. Two cervices are most commonly associated with uterus didelphys, but not always. E.g.: "uterus didelphys unicollis."
  • Unicornuate: UU. An asymmetrical uterine anomaly in which one of the two müllerian ducts has failed to form properly. The hemiuterus is a small, banana-shaped organ frequently accompanied by an anlage, or rudimentary uterine horn or bud, which may or may not have an open endometrial cavity. Pregnancies in the smaller horn almost invariably rupture, and to prevent this, a hemihysterectomy may be recommended. Women with UU usually have bilateral ovaries, and may have an associated kidney anomaly on the side with the anlage.
  • US: Abbreviation for "ultrasound."
  • Uterus Didelphys: Term for the existence in a woman of two separate hemi-uteri, usually each with its own cervix. Some degree of vaginal duplication may also be present. Literally means "two wombs."
  • UU: Abbreviation for unicornuate uterus, a.k.a. "uterus unicornis."
  • Vaginoplasty: Any surgery done to alter the shape of the vagina. With some müllerian anomalies, especially UD and total SU, there may be a septum present in the upper end of the vagina.