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Uterine Septum |
Unicornuate Uterus |
Bicornuate Uterus |
Didelphic Uterus Case: 28 year old G2 S2 undergoes a basic clinical evaluation for recurrent pregnancy loss and the only abnormal finding is a hysterosalpinogram that identifies a wedge shaped defect at the top (fundus) of the uterus that is consistent with either a uterine septum or a bicornuate uterus. Question: Should this woman undergo treatment for this abnormality? Answer: Hysteroscopic transection of a uterine septum can be performed on a “same day surgery” basis, has minimal surgical risk, and the postoperative recovery period is generally a few days. Laparotomy with reconstruction of the uterine wall for a bicornuate uterus requires a hospital stay (a few days), has relatively low surgical risk, and the postoperative recovery period is about 6 weeks. Septum transection may dramatically improve the chance to carry a pregnancy to term. Reportedly, the pregnancy loss rate is as high as 90% without repair and as low as 10% following repair. The surgery involves laparoscopy (to rule out a bicornuate uterus and avoid uterine perforation during hysteroscopy) and hysteroscopy (to transect the septum), both of which are same day surgical procedures. Patients usually do very well postoperatively, have very little discomfort, and rapidly return to work. In this situation, I suggest the couple consider the minor surgery to assess the situation thoroughly. If a bicornuate uterus is ruled out and a septum identified, then I suggest transection since the recovery time is not increased. I usually provide the patient with 1-2 months of higher dose estrogen (with progesterone withdrawal) to re-surface the lining over the transected defect. If a bicornuate uterus is identified, then a laparotomy (larger abdominal incision with longer recovery period) is required and I leave this decision up to the couple. If they decide (preoperatively) that they would want to have a reunification procedure with laparotomy if a bicornuate uterus is found, then I proceed directly to an open case. If they decide that they want to stop with the minor surgery, then the pelvic evaluation is completed and if a bicornuate uterus is identified I stop at this point. If the patient continues to have pregnancy losses with a bicornuate uterus and all other factors have been ruled out or appropriately treated then I would suggest (re-) consideration of surgical repair (a uterine reunification procedure). Case: 25 year old G2 S2 with recurrent pregnancy losses has a uterine defect (septum versus bicornuate uterus) on hysterosalpingography (HSG) and also has a luteal phase defect (progesterone insufficiency problem) on endometrial biopsy. Question: Should this patient have a hysteroscopic transection of the septum prior to correction of the luteal phase defect, visa versa, or some other plan? Answer: Many evaluations for subfertility or recurrent pregnancy loss will find more than just one isolated abnormality. In these situations, developing a treatment plan for the identified problems depends on the risk to benefit assessments for the treatment of each identified problem and the couple’s overall desires. In this situation, the wedge shaped defect seen on HSG may be due to a bicornuate uterus—which do not need to be repaired unless there are recurrent losses after all other causes of recurrent pregnancy loss have been treated. I would suggest treatment of the progesterone insufficiency problem if a bicornuate uterus is identified. In this case, a uterine septum may have caused the wedge shaped defect on HSG. Septums are associated with a high rate of pregnancy loss and so I would suggest initially transecting the uterine septum hysteroscopically (if found to be present). Pelvic evaluation (laparoscopy and hysteroscopy) is the most definitive way to determine whether the defect identified on HSG represents a uterine septum or bicornuate uterus. Another possible way to distinguish the two is an MRI, which theoretically can see the soft tissue changes associated with either of these two abnormalities. I personally have not had good experiences with the local MRI centers near my office(s), since many times the patient will have an MRI and the diagnosis remains uncertain. This couple could (reasonably) consider an MRI to determine whether there is a septate or bicornuate uterus. If a septate uterus is identified or the diagnosis is unclear following the MRI, then a pelvic evaluation can be useful to identify and treat a possible uterine septum. If a bicornuate uterus is identified on MRI, then treatment of the luteal phase defect may (reasonably) be attempted initially. Case: 26 year old G0 with a history of an infertility evaluation identifying a wedge shaped defect at the top (fundus) of the uterus. Question: If there have been no prior pregnancy losses, should a uterine septum be transected if identified? Answer: Uterine septums generally do not inhibit one from getting pregnant, rather, they usually lead to recurrent pregnancy loss. If a uterine septum has been suggested on HSG and is confirmed on pelvic evaluation (when performed as part of an infertility evaluation) then I would generally suggest transecting the septum since it carries a high risk for pregnancy loss. If a uterine septum is suggested on HSG and is confirmed with MRI (if a bicornuate uterus is ruled out) then I would discuss the treatment options with the couple in detail. If the couple wants to minimize their chance for a future pregnancy loss then I would suggest transecting the septum. If the couple would rather try to get pregnant without prior surgery, then I would only suggest hysteroscopic transection if the couple experiences pregnancy losses in the future. Case: 32 year old G3 S3 undergoes transection of a deep uterine septum. Question: Should the patient take medication to minimize the risk for adhesion (scar) formation within the uterine cavity? Answer: I routinely provide my patients with 1-2 months of high dose estrogen (with progestin withdrawal), such as premarin (2.5 mg a day x 30 days, possibly repeated once) and provera (10 mg a day on days 21 to 30 of premarin, possibly repeated once). I have not had difficulty with postoperative adhesion (scar) formation within the uterine cavity using this protocol. Other reproductive surgeons may suggest use of mechanical barriers (with antibiotics) to reduce adhesion formation. Case: 15 year old with progressive and debilitating pain during menses, a normal pelvic examination at her gynecologist’s office, and an ultrasound revealing a normal uterus deviated to the left side with a solid right adnexal mass. Question: What other tests could be done to identify the cause of this woman’s pain? Answer: 15 years of age is young to have debilitating pain with menses. When this does occur, it is often associated with a Mullerian anomaly possibly in conjunction with endometriosis. A unicornuate uterus with a second largely degenerate noncommunicating uterine horn (that has an endometrial cavity that is able to respond to estrogen and progesterone hormonal stimulation) may result in blood becoming “entrapped” within the noncommunicating horn during menses. This entraped blood can cause severe pain. A noncommunicating uterine horn with a patent fallopian tube can result in the release of abundant menstrual blood and endometrium into the pelvis, where it can result in extensive implants of endometriosis. This can also cause a great deal of discomfort. I suggest a hysterosalpingogram for young women with this sort of history. If the patient is intolerant to this test, then an MRI may also be useful. In this case, the woman was found to have a unicornuate uterus (may seem to be normal on examination and ultrasound) and the adnexal mass was a noncommunicating uterine horn. Case: 24 year old G0 with a history of a unicornuate uterus with a noncommunicating uterine horn, no dysmenorrhea (pain with menses), and subfertility. Question: Does this patient require surgery to treat the noncommunicating horn? Answer: Noncommunicating uterine horns may cause intense pain with menses due to the presence of blood that becomes “entrapped” within the uterus, which causes severe prolonged uterine contractions. If there is no pain with menses, then one must assess the need for surgery carefully while weighing its risks and benefits. Noncommunicating uterine horns that do not cause significant pain will usually have patent (open) fallopian tubes. The monthly endometrial growth would then be expelled into the pelvis and would predispose the woman to developing endometriosis. Since there is a discrepancy between the amount of endometriosis present and the amount of pain that is caused by the endometriosis, one cannot use the lack of menstrual pain to suggest an absence of endometriosis. If the infertility evaluation is otherwise normal following an assessment of ovulation, the sperm, the hysterosalpingogram, and the postcoital test, then I recommend consideration of a pelvic evaluation (to identify and treat such pelvic pathology as adhesions and endometriosis). In this situation, if the pelvic evaluation were normal except for the presence of a noncommunicating horn, then I would not recommend removal of this horn. Case: 18 year old G0 with a unicornuate uterus and a small communication to a second (partially distended) uterine horn, absence of pain with menses, subfertility, and an otherwise normal basic infertility evaluation. Question: Is surgery advisable to improve fertility? Answer: The distension of the uterus by entrapped blood (hematometra) often causes pain due to expansion of the uterine wall and subsequent uterine contractions. If there is a communication between the uterine horns, then this discomfort may be minimal. The distended uterine horn may lead to a vaginal septum that causes a vaginal obstruction of blood (hemotocolpos). This situation typically leads to great discomfort especially around menses. If there is a communication between the uterine horns and the second horn does not have a connection (through a cervix) to the vagina, then the contents of the second horn may cause significant pain or subfertility. In these circumstances, the distended horn can be removed or opened and re-unified to dominant horn. Surgery may be indicated if there is significant pain or subfertility that is not attributable to other pathology. Surgical repair involving unicornuate uteri should only be initiated by surgeons with deep experience in these sorts of cases. I typically would refer women with very rare anomalies from my office in New Jersey to a surgeon in Georgia since there are few surgeons in the USA with extensive experience in these types of cases. Case: 22 year old G2 S2 with a probable unicornuate uterus on hysterosalpingogram. Question: Are other diagnostic tests indicated once a unicornuate uterus is identified and is there any effective treatment for the unicornuate uterus? Answer: The size of the unicornuate uterus’s cavity is of central importance in predicting the woman’s reproductive potential. However, the size of the uterine (unicornuate) cavity is not entirely reliable at determining future reproductive potential since some women with a very small appearing cavity deliver at term without apparent difficulty and others have a large cavity with recurrent pregnancy losses. There is no surgical way to safely and effectively “open up” the uterine cavity of a unicornuate uterus. When a woman with a known unicornuate uterus does become pregnant, the Obstetrician should carefully consider the utility of a cerclage procedure since many of these uteruses have a “weak” or incompetent cervix. During pregnancy, there is a high risk for abnormal fetal positions (“lie”) such as breech or transverse. Many of these pregnancies within unicornuate uteri require Cesarean Section for delivery. There is a high risk of renal agenesis (absence of a kidney) on the side of the undeveloped Mullerian duct. Women with a known unicornuate uterus should have an ultrasound of the kidneys to assess this possibility. Case: 28 year old G2 S2 with an unicornuate uterus, a spontaneous loss at 10 weeks during the initial pregnancy, and a spontaneous loss with “painless dilatation” of the cervix at 18 weeks for the second pregnancy. Question: What can be expected during future pregnancies for this woman and what should be done to limit the chance of pregnancy loss in the future? Answer: Painless dilatation is strongly associated with an incompetent cervix. An incompetent cervix is also known to be associated with an unicornuate uterus. A cerclage may effectively treat this condition. Sometimes, women with a Mullerian abnormality will carry each subsequent pregnancy further and further through gestation before suffering a loss. This seems to be due to each subsequent pregnancy “expanding” the cavity a little bit, possibly by mechanical pressure or possibly due to changes that naturally occur in pregnancy. Case: 32 year old G3 S3 (all within the first trimester) with a bicornuate uterus and an otherwise normal recurrent pregnancy loss evaluation. Question: Should a bicornuate uterus be repaired surgically in this situation? Answer: A bicornuate uterus is not always associated with recurrent pregnancy loss, so repair is often delayed until a problem presents. Some prominent reproductive endocrinologists recommend repair of a bicornuate uterus whenever it is identified and fertility is desired. The rationale for this recommendation is that these anomalies are associated with an increased risk for pregnancy wastage (although many women with a bicornuate uterus deliver at term without a history of pregnancy loss) and malpresentation of the fetus during labor (breech or transverse lie of the fetus increases the need to consider Cesarean delivery). In the situation described here, it appears that the recurrent losses may be due to the bicornuate uterus. It is also possible that the losses suffered by this couple are due to problems that are (currently) poorly understood (such as genetic or immunologic problems, where some of the testing available is still of unclear clinical value or “experimental”). My recommendation to this couple would be to consider the risks and benefits of the reconstruction procedure and the experimental treatments for immunologic causes of pregnancy loss. I generally would suggest the surgery over experimental treatments for immunological abnormalities because its benefit has been more clearly demonstrated in the available literature. Case: 19 year old G0 with 2 cervices (the cervix is the “mouth of the uterus” that normally extends into the vaginal vault, through which the fetus passes during labor and delivery) on vaginal speculum exam. Question: What should be recommended regarding further testing or treatment for this? Answer: If the Mullerian ducts fail to fuse at all during a female’s embryonic development (while growing within her mother’s uterus) then a complete duplication of the uterus (double uterus), cervix (double cervix) and upper vagina (double vagina) is possible. Occasionally, the vaginal septum will be connected to the lateral vaginal wall such that there is an obstruction that does not allow communication between the cervix (on that side) and the vaginal vault. This typically will result in severe pain with menses due to the entrapped blood and should be repaired surgically. If there is a duplication of the uterus, cervix and upper vagina without a vaginal obstruction then the need for repair is less obvious. The didelphic uterus is associated with an increased risk for abnormal presentation at labor and delivery (breech or transverse lie) and preterm labor, so that some reproductive endocrinologists may suggest repair upon identification. I generally suggest waiting to see whether a problem presents since the repair is very difficult technically and the results of the repair are not always beneficial. A surgeon with deep experience in such repairs should be sought if repair is considered. The didelphic uterus is reportedly associated with one of the highest chances for normal reproductive potential among the Mullerian anomalies. This patient should have independent pap smears of each cervix during routine gynecologic care. Case: 28 G0 with subfertility, an abnormal postcoital test, and a didelphic uterus (with double cervix and uterus). Question: If intrauterine inseminations are suggested, should a portion of the washed sperm be placed within each uterine horn at the time of intrauterine insemination? Answer: A reproductive age woman generally produces one mature egg per month. The twinning rate in the USA is about 1 in 90 so that about 1% of women may produce 2 mature eggs in a cycle. If there are two uterine horns each connected to their respective cervix and fallopian tube, then it is possible to perform an ultrasound to determine which ovary contains that month’s dominant follicle (and mature egg) and sperm can be placed into the cavity on that side. Otherwise, it is reasonable to place half of the washed specimen of sperm into each of the cavities since the concentration of post washed sperm is normally high (several million sperm per mL). Case: 36 year old G3 S3 with a known history of DES exposure in utero and a small caliber T shaped uterus on hysterosalpingogram. Each loss was associated with painful contractions. Question: What treatment options are available and which should be suggested? Answer: An abnormal size and shape of the uterine cavity is strongly associated with recurrent pregnancy loss. Surgical repair has been attempted historically with transection of the lateral wall of the uterine cavity. The uterine artery approaches the uterus from the lateral broad ligament at the level of the cervix and branches superiorly (towards the head) and inferiorly (towards the feet) where it normally courses through the muscular wall of the uterus. If surgical transection of the lateral wall of the uterus is attempted and major branches of the uterine artery are severed then the amount of bleeding that results may be enormous. This puts the woman at a high risk for hysterectomy and significant morbidity or even mortality. I do not recommend surgical repair of a small T shaped DES uterus since the benefits have not been established and the risks are high. If the uterus is not able to carry a pregnancy to term, consideration of a surrogate uterus may be considered. Surrogacy is complicated (from a legal point of view) within the USA, especially if a custody dispute evolves before the offspring is born. Courts sometimes may award custody to the surrogate (woman carrying the baby) rather than the “biologic parents.” Adoption is another possibility that can be considered. Case: 38 year old G2 S2 with a known history of DES exposure, a cockscomb cervix and a relatively normal appearing uterine cavity on hysterosalpingogram. Painless dilatation of the cervix was associated with the pregnancy losses. Question: What should be suggested for this couple? Answer: With two pregnancy losses a basic recurrent loss evaluation is appropriate, with treatment of identified problems. The structural abnormality of the cervix (cockscomb shaped cervical hood) is associated with DES exposure in utero and is also associated with an incompetent cervix. In this situation, a cervical cerclage is also indicated to maintain closure of the cervix until labor and delivery. Case: 24 year old G0 with a large irregular uterus on exam (about equivalent to an 18 week size pregnant uterus) with a constant pressure (but no pain) within the pelvis, frequent urinary urge but no compression of the ureters (or dilatation of the kidneys) on ultrasound, and a general desire to preserve reproductive potential for the future. Question: Should surgery to remove the fibroid(s) be considered at this time? Answer: Uterine fibroids are common. Larger fibroids are encountered less often. If the fibroid mass is not causing a known cosmetic or medical problem, then removal is usually not necessary. The more common medical issues with larger fibroids are compression of the urinary system (which can result in the need to empty the bladder often, compression and dilatation of the ureter(s), dilatation of the kidneys), pelvic (or abdominal) pressure or pain (pain is most often associated with central cystic degeneration of the fibroid), inability to assess the other pelvic organs on examination (a fibroid greater than 12 weeks pregnancy size has historically been considered an independent reason for surgical removal since assessment of the ovaries becomes difficult on gynecologic exam, but currently assessment of the ovaries is possible using ultrasonography), heavy uterine bleeding with menses (large transmural fibroids or submucosal fibroids may result in distortion of the endometrial cavity and heavy menstrual flows), or rapid growth (rapid growth of tissue may suggest a greater possibility of malignant degeneration or a “cancerous” uterine sarcoma). I often suggest a hysterosalpingogram for fertility seeking women with a fibroid uterus since the shape of the uterine cavity may be tremendously distorted with fibroids. If the uterine cavity’s shape is normal, then surgery is generally not recommended from the reproductive point of view. Case: 26 year old G2 S2 with an ultrasound identifying a fibroid in the wall of the uterus (3cm diameter without apparent inward extension to the uterine cavity = lining). Question: Should removal of the fibroid be suggested? Answer: I routinely complete a basic recurrent pregnancy loss evaluation when such an evaluation is deemed appropriate. I usually suggest such an evaluation after 3 consecutive pregnancy losses if it has not been performed earlier (patient’s often request an evaluation after 2 losses). Fibroids are usually of unclear reproductive importance. Submucosal fibroids (that present into the cavity) may be associated with recurrent pregnancy loss and I suggest removing these masses. Submucosal fibroids are usually missed (not seen) on ultrasound examination but generally are seen during a hysterosalpingogram. Fibroids that compress and occlude the fallopian tubes, or those that distort the uterine cavity on HSG are reasonable to remove based on their (negative) impact on reproduction. If the recurrent pregnancy loss evaluation is completely normal except for the presence of a uterine fibroid and the fibroid extends to the uterine cavity, then removal of the fibroid is often considered. Case: 31 year old with unexplained infertility undergoes a pelvic evaluation (laparoscopy and hysteroscopy) to identify and treat a possible pelvic factor. On hysteroscopy, a small filmy polypoid structure within the uterine cavity is identified. Question: Should the irregularity within the cavity be removed? Answer: I often encounter filmy or dense irregularities within the uterine cavity on hysteroscopy. The pathology reports (which are based on the tissue’s appearance under the microscope) obtained from this tissue vary from “endometrium” to “polyp.” I usually cannot predict which diagnosis (normal endometrium or polyp) will be given since the visual appearance (on gross inspection) of the tissue can look similar. Generally, polyps are organized overgrowths of the lining of the uterus (endometrium) that have developed their own localized blood supply. Some abnormalities of the endometrium are more clearly polyps, but most of the abnormalities that I see are filmy and not clearly polyps. I routinely try to “smooth out” the lining of the uterus by removing any polypoid structures or irregularities that I encounter on hysteroscopy. It is possible that many of these irregularities are transient and would be removed naturally by the patient (possibly with the next menstrual flow). However, since I cannot predict whether these irregularities will persist if untreated I simply remove them when identified. Case: 33 year old G3 P1 (1995) S2 (1998 and 1999) with a small (1cm) rounded filling defect identified on hysterosalpingogram (HSG). Question: Should a hysteroscopy be performed to assess and treat the filling defect? Answer: A filling defect identified within the uterine cavity on hysterosalpingography is potentially of critical (reproductive) importance, since it may be a cause for infertility or pregnancy losses. The size of the filling defect is not really important, since even a small irregularity within the uterine cavity can interfere with implantation or the development of a normal pregnancy. I suggest removal of any persistent filling defect identified on HSG. Radiologists occasionally point out that the defect looks small, but from the reproductive point of view, even small defects can have importance. Many women with filling defects on HSG may have had a normal pregnancy in the past. Endometrial polyps and/or fibroids grow over time. Having a history of a normal pregnancy does not mean that the filling defect currently seen is unimportant, since it may have grown since the successful pregnancy. Case: 27 year old G2 S2 with a history of 2 prior D+Cs (dilitation and curettage procedures) now with irregular “string like” filling defects on hysterosalpingogram (HSG) and an otherwise normal recurrent pregnancy loss evaluation. Question: Should hysteroscopy be performed to further assess and treat the defects within the uterine cavity that were identified on the HSG? Answer: I suggest a hysteroscopic evaluation and treatment of the uterine cavity when significant defects are identified on HSG. The string like defects seen on this HSG may be artifact or may represent intrauterine adhesion (scar) formation (Asherman’s syndrome). Asherman’s syndrome may involve a spectrum of abnormalities, from thin string like scar tissue to thick very dense adhesions. The filmy adhesions can usually be removed easily with hysteroscopy while the very dense adhesions may require multiple hysteroscopic procedures for complete removal. Following repair of Asherman’s syndrome, there is an increased risk of placenta accreta (invasion of the placenta into the muscular wall of the uterus, which is difficult to remove at delivery), preterm labor, and postpartum bleeding. Case: 31 year old G0 with subfertility status post transmural myomectomy (removal of a fibroid from the wall of the uterus) during which time the uterine cavity was entered and repaired, now with an irregular area of filling defect (of the uterine cavity) on hysterosalpingography (HSG). There is no other identified cause of subfertility. Question: Should this defect seen on HSG be repaired? Answer: Surgical repair of any defect seen on HSG should be considered. In this situation, the defect identified on HSG appears to be related to postsurgical changes following a myomectomy. The defect now seen within the uterine cavity may represent an area of compression due to (sutures placed during) the reconstruction of the uterine wall or may represent postoperative adhesion (scar) formation. Given that the uterus has had adequate time to heal following the removal of a fibroid, I would suggest assessing the cavity directly with hysteroscopy with surgical transection of scar tissue or gross irregularities that are identified. This is usually a straightforward and low risk procedure using a type of hysteroscope called a resectoscope. Case: 25 year old G3 S2 at 8 weeks gestation (2 months pregnant) with a history of painless dilatation of the cervix (opening of the mouth of the uterus without painful contractions of the uterus) immediately prior to her 2 spontaneous pregnancy losses. The prior losses occurred at 14-16 weeks gestation (3-4 months pregnant). Question: Should a cerclage be placed for this pregnancy? Answer: Generally, the diagnosis of cervical incompetence (the inability of the cervix to maintain normal closure throughout pregnancy) is difficult and often uncertain. The history of painless dilatation of the cervix with pregnancy losses is very suggestive of cervical incompetence. Confirmation of the diagnosis may additionally involve testing the cervix when the woman is not pregnant (with either a hysterosalpingogram or a uterine dilator) or during pregnancy (with ultrasound evaluation of the cervix for shortening or dilatation). Given this woman’s history, which strongly suggests cervical incompetence, I believe that it would be prudent to suggest a cerclage regardless of additional testing. If there were any question about the utility of a cerclage in this situation, I would generally recommend a consultation (second opinion) with a perinatologist (high risk pregnancy expert). Case: 29 year old G3 S2 is now at 39 weeks gestation (full term pregnancy) with a cerclage in place for cervical incompetence, has painful intermittent contractions every 10-15 minutes. Question: Should the cerclage be removed? Answer: Shirodkar cerclages are generally not removed and require the patient to deliver via Cesarean Section. McDonald’s cerclages are generally removed when the patient is at term or goes into labor. If the patient labors with a cerclage in place, the suture material within the cervix can tear through the cervical tissue to cause trauma and possibly heavy bleeding. | |||||||||||
