Mullerian Duct Anomalies

Updated: May 17, 2024

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Background

MĂĽllerian duct abnormalities (MDA) are the birth defects of female reproductive system that had fallen during germs formation. Besides abnormalities of the female reproductive system, like the fallopian tubes, the cervix, and the vagina, this issue can also be manifest in various ways. In about 2 to 4% cases in the public in the general population, they are known to be very pervasive.

They are classified in a number of ways, but the American Society for Reproductive Medicine (ASRM) classification scheme is one of the most used and divides MDAs into seven primary subtypes:

Uterine agenesis/hypoplasia: This occurs when the uterus is small or missing.

Unicornuate uterus: The uterus, that is the only one on the whole length of her early embryo form, is being there.

Uterine didelphys: I delivered my own baby needing only an episiotomy and the only part of the reproductive tract that was duplicated was my cervix.

Uterus that is bicornuate: It features a fundus depression that gives the organ its typical heart-shaped appearance, resulting in a deeply concave appearance from the top.

Septate uterus: That septum that either totally or partly divides the uterus is found to exist.

Epidemiology

The degree of true incidence and prevalence of MĂĽllerian duct anomalies (MDA) in general population is not very well known because a huge selection bias and under reporting exists. The rate of incidence widely affects studies’ results that are usually between 0.1% and 3.5%. On the other hand, the risk is slightly higher in women with IVF treatment issues such as repeated pregnancy losses and infertility problems, ranging from 3% to 10%. This also varies considerably as reports cite values between 0.16% and 10%.

Anatomy

Pathophysiology

MĂĽllerian duct abnormalties (MDAs) is a whole spectrum of structural anomalies in the second type of female genital tract that arise because of disruptions in the embryonic development. In the refining years of fetal development, the MĂĽllerian ducts go through a range of fusion, resorption, and differentiation processes, in the end forming the uterus, fallopian tubes, cervix and upper vagina. Even a single unplanned incident or aberration in running the process could result in a spectrum of defects. As such, a septate uterus is developed due to inadequate re- adsorption of the uterovaginal septum which makes uterine cavity be into two divisions. Another congenital defect that results in hemispherical uterus is bicornuate uterus, which results from an incomplete fusion of the Mullerian ducts at the fundus, giving the uterine appearance of a heart-shaped figure.

Etiology

This disease that involves the structure of the MĂĽllerian ducts is multifactorial. The genetic background, the environment and embryo development can cause anomalies. Accordingly, the exact cause is not clear, but it is considered that genetic susceptibility and environmental factors affect the development of MĂĽllerian ducts in the wrong way. It has been found that the development of the MDAs was affected by the use of some substances such as DES by pregnant women at the embryonic stage.

Genetics

Prognostic Factors

The prognosis of MDA can be different for each kind and severity of the congenital anomaly. In this regard some MDAs women may be asymptomatic and do not need treatment while others may go through obstruction and require surgery that is destructive of their fertility potential.

Clinical History

Age group: MDAs can be present at different ages and it is determined by the type and severity of the deformity. For instance, pains that experience during puberty or young adulthood and even cause severe pain and affect menstrual cycle may be the side effects of certain drugs. The fact that you are not as fertile as you were before is clearly seen when a woman has problems conceiving or carrying the pregnancy.

Physical Examination

External Genitalia: Palpation of the external genitalia can disclose various anomalies such as abnormalities in external vaginal or cervix and also can give the clue of presence of MĂĽllerian duct anomalies.

Pelvic Organs: Manual palpation of the pelvic organs like uterus and ovaries can help in detection of any structural abnormalities or asymmetries that could be of MĂĽllerian ducts development related.

Associated Structures: Clinical assessment of the kidney and the urinary tract will be done because; renal abnormalities are rarely found with the Mullerian Duct Anomalies.

Age group

Associated comorbidity

The following MDAs may go along with several other regenerative conditions such as urinary tract anomalies, renal anomalies, and abdominal or pelvic pain. These comorbidities cannot be ruled out to affect the clinical manifestation of MDAs.

Associated activity

Acuity of presentation

The intensity of presentation of symptomatic MDA can fluctuate between asymptomatic and severe. Such as MDAs presenting with abdominal pain and obstruction may warrant surgical therapy while some asymptomatic MDAs don’t require medication.

Differential Diagnoses

  • Endometriosis
  • Uterine fibroids
  • Adenomyosis
  • Ovarian cysts

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Observation: A gynecologist may say that a gynaecological exam would be done and imaging studies would be ordered if the symptoms or any complications change.

Medical Management: Enhancing the symptomatic care or the treatment of other pertinent conditions like pain management, hormone therapy or medication.

Surgical Management: Many different chronic diseases caused by these structural problems require surgical interventions that are designed to restore normal uterine anatomy, improve reproductive results, eliminate symptoms, and avoid further complications.

Assisted Reproductive Technologies (ART): IVF can be broadly employed to avoid anatomical obstacles as well as make up for fertility deficits. Severe uterine anomalies may require the adoption of IVF fertilization with blastocyst culture and embryo placement into the womb of surrogate mother.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-a-non-pharmacological-approach-for-treating-mullerian-duct-abnormalities

Observation can be an option in the case of an asymptomatic individual or one having a small number of symptoms. These situations can call for a monitoring plan through regular checkups with a healthcare provider who can develop plans to control the condition effectively.

Lifestyle changes may also include physical therapy on the pelvic floor that can ease the discomfort of dysmenorrhea and the pelvic pain typically associated with MDAs. Patients may also benefit from counseling and education on their condition so that they are able to cope with any psychological or emotional effects.

Surgery may be recommended to patients suffering from these conditions or who have had a previous miscarriage or infertility problems. The available surgical options may include hysteroscopic metroplasty, laparoscopic metroplasty or open metroplasty, differentiating depending on the type of MDA and the patients\’ individual needs. To cure these structural abnormalities of the MĂĽllerian ducts these procedures aim to increase the possibilities related to the fertility outcomes.

Role of hormonal therapy in the treatment of MĂĽllerian duct anomalies

Hormonal therapy is one of the treatment modalities that have been established as the mainstay for managing menstrual abnormalities. While it may not restore the usual functioning of an organ or system, it can effectively correct the abnormality and help those suffering with this condition to achieve a better quality of life. Hormonal agents including COCs containing estrogen and progesterone would be one of the many useful ways to treat some dysfunctions of the menstrual cycle, specifically abnormal bleeding disorders. Dysmenorrhea which is usually seen is one of the symptoms that could be fought by decreasing the intensity and duration to a minimum at the peak of menstrual cramping. Addition of a progestogen often occurs, to prevent any endometrial injury due to estrogen alone, which can be in form of endometrial hyperplasia or cancer.

use-of-intervention-with-a-procedure-in-treating-mullerian-duct-anomalies

  • Hysteroscopic Metroplasty: Hysteroscopic metroplasty is one of the minimally invasive operations used in correction of septate uteri or bicornuate uteri, both of which are the types of MDAs that are subdivided by a septum the uterus. It is performed by inserting a hystescope via the cervix into the uterine cavity. Precisionist surgical instruments are employed cutting or tunnelling through the septate endometrium to create a single normal uterus. It is recognized for its superior results, including the minimal complication risk and helping individual to receive care as an outpatient.
  • Laparoscopic Surgery: Laparoscopic surgeries will be only relevant where complex MDAs would exist, wherein there exists uterus with rudimentary horn and/or very severe uterine anomalies that are associated with high degree of pelvic adhesions In the laparoscopic surgery a small incision is made in the abdomen and the laparoscopes or the thin lighted tube with a camera are inserted along with special surgical instruments to access and fix the anomalies. As for laparoscopic surgical procedures for MDAs, they may include the resection of a rudimentary uterine horn, surgery to excise the adhesions from a pelvic cavity, or reconstruction of the uterine axis.
  • Laparotomy: In cases where simple or complex diagnosis cannot be adequately treated with minimally invasive medical approaches, laparotomy (open abdominal surgery) is done. Although the laparotomy method has more benefits for patients with extensive surgical treatments, such as correction of uterine defects or removal of congenital uterine abnormalities, it is also advantageous in approach and the visualization of the pelvic organs.
  • Fertility-enhancing Procedures: Women with MDAs who develop infertility may undergo interventions such as tubal cannulation or reconstruction of the tubes as these are procedures that are estimated to increase tubal patency thus, infertility would be reduced. These procedures are used to resolve the cases of interstitial tubal obstructions or dysfunctions that are thought to play a role in infertility in women with MDAs.
  • Uterine Transplantation: It is one of the recent and advanced, highly-specialized procedures which can be considered in women with absolute uterine factor infertility, including those who have MDAs which resulted in uterine absence or dysfunction.

use-of-phases-in-managing-mullerian-duct-anomalies

The Mullerian duct anomalies (MDAs) are a particular type of uterine anomaly that can cause the woman to have pelvic pain, abnormalities in the menstruation bleeding or inability to get pregnant. Besides the initial phase which consists of a complete diagnostic and assessment process, including a medical history, physical examination, imaging studies, and genetic testing, there is the stage of creating and developing a treatment plan. Symptom Management is a practice of controlling symptoms including pelvic pain, abnormal uterine bleeding, and infertility using the contemporary medicine and the changes in daily routine. Fertility indicators, including ovarian reserve and the other factors influencing fertility, need to be evaluated and counseling provided to the women who are going to be mothers, to prevent difficult situations and the lack of opportunities.

Based on the assessment results and patient representatives, the treatment plan is established through which a patient could undergo either conservative management, pharmacological therapy, interventional procedures, or surgical corrections.

Among intervention procedures as hysteroscopic metroplasty, laparoscopic surgery, or uterine transplantation, may be chosen when other options including behavioral and pharmacological therapies prove inadequate. It is the routine follow-up and observational analysis that is the need of the hour to assess treatments, check for recurrence of symptoms or complications and answer any queries.

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Mullerian Duct Anomalies

Updated : May 17, 2024

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MĂĽllerian duct abnormalities (MDA) are the birth defects of female reproductive system that had fallen during germs formation. Besides abnormalities of the female reproductive system, like the fallopian tubes, the cervix, and the vagina, this issue can also be manifest in various ways. In about 2 to 4% cases in the public in the general population, they are known to be very pervasive.

They are classified in a number of ways, but the American Society for Reproductive Medicine (ASRM) classification scheme is one of the most used and divides MDAs into seven primary subtypes:

Uterine agenesis/hypoplasia: This occurs when the uterus is small or missing.

Unicornuate uterus: The uterus, that is the only one on the whole length of her early embryo form, is being there.

Uterine didelphys: I delivered my own baby needing only an episiotomy and the only part of the reproductive tract that was duplicated was my cervix.

Uterus that is bicornuate: It features a fundus depression that gives the organ its typical heart-shaped appearance, resulting in a deeply concave appearance from the top.

Septate uterus: That septum that either totally or partly divides the uterus is found to exist.

The degree of true incidence and prevalence of MĂĽllerian duct anomalies (MDA) in general population is not very well known because a huge selection bias and under reporting exists. The rate of incidence widely affects studies’ results that are usually between 0.1% and 3.5%. On the other hand, the risk is slightly higher in women with IVF treatment issues such as repeated pregnancy losses and infertility problems, ranging from 3% to 10%. This also varies considerably as reports cite values between 0.16% and 10%.

MĂĽllerian duct abnormalties (MDAs) is a whole spectrum of structural anomalies in the second type of female genital tract that arise because of disruptions in the embryonic development. In the refining years of fetal development, the MĂĽllerian ducts go through a range of fusion, resorption, and differentiation processes, in the end forming the uterus, fallopian tubes, cervix and upper vagina. Even a single unplanned incident or aberration in running the process could result in a spectrum of defects. As such, a septate uterus is developed due to inadequate re- adsorption of the uterovaginal septum which makes uterine cavity be into two divisions. Another congenital defect that results in hemispherical uterus is bicornuate uterus, which results from an incomplete fusion of the Mullerian ducts at the fundus, giving the uterine appearance of a heart-shaped figure.

This disease that involves the structure of the MĂĽllerian ducts is multifactorial. The genetic background, the environment and embryo development can cause anomalies. Accordingly, the exact cause is not clear, but it is considered that genetic susceptibility and environmental factors affect the development of MĂĽllerian ducts in the wrong way. It has been found that the development of the MDAs was affected by the use of some substances such as DES by pregnant women at the embryonic stage.

The prognosis of MDA can be different for each kind and severity of the congenital anomaly. In this regard some MDAs women may be asymptomatic and do not need treatment while others may go through obstruction and require surgery that is destructive of their fertility potential.

Age group: MDAs can be present at different ages and it is determined by the type and severity of the deformity. For instance, pains that experience during puberty or young adulthood and even cause severe pain and affect menstrual cycle may be the side effects of certain drugs. The fact that you are not as fertile as you were before is clearly seen when a woman has problems conceiving or carrying the pregnancy.

External Genitalia: Palpation of the external genitalia can disclose various anomalies such as abnormalities in external vaginal or cervix and also can give the clue of presence of MĂĽllerian duct anomalies.

Pelvic Organs: Manual palpation of the pelvic organs like uterus and ovaries can help in detection of any structural abnormalities or asymmetries that could be of MĂĽllerian ducts development related.

Associated Structures: Clinical assessment of the kidney and the urinary tract will be done because; renal abnormalities are rarely found with the Mullerian Duct Anomalies.

The following MDAs may go along with several other regenerative conditions such as urinary tract anomalies, renal anomalies, and abdominal or pelvic pain. These comorbidities cannot be ruled out to affect the clinical manifestation of MDAs.

The intensity of presentation of symptomatic MDA can fluctuate between asymptomatic and severe. Such as MDAs presenting with abdominal pain and obstruction may warrant surgical therapy while some asymptomatic MDAs don’t require medication.

  • Endometriosis
  • Uterine fibroids
  • Adenomyosis
  • Ovarian cysts

Observation: A gynecologist may say that a gynaecological exam would be done and imaging studies would be ordered if the symptoms or any complications change.

Medical Management: Enhancing the symptomatic care or the treatment of other pertinent conditions like pain management, hormone therapy or medication.

Surgical Management: Many different chronic diseases caused by these structural problems require surgical interventions that are designed to restore normal uterine anatomy, improve reproductive results, eliminate symptoms, and avoid further complications.

Assisted Reproductive Technologies (ART): IVF can be broadly employed to avoid anatomical obstacles as well as make up for fertility deficits. Severe uterine anomalies may require the adoption of IVF fertilization with blastocyst culture and embryo placement into the womb of surrogate mother.

Observation can be an option in the case of an asymptomatic individual or one having a small number of symptoms. These situations can call for a monitoring plan through regular checkups with a healthcare provider who can develop plans to control the condition effectively.

Lifestyle changes may also include physical therapy on the pelvic floor that can ease the discomfort of dysmenorrhea and the pelvic pain typically associated with MDAs. Patients may also benefit from counseling and education on their condition so that they are able to cope with any psychological or emotional effects.

Surgery may be recommended to patients suffering from these conditions or who have had a previous miscarriage or infertility problems. The available surgical options may include hysteroscopic metroplasty, laparoscopic metroplasty or open metroplasty, differentiating depending on the type of MDA and the patients\’ individual needs. To cure these structural abnormalities of the MĂĽllerian ducts these procedures aim to increase the possibilities related to the fertility outcomes.

Hormonal therapy is one of the treatment modalities that have been established as the mainstay for managing menstrual abnormalities. While it may not restore the usual functioning of an organ or system, it can effectively correct the abnormality and help those suffering with this condition to achieve a better quality of life. Hormonal agents including COCs containing estrogen and progesterone would be one of the many useful ways to treat some dysfunctions of the menstrual cycle, specifically abnormal bleeding disorders. Dysmenorrhea which is usually seen is one of the symptoms that could be fought by decreasing the intensity and duration to a minimum at the peak of menstrual cramping. Addition of a progestogen often occurs, to prevent any endometrial injury due to estrogen alone, which can be in form of endometrial hyperplasia or cancer.

  • Hysteroscopic Metroplasty: Hysteroscopic metroplasty is one of the minimally invasive operations used in correction of septate uteri or bicornuate uteri, both of which are the types of MDAs that are subdivided by a septum the uterus. It is performed by inserting a hystescope via the cervix into the uterine cavity. Precisionist surgical instruments are employed cutting or tunnelling through the septate endometrium to create a single normal uterus. It is recognized for its superior results, including the minimal complication risk and helping individual to receive care as an outpatient.
  • Laparoscopic Surgery: Laparoscopic surgeries will be only relevant where complex MDAs would exist, wherein there exists uterus with rudimentary horn and/or very severe uterine anomalies that are associated with high degree of pelvic adhesions In the laparoscopic surgery a small incision is made in the abdomen and the laparoscopes or the thin lighted tube with a camera are inserted along with special surgical instruments to access and fix the anomalies. As for laparoscopic surgical procedures for MDAs, they may include the resection of a rudimentary uterine horn, surgery to excise the adhesions from a pelvic cavity, or reconstruction of the uterine axis.
  • Laparotomy: In cases where simple or complex diagnosis cannot be adequately treated with minimally invasive medical approaches, laparotomy (open abdominal surgery) is done. Although the laparotomy method has more benefits for patients with extensive surgical treatments, such as correction of uterine defects or removal of congenital uterine abnormalities, it is also advantageous in approach and the visualization of the pelvic organs.
  • Fertility-enhancing Procedures: Women with MDAs who develop infertility may undergo interventions such as tubal cannulation or reconstruction of the tubes as these are procedures that are estimated to increase tubal patency thus, infertility would be reduced. These procedures are used to resolve the cases of interstitial tubal obstructions or dysfunctions that are thought to play a role in infertility in women with MDAs.
  • Uterine Transplantation: It is one of the recent and advanced, highly-specialized procedures which can be considered in women with absolute uterine factor infertility, including those who have MDAs which resulted in uterine absence or dysfunction.

The Mullerian duct anomalies (MDAs) are a particular type of uterine anomaly that can cause the woman to have pelvic pain, abnormalities in the menstruation bleeding or inability to get pregnant. Besides the initial phase which consists of a complete diagnostic and assessment process, including a medical history, physical examination, imaging studies, and genetic testing, there is the stage of creating and developing a treatment plan. Symptom Management is a practice of controlling symptoms including pelvic pain, abnormal uterine bleeding, and infertility using the contemporary medicine and the changes in daily routine. Fertility indicators, including ovarian reserve and the other factors influencing fertility, need to be evaluated and counseling provided to the women who are going to be mothers, to prevent difficult situations and the lack of opportunities.

Based on the assessment results and patient representatives, the treatment plan is established through which a patient could undergo either conservative management, pharmacological therapy, interventional procedures, or surgical corrections.

Among intervention procedures as hysteroscopic metroplasty, laparoscopic surgery, or uterine transplantation, may be chosen when other options including behavioral and pharmacological therapies prove inadequate. It is the routine follow-up and observational analysis that is the need of the hour to assess treatments, check for recurrence of symptoms or complications and answer any queries.

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