Congenital Mullerian Anomalies with two Cavitated, Non-Communicating Rudimentary Horns


Case Presentation:

We present an instance of Congenital Müllerian anomalies with two cavitated, non-communicating rudimentary horns diagnosed at World Laparoscopy Hospital by Prof. R.K. Mishra. To our knowledge, this represents the very first reported video on web with Congenital Müllerian anomalies. Not all the types of uterine abnormalities can affect fertility & also not all the abnormalities correctable surgically. Müllerian duct anomalies are an uncommon but often non treatable cause of infertility. Patients with Müllerian duct anomalies are known to have a higher incidence of infertility compare to other anatomical defect, repeated first trimester spontaneous abortions, fetal intrauterine growth retardation, fetal malposition, preterm labor, and retained placenta. The role of imaging like MRI, Ultrasound is to help detect, diagnose, and distinguish surgically correctable forms of Müllerian duct anomalies from inoperable forms. In some correctable lesions, the laparoscopic diagnosis is very helpful and minimal access surgical approach is altered based on imaging findings

The uterus, fallopian tubes and upper vagina are made up of two partially fused tubes, which, within the embryo, are classified as müllerian ducts, named for physiologist Johannes Peter Müller, who first described them in 1830. They are also referred to as paramesonephric ducts, and therefore are in the beginning present in embryos of both sexes.

Normally, these ducts run down vertically from flank to pelvic floor in the young embryo and finally fuse right into a double-barreled tube with two loose ends, known as the uterovaginal primordium, or UVP. The double UVP will ultimately merge into a single-barreled uterus, cervix and upper vagina, while the loose ends develop into the fallopian tubes. In adulthood, these organs are known as the müllerian tract and congenital malformations of the tract are known as müllerian anomalies.

Müllerian duct anomalies are estimated to occur in 0.1-0.5% of ladies. The true prevalence is unknown since the anomalies usually are discovered in patients presenting with infertility. The existence of a müllerian duct anomaly is not associated with significantly increased mortality when compared to general population. Certain kinds of the anomaly can increase morbidity, for example in patients with obstructed or partially obstructed müllerian systems who present with hematosalpinx, hematocolpos, retrograde menses, and endometriosis.

Mullerian Anomalies may be diagnosed in infancy, adolescence, or young adulthood. Female patients may present having a mass resulting from an obstructed müllerian system as infants (mucocolpos); with primary amenorrhea, mass (hematocolpos), or delayed onset of menarche as adolescents; or with problems of fertility and/or carrying pregnancy to term as adults.

Ovaries and the lower vagina are not derived from the müllerian system. The ovaries are based on germ cells that migrate in the primitive yolk sac into the mesenchyme from the peritoneal cavity and subsequently develop into ova and supporting cells. The lower vagina arises from the sinovaginal bulb, which fuses with the müllerian-derived upper sixty-six per cent to form the complete vagina.

Types of Mullerian abnormalities:

  1. Agenesis & hypoplasia: Mayer-Rokitansky-Kuster-Hauser syndrome is most typical. Any part of the müllerian tract fails to form, or is extremely underdeveloped. For example, a cervix can be a tissue-thin membrane rather than tough, fibrous "donut" several centimeters thick. Most women struggling with agenesis or extreme hypoplasia have severe fertility problems, simply by lacking sufficient tissue to aid a growing pregnancy. A common diagnosis was previously "infantile uterus," but it simply means a smaller-than-average uterus and does not make reference to the MA described above.
  2. Unicornuate uterus: When one müllerian duct is underdeveloped or does not develop, a banana-shaped half-uterus is created. It might or may not be accompanied by a rudimentary horn, and that other horn may or may not come with an endometrial cavity or contact 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 can be found in an odd place, further up through the ribs.
  3. Uterus didelphys: The müllerian tract does not fuse along all or the majority of its length. There may be complete duplication of the vagina, cervix and uterus, and also the two halves may be divided by a ligament of ligament.
  4. Bicornuate uterus: The uterine fundus fails to fuse and a myometrial division extends right down to the cervix inside a complete bicornuate uterus, or part method to the cervix in a partial bicornuate uterus. The division is visible on the outside of the uterus, evidenced with a groove or cleft in the uterine dome exceeding 1.5 centimeters. Cervix and vagina are often single but might be septate or duplicate.
  5. Septate uterus: The müllerian tract has fused properly and the uterus looks single from the outside, however the inner duct wall has failed to dissolve around 20 weeks of gestation, and also the uterus retains a double cavity. There might or may not be a shallow groove of just one.5 centimeters or less about the outer uterine dome, and sometimes even a whitish triangle of tissue, the septum itself, is seen.

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