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Müllerian agenesis, also known as Müllerian aplasia, vaginal agenesis, or Mayer–Rokitansky–Küster–Hauser syndrome (MRKH syndrome), is a congenital malformation characterized by a failure of the Müllerian ducts to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion.
The most prevalent form of vaginal agenesis is Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome and results in congenital aplasia or hypoplasia of Müllerian derived structures. [9] MRKH syndrome account for 5% to 10% of all Müllerian anomalies. While septate uterus or class II uterine anomalies account for 3% to 7% of all Müllerian ...
MURCS association (a variant of Mayer-Rokitansky-Küster-Hauser syndrome) is a very rare developmental disorder [2] that primarily affects the reproductive and urinary systems involving MUllerian agenesis, Renal agenesis, Cervicothoracic Somite abnormalities. [3] It affects only females.
The distinction between an arcuate uterus and a septate uterus is not standardized. A rudimentary uterus is a uterine remnant not connected to cervix and vagina and may be found on the other side of a unicornuate uterus. Patients with uterine abnormalities may have associated renal abnormalities including unilateral renal agenesis. [1]
Müllerian agenesis (absent uterus). Uterus is not present, vagina only rudimentary or absent. [12] [13] Uterus didelphys, also uterus didelphis (double uterus). transverse vaginal septum; Septated uterus (uterine septum or partition). With a complete vaginal septum. Rudimentary uterus is a uterine remnant not connected to cervix and vagina.
Uterine agenesis is the second most common cause (15% of primary amenorrhea patients, 1 in 4500 births). It is a congenital absence of the uterus (Rokitansky-Kuster-Hauser syndrome), but the female still has ovaries, a cyclic ovulation, pubic/axillary hair and a normal range of hormones.
The paramesonephric ducts play a critical role in the female reproductive tract and differentiate to form the uterine tubes, uterus, superior vagina as well as the uterine cervix. Many types of disorders can occur when this system is disrupted ranging from uterine and vaginal agenesis to the duplication of unwanted cells of the uterus and vagina.
Uterus didelphys (from Ancient Greek di- 'two' and delphus 'womb'; sometimes also uterus didelphis) represents a uterine malformation where the uterus is present as a paired organ when the embryogenetic fusion of the Müllerian ducts fails to occur. As a result, there is a double uterus with two separate cervices, and possibly a double vagina ...