Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder that affects women. It is characterized by the failure of the uterus and the vagina to develop properly in women who have normal ovarian function and normal external genitalia. Women with this disorder develop normal secondary sexual characteristics during puberty (e.g., breast development and pubic hair), but do not have a menstrual cycle (primary amenorrhea). Often, the failure to begin the menstrual cycle is the initial clinical sign of MRKH syndrome. The range and severity of MRKH syndrome can vary greatly and the disorder is generally broken down into type I, which occurs as an isolated finding, and type II, which occurs with abnormalities of additional organ systems including mainly the kidneys and the skeleton. Because of the nature of the disorder, MRKH syndrome can cause significant psychological challenges and counseling is recommended. The exact cause of MRKH syndrome remains largely unknown, but there is now no doubt of a genetic origin. In this respect, an update on the most recent research publications shows the involvement of several chromosomal segments, some of them including genes likely to account for the disorder.

Irregular formation of female reproductive parts affecting the system is known as Mullerian Duct-Renal-Cervicothoracic-up…

Causes:
There is not just one cause of MURCS anomalies available. It could occur due to any of the below reasons:

1. Developmental defect: The defect sometimes is mild with the detection of only a few problems or no problems. It happens when the issues provoke back problems, spinal issues, and menstrual issues in later stages of life.

2. Hereditary Defects: These are detected at the time of birth or right before the birth of a baby. Details of underdeveloped ducts and uterus are found in sonography of developmental checks.

3. Gene Mutation: Changes in gene formation or incomplete count of chromosomes give rise to these congenital defects. They occur due to mutations in the parent’s genes and create defects in a child.

Inheritance:
1. Disorders that are so rare are diagnosed and given treatment. The reason why MURCS doesn’t have any specific drug invented is the rarity of this disease and undetectable risks.

2. It occurs to one in 5000 females as the defect is related to female reproductive parts and later problems due to the same.

3. There are two types of Mullerian duct syndromes: Type 1 with vaginal failed development and Type 2 with extragenital manifestations.

Symptoms
Abnormalities in female reproductive organs, neck, mouth, skeletal limbs, and nervous systems
Conditions
Underdeveloped genetic organs,Defects in uterus, fallopian tubes, cervix,Issues in growth of height, spine, kidneys, and face
Drugs
NA

Mullerian Duct-Renal-Cervicothoracic-up… is usually recognized at the beginning of puberty ages when a girl crosses or is in her teens. It is the time when a young female either gets difficulty in their menstrual cycle or fails to get one.

Diagnosis:
Following medical procedures can help in detecting the defect before it turns into cancer:

1. Ultrasound: It confirms abnormality of a genital tract and should perform at the time of the menstrual cycle.
2. MRI: It evaluates female pelvic anatomy.


Treatments:
1. When the condition is mild and does not affect the reproduction process, there is no need for treatment required to cure the defect.

2. The crucial problems due to this defect occur mainly in menstruation areas with unbearable pains and blood loss. This issue can only be resolved by Surgical Intervention.

3. Surgeries depend on the severity of symptoms in an individual’s body and the body’s reaction accordingly.

4. Further, the treatment can extend or change according to damages to the uterine walls and duct issues:

- Vaginal Agenesis management: This process corrects vaginal defects and development failures. It also includes counseling with patients for a better understanding of a patient.

- Infertility and Uterus Transplantation: Major surgeries include transplanting the uterus and improving infertility problems. Uterus transplant has emerged as the first infertility treatment for women with MRKH syndrome.

Symptoms
Abnormalities in female reproductive organs, neck, mouth, skeletal limbs, and nervous systems
Conditions
Underdeveloped genetic organs,Defects in uterus, fallopian tubes, cervix,Issues in growth of height, spine, kidneys, and face
Drugs
NA

Mullerian Duct-Renal-Cervicothoracic-up… can arise when a woman finds trouble conceiving or sustaining a pregnancy. Many problems in pelvic muscles or abdominal region during sex or menstruation are indicating MURCS syndrome.

The defects generally caused by a birth defect or issues that occur in the development of female reproductive organs, are all Mullerian Duct-Renal-Cervicothoracic-Up.

Types and risk factors of MURCS:
There are different stages of developmental issues in ducts connected to fallopian tubes and other abdominal organs:

1. Unicornuate Uterus (UU): Underdeveloped Mullerian duct causes a banana-shaped uterus and incomplete kidney formation. This results in two subtypes of this kind:
2. Uterus Didelphys: It is like complete duplication of the uterus with a “double uterus” with the vagina, cervix, and uterus divided into two halves.
3. Bicornuate Uterus: Most common of both, the form is described with two horns. In this case, the womb is heart-shaped instead of pear-shaped.
4. Septate Uterus: It extends to only one part of the uterus and may reach as far as the cervix.
5. Hypoplasia: All parts of the duct in this type fail to form and are underdeveloped.
6. Arcuate Uterus: Uterus shape defect occurs in this type with fundus intended slightly inside and outside. But it is so slight that it is considered normal.
7. DES-related Uterus: There T-shaped uterine cavity, dilated horns, and a malformed cervix and upper vagina may sort this anomaly.


Symptoms
Abnormalities in female reproductive organs, neck, mouth, skeletal limbs, and nervous systems
Conditions
Underdeveloped genetic organs,Defects in uterus, fallopian tubes, cervix,Issues in growth of height, spine, kidneys, and face
Drugs
NA

Mullerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. Most Mullerian duct-renal-cervicothoracic-upp... patients are diagnosed with primary amenorrhea from late adolescence. However, a few cases with MURCS association are not well diagnosed during childhood, and long-term outcomes are not well reported.

Management of patients with mullerian agenesis includes psychosocial counseling as well as treatment of the anatomic anomalies. Options include vaginal elongation and the surgical creation of a neovagina.

1. Vaginal Elongation: Primary vaginal elongation by dilation is the appropriate first-line approach in most patients because it is safer, patient-controlled, and more cost-effective than surgery. When well-counseled and emotionally prepared, almost all patients (90-96%) will be able to achieve anatomic and functional success by primary vaginal dilation

2. Surgical Creation of a Neovagina: The surgical creation of a vagina requires ongoing postoperative dilation or vaginal intercourse to maintain adequate vaginal length and diameter; therefore, it is not a method to avoid vaginal dilator therapy. Because primary vaginal dilation is successful for more than 90-96% of patients, surgery should be reserved for the rare patient who is unsuccessful with a primary dilator

3. General Gynecologic Care: Health care providers should be aware that some routine gynecology questions, including the date of last menstrual period, are unnecessary and may make patients have less confidence in the health care team. The patient should be asked about any vaginal discharge, bleeding, pelvic pain, or dyspareunia. Pelvic examinations should be performed if there are concerns about complications, vaginal stricture, or stenosis.

Symptoms
Abnormalities in female reproductive organs, neck, mouth, skeletal limbs, and nervous systems
Conditions
Underdeveloped genetic organs,Defects in uterus, fallopian tubes, cervix,Issues in growth of height, spine, kidneys, and face
Drugs
NA