Materiały źródłowe

• #1 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  Mllerian agenesis, also referred to as mllerian aplasia, Mayer-Rokitansky-Kster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,5005,000 females. Mllerian agenesis is caused by embryologic underdevelopment of the mllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with mllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. […] The most important steps in the effective management of mllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. […] 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.

• #2 Müllerian agenesis – Wikipedia

  https://en.wikipedia.org/wiki/M%C3%BCllerian_agenesis

  Mllerian agenesis, also known as vaginal agenesis, is a congenital malformation characterized by a failure of the Mllerian ducts to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Mllerian agenesis (including absence of the uterus, cervix and/or vagina) is the cause in 15% of cases of primary amenorrhoea. […] A female with this condition is hormonally normal; that is, the woman will enter puberty with development of secondary sexual characteristics including thelarche (breast development) and pubarche (pubic hair). The woman’s karyotype will be 46,XX. At least one ovary is intact, if not both, and ovulation usually occurs. Typically, the vagina is shortened and intercourse may, in some cases, be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina. […] A woman with Mllerian agenesis typically discovers the condition when, during puberty years, the menstrual cycle does not start (primary amenorrhoea). Some find out earlier through surgeries for other conditions, such as a hernia.

• #3 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #4 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #5 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #6 Vaginal agenesis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/diagnosis-treatment/drc-20355741

  Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Some patients report problems with urinating and with vaginal bleeding and pain, especially in the beginning. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #7 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  During pregnancy, a baby’s reproductive system may not finish developing in the mother’s uterus. She may be born without a vagina and have other absent reproductive organs. This condition is called vaginal agenesis. […] Sometimes vaginal agenesis is recognized at birth. Most times, the condition isn’t diagnosed until puberty, when the teen notices she hasn’t started her period and seeks medical advice. […] Young women with vaginal agenesis have normal ovaries and normal external genitalia and thus go through puberty and develop breasts, under arm and pubic hair, except they will not have a periods. […] The symptoms of vaginal agenesis include: Small pouch or dimple where vaginal opening should be, Lack of menstrual cycle (amenorrhea), Lower abdominal pain if a uterus is present without a connection to a vaginal canal.

• #8 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #9 Mayer-Rokitansky-Küster-Hauser syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/mayer-rokitansky-kuster-hauser-syndrome/

  Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder that causes the vagina and uterus to be underdeveloped or absent. Without a uterus, affected women do not have menstrual periods. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 15 (primary amenorrhea). […] Girls and women with MRKH syndrome have a 46,XX chromosome pattern. They have normal external genitalia and functioning ovaries. They also have normal breast and pubic hair development. Although women with this condition are unable to carry a pregnancy, they may be able to have children with the help of assisted reproductive technologies or a uterus transplant. […] MRKH syndrome is often divided into two types. MRKH syndrome type 1 affects only the reproductive organs. MRKH syndrome type 2 also affects other parts of the body. In this form of the condition, the kidneys may be abnormally formed or positioned, or one kidney may fail to develop (unilateral renal agenesis). Additionally, hearing loss, heart defects, or skeletal abnormalities, particularly of the spinal bones (vertebrae), can occur in those with MRKH syndrome type 2.

• #10 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #11 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #12 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  During pregnancy, a baby’s reproductive system may not finish developing in the mother’s uterus. She may be born without a vagina and have other absent reproductive organs. This condition is called vaginal agenesis. […] Sometimes vaginal agenesis is recognized at birth. Most times, the condition isn’t diagnosed until puberty, when the teen notices she hasn’t started her period and seeks medical advice. […] Young women with vaginal agenesis have normal ovaries and normal external genitalia and thus go through puberty and develop breasts, under arm and pubic hair, except they will not have a periods. […] The symptoms of vaginal agenesis include: Small pouch or dimple where vaginal opening should be, Lack of menstrual cycle (amenorrhea), Lower abdominal pain if a uterus is present without a connection to a vaginal canal.

• #13 Vaginal and Mullerian Agenesis | Norton Children’s

  https://nortonchildrens.com/services/gynecology/conditions/vaginal-mullerian-agenesis/

  Vaginal agenesis is a rare disorder that occurs when the vagina doesn’t develop and the uterus may only develop partially or not at all. The condition is present before birth. […] Girls with vaginal agenesis typically have normal external genitalia and ovaries. As a result, they go through puberty and develop breasts and pubic hair, but they will not have periods. There may be a small pouch or dimple where a vaginal opening should be. […] Sometimes vaginal agenesis is recognized at birth. Most times it doesn’t present itself until puberty, when a teen notices she hasn’t started her period. […] If the child was born with an incomplete vagina but has a normal size uterus, she likely will be able to become pregnant. […] A normal sex life is possible for women born with vaginal agenesis because typically it does not affect external genitalia.

• #14 Vaginal Atresia in Children: Signs, Diagnosis, Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/vaginal-atresia

  Symptoms May Include: […] A small pouch or dimple where the vaginal opening should be […] Failure to start having menstrual periods […] Cyclical abdominal pain […] Pelvic mass, if the upper vagina fills with menstrual blood.

• #15 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #16 Vaginal agenesis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/vaginal-agenesis

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #17 Vaginal Agenesis | Texas Children’s

  https://www.texaschildrens.org/content/conditions/vaginal-agenesis

  Vaginal agenesis occurs when a girl is born without a vagina. […] Symptoms may include: […] A small pouch or dimple where the vaginal opening should be […] Failure to start having periods at puberty (primary amenorrhea) […] Because the outward genitalia appear normal, the condition is often not diagnosed until puberty, when a girl fails to begin having periods and visits her doctor. […] Treatment depends on the individual patient and what symptoms are present.

• #18 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #19 Müllerian agenesis – Wikipedia

  https://en.wikipedia.org/wiki/M%C3%BCllerian_agenesis

  Mllerian agenesis, also known as vaginal agenesis, is a congenital malformation characterized by a failure of the Mllerian ducts to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Mllerian agenesis (including absence of the uterus, cervix and/or vagina) is the cause in 15% of cases of primary amenorrhoea. […] A female with this condition is hormonally normal; that is, the woman will enter puberty with development of secondary sexual characteristics including thelarche (breast development) and pubarche (pubic hair). The woman’s karyotype will be 46,XX. At least one ovary is intact, if not both, and ovulation usually occurs. Typically, the vagina is shortened and intercourse may, in some cases, be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina. […] A woman with Mllerian agenesis typically discovers the condition when, during puberty years, the menstrual cycle does not start (primary amenorrhoea). Some find out earlier through surgeries for other conditions, such as a hernia.

• #20 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #21 Vaginal Agenesis: Causes, Symptoms, Diagnosis, Treatment and Complications

  https://www.prepladder.com/neet-pg-study-material/obstetrics-and-gynaecology/vaginal-agenesis-causes-symptoms-diagnosis-treatment-and-complications

  Vaginal agenesis is an uncommon disorder in which the vagina does not develop, but the uterus (womb) may develop partially or not at all. […] A female is often diagnosed with vaginal agenesis when she does not begin menstruation at puberty. […] Many times, a lady with vaginal agenesis who does not menstruate (amenorrhoea) is not diagnosed until she is in her teens. Other signs of puberty often follow typical female growth. […] Vaginal agenesis symptoms may include: The vagina may be nonexistent or have a tiny indentation where a vaginal entrance should be, or it may be shorter and not have a cervix at the end. […] The uterus may be partially formed or absent. Monthly cramps or chronic abdominal pain may be caused by the endometrium, the tissue lining the uterus. […] Vaginal agenesis may have an impact on your sexual interactions, but with treatment, your vagina will typically function normally for sexual activity.

• #22 Vaginal Agenesis | Intimate Wellness Institute

  https://iwiva.com/home-page/womens-speciality-care/advanced-gynecology/congenital-abnormalities/vaginal-agenesis/

  Vaginal agenesis is a congenital anomaly (birth defect). It happens when the vagina of a fetus doesnt develop properly in your uterus. They may be missing all or part of their vagina. Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short.

• #23 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #24 Vaginal Agenesis – Clinical Tree

  https://clinicalpub.com/vaginal-agenesis/

  Vaginal agenesis is the congenital absence of the vagina, most often associated with an absence of the uterus (MayerRokitanskyKsterHauser [MRKH] syndrome). […] Vaginal obstruction (absence) […] Primary amenorrhea […] Cyclic abdominal pain […] Hematometra (if a uterus or uterine remnant is present).

• #25 Vaginal Agenesis Treatment in Turkey – Vaginoplasty Clinic

  https://www.vaginoplastyturkey.com/en/vaginal-agenesis-treatment-in-turkey

  Patients with vaginal agenesis have normal growth and pubertal development such as pubic and axillary hair and breast maturation. However they cannot have menstruation which is called primary amenorrhea. If they have a uterus but no vagina, menstrual bleeding will accumulate in the uterus (hematometra) since it cannot flow out, this causes sharp lower abdominal pain monthly. […] Typically women with MRKH syndrome have ovaries and eggs so that they ovulate. They may have symptoms related to ovulation such as mid-cycle pain and premenstrual syndrome but no menstrual bleeding. […] Since girls with MRKH syndrome have ovaries, they do ovulate. […] Women with MRKH have ovaries and the eggs will be expelled into the abdominal cavity during ovulation, the same way as in other patients who have a uterus. The difference is that the eggs will not be able form an embryo in the fallopian tube, and there is no uterus for an embryo to be implanted. […] Orgasm in MRKH syndrome patients can be achieved by stimulation of the clitoris. Women do not necessarily need to have a vagina to achieve orgasm. After vaginal agnesis treatment, vaginal intercourse will also make it possible to have an orgasm through penetration.

• #26 Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay | Journal of the Belgian Society of Radiology

  https://jbsr.be/articles/10.5334/jbsr.3660

  Hematocolpos is a medical condition in which menstrual blood or secretory fluid accumulates in the vagina due to vaginal obstruction. […] The main symptoms are periodic abdominal pain and primary amenorrhea. […] Late complications include tubal infection, adhesion, pelvic endometriosis, infertility, and renal failure due to hydronephrosis. […] Distal vaginal atresia and transverse vaginal septum involve segmental vaginal obstruction, with controversial terminology and embryology. […] Both typically present similarly and are not associated with other urogenital anomalies. […] Hematocolpos results from menstrual blood accumulation due to vaginal obstruction, mainly from congenital causes. Accurate differential diagnosis requires clinical examination, ultrasonography, and MRI. Early diagnosis and surgical treatment are crucial to prevent complications and preserve fertility.

• #27 Vaginal atresia: Causes, symptoms, treatments, and more

  https://www.medicalnewstoday.com/articles/vaginal-atresia

  A person may not receive a diagnosis of vaginal atresia until they reach puberty. […] A female entering puberty may experience: a small dimple or pouch where the vaginal opening would be, a lack of periods, abdominal pain that appears in cycles. […] Symptoms include a lack of menstruation, abdominal pain, and a dimple or pouch where the vagina should be. A person may also experience a pelvic mass filled with menstruation blood.

• #28 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #29 Vaginal Agenesis | Intimate Wellness Institute

  https://iwiva.com/home-page/womens-speciality-care/advanced-gynecology/congenital-abnormalities/vaginal-agenesis/

  Vaginal agenesis is a congenital anomaly (birth defect). It happens when the vagina of a fetus doesnt develop properly in your uterus. They may be missing all or part of their vagina. Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short.

• #30 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Vaginal agenesis, also known as Mullerian agenesis, Mullerian aplasia, Bardet-Biedl syndrome, Fraser syndrome, or Mayer-Rokitansky-Kuster-Hauser syndrome, is a congenital birth defect in which the vagina is either short, doesnt develop fully, or be absent altogether. […] The characteristic symptom of Vaginal agenesis is often related to a condition called amenorrhea which is the lack of menstrual cycle during puberty. […] Although girls with vaginal agenesis grow like normal individuals, they usually experience the following signs and symptoms, which includes: Vagina may be short without a cervix at the end, or absent and marked only by a slight indentation where typically a vaginal opening should be located. […] Painful amenorrhea (seen in girls with a uterus). […] Painless amenorrhea (seen in girls without a uterus).

• #31 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #32 Lower urinary tract symptoms in women with vaginal agenesis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/22797462/

  Urinary symptoms were present in 53% of women prior to treatment. […] Women with vaginal agenesis had significant levels of urinary symptoms and to our knowledge this has not been reported before. The majority of symptoms were not altered by treatment.

• #33

  https://link.springer.com/article/10.1007/s00192-012-1870-4

  Urinary symptoms were present in 53% of women prior to treatment. […] Women with vaginal agenesis had significant levels of urinary symptoms and to our knowledge this has not been reported before. […] The majority of symptoms were not altered by treatment.

• #34 What Is MRKH Syndrome?

  https://www.verywellhealth.com/what-is-mrkh-syndrome-4170044

  Mayer-Rokitansky-Kster-Hauser Syndrome is also known as vaginal agenesis. […] MRKH syndrome is the most common type of vaginal agenesis. […] MRKH syndrome is a lifelong condition that may be associated with other complications. People with MRKH usually do not menstruate, for example, and may have urinary problems. […] Because the vulva appears normal, a woman may only discover she has MRKH syndrome when they fail to get their menstrual period. Or, they may try and fail to have vaginal intercourse. […] Most people with MRKH can urinate with their existing vagina. However, it’s not uncommon to have urinary problems. One study found that 70% of the 614 people studied (about half of whom had undergone dilation, surgery, or both) had urinary pain, urgency, and other symptoms, including frequent urinary tract infections.

• #35 Vaginal Anomalies (Vaginal Septum, Vaginal Agenesis/Atresia) | Nationwide Children’s

  https://www.nationwidechildrens.org/conditions/vaginal-anomalies

  Vaginal agenesis/atresia is a disorder present before birth in which the vagina stops developing. Some patients may have a shorter vagina, a part of a vagina or no vagina at all. This affects one in 5,000 females. […] With vaginal agenesis/atresia or a transverse vaginal septum, the most common symptom is a lack of a menstrual period. Typically, patients progress through puberty normally but never have a menstrual period. If there is menstrual blood collecting in the upper vagina, the patient may present with abdominal or pelvic pain. If the upper vagina is quite distended with menstrual blood, the patient may also have urinary symptoms such as urinary frequency, urinary urgency, or feelings of incomplete voiding. […] In cases of a longitudinal vaginal septum, patients will menstruate normally, but may have difficulty with tampon insertion or vaginal intercourse. They may also report that they can insert a tampon, continue to notice menstrual blood leaking around the tampon. This happens because the tampon is inserted on one side of the longitudinal vaginal septum and menstrual blood is continuing to flow out on the other side of the septum.

• #36

  https://link.springer.com/article/10.1007/s00192-012-1870-4

  Urinary symptoms were present in 53% of women prior to treatment. […] Women with vaginal agenesis had significant levels of urinary symptoms and to our knowledge this has not been reported before. […] The majority of symptoms were not altered by treatment.

• #37 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #38 Mayer-Rokitansky-Küster-Hauser syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/mayer-rokitansky-kuster-hauser-syndrome/

  Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder that causes the vagina and uterus to be underdeveloped or absent. Without a uterus, affected women do not have menstrual periods. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 15 (primary amenorrhea). […] Girls and women with MRKH syndrome have a 46,XX chromosome pattern. They have normal external genitalia and functioning ovaries. They also have normal breast and pubic hair development. Although women with this condition are unable to carry a pregnancy, they may be able to have children with the help of assisted reproductive technologies or a uterus transplant. […] MRKH syndrome is often divided into two types. MRKH syndrome type 1 affects only the reproductive organs. MRKH syndrome type 2 also affects other parts of the body. In this form of the condition, the kidneys may be abnormally formed or positioned, or one kidney may fail to develop (unilateral renal agenesis). Additionally, hearing loss, heart defects, or skeletal abnormalities, particularly of the spinal bones (vertebrae), can occur in those with MRKH syndrome type 2.

• #39 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  Mllerian agenesis, also termed mllerian aplasia, is characterized by an absence or hypoplasia of the uterus, proximal vagina, and, in some cases, the fallopian tubes. The condition has been also referred to as the Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome, when it occurs in its most extreme form, both uterine and vaginal aplasia. […] Mllerian agenesis can be partial or complete. Partial mllerian agenesis is more rarely encountered and is characterized by a normal uterus and small vaginal pouch distal to the cervix. Complete mllerian agenesis (MRKH syndrome) is the most common variant encountered, and it is characterized by congenital absence of the vagina and the uterus in 90-95% of cases. The fallopian tubes are normal, and the ovaries have normal endocrine and oocyte function. […] The incidence of associated urologic abnormalities ranges between 15% and 40%, and skeletal anomalies, such as congenital fusion or absence of vertebrae, occur in approximately 12-50% of cases.

• #40 Female Genital Birth Defects – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-urinary-tract-and-genitals/female-genital-birth-defects

  Girls who have vaginal agenesis also may have kidney abnormalities. For example, they may be missing a kidney. They may also have problems with bones or abdominal wall abnormalities. […] No immediate treatment is needed unless girls have pain due to blocked flow of menstrual blood. Once girls are ready to begin treatment, they will use a vaginal dilator to stretch their vaginal canal. A vaginal dilator is a smooth plastic rod shaped similar to a tampon. Girls insert a dilator and, over time, it stretches the vagina.

• #41 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  Mllerian agenesis, also termed mllerian aplasia, is characterized by an absence or hypoplasia of the uterus, proximal vagina, and, in some cases, the fallopian tubes. The condition has been also referred to as the Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome, when it occurs in its most extreme form, both uterine and vaginal aplasia. […] Mllerian agenesis can be partial or complete. Partial mllerian agenesis is more rarely encountered and is characterized by a normal uterus and small vaginal pouch distal to the cervix. Complete mllerian agenesis (MRKH syndrome) is the most common variant encountered, and it is characterized by congenital absence of the vagina and the uterus in 90-95% of cases. The fallopian tubes are normal, and the ovaries have normal endocrine and oocyte function. […] The incidence of associated urologic abnormalities ranges between 15% and 40%, and skeletal anomalies, such as congenital fusion or absence of vertebrae, occur in approximately 12-50% of cases.

• #42 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #43 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #44 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #45 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #46 Mayer-Rokitansky-Küster-Hauser syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/mayer-rokitansky-kuster-hauser-syndrome/

  Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder that causes the vagina and uterus to be underdeveloped or absent. Without a uterus, affected women do not have menstrual periods. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 15 (primary amenorrhea). […] Girls and women with MRKH syndrome have a 46,XX chromosome pattern. They have normal external genitalia and functioning ovaries. They also have normal breast and pubic hair development. Although women with this condition are unable to carry a pregnancy, they may be able to have children with the help of assisted reproductive technologies or a uterus transplant. […] MRKH syndrome is often divided into two types. MRKH syndrome type 1 affects only the reproductive organs. MRKH syndrome type 2 also affects other parts of the body. In this form of the condition, the kidneys may be abnormally formed or positioned, or one kidney may fail to develop (unilateral renal agenesis). Additionally, hearing loss, heart defects, or skeletal abnormalities, particularly of the spinal bones (vertebrae), can occur in those with MRKH syndrome type 2.

• #47 Vaginal agenesis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/diagnosis-treatment/drc-20355741

  Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Some patients report problems with urinating and with vaginal bleeding and pain, especially in the beginning. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #48 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  Mllerian agenesis, also termed mllerian aplasia, is characterized by an absence or hypoplasia of the uterus, proximal vagina, and, in some cases, the fallopian tubes. […] Mllerian agenesis can be partial or complete. Partial mllerian agenesis is more rarely encountered and is characterized by a normal uterus and small vaginal pouch distal to the cervix. Complete mllerian agenesis (MRKH syndrome) is the most common variant encountered, and it is characterized by congenital absence of the vagina and the uterus in 90-95% of cases. […] Mllerian agenesis is usually diagnosed at puberty when adolescents present to the gynecologist with primary amenorrhea. It is the second most common cause of primary amenorrhea in adolescents. […] Appearances of both introitus and vagina can vary significantly in young adolescents. The vaginal vault can be either completely absent or a short vaginal pouch can be present.

• #49 Vaginal agenesis – Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.

  https://www.vejthani.com/diseases-conditions/vaginal-agenesis/

  Vaginal agenesis can show a few different signs. One common sign is amenorrhea, which is when someone does not start having a period by age 15. […] Other signs of vaginal agenesis include: The vagina might be shorter than usual, without a cervix at the end, or it may be absent, with only a slight indentation where the vaginal opening would normally be. […] Its crucial to seek medical advice if a girl hasnt begun menstruating by the age of 15. Any abnormalities in the vaginal region warrant prompt medical attention.

• #50 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. This profile helps distinguish the MRKH syndrome from androgen insensitivity syndromes in which postpubertal testosterone is elevated. […] Surgical treatment should be considered only when the patient can participate in the decision-making, wishes to become sexually active, and is highly motivated to use a vaginal prosthesis for several months after surgery. […] The aim of surgical treatment is to create a neovagina. Although several vaginoplasty methods have been developed, refined, and modified, no definitive surgical approach has been established. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty.

• #51 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #52 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #53 MRKH Diagnosis | MRKH Symptoms | MRKH Syndrome

  https://www.miklosandmoore.com/mrkh-atlanta/diagnosis/

  MRKH syndrome is often associated with urinary tract, skeletal and auditory anomalies. […] Diagnosis is usually delayed until adolescence when the young woman does not have her period. The confirmation of the diagnosis is inevitably a psychological shock for the patient and her family. The absence of a vagina has a profound impact on the young womans sense of femininity and her future sexuality, thoughts about her future ability to bear children and have a family and the immediate feelings and shock of being told she does not have a normal vagina or uterus. […] Typically, vaginal agenesis patients do not have a functioning uterus and cannot become pregnant. Most have normal functioning ovaries and can become biologic parents through in-vitro fertilization of their own eggs with the pregnancy carried by a surrogate mother.

• #54 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #55 Study on depressive symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: an analysis of 141 cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01405-9

  Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome is a rare congenital disease characterized by uterovaginal agenesis. […] The diagnosis of MRKH syndrome generally leads to considerable emotional burdens on patients. […] The aim of this study was to investigate the prevalence of depressive symptoms in Chinese patients with MRKH syndrome and to identify the factors associated with depressive symptoms. […] A total of 75.2% of MRKH patients suffered from depressive symptoms, and 34.0% reached a moderate to severe level, while the proportion in the control group was 61.2 and 24.2% respectively. […] Three-quarters of MRKH patients show depressive symptoms, and one-third of these individuals are even at risk for depressive disorders. […] Therefore, depressive symptom screening and proper psychotherapy in MRKH patients are of great importance.

• #56 Study on depressive symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: an analysis of 141 cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01405-9

  Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome is a rare congenital disease characterized by uterovaginal agenesis. […] The diagnosis of MRKH syndrome generally leads to considerable emotional burdens on patients. […] The aim of this study was to investigate the prevalence of depressive symptoms in Chinese patients with MRKH syndrome and to identify the factors associated with depressive symptoms. […] A total of 75.2% of MRKH patients suffered from depressive symptoms, and 34.0% reached a moderate to severe level, while the proportion in the control group was 61.2 and 24.2% respectively. […] Three-quarters of MRKH patients show depressive symptoms, and one-third of these individuals are even at risk for depressive disorders. […] Therefore, depressive symptom screening and proper psychotherapy in MRKH patients are of great importance.

• #57 Study on depressive symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: an analysis of 141 cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01405-9

  Due to emotional burdens caused by the diagnosis, management and treatment of MRKH syndrome, depressive symptoms appear frequently. […] Patients have reported depressive emotions, even suicidal thoughts, in reaction to the diagnosis of this condition. […] The PHQ-9 score was significantly higher in MRKH patient group than the age-matched control group (7.0 (4.511.0) vs. 6.0 (3.09.0)), median and IQRs) (P=0.015). […] According to the last question in the PHQ-9, 11 (7.8%) patients with MRKH syndrome admitted that they had self-injury or suicidal ideas more than half of the time in the past 2 weeks, and 5 (3.5%) patients had these ideas almost every day. […] Negative self-evaluation of femininity (OR 3.96, 95% CI 1.3711.46); neurotic personality traits (OR 1.19 95% CI 1.111.28) and sexual dysfunction (compared with those who had no sexual activity, OR 4.81, 95% CI 1.2418.72) were potential risk factors for depressive symptoms in patients with MRKH syndrome.

• #58 Study on depressive symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: an analysis of 141 cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01405-9

  Due to emotional burdens caused by the diagnosis, management and treatment of MRKH syndrome, depressive symptoms appear frequently. […] Patients have reported depressive emotions, even suicidal thoughts, in reaction to the diagnosis of this condition. […] The PHQ-9 score was significantly higher in MRKH patient group than the age-matched control group (7.0 (4.511.0) vs. 6.0 (3.09.0)), median and IQRs) (P=0.015). […] According to the last question in the PHQ-9, 11 (7.8%) patients with MRKH syndrome admitted that they had self-injury or suicidal ideas more than half of the time in the past 2 weeks, and 5 (3.5%) patients had these ideas almost every day. […] Negative self-evaluation of femininity (OR 3.96, 95% CI 1.3711.46); neurotic personality traits (OR 1.19 95% CI 1.111.28) and sexual dysfunction (compared with those who had no sexual activity, OR 4.81, 95% CI 1.2418.72) were potential risk factors for depressive symptoms in patients with MRKH syndrome.

• #59 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  Vaginal agenesis involves issues of physical abnormality, body image, sexual identity, and sexual/reproductive functioning that require long-term medical and psychological management. […] Vaginal agenesis, which occurs in approximately 1 in every 5,000 to 7,000 female births, is a significant threat to the mental health and well-being of an otherwise normal, healthy young woman. […] In all of these patients, the external genitalia are essentially normal. In place of the vagina, there is typically a small pouch or dimple that is 1 to 4 cm in depth. […] During arousal, the woman with vaginal agenesis experiences pelvic congestion with resultant lubrication in the genital area and will have an orgasm in response. […] Any adolescent girl has many psychosexual concerns and questions. […] When vaginal agenesis is diagnosed in adolescence–often as the result of an investigation of primary amenorrhea–the girl may react with shock and disbelief.

• #60 Vaginal Agenesis- Causes, Symptoms and Treatment – Best Fertility Centre in Chennai

  https://aakashfertilitycentre.in/vaginal-agenesis-causes-symptoms-and-treatment/

  Vaginal agenesis, medically known as Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome, is a rare congenital disorder characterized by either incomplete or complete absence of the vagina. […] The hallmark symptom of vaginal agenesis is the complete absence or partial development of the vaginal canal. […] Women with vaginal agenesis may experience primary amenorrhea (lack of menstruation) due to the absence of a functional uterus-vagina connection. […] Incomplete vaginal development can lead to difficulties with sexual intercourse, including pain or discomfort. […] The condition can have profound effects on self-image, identity, and mental well-being, leading to feelings of inadequacy, anxiety, or depression.

• #61 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #62 Vaginal Agenesis Treatment in West Orange, NJ

  https://njgyncancer.com/what-we-treat/vaginal-agenesis.php

  Vaginal agenesis is an uncommon birth defect that causes abnormal development of the vagina. Such abnormalities may include a shallow, remnant, or nonexistent vagina. […] Women with vaginal agenesis typically experience few symptoms, other than those related to the menstrual cycle. This is due to the connection between the uterus and the vagina. If the menstrual flow has no way to exit the uterus through the vagina, or if this flow is blocked in any way, painful symptoms will occur. […] Vaginal agenesis can also make it difficult or painful to engage in sex. Pregnancy is often very challenging if not impossible as well. […] Cases of vaginal agenesis that affect the patient’s quality of life will often lead to some type of vaginal reconstructive surgery. The specifics of this surgery will be dependent upon the extent of the vaginal deformities caused by vaginal agenesis.

• #63 Vaginal Agenesis | Intimate Wellness Institute

  https://iwiva.com/home-page/womens-speciality-care/advanced-gynecology/congenital-abnormalities/vaginal-agenesis/

  Vaginal agenesis is a congenital anomaly (birth defect). It happens when the vagina of a fetus doesnt develop properly in your uterus. They may be missing all or part of their vagina. Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short.

• #64 Mayer-Rokitansky-Küster-Hauser Syndrome: Overview

  https://my.clevelandclinic.org/health/diseases/23380-mayer-rokitansky-kuster-hauser-syndrome

  Mayer-Rokitansky-Kster-Hauser syndrome (MRKH syndrome) is a congenital condition that causes an infant to be born with an underdeveloped or missing uterus and/or vagina. […] People typically discover they have MRKH syndrome during their teen years when they never have a menstrual period. This is because they dont have a fully formed uterus and vagina. Other times, sexual intercourse is painful or impossible due to your vaginal canal being short and narrow. […] Symptoms of MRKH syndrome can vary depending on what type you have. […] In many cases, not getting a menstrual period (amenorrhea) by age 16 may be a sign your uterus or vagina isnt developed. […] You may also experience difficulty or pain when you try to have vaginal intercourse for the first time. This is because your vagina is thinner, narrower and shorter than a typical vagina.

• #65 Vertical/Complete Vaginal Septum | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vertical-complete-vaginal-septum

  Your daughter may realize she has duplicate vaginas when she inserts a tampon during her period and blood still leaks from her vagina. […] Common symptoms include: Pain upon inserting a tampon, Menstrual blood leaks out even with a tampon inserted. […] Having a vertical or complete vaginal septum may not affect her sexual life at all. However, some women with a vertical septum experience pain during sex.

• #66 Vaginal Agenesis – Giggles – Giggles

  http://giggles.co.in/departments/vaginal-agenesis/

  Vaginal Agenesis is a rare congenital defect that affects the lives of many women around the world. The condition occurs when the vagina is not formed fully during the foetal stage. Women with Vaginal Agenesis often struggle with feelings of shame and inadequacy and may face significant barriers in relationships and intimacy. […] Vaginal Agenesis is a rare congenital condition affecting female babies. Nearly 1 out of every 5000-7000 female infants are born with this condition. The condition occurs when a foetuss vagina doesnt develop properly in the uterus. […] While females with Vaginal Agenesis may be born without all or part of their vagina, the outer sexual organs may look completely normal. Therefore Vaginal Agenesis often goes unrecognised at birth. The symptoms may start to appear as these women reach puberty. Common symptoms include:

• #67 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #68 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  Vaginal agenesis involves issues of physical abnormality, body image, sexual identity, and sexual/reproductive functioning that require long-term medical and psychological management. […] Vaginal agenesis, which occurs in approximately 1 in every 5,000 to 7,000 female births, is a significant threat to the mental health and well-being of an otherwise normal, healthy young woman. […] In all of these patients, the external genitalia are essentially normal. In place of the vagina, there is typically a small pouch or dimple that is 1 to 4 cm in depth. […] During arousal, the woman with vaginal agenesis experiences pelvic congestion with resultant lubrication in the genital area and will have an orgasm in response. […] Any adolescent girl has many psychosexual concerns and questions. […] When vaginal agenesis is diagnosed in adolescence–often as the result of an investigation of primary amenorrhea–the girl may react with shock and disbelief.

• #69 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #70 Vaginal Agenesis Treatment in Turkey – Vaginoplasty Clinic

  https://www.vaginoplastyturkey.com/en/vaginal-agenesis-treatment-in-turkey

  Patients with vaginal agenesis have normal growth and pubertal development such as pubic and axillary hair and breast maturation. However they cannot have menstruation which is called primary amenorrhea. If they have a uterus but no vagina, menstrual bleeding will accumulate in the uterus (hematometra) since it cannot flow out, this causes sharp lower abdominal pain monthly. […] Typically women with MRKH syndrome have ovaries and eggs so that they ovulate. They may have symptoms related to ovulation such as mid-cycle pain and premenstrual syndrome but no menstrual bleeding. […] Since girls with MRKH syndrome have ovaries, they do ovulate. […] Women with MRKH have ovaries and the eggs will be expelled into the abdominal cavity during ovulation, the same way as in other patients who have a uterus. The difference is that the eggs will not be able form an embryo in the fallopian tube, and there is no uterus for an embryo to be implanted. […] Orgasm in MRKH syndrome patients can be achieved by stimulation of the clitoris. Women do not necessarily need to have a vagina to achieve orgasm. After vaginal agnesis treatment, vaginal intercourse will also make it possible to have an orgasm through penetration.

• #71 Vaginal agenesis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/vaginal-agenesis

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Vaginal agenesis may impact your sexual relationships, but after treatment, your vagina will typically function well for sexual activity.

• #72 Vaginal agenesis – Hancock Health

  https://www.hancockhealth.org/mayo-health-library/vaginal-agenesis/

  Vaginal agenesis is often identified at puberty when a female does not begin menstruating. […] Vaginal agenesis often goes unnoticed until females reach their teens, but dont menstruate (amenorrhea). Other signs of puberty usually follow typical female development. […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods dont start, even after youve developed breasts and have underarm and pubic hair. […] Vaginal agenesis may impact your sexual relationships, but after treatment, your vagina will typically function well for sexual activity. […] Treatment for vaginal agenesis often occurs in the late teens or early 20s, but you may wait until youre older and you feel motivated and ready to participate in treatment.

• #73 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #74 Vaginal and Mullerian Agenesis | Norton Children’s

  https://nortonchildrens.com/services/gynecology/conditions/vaginal-mullerian-agenesis/

  Vaginal agenesis is a rare disorder that occurs when the vagina doesn’t develop and the uterus may only develop partially or not at all. The condition is present before birth. […] Girls with vaginal agenesis typically have normal external genitalia and ovaries. As a result, they go through puberty and develop breasts and pubic hair, but they will not have periods. There may be a small pouch or dimple where a vaginal opening should be. […] Sometimes vaginal agenesis is recognized at birth. Most times it doesn’t present itself until puberty, when a teen notices she hasn’t started her period. […] If the child was born with an incomplete vagina but has a normal size uterus, she likely will be able to become pregnant. […] A normal sex life is possible for women born with vaginal agenesis because typically it does not affect external genitalia.

• #75 Mayer-Rokitansky-Küster-Hauser Syndrome: Overview

  https://my.clevelandclinic.org/health/diseases/23380-mayer-rokitansky-kuster-hauser-syndrome

  If you have the second type of MRKH syndrome, you may also have kidney complications or missing one or both kidneys. […] Reproductive health complications are common in people with MRKH. Other than being unable to carry a pregnancy or have a biological child, conditions like endometriosis can occur in people with an underdeveloped uterus.

• #76 MRKH Syndrome in Children | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/mayer-rokitansky-kuster-hauser-syndrome/

  Girls with MRKH have typical breast and pubic hair development during puberty but will not have menstrual periods. […] Because the ovaries are present and functioning normally, girls with MRKH may still experience cyclic hormonal symptoms such as mood changes and bloating. […] If a uterine remnant (or very small uterus) is present with a functional uterine lining (period lining), girls may have cyclic pelvic pain due to obstructed menstrual flow. […] Some girls with MRKH will also have kidney abnormalities, such as an absent kidney or misplaced kidney. […] Women born without a uterus or a uterine remnant cannot carry their own pregnancies.

• #77 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #78 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #79 MRKH Diagnosis | MRKH Symptoms | MRKH Syndrome

  https://www.miklosandmoore.com/mrkh-atlanta/diagnosis/

  MRKH syndrome is often associated with urinary tract, skeletal and auditory anomalies. […] Diagnosis is usually delayed until adolescence when the young woman does not have her period. The confirmation of the diagnosis is inevitably a psychological shock for the patient and her family. The absence of a vagina has a profound impact on the young womans sense of femininity and her future sexuality, thoughts about her future ability to bear children and have a family and the immediate feelings and shock of being told she does not have a normal vagina or uterus. […] Typically, vaginal agenesis patients do not have a functioning uterus and cannot become pregnant. Most have normal functioning ovaries and can become biologic parents through in-vitro fertilization of their own eggs with the pregnancy carried by a surrogate mother.

• #80 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #81 Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay | Journal of the Belgian Society of Radiology

  https://jbsr.be/articles/10.5334/jbsr.3660

  Hematocolpos is a medical condition in which menstrual blood or secretory fluid accumulates in the vagina due to vaginal obstruction. […] The main symptoms are periodic abdominal pain and primary amenorrhea. […] Late complications include tubal infection, adhesion, pelvic endometriosis, infertility, and renal failure due to hydronephrosis. […] Distal vaginal atresia and transverse vaginal septum involve segmental vaginal obstruction, with controversial terminology and embryology. […] Both typically present similarly and are not associated with other urogenital anomalies. […] Hematocolpos results from menstrual blood accumulation due to vaginal obstruction, mainly from congenital causes. Accurate differential diagnosis requires clinical examination, ultrasonography, and MRI. Early diagnosis and surgical treatment are crucial to prevent complications and preserve fertility.

• #82 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #83 Mayer-Rokitansky-Küster-Hauser Syndrome: Overview

  https://my.clevelandclinic.org/health/diseases/23380-mayer-rokitansky-kuster-hauser-syndrome

  If you have the second type of MRKH syndrome, you may also have kidney complications or missing one or both kidneys. […] Reproductive health complications are common in people with MRKH. Other than being unable to carry a pregnancy or have a biological child, conditions like endometriosis can occur in people with an underdeveloped uterus.

• #84 Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay | Journal of the Belgian Society of Radiology

  https://jbsr.be/articles/10.5334/jbsr.3660

  Hematocolpos is a medical condition in which menstrual blood or secretory fluid accumulates in the vagina due to vaginal obstruction. […] The main symptoms are periodic abdominal pain and primary amenorrhea. […] Late complications include tubal infection, adhesion, pelvic endometriosis, infertility, and renal failure due to hydronephrosis. […] Distal vaginal atresia and transverse vaginal septum involve segmental vaginal obstruction, with controversial terminology and embryology. […] Both typically present similarly and are not associated with other urogenital anomalies. […] Hematocolpos results from menstrual blood accumulation due to vaginal obstruction, mainly from congenital causes. Accurate differential diagnosis requires clinical examination, ultrasonography, and MRI. Early diagnosis and surgical treatment are crucial to prevent complications and preserve fertility.

• #85 Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay | Journal of the Belgian Society of Radiology

  https://jbsr.be/articles/10.5334/jbsr.3660

  Hematocolpos is a medical condition in which menstrual blood or secretory fluid accumulates in the vagina due to vaginal obstruction. […] The main symptoms are periodic abdominal pain and primary amenorrhea. […] Late complications include tubal infection, adhesion, pelvic endometriosis, infertility, and renal failure due to hydronephrosis. […] Distal vaginal atresia and transverse vaginal septum involve segmental vaginal obstruction, with controversial terminology and embryology. […] Both typically present similarly and are not associated with other urogenital anomalies. […] Hematocolpos results from menstrual blood accumulation due to vaginal obstruction, mainly from congenital causes. Accurate differential diagnosis requires clinical examination, ultrasonography, and MRI. Early diagnosis and surgical treatment are crucial to prevent complications and preserve fertility.

• #86 Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay | Journal of the Belgian Society of Radiology

  https://jbsr.be/articles/10.5334/jbsr.3660

  Hematocolpos is a medical condition in which menstrual blood or secretory fluid accumulates in the vagina due to vaginal obstruction. […] The main symptoms are periodic abdominal pain and primary amenorrhea. […] Late complications include tubal infection, adhesion, pelvic endometriosis, infertility, and renal failure due to hydronephrosis. […] Distal vaginal atresia and transverse vaginal septum involve segmental vaginal obstruction, with controversial terminology and embryology. […] Both typically present similarly and are not associated with other urogenital anomalies. […] Hematocolpos results from menstrual blood accumulation due to vaginal obstruction, mainly from congenital causes. Accurate differential diagnosis requires clinical examination, ultrasonography, and MRI. Early diagnosis and surgical treatment are crucial to prevent complications and preserve fertility.

• #87 Mayer-Rokitansky-Küster-Hauser Syndrome: Overview

  https://my.clevelandclinic.org/health/diseases/23380-mayer-rokitansky-kuster-hauser-syndrome

  If you have the second type of MRKH syndrome, you may also have kidney complications or missing one or both kidneys. […] Reproductive health complications are common in people with MRKH. Other than being unable to carry a pregnancy or have a biological child, conditions like endometriosis can occur in people with an underdeveloped uterus.

• #88 Vaginal agenesis – Hancock Health

  https://www.hancockhealth.org/mayo-health-library/vaginal-agenesis/

  Vaginal agenesis is often identified at puberty when a female does not begin menstruating. […] Vaginal agenesis often goes unnoticed until females reach their teens, but dont menstruate (amenorrhea). Other signs of puberty usually follow typical female development. […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods dont start, even after youve developed breasts and have underarm and pubic hair. […] Vaginal agenesis may impact your sexual relationships, but after treatment, your vagina will typically function well for sexual activity. […] Treatment for vaginal agenesis often occurs in the late teens or early 20s, but you may wait until youre older and you feel motivated and ready to participate in treatment.

• #89

• #90 Female Genital Birth Defects – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-urinary-tract-and-genitals/female-genital-birth-defects

  Girls who have vaginal agenesis also may have kidney abnormalities. For example, they may be missing a kidney. They may also have problems with bones or abdominal wall abnormalities. […] No immediate treatment is needed unless girls have pain due to blocked flow of menstrual blood. Once girls are ready to begin treatment, they will use a vaginal dilator to stretch their vaginal canal. A vaginal dilator is a smooth plastic rod shaped similar to a tampon. Girls insert a dilator and, over time, it stretches the vagina.

• #91 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. This profile helps distinguish the MRKH syndrome from androgen insensitivity syndromes in which postpubertal testosterone is elevated. […] Surgical treatment should be considered only when the patient can participate in the decision-making, wishes to become sexually active, and is highly motivated to use a vaginal prosthesis for several months after surgery. […] The aim of surgical treatment is to create a neovagina. Although several vaginoplasty methods have been developed, refined, and modified, no definitive surgical approach has been established. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty.

• #92 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. This profile helps distinguish the MRKH syndrome from androgen insensitivity syndromes in which postpubertal testosterone is elevated. […] Surgical treatment should be considered only when the patient can participate in the decision-making, wishes to become sexually active, and is highly motivated to use a vaginal prosthesis for several months after surgery. […] The aim of surgical treatment is to create a neovagina. Although several vaginoplasty methods have been developed, refined, and modified, no definitive surgical approach has been established. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty.

• #93 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  Mllerian agenesis, also referred to as mllerian aplasia, Mayer-Rokitansky-Kster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,5005,000 females. Mllerian agenesis is caused by embryologic underdevelopment of the mllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with mllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. […] The most important steps in the effective management of mllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. […] 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.

• #94 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #95 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  Mllerian agenesis, also referred to as mllerian aplasia, Mayer-Rokitansky-Kster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,5005,000 females. Mllerian agenesis is caused by embryologic underdevelopment of the mllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with mllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. […] The most important steps in the effective management of mllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. […] 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.

• #96 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #97 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #98 Müllerian Anomalies | Congenital Female Tract Abnormalities | Duke Health

  https://www.dukehealth.org/treatments/obstetrics-and-gynecology/mullerian-anomalies

  Vaginal agenesis, vaginal septum, and congenital uterine abnormalities often go undetected and may cause no symptoms. […] However, that may change at the start of menses or sexual activity, when trying to become pregnant, or during pregnancy. It’s at that point that either menstruation doesn’t start or a woman experiences pain and difficulty having sex, getting pregnant, or maintaining a pregnancy. […] Dilator treatment is often the first treatment recommended. It does not require surgery and may be prescribed to create an indent in the vagina when MRKH or vaginal agenesis is present. A hard, smooth, plastic, tampon-shaped device is applied daily for 20 to 30 minutes with hand pressure to stretch the vaginal canal. This process must be done regularly or the indent will disappear. […] Surgery to create a functional vagina may be performed when a young woman wants a longer, more functional vagina. An opening is made in the vaginal area. A graft of skin is taken from the inside of the cheek, buttocks, or leg and placed inside the opening. A mold is also placed inside to maintain the vaginal canal during healing. The mold is later removed. Regular use of vaginal dilation and sexual intercourse helps to maintain the shape.

• #99 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #100 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #101

• #102 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. This profile helps distinguish the MRKH syndrome from androgen insensitivity syndromes in which postpubertal testosterone is elevated. […] Surgical treatment should be considered only when the patient can participate in the decision-making, wishes to become sexually active, and is highly motivated to use a vaginal prosthesis for several months after surgery. […] The aim of surgical treatment is to create a neovagina. Although several vaginoplasty methods have been developed, refined, and modified, no definitive surgical approach has been established. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty.

• #103 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-overview

  The hormonal profile is that of a normal female with age-appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. […] The first-line approach in most patients should be primary vaginal elongation by dilation. Compared with surgery, it is safer, patient-controlled, and more cost-effective. […] Primary vaginal dilation is successful for more than 90-96% of patients. […] The aim of surgical treatment is to create a neovagina. […] The modified McIndoe procedure remains the most common surgical approach to vaginoplasty. […] The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #104 Müllerian Anomalies | Congenital Female Tract Abnormalities | Duke Health

  https://www.dukehealth.org/treatments/obstetrics-and-gynecology/mullerian-anomalies

  Vaginal agenesis, vaginal septum, and congenital uterine abnormalities often go undetected and may cause no symptoms. […] However, that may change at the start of menses or sexual activity, when trying to become pregnant, or during pregnancy. It’s at that point that either menstruation doesn’t start or a woman experiences pain and difficulty having sex, getting pregnant, or maintaining a pregnancy. […] Dilator treatment is often the first treatment recommended. It does not require surgery and may be prescribed to create an indent in the vagina when MRKH or vaginal agenesis is present. A hard, smooth, plastic, tampon-shaped device is applied daily for 20 to 30 minutes with hand pressure to stretch the vaginal canal. This process must be done regularly or the indent will disappear. […] Surgery to create a functional vagina may be performed when a young woman wants a longer, more functional vagina. An opening is made in the vaginal area. A graft of skin is taken from the inside of the cheek, buttocks, or leg and placed inside the opening. A mold is also placed inside to maintain the vaginal canal during healing. The mold is later removed. Regular use of vaginal dilation and sexual intercourse helps to maintain the shape.

• #105 Mullerian Duct Anomalies: Overview, Incidence and Prevalence, Embryology

  https://emedicine.medscape.com/article/954110-clinical

  The patient is gradually allowed to ambulate within 24 hours after surgery, but she must remain on bedrest in upright and flat positions for 1 week. […] The neovagina is irrigated with warm sodium chloride solution and carefully inspected to determine whether the graft has taken satisfactorily. […] The procedures for both thick and thin vaginal septa result in a vagina that is only slightly shorter than average.

• #106 Vaginal agenesis | Altru Health System

  https://www.altru.org/health-library/conditions/vaginal-agenesis

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Treatment for vaginal agenesis often occurs in the late teens or early 20s, but you may wait until you’re older and you feel motivated and ready to participate in treatment. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #107 Vaginal agenesis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/diagnosis-treatment/drc-20355741

  Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Some patients report problems with urinating and with vaginal bleeding and pain, especially in the beginning. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #108 Vaginal agenesis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/diagnosis-treatment/drc-20355741

  Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Some patients report problems with urinating and with vaginal bleeding and pain, especially in the beginning. […] After surgery, use of a mold, dilation or frequent sexual intercourse is needed to maintain a functional vagina.

• #109 Congenital Anomalies of the Vagina – Brigham and Women’s Hospital

  https://www.brighamandwomens.org/obgyn/infertility-reproductive-surgery/congenital-anomalies/vagina-anomalies

  Alternatively, a vagina can be created with the utilization of a skin graft and this procedure is called a McIndoe procedure. […] The utilization of a skin graft can thus result in a normal, functional vagina. Some women find that they need to utilize water-based lubricants for sexual activity, as the skin of the newly created vagina may be dry. […] Another option for creation of a vagina is with the utilization of bowel. […] Additionally, concerns exist regarding the utilization of bowel for the creation of a vagina due to the risk of sexually transmitted diseases and the fact that bowel is a poor protective barrier against sexually transmitted diseases when compared to skin. […] Vaginal agenesis can be diagnosed on physical examination with additional information gathered from ultrasound or MRI. […] Women with vaginal agenesis from Mayer-von RokitanskyKster-Hausers syndrome have a karyotype of 46, XX which is the most common for women. Thus obtaining a karyotype can also be helpful in making a definitive diagnosis; additionally a testosterone level can also be helpful in making a diagnosis.

• #110 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #111 Vaginal agenesis – Hancock Health

  https://www.hancockhealth.org/mayo-health-library/vaginal-agenesis/

  Vaginal agenesis is often identified at puberty when a female does not begin menstruating. […] Vaginal agenesis often goes unnoticed until females reach their teens, but dont menstruate (amenorrhea). Other signs of puberty usually follow typical female development. […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods dont start, even after youve developed breasts and have underarm and pubic hair. […] Vaginal agenesis may impact your sexual relationships, but after treatment, your vagina will typically function well for sexual activity. […] Treatment for vaginal agenesis often occurs in the late teens or early 20s, but you may wait until youre older and you feel motivated and ready to participate in treatment.

• #112 What Is Agenesis of the Vagina?

  https://www.icliniq.com/articles/womens-health/agenesis-of-the-vagina

  Vaginal agenesis is seen associated with other symptoms, such as: Problems with the development of the kidneys and urinary tract. Developmental changes in the spine, wrists, and rib bones. Hearing trouble. It can also include other congenital conditions of the gastrointestinal tract, heart, and limb growth. […] A vaginal dilator for three months after the surgery is recommended for most patients. It can be removed during showers, during bowel movements, and during sexual intercourse. After three months dilators are recommended to wear for another three months at night. […] Agenesis of the vagina is a rare condition, occurring before birth. It is often left unnoticed until the puberty period of a girl as menstruation is not possible due to a lack of vaginal opening. The treatment for vaginal agenesis is using a vaginal dilator or surgery to create a vaginal opening. Most doctors recommend vaginal dilators as an initial treatment option as it is an invasive method.

• #113 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #114 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  The adolescent girl and her parents often ask questions about physiology and anatomy (eg, absence of menarche, failure of the vagina to lengthen). […] The physician should reassure the patient and her parents by citing the normal development of her breasts, ovaries, pubic hair, vulva, and clitoris. […] The patient can be told that she will be able to experience normal orgasmic responses. […] Women with vaginal agenesis who have a 46,XX karyotype have normal female levels of the hormones estrogen and progesterone, and they should be reassured concerning ovarian function. […] Concerns about personal and sexual relationships should be addressed with a review of the patient’s normal sex characteristics, hormonal activity, and orgasmic function. […] Some women initially report a feeling of pressure, tightness, or soreness during or after intercourse, but these symptoms abate with repeated activity. […] The importance of referral to a certified sex therapist cannot be overemphasized, since the physician and counselor form complementary relationships with the patient.

• #115 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  The adolescent girl and her parents often ask questions about physiology and anatomy (eg, absence of menarche, failure of the vagina to lengthen). […] The physician should reassure the patient and her parents by citing the normal development of her breasts, ovaries, pubic hair, vulva, and clitoris. […] The patient can be told that she will be able to experience normal orgasmic responses. […] Women with vaginal agenesis who have a 46,XX karyotype have normal female levels of the hormones estrogen and progesterone, and they should be reassured concerning ovarian function. […] Concerns about personal and sexual relationships should be addressed with a review of the patient’s normal sex characteristics, hormonal activity, and orgasmic function. […] Some women initially report a feeling of pressure, tightness, or soreness during or after intercourse, but these symptoms abate with repeated activity. […] The importance of referral to a certified sex therapist cannot be overemphasized, since the physician and counselor form complementary relationships with the patient.

• #116 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  The adolescent girl and her parents often ask questions about physiology and anatomy (eg, absence of menarche, failure of the vagina to lengthen). […] The physician should reassure the patient and her parents by citing the normal development of her breasts, ovaries, pubic hair, vulva, and clitoris. […] The patient can be told that she will be able to experience normal orgasmic responses. […] Women with vaginal agenesis who have a 46,XX karyotype have normal female levels of the hormones estrogen and progesterone, and they should be reassured concerning ovarian function. […] Concerns about personal and sexual relationships should be addressed with a review of the patient’s normal sex characteristics, hormonal activity, and orgasmic function. […] Some women initially report a feeling of pressure, tightness, or soreness during or after intercourse, but these symptoms abate with repeated activity. […] The importance of referral to a certified sex therapist cannot be overemphasized, since the physician and counselor form complementary relationships with the patient.

• #117 Care and Counseling of the Patient with Vaginal Agenesis | Intersex Society of North America

  https://isna.org/node/83/

  The adolescent girl and her parents often ask questions about physiology and anatomy (eg, absence of menarche, failure of the vagina to lengthen). […] The physician should reassure the patient and her parents by citing the normal development of her breasts, ovaries, pubic hair, vulva, and clitoris. […] The patient can be told that she will be able to experience normal orgasmic responses. […] Women with vaginal agenesis who have a 46,XX karyotype have normal female levels of the hormones estrogen and progesterone, and they should be reassured concerning ovarian function. […] Concerns about personal and sexual relationships should be addressed with a review of the patient’s normal sex characteristics, hormonal activity, and orgasmic function. […] Some women initially report a feeling of pressure, tightness, or soreness during or after intercourse, but these symptoms abate with repeated activity. […] The importance of referral to a certified sex therapist cannot be overemphasized, since the physician and counselor form complementary relationships with the patient.

• #118 MRKH: Commonly Asked Questions – Center for Young Women’s Health

  https://youngwomenshealth.org/guides/mrkh-frequently-asked-questions/

  Girls are often diagnosed between the ages of 15-18 when they dont get a period. […] General gynecologic care is important but your visit may vary depending on your symptoms and history. Pelvic examinations may be important if there are concerns about complications or symptoms such as vaginal narrowing or discharge. […] Some women with MRKH may have a tiny uterus called a uterine remnant or uterine horn. This type of uterus or womb is not big enough to carry a baby but sometimes it can cause pelvic pain if blood leaks into the pelvic cavity. […] If you have vaginal intercourse before your vagina is created using dilators or surgery, sex will likely be very painful. It could cause a tear in your vagina and bleeding. […] Typically, women do not experience pain with intercourse after they have completed dilation treatment. If you feel any discomfort after treatment it may be that you need to use a lubricant such as KY jelly before vaginal intercourse.

• #119 Study on depressive symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: an analysis of 141 cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01405-9

  Due to emotional burdens caused by the diagnosis, management and treatment of MRKH syndrome, depressive symptoms appear frequently. […] Patients have reported depressive emotions, even suicidal thoughts, in reaction to the diagnosis of this condition. […] The PHQ-9 score was significantly higher in MRKH patient group than the age-matched control group (7.0 (4.511.0) vs. 6.0 (3.09.0)), median and IQRs) (P=0.015). […] According to the last question in the PHQ-9, 11 (7.8%) patients with MRKH syndrome admitted that they had self-injury or suicidal ideas more than half of the time in the past 2 weeks, and 5 (3.5%) patients had these ideas almost every day. […] Negative self-evaluation of femininity (OR 3.96, 95% CI 1.3711.46); neurotic personality traits (OR 1.19 95% CI 1.111.28) and sexual dysfunction (compared with those who had no sexual activity, OR 4.81, 95% CI 1.2418.72) were potential risk factors for depressive symptoms in patients with MRKH syndrome.

• #120 MRKH: Commonly Asked Questions – Center for Young Women’s Health

  https://youngwomenshealth.org/guides/mrkh-frequently-asked-questions/

  Orgasm is the result of sexual stimulation that is a combination of emotional, psychological and physical pleasure. There are many reasons why women may not experience orgasm at any given time, but none that are directly related to having MRKH. […] If you find yourself beginning to be overwhelmed or concerned about something, the sooner you seek help the better.

• #121 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #122 Vaginal Agenesis: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/vaginal-agenesis

  The long-term prognosis for individuals with vaginal agenesis can vary based on a variety of factors, including the severity of the condition, the effectiveness of treatment, and the presence of any associated medical conditions. However, with timely and appropriate medical intervention such as surgical intervention and ongoing medical management, patients with vaginal agenesis can generally expect to achieve good quality of life and reproductive outcomes. Long-term follow-up and management are essential to manage any potential complications or concerns that may arise.

• #123 Vaginal Agenesis: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/vaginal-agenesis

  Early diagnosis is crucial in improving the prognosis of vaginal agenesis. It allows for early intervention and treatment to prevent potential complications such as urinary or gynecologic problems. Early diagnosis also allows for patients to receive appropriate counseling and support throughout their lifetime. […] Surgery can be an effective treatment option for vaginal agenesis. Procedures such as vaginal dilation or reconstruction can help create a functional vaginal canal. Surgical outcomes depend on various factors such as the severity of the condition, the specific surgical technique utilized, and the individual patient`s recovery. Therefore, it is essential to discuss all available options and the associated risks and benefits with a medical professional. […] Specific factors that can affect the prognosis of vaginal agenesis include the type and severity of the condition, the presence of other congenital anomalies or associated medical conditions, the age at diagnosis, and the timing and effectiveness of treatment. Those with a milder form of vaginal agenesis and those who receive early and appropriate treatment generally have a better prognosis.

• #124 Vaginal and Mullerian Agenesis | Norton Children’s

  https://nortonchildrens.com/services/gynecology/conditions/vaginal-mullerian-agenesis/

  Vaginal agenesis is a rare disorder that occurs when the vagina doesn’t develop and the uterus may only develop partially or not at all. The condition is present before birth. […] Girls with vaginal agenesis typically have normal external genitalia and ovaries. As a result, they go through puberty and develop breasts and pubic hair, but they will not have periods. There may be a small pouch or dimple where a vaginal opening should be. […] Sometimes vaginal agenesis is recognized at birth. Most times it doesn’t present itself until puberty, when a teen notices she hasn’t started her period. […] If the child was born with an incomplete vagina but has a normal size uterus, she likely will be able to become pregnant. […] A normal sex life is possible for women born with vaginal agenesis because typically it does not affect external genitalia.

• #125 Vaginal agenesis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/vaginal-agenesis

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. […] Vaginal agenesis is typically diagnosed during puberty when your menstrual periods don’t start, even after you’ve developed breasts and have underarm and pubic hair. […] Vaginal agenesis may impact your sexual relationships, but after treatment, your vagina will typically function well for sexual activity.

• #126 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #127 Müllerian Anomalies | University of Colorado OB-GYN | Denver | Boulder

  https://obgyn.coloradowomenshealth.com/health-info/conditions/mullerian-anomalies

  Cervical agenesis happens when a baby is born without a cervix, the opening in the uterus that connects to the vagina. Typically, this disorder occurs in conjunction with vaginal agenesis. Symptoms include abdominal pain and lack of menstruation during puberty. […] Mllerian agenesis is absence of a uterus and upper vagina and is also known as Mayer-Rokitansky-Kster-Hauser syndrome. A person born without a uterus or a very small uterus (also known as a uterine remnant) cannot carry their own pregnancies. However, since ovaries are normal, assisted reproduction options including in vitro fertilization (IVF) and gestational carriers are available. Vaginal creation or lengthening can be considered when desired by the patient.

• #128 Müllerian Agenesis: Room for Improvement in Care, Counseling – Research Horizons

  https://scienceblog.cincinnatichildrens.org/mullerian-agenesis-room-for-improvement-in-care-counseling/

  Müllerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a congenital condition that results in underdevelopment of the uterus, cervix and upper two-thirds of the vagina. MRKH affects 1 in 5,000 individuals assigned female sex at birth. This condition results in infertility and can have a significant psychosocial impact. The condition also results in a shortened vaginal canal. Individuals with MRKH are often diagnosed by and seek care from gynecologists. This care can include lengthening of the vaginal canal through dilation and surgery when indicated. […] Overall, 616 individuals with Müllerian agenesis from 40 countries responded to an in-depth survey about their experiences. Of the respondents, 54% received one or more vaginal lengthening treatments. The most common treatments were dilator use (72%), coital dilation (34%) and surgery (39%). Notably, 46% of the respondents reported seeking no vaginal lengthening treatment. This varied by geography, with 59% of respondents in North America and Europe reporting treatments compared to 16% among women from Africa, Asia and South America. Shared themes showed both positive and challenging aspects of vaginal lengthening including difficult physical symptoms, practical and psychosocial challenges, intimate relationships and sexual satisfaction, and impact of experiences with healthcare providers. […] “Our findings show room for improvement in the counseling and care of individuals with MRKH, particularly around vaginal lengthening,” Pennesi says. “There is a need to educate healthcare providers so they can support patients in accessing appropriate, comprehensive and longitudinal care.”

• #129 MRKH Syndrome | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/m/mrkh-syndrome

  Around half of people with MRKH have related congenital conditions. As a result, those with MRKH may also have bowel symptoms, congenital spine and conditions, kidney abnormalities, hearing loss, and urinary symptoms. […] People with MRKH syndrome can expect to live a full life. There is currently no data on early menopause since ovarian function is not affected.

• #130 Vaginal Agenesis: Causes, Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/23458-vaginal-agenesis

  Vaginal agenesis is a congenital abnormality (birth defect). It happens when a fetus’s vagina doesnt develop properly in the uterus. […] Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women dont learn that they have the condition until their teenage years. It becomes apparent when they dont start their monthly period (menstruation). […] Signs of this condition include: Amenorrhea, no monthly period by age 15. Cryptomenorrhea, when menstrual blood pools inside your body because there isnt a way for it to exit your body. This buildup can cause cramping and abdominal pain. Dimple or divot where the vaginal opening should be. Pain during sex, which can happen when the vagina is too short. […] Your outlook depends on many factors. These include the treatment you received and which of your reproductive organs the condition affected. With treatment, many people have a satisfying sex life.

• #131 Vaginal agenesis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

  Vaginal agenesis often goes unnoticed until females reach their teens, but don’t menstruate (amenorrhea). […] Vaginal agenesis may have these features: The genitals look like a typical female. The vagina may be shortened without a cervix at the end, or absent and marked only by a slight indentation where a vaginal opening would typically be located. There may be no uterus or one that’s only partially developed. If there’s tissue lining the uterus (endometrium), monthly cramping or chronic abdominal pain may occur. The ovaries typically are fully developed and functional, but they may be in an unusual location in the abdomen. Sometimes the pair of tubes that eggs travel through to get from the ovaries to the uterus (fallopian tubes) are absent or do not develop typically. […] Vaginal agenesis may also be associated with other issues, such as: Problems with development of the kidneys and urinary tract, Developmental changes in the bones of the spine, ribs and wrists, Hearing problems, Other congenital conditions that also involve the heart, gastrointestinal tract and limb growth.

• #132 Vaginal Agenesis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/vaginal-agenesis-causes-symptoms-and-treatment?srsltid=AfmBOoo8YtMQNcC6WYrK5RvCOMDSMylyYbM5IKmbufCt2mwUvlmNA4iL

  Abdominal pain and intense cramping (due to build-up of menstrual fluid from the obstruction caused owing to the missing vagina). […] Formation of a pelvic mass (if the upper vagina fills with menstrual blood). […] In some cases, Vaginal agenesis often causes other symptoms, including: Issues with the development of the kidneys and urinary tract. […] Structural changes in the bones of the spine, ribs, and wrists. […] Problems with hearing. […] Other congenital conditions that also involve the heart, gastrointestinal tract, and growth of limbs. […] In case Vaginal agenesis is not treated on time, it often disrupts the routine life of many women. […] It also impacts the sexual relationship with the partner and often leads to women failing to bear a child.

• #133 Mayer-Rokitansky-Küster-Hauser Syndrome: Overview

  https://my.clevelandclinic.org/health/diseases/23380-mayer-rokitansky-kuster-hauser-syndrome

  If you have the second type of MRKH syndrome, you may also have kidney complications or missing one or both kidneys. […] Reproductive health complications are common in people with MRKH. Other than being unable to carry a pregnancy or have a biological child, conditions like endometriosis can occur in people with an underdeveloped uterus.

• #134 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  Mllerian agenesis, also referred to as mllerian aplasia, Mayer-Rokitansky-Kster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,5005,000 females. Mllerian agenesis is caused by embryologic underdevelopment of the mllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with mllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. […] The most important steps in the effective management of mllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. […] 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.

• #135 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #136 Vaginal Agenesis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vaginal-agenesis

  Depending on the reproductive organs affected, your daughter’s sexual life and fertility could be impacted in a variety of ways. If she was born with a normal-sized uterus, she may be able to become pregnant and deliver a baby. […] Much of female sexual pleasure comes from stimulation of the clitoris. Since patients with vaginal agenesis have normal external genitalia, she should be able to experience normal orgasmic function. […] It’s important to understand that if your daughter has MRKH, she has a karyotype of 46 XX, meaning she is genetically female. […] A vaginal dilator is hard, smooth plastic and shaped similar to a tampon. When pressure is applied by hand over time it stretches the vagina. […] It should be noted that a young woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally.

• #137 Müllerian Agenesis: Diagnosis, Management, and Treatment | ACOG

  https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment

  When well-counseled and emotionally prepared, almost all patients (9096%) will be able to achieve anatomic and functional success by primary vaginal dilation. […] Evaluation for associated congenital anomalies is essential because up to 53% of patients with mllerian agenesis have concomitant congenital malformations, especially of the urinary tract and skeleton. […] The psychologic effect of the diagnosis of mllerian agenesis should not be underestimated. All patients with mllerian agenesis should be offered counseling and encouraged to connect with peer support groups. […] Future options for having children should be addressed with patients, including adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with mllerian agenesis.

• #138 Vaginal Agenesis: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/vaginal-agenesis

  Early diagnosis is crucial in improving the prognosis of vaginal agenesis. It allows for early intervention and treatment to prevent potential complications such as urinary or gynecologic problems. Early diagnosis also allows for patients to receive appropriate counseling and support throughout their lifetime. […] Surgery can be an effective treatment option for vaginal agenesis. Procedures such as vaginal dilation or reconstruction can help create a functional vaginal canal. Surgical outcomes depend on various factors such as the severity of the condition, the specific surgical technique utilized, and the individual patient`s recovery. Therefore, it is essential to discuss all available options and the associated risks and benefits with a medical professional. […] Specific factors that can affect the prognosis of vaginal agenesis include the type and severity of the condition, the presence of other congenital anomalies or associated medical conditions, the age at diagnosis, and the timing and effectiveness of treatment. Those with a milder form of vaginal agenesis and those who receive early and appropriate treatment generally have a better prognosis.

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