| Vaginal hypoplasia | |
|---|---|
| Other names | Congenital absence of vagina |
 | |
| Vaginal hypoplasia in a patient with Müllerian agenesis | |
| Specialty | Gynecology, medical genetics |
Vaginal hypoplasia is the underdevelopment or incomplete development of the vagina. It is a birth defect or congenital abnormality of the female genitourinary system.
Vaginal hypoplasia can vary in severity from being smaller than normal to being completely absent. The absence of a vagina is a result of vaginal agenesis. Diagnostically, it may look similar to a vaginal obstruction such as can be caused by an imperforate hymen or, less commonly, a transverse vaginal septum.[ citation needed ]
It is frequently associated with Mayer-Rokitansky-Küstner-Hauser syndrome, in which the most common result is an absent uterus in conjunction with a deformed or missing vagina, despite the presence of normal ovaries and normal external genitalia. It is also associated with cervical agenesis, in which the uterus is present but the uterine cervix is absent. The situation is most urgent where there is a menstruating uterus with an obstructed uterovaginal outflow, leading to hematometra. In this case prompt medical action is required.[ citation needed ]
The main causes are Müllerian agenesis and complete androgen insensitivity syndrome. [1]
In order to facilitate sexual intercourse, the main treatments are self-dilation methods (using intra-vaginal cylinders or inflatable expanders (vaginal stents) of increasing size) and surgical vaginoplasty to lengthen the vagina. [1] Self-dilation has a high success rate, estimated at 75%, and is usually the first-line treatment due to low surgical invasiveness. [1] Overall, the complication rates are significantly lower with dilation than with vaginoplasty. [1]
Surgery is indicated when there is inability or reluctance to perform self-dilation, or where it is performed but with failed result. The vaginoplasty is performed around the inflatable expander which maintains the neovagina against the pelvic wall after the surgery and favors the process of microscopic neovascularization while reducing the risks for hematoma. [2] [3] One appropriate surgical variant is the Vecchietti technique. In this procedure, an olive-shaped pressure device is pressed towards the potential vaginal space by a thread that goes through the skin, behind the urinary bladder and pubic bone and exits the skin in the hypogastrium, where it is attached to a plate that provides counter-traction. [1] Vaginoplasty can also be performed using a skin graft or an intestinal graft. Traction vaginoplasty such as the Vecchietti technique seems to have the highest success rates both anatomically (99%) and functionally (96%), whereas skin graft procedures and intestinal procedures have the lowest successful outcomes (83–95%). [1]
After vaginoplasty, available evidence suggests that continued self-dilation is needed to maintain the patency in periods of coital inactivity. [1] A vaginal expander can be used regularly to prevent post-operative vaginal retraction. [4]
Vaginal hypoplasia is estimated to occur in 1 in 4,000–5,000 live female births. It is often unnoticed until adolescence when pain and a lack of menstrual flow indicates the condition. [5]
The cervix or cervix uteri is a dynamic fibromuscular organ of the female reproductive system that connects the vagina with the uterine cavity. The human cervix has been documented anatomically since at least the time of Hippocrates, over 2,000 years ago[citation needed]. The cervix is approximately 4 cm long with a diameter of approximately 3 cm and tends to be described as a cylindrical shape, although the front and back walls of the cervix are contiguous. The size of the cervix changes throughout a women's life cycle. For example, during their fertile years of the reproductive cycle, females tend to have a larger cervix vis á vis postmenopausal females; likewise, females who have produced offspring have a larger sized cervix than females who have not produced offspring.
Gender-affirming surgery for female-to-male transgender people includes a variety of surgical procedures that alter anatomical traits to provide physical traits more comfortable to the trans man's male identity and functioning.
Gender-affirming surgery for male-to-female transgender women or transfeminine non-binary people describes a variety of surgical procedures that alter the body to provide physical traits more comfortable and affirming to an individual's gender identity and overall functioning.
Vaginoplasty is any surgical procedure that results in the construction or reconstruction of the vagina. It is a type of genitoplasty. Pelvic organ prolapse is often treated with one or more surgeries to repair the vagina. Sometimes a vaginoplasty is needed following the treatment or removal of malignant growths or abscesses to restore a normal vaginal structure and function. Surgery to the vagina is done to correct congenital defects to the vagina, urethra and rectum. It may correct protrusion of the urinary bladder into the vagina (cystocele) and protrusion of the rectum (rectocele) into the vagina. Often, a vaginoplasty is performed to repair the vagina and its attached structures due to trauma or injury.
The paramesonephric ducts are paired ducts of the embryo in the female reproductive system that run down the lateral sides of the genital ridge and terminate at the sinus tubercle in the primitive urogenital sinus. In the female, they will develop to form the fallopian tubes, uterus, cervix, and the upper one-third of the vagina.
Müllerian agenesis, also known as Müllerian aplasia, vaginal agenesis, or Mayer–Rokitansky–Küster–Hauser syndrome, is a congenital malformation characterized by a failure of the Müllerian ducts to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Müllerian agenesis is the cause in 15% of cases of primary amenorrhoea. Because most of the vagina does not develop from the Müllerian duct, instead developing from the urogenital sinus, along with the bladder and urethra, it is present even when the Müllerian duct is completely absent. Because ovaries do not develop from the Müllerian ducts, affected people might have normal secondary sexual characteristics but are infertile due to the lack of a functional uterus. However, biological motherhood is possible through uterus transplantation or use of gestational surrogates.
A uterine malformation is a type of female genital malformation resulting from an abnormal development of the Müllerian duct(s) during embryogenesis. Symptoms range from amenorrhea, infertility, recurrent pregnancy loss, and pain, to normal functioning depending on the nature of the defect.
Pelvic organ prolapse (POP) is characterized by descent of pelvic organs from their normal positions into the vagina. In women, the condition usually occurs when the pelvic floor collapses after gynecological cancer treatment, childbirth or heavy lifting. Injury incurred to fascia membranes and other connective structures can result in cystocele, rectocele or both. Treatment can involve dietary and lifestyle changes, physical therapy, or surgery.
Labiaplasty is a plastic surgery procedure for creating or altering the labia minora and the labia majora, the folds of skin of the human vulva. It is a type of vulvoplasty. There are two main categories of women seeking cosmetic genital surgery: those with conditions such as intersex, and those with no underlying condition who experience physical discomfort or wish to alter the appearance of their vulvas because they believe they do not fall within a normal range.
Vaginal atresia is a condition in which the vagina is abnormally closed or absent. The main causes can either be complete vaginal hypoplasia, or a vaginal obstruction, often caused by an imperforate hymen or, less commonly, a transverse vaginal septum. It results in uterovaginal outflow tract obstruction. This condition does not usually occur by itself within an individual, but coupled with other developmental disorders within the female. The disorders that are usually coupled with a female who has vaginal atresia are Mayer-Rokitansky-Küster-Hauser syndrome, Bardet-Biedl syndrome, or Fraser syndrome. One out of every 5,000 women have this abnormality.
Vaginectomy is a surgery to remove all or part of the vagina. It is one form of treatment for individuals with vaginal cancer or rectal cancer that is used to remove tissue with cancerous cells. It can also be used in gender-affirming surgery. Some people born with a vagina who identify as trans men or as nonbinary may choose vaginectomy in conjunction with other surgeries to make the clitoris more penis-like (metoidioplasty), construct of a full-size penis (phalloplasty), or create a relatively smooth, featureless genital area.
A vaginal dilator is an instrument used to gently stretch the vagina. They are used when the vagina has become narrowed, such as after brachytherapy for gynecologic cancers, and as therapy for vaginismus and other forms of dyspareunia.
Hematocolpos is a medical condition in which the vagina is pooled with menstrual blood due to multiple factors leading to the blockage of menstrual blood flow. The medical definition of hematocolpos is 'an accumulation of blood within the vagina'. It is often caused by the combination of menstruation with an imperforate hymen. It is sometimes seen in Robinow syndrome, uterus didelphys, or other vaginal anomalies.
Hematometra is a medical condition involving collection or retention of blood in the uterus. It is most commonly caused by an imperforate hymen or a transverse vaginal septum.
A vaginal disease is a pathological condition that affects part or all of the vagina.
Complete androgen insensitivity syndrome (CAIS) is an AIS condition that results in the complete inability of the cell to respond to androgens. As such, the insensitivity to androgens is only clinically significant when it occurs in individuals who are exposed to significant amounts of testosterone at some point in their lives. The unresponsiveness of the cell to the presence of androgenic hormones prevents the masculinization of male genitalia in the developing fetus, as well as the development of male secondary sexual characteristics at puberty, but does allow, without significant impairment, female genital and sexual development in those with the condition.
Vaginal transplantation is procedure whereby donated or laboratory-grown vagina tissue is used to create a 'neovagina'. It is most often used in women who have vaginal aplasia.
Cervical agenesis is a congenital disorder of the female genital system that manifests itself in the absence of a cervix, the connecting structure between the uterus and vagina. Milder forms of the condition, in which the cervix is present but deformed and nonfunctional, are known as cervical atresia or cervical dysgenesis.
Müllerian duct anomalies are those structural anomalies caused by errors in Müllerian duct development during embryonic morphogenesis. Factors that precipitate include genetics, and maternal exposure to teratogens.
Vaginal anomalies are abnormal structures that are formed during the prenatal development of the female reproductive system and are rare congenital defects that result in an abnormal or absent vagina.
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