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The American Fertility Society (now American Society of Reproductive Medicine) Classification distinguishes:
- Class I: Müllerian agenesis (absent uterus).
- Uterus is not present, vagina only rudimentary or absent. The condition is also called Mayer-Rokitansky-Kuster-Hauser syndrome. The patient with MRKH syndrome will have primary amenorrhea.
- Class II: Unicornuate uterus (a one-sided uterus).
- Only one side of the Müllerian duct forms. The uterus has a typical "banana shape" on imaging systems.
- Class III: Uterus didelphys, also uterus didelphis (double uterus).
- Both Müllerian ducts develop but fail to fuse, thus the patient has a "double uterus". This may be a condition with a double cervix and a vaginal partition (v.i.), or the lower Müllerian system fused into its unpaired condition. See Triplet-birth with Uterus didelphys for a case of a woman having spontaneous birth in both wombs with twins.
- Class IV: Bicornuate uterus (uterus with two horns).
- Only the upper part of that part of the Müllerian system that forms the uterus fails to fuse, thus the caudal part of the uterus is normal, the cranial part is bifurcated. The uterus is "heart-shaped".
- Class V: Septated uterus (uterine septum or partition).
- The two Müllerian ducts have fused, but the partition between them is still present, splitting the system into two parts. With a complete septum the vagina, cervix and the uterus can be partitioned. Usually the septum affects only the cranial part of the uterus. A uterine septum is the most common uterine malformation and a cause for miscarriages. It is diagnosed by medical image techniques, i.e. ultrasound or an MRI. MRI is considered the preferred modality due to its multiplanar capabilities as well as its ability to evaluate the uterine contour, junctional zone, and other pelvic anatomy. A hysterosalpingogram is not considered as useful due to the inability of the technique to evaluate the exterior contour of the uterus and distinguish between a bicornuate and septate uterus.
A uterine septum can be corrected by hysteroscopic surgery.
- Class VI: DES uterus.
- The uterine cavity has a "T-shape" as a result of fetal exposure to diethylstilbestrol.
An additional variation is the arcuate uterus where there is a concave dimple in the uterine fundus within the cavity.
A rudimentary uterus is a uterine remnant not connected to cervix and vagina and may be found on the other side of an unicornuate uterus.
Patients with uterine abnormalities may have associated renal abnormalities including unilateral renal agenesis.
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.
The arcuate uterus is a form of a uterine anomaly or variation where the uterine cavity displays a concave contour towards the fundus. Normally the uterine cavity is straight or convex towards the fundus on anterior-posterior imaging, but in the arcuate uterus the myometrium of the fundus dips into the cavity and may form a small septation. The distinction between an arcuate uterus and a septate uterus is not standardized.
A unicornuate uterus represents a uterine malformation where the uterus is formed from one only of the paired Müllerian ducts while the other Müllerian duct does not develop or only in a rudimentary fashion. The sometimes called "hemi-uterus" has a single horn linked to the ipsilateral fallopian tube that faces its ovary.
A bicornuate uterus or bicornate uterus (from the Latin "cornū", meaning "horn"), commonly referred to as a "heart-shaped" uterus, is a uterus composed of two "horns" separated by a septum. In humans, a bicornuate uterus is a type of uterine malformation, but in some other mammalian species, including rodents and pigs, it is normal.
A uterine septum is a form of a congenital malformation where the uterine cavity is partitioned by a longitudinal septum; the outside of the uterus has a normal typical shape. The wedge-like partition may involve only the superior part of the cavity resulting in an "incomplete septum" or a "subseptate uterus", or less frequently the total length of the cavity ("complete septum") and the cervix resulting in a double cervix. The septation may also continue caudally into the vagina resulting in a "double vagina".
It is possible to diagnose a bicornuate uterus using gynecologic ultrasonography, specifically sonohysterography, and MRI. However, as there is no indication to do such procedures on asymptomatic women, the presence of a bicornuate uterus may not be detected until pregnancy or delivery.
In a C-section (usually done due to malpresentation), the irregular shape of the uterus will be apparent.
Other less reliable diagnostic imaging methods include hysterosalpingography and hysteroscopy; these procedures are typically done during the course of an infertility investigation.
Uterine hypoplasia, also known as naive uterus or infantile uterus, is a reproductive disorder characterized by hypoplasia of the uterus. It is usually due to pubertal failure/hypogonadism and may be treated with puberty induction using estrogens and/or progestogens.
Women with the condition may be asymptomatic and unaware of having a uniconuate uterus; normal pregnancy may occur. In a review of the literature Reichman et al. analyzed the data on pregnancy outcome of 290 women with a unicornuate uterus. 175 women had conceived for a total of 468 pregnancies. They found that about 50% of patients delivered a live baby. The rates for ectopic pregnancy was 2.7%, for miscarriage 34%, and for preterm delivery 20%, while the intrauterine demise rate was 10%. Thus patients with a unicornuate uterus are at a higher risk for pregnancy loss and obstetrical complications.
A specific association of uterus didelphys (double uterus), unilateral hematocolpos (inadequate draining of menstrual blood) and ipsilateral renal agenesis (having only one kidney) has been described.
Uterus didelphys (sometimes also "uterus didelphis") represents a uterine malformation where the uterus is present as a paired organ when the embryogenetic fusion of the Müllerian ducts fails to occur. As a result, there is a double uterus with two separate cervices, and rarely a double vagina as well. Each uterus has a single horn linked to the ipsilateral fallopian tube that faces its ovary.
In non human species ("e.g." nematodes), a didelphic genital tract may be normal rather than a malformation. Such species are described as didelphic, as opposed to monodelphic, with a single tract.
Adenomyosis can vary widely in the type and severity of symptoms that it causes, ranging from being entirely asymptomatic 33% of the time to being a severe and debilitating condition in some cases. Women with adenomyosis typically first report symptoms when they are between 40 and 50, but symptoms can occur in younger women.
Symptoms and the estimated percent affected may include:
- Chronic pelvic pain (77%)
- Heavy menstrual bleeding (40-60%), which is more common with in women with deeper adenomyosis. Blood loss may be significant enough to cause anemia, with associated symptoms of fatigue, dizziness, and moodiness.
- Abnormal uterine bleeding
- Painful cramping menstruation (15-30%)
- Painful vaginal intercourse (7%)
- A 'bearing' down feeling
- Pressure on bladder
- Dragging sensation down thighs and legs
Clinical signs of adenomyosis may include:
- Uterine enlargement (30%), which in turn can lead to symptoms of pelvic fullness.
- Tender uterus
- Infertility or sub-fertility (11-12%) - In addition, adenomyosis is associated with an increased incidence of preterm labour and premature rupture of membranes.
Women with adenomyosis are also more likely to have other uterine conditions, including:
- Uterine fibroids (50%)
- Endometriosis (11%)
- Endometrial polyp (7%)
The following table distinguishes among some of the terms used for the position of the uterus:
A retroverted uterus should be distinguished from the following:
Additional terms include:
- "retrocessed uterus:" both the superior and inferior ends of the uterus are pushed posteriorly
- "severely anteflexed uterus:" the uterus is in the same position as "normal" and bends in the same direction (concave is anterior) but the bend is much more pronounced
- "vertical uterus:" the fundus (top of the uterus) is straight up.
In most cases, a retroverted uterus is genetic and is perfectly normal but there are other factors that can cause the uterus to be retroverted. Some cases are caused by pelvic surgery, pelvic adhesions, endometriosis, fibroids, pelvic inflammatory disease, or the labor of childbirth.
Patients with cervical agenesis typically present in early adolescence, around the time of menarche, with amenorrhea and cyclic pelvic pain caused by the obstruction of menstrual flow from the uterus.
Adenomyosis is a gynecologic medical condition characterized by the abnormal presence of endometrial tissue (the inner lining of the uterus) within the myometrium (the thick, muscular layer of the uterus). In contrast, when endometrial tissue is present entirely outside the uterus, it represents a similar but distinct medical condition called endometriosis. The two conditions are found together in many cases, but often occur independently. Before being recognized as its own condition, adenomyosis used to be called "endometriosis interna". Additionally, the less-commonly used term "adenomyometritis" is a more specific name for the condition, specifying involvement of the uterus.
The condition is typically found in women between the ages of 35 and 50 but can also be present in younger women. Patients with adenomyosis often present with painful and/or profuse menses (dysmenorrhea & menorrhagia, respectively). Other possible symptoms are pain during sexual intercourse, chronic pelvic pain and irritation of the urinary bladder.
In adenomyosis, "basal" endometrium penetrates into hyperplastic myometrial fibers. Therefore, unlike functional layer, basal layer does not undergo typical cyclic changes with menstrual cycle.
Adenomyosis may involve the uterus focally, creating an adenomyoma. With diffuse involvement, the uterus becomes bulky and heavier.
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.
A pelvic examination may reveal a double vagina or double cervix that should be further investigated and may lead to the discovery of a uterine septum. In most patients, however, the pelvic examination is normal. Investigations are usually prompted on the basis of reproductive problems.
Helpful techniques to investigate a septum are transvaginal ultrasonography and sonohysterography, MRI, and hysteroscopy. More recently 3-D ultrasonography has been advocated as an excellent non-invasive method to delineate the condition. Prior to modern imaging hysterosalpingography was used to help diagnose the uterine septum, however, a bicornuate uterus may deliver a similar image.
An important category of septate uterus is the hybrid type a variant that may be misdiagnosed as bicornuate uterus when seen by laparoscopy Professor El Saman From Egypt was the first to describe this anomaly and warned gynecologist about this common misdiagnosis, whenever there is a uterine fundus depression on laparoscopy gynecologists should compare the depth of this depression with the depth of the dividing internal interface. Hybrid septate uterus benefit from hysteroscopic metroplasty under laparoscopic control.
The major differential diagnosis is the uterine septum. The lack of agreement to separate these two entities makes it difficult to assess the results in the literature.
An individual with this condition is hormonally normal; that is, the person will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche (pubic hair). The person's chromosome constellation 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.
If there is no uterus, a person with MRKH cannot carry a pregnancy without intervention. It is possible for the person to have genetic offspring by in vitro fertilization (IVF) and surrogacy. Successful uterine transplant has been performed in limited numbers of patients, resulting in several live births, but the technique is not widespread or accessible to many women.
A person with MRKH 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.
Hematometra typically presents as cyclic, cramping pain in the midline of the pelvis or lower abdomen. Patients may also report urinary frequency and urinary retention. Premenopausal women with hematometra often experience abnormal vaginal bleeding, including dysmenorrhea (pain during menstruation) or amenorrhea (lack of menstruation), while postmenopausal women are more likely to be asymptomatic. Due to the accumulation of blood in the uterus, patients may develop low blood pressure or a vasovagal response. When palpated, the uterus will typically feel firm and enlarged.
A boggy uterus is a finding upon physical examination where the uterus is more flaccid than would be expected.
It can be associated with uterine atony.
It may also be associated with adenomyosis.
The condition may be asymptomatic. The predominant symptoms are:
- Abnormal lochial discharge either excessive or prolonged
- Irregular or at times excessive uterine bleeding
- Irregular cramp like pain is cases of retained products or rise of temperature in sepsis
Uterine hyperplasia, or enlarged uterus, is a medical symptom in which the volume and size of the uterus in a female is abnormally high. It can be a symptom of medical conditions such as adenomyosis, uterine fibroids, ovarian cysts, and endometrial cancer.
When the involution is impaired or retarded it is called subinvolution. The uterus is the most common organ affected by subinvolution. As it is the most accessible organ to be measured per abdomen, the uterine involution is considered clinically as an index to assess subinvolution.