Uterine Didelphys
Encyclopedia
Uterus didelphys represents a uterine malformation
where the uterus is present as a paired organ as the embryogenetic fusion of the mullerian duct
s failed to occur. As a result there is a double uterus with two separate cervices
, and often a double vagina as well. Each uterus has a single horn linked to the ipsilateral fallopian tube
that faces its ovary
.
by the fusion of the two paramesonephric ducts (also called mullerian ducts). This process usually fuses the two mullerian ducts into a single uterine body but fails to take place in these affected women who maintain their double mullerian systems. A didelphic uterus will have a double cervix
and is usually associated with a double vagina. Causes for the failure to fuse are not known.
Associated defects may affect the vagina, the renal system, and less commonly, the skeleton
.
The condition is less common than these other uterine malformations: arcuate uterus
, septate uterus, and bicornuate uterus
. It has been estimated to occur in 1/3,000 women.
and dyspareunia
. All patients displayed a double vagina. The fetal survival rate in 18 patients who delivered was 67.5%. Breech presentation was present in 43% and premature delivery common (21%).
Helpful techniques to investigate the uterine structure are transvaginal ultrasonography and sonohysterography, hysterosalpingography
, MRI, and hysteroscopy
. More recently 3-D ultrasonography has been advocated as an excellent non-invasive method to evaluate uterine malformations.
Uterus didelphys is often confused with a complete uterine septum. Often more than one method of investigation is necessary to accurately diagnose the condition. Correct diagnosis is crucial as treatment for these two conditions is very different. Whereas most doctors recommend removal of a uterine septum, they generally concur that it is better not to operate on a uterus didelphys. In either case, a highly qualified reproductive endocrinologist should be consulted.
and malpresentation are common. Cesarean section was performed in 82% of patients reported by Heinonen.
Uterus didelphys, in certain studies, has also been found associated with higher rate of infertility, spontaneous abortion, intrauterine growth retardation, and postpartum bleed.
and ipsilateral renal agenesis has been described.
woman with a double uterus gave birth to triplets
in 2006. Hannah Kersey, of Northam in Devon, gave birth to a pair of identical twins from an egg that implanted into one womb and then divided, and to an infant from a single egg that implanted into the other womb. This was the first known birth of viable triplets in a woman with a double uterus. It is estimated that the possibility of such a birth is about 1 in 25 million. A triplet pregnancy in a woman with uterus didelphys was reported from Israel in 1981; one baby died in utero, and of the remaining babies, one was delivered at 27 weeks gestation and the other 72 days later.
Uterine malformation
A uterine malformation is a type of female genital malformation resulting from an abnormal development of the Müllerian duct during embryogenesis...
where the uterus is present as a paired organ as the embryogenetic fusion of the mullerian duct
Müllerian duct
Müllerian ducts are paired ducts of the embryo that run down the lateral sides of the urogenital ridge and terminate at the Müllerian eminence in the primitive urogenital sinus. In the female, they will develop to form the Fallopian tubes, uterus, cervix, and the upper two-third of the vagina; in...
s failed to occur. As a result there is a double uterus with two separate cervices
Cervix
The cervix is the lower, narrow portion of the uterus where it joins with the top end of the vagina. It is cylindrical or conical in shape and protrudes through the upper anterior vaginal wall...
, and often a double vagina as well. Each uterus has a single horn linked to the ipsilateral fallopian tube
Fallopian tube
The Fallopian tubes, also known as oviducts, uterine tubes, and salpinges are two very fine tubes lined with ciliated epithelia, leading from the ovaries of female mammals into the uterus, via the utero-tubal junction...
that faces its ovary
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy:Ovaries...
.
Etiology
The uterus is formed during embryogenesisEmbryogenesis
Embryogenesis is the process by which the embryo is formed and develops, until it develops into a fetus.Embryogenesis starts with the fertilization of the ovum by sperm. The fertilized ovum is referred to as a zygote...
by the fusion of the two paramesonephric ducts (also called mullerian ducts). This process usually fuses the two mullerian ducts into a single uterine body but fails to take place in these affected women who maintain their double mullerian systems. A didelphic uterus will have a double cervix
Cervix
The cervix is the lower, narrow portion of the uterus where it joins with the top end of the vagina. It is cylindrical or conical in shape and protrudes through the upper anterior vaginal wall...
and is usually associated with a double vagina. Causes for the failure to fuse are not known.
Associated defects may affect the vagina, the renal system, and less commonly, the skeleton
Skeleton
The skeleton is the body part that forms the supporting structure of an organism. There are two different skeletal types: the exoskeleton, which is the stable outer shell of an organism, and the endoskeleton, which forms the support structure inside the body.In a figurative sense, skeleton can...
.
The condition is less common than these other uterine malformations: arcuate uterus
Arcuate uterus
The arcuate uterus is a form of a uterine anomaly or variation where the uterine fundus displays a concave contour towards the uterine cavity. Normally the fundus of the uterus is straight or convex on anterior-posterior imaging but in the arcuate uterus it dips into the cavity and may form a small...
, septate uterus, and bicornuate uterus
Bicornuate uterus
A bicornuate uterus or bicornate uterus, commonly referred to as a "heart-shaped" uterus, is a type of uterine malformation where two "horns" form at the upper part of the uterus.-Pathophysiology:A bicornuate uterus is formed during embryogenesis....
. It has been estimated to occur in 1/3,000 women.
Presentation
Women with the condition may be asymptomatic and unaware of having a double uterus. However, a study by Heinonen showed that certain conditions are more common. In his study of 26 women with a double uterus gynecological complaints included dysmenorrheaDysmenorrhea
Dysmenorrhea is a gynecological medical condition of pain during menstruation that interferes with daily activities, as defined by ACOG and others. Still, dysmenorrhea is often defined simply as menstrual pain, or at least menstrual pain that is excessive...
and dyspareunia
Dyspareunia
Dyspareunia is painful sexual intercourse, due to medical or psychological causes. The symptom is reported almost exclusively by women, although the problem can also occur in men. The causes are often reversible, even when long-standing, but self-perpetuating pain is a factor after the original...
. All patients displayed a double vagina. The fetal survival rate in 18 patients who delivered was 67.5%. Breech presentation was present in 43% and premature delivery common (21%).
Diagnosis
A pelvic examination will typically reveal a double vagina and a double cervix. Investigations are usually prompted on the basis of such findings as well as when reproductive problems are encountered. Not all cases of uterus didelphys involve duplication of the cervix and vagina.Helpful techniques to investigate the uterine structure are transvaginal ultrasonography and sonohysterography, hysterosalpingography
Hysterosalpingography
Hysterosalpingography is a radiologic procedure to investigate the shape of the uterine cavity and the shape and patency of the fallopian tubes. It entails the injection of a radio-opaque material into the cervical canal and usually fluoroscopy with image intensification...
, MRI, and hysteroscopy
Hysteroscopy
Hysteroscopy is the inspection of the uterine cavity by endoscopy with access through the cervix. It allows for the diagnosis of intrauterine pathology and serves as a method for surgical intervention .-Method:...
. More recently 3-D ultrasonography has been advocated as an excellent non-invasive method to evaluate uterine malformations.
Uterus didelphys is often confused with a complete uterine septum. Often more than one method of investigation is necessary to accurately diagnose the condition. Correct diagnosis is crucial as treatment for these two conditions is very different. Whereas most doctors recommend removal of a uterine septum, they generally concur that it is better not to operate on a uterus didelphys. In either case, a highly qualified reproductive endocrinologist should be consulted.
Frequency
In the United States, uterus didelphys is reported to occur in 0.1-0.5% of women. It is difficult to know the exact occurrence of this anomaly, as it may go undetected in the absence of medical and reproductive complications.Management
Patients with a double uterus may need special attention during pregnancy as premature birthPremature birth
In humans preterm birth refers to the birth of a baby of less than 37 weeks gestational age. The cause for preterm birth is in many situations elusive and unknown; many factors appear to be associated with the development of preterm birth, making the reduction of preterm birth a challenging...
and malpresentation are common. Cesarean section was performed in 82% of patients reported by Heinonen.
Uterus didelphys, in certain studies, has also been found associated with higher rate of infertility, spontaneous abortion, intrauterine growth retardation, and postpartum bleed.
Syndrome
A specific association of uterus didelphys, unilateral hematocolposHematocolpos
Hematocolpos is a medical condition in which the vagina fills with menstrual blood. It is often caused by the combination of menstruation with an imperforate hymen....
and ipsilateral renal agenesis has been described.
Multiple pregnancy
A number of twin gestations have occurred where each uterus carried its pregnancy separately. A recent example occurred on February 26, 2009, when Sarah Reinfelder of Sault Ste. Marie, Michigan delivered two healthy, although seven weeks premature, infants by cesarean section at Marquette General Hospital. It is possible that the deliveries occur at different times, thus the delivery interval could be days or even weeks.Triplets
A UKUnited Kingdom
The United Kingdom of Great Britain and Northern IrelandIn the United Kingdom and Dependencies, other languages have been officially recognised as legitimate autochthonous languages under the European Charter for Regional or Minority Languages...
woman with a double uterus gave birth to triplets
Multiple birth
A multiple birth occurs when more than one fetus is carried to term in a single pregnancy. Different names for multiple births are used, depending on the number of offspring. Common multiples are two and three, known as twins and triplets...
in 2006. Hannah Kersey, of Northam in Devon, gave birth to a pair of identical twins from an egg that implanted into one womb and then divided, and to an infant from a single egg that implanted into the other womb. This was the first known birth of viable triplets in a woman with a double uterus. It is estimated that the possibility of such a birth is about 1 in 25 million. A triplet pregnancy in a woman with uterus didelphys was reported from Israel in 1981; one baby died in utero, and of the remaining babies, one was delivered at 27 weeks gestation and the other 72 days later.
External links
- http://www.gfmer.ch/selected_images_v2/detail_list.php?cat1=17&cat3=311&stype=d Images
- http://uterusdidelphys.webs.com - a social website designed to help those with UD and inform others of this condition.