Unicornuate Uterus
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A unicornuate uterus represents a
uterine malformation 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 f ...
where the uterus is formed from only one of the paired
Müllerian duct The paramesonephric ducts (or Müllerian ducts) are paired ducts of the embryo in the reproductive system of humans and other mammals that run down the lateral sides of the genital ridge and terminate at the sinus tubercle in the primitive uroge ...
s while the other Müllerian duct does not develop or only in a rudimentary fashion. The sometimes called hemi-uterus has a single
horn Horn may refer to: Common uses * Horn (acoustic), a tapered sound guide ** Horn antenna ** Horn loudspeaker ** Vehicle horn ** Train horn *Horn (anatomy), a pointed, bony projection on the head of various animals * Horn (instrument), a family ...
linked to the ipsilateral
fallopian tube The fallopian tubes, also known as uterine tubes, oviducts or salpinges (: salpinx), are paired tubular sex organs in the human female body that stretch from the Ovary, ovaries to the uterus. The fallopian tubes are part of the female reproduct ...
that faces its
ovary The ovary () is a gonad in the female reproductive system that produces ova; when released, an ovum travels through the fallopian tube/ oviduct into the uterus. There is an ovary on the left and the right side of the body. The ovaries are end ...
.


Signs and symptoms

Women with the condition may be asymptomatic and unaware of having a unicornuate 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 Complications of pregnancy are health problems that are related to or arise during pregnancy. Complications that occur primarily during childbirth are termed obstetric labor complications, and problems that occur primarily after childbirth are term ...
.


Cause

The uterus is normally formed during
embryogenesis An embryo ( ) is the initial stage of development for a multicellular organism. In organisms that reproduce sexually, embryonic development is the part of the life cycle that begins just after fertilization of the female egg cell by the male ...
by the fusion of the two
Müllerian duct The paramesonephric ducts (or Müllerian ducts) are paired ducts of the embryo in the reproductive system of humans and other mammals that run down the lateral sides of the genital ridge and terminate at the sinus tubercle in the primitive uroge ...
s. If one of the ducts does not develop, only one Müllerian duct contributes to the uterine development. This uterus may or may not be connected to Müllerian structure on the opposite site if the Müllerian duct on that site undergoes some development. A unicornuate uterus has a single cervix and vagina. Associated defects may affect the renal system, and less common, the
skeleton A skeleton is the structural frame that supports the body of most animals. There are several types of skeletons, including the exoskeleton, which is a rigid outer shell that holds up an organism's shape; the endoskeleton, a rigid internal fra ...
. The condition is much less common than these other uterine malformations: arcuate uterus, septate uterus, and
bicornuate uterus A bicornuate uterus or bicornate uterus (from the Latin ''cornū'', meaning "horn"), is a type of müllerian anomalies, Müllerian anomaly in the human uterus, where there is a deep indentation at the Uterus#Structure, fundus (top) of the uterus. ...
. While the uterus didelphys is estimated to occur in 1/3,000 women, the unicornuate uterus appears to be even more infrequent with an estimated occurrence of about 1/4,000.


Diagnosis

A pelvic examination will typically reveal a single vagina and a single cervix. Investigations are usually prompted on the basis of reproductive problems. Helpful techniques to investigate the uterine structure are transvaginal ultrasonography and sonohysterography,
hysterosalpingography Hysterosalpingography (HSG), also known as uterosalpingography, is a radiology, radiologic procedure to investigate the shape of the uterine cavity and the shape and patency of the fallopian tubes. It is a special x-ray procedure using Contrast ...
,
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
, and hysteroscopy. More recently 3-D ultrasonography has been advocated as an excellent non-invasive method to evaluate uterine malformations.


Rudimentary horn

A unicornuate uterus may be associated with a rudimentary horn on the opposite site. This horn may be communicating with the uterus, and linked to the ispilateral tube. Occasionally a pregnancy may implant into such a horn setting up a dangerous situation as such pregnancy can lead to a potentially fatal uterine rupture. The majority of pregnancies in a rudimentary horn result in torsion and rupture, although there are still some cases of full-term pregnancies. Surgical resection of the horn is indicated.


Management

Patients with a unicornuate uterus may need special attention during pregnancy as
miscarriage Miscarriage, also known in medical terms as a spontaneous abortion, is an end to pregnancy resulting in the loss and expulsion of an embryo or fetus from the womb before it can fetal viability, survive independently. Miscarriage before 6 weeks ...
, fetal demise,
premature birth Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is betwee ...
, and malpresentation are more common. It is unproven that cerclage procedures are helpful. Although it is unclear whether interventions before conception or early in pregnancy such as resection of the rudimentary horn and prophylactic cervical cerclage decidedly improve obstetrical outcomes, current practice suggests that such interventions may be helpful.


References


External links

{{DEFAULTSORT:Unicornuate Uterus Anatomical pathology Congenital malformations of uterus and cervix